Editors

Franck Billmann and Tobias Keck

Essentials of Visceral Surgery

For Residents and Fellows
Editors
Franck Billmann
Department of General, Visceral and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Germany

Tobias Keck
Department of Surgery, University Hospital Schleswig-Holstein, Lübeck,
Germany

ISBN 978-3-662-66734-7 e-ISBN 978-3-662-66735-4

https://doi.org/10.1007/978-3-662-66735-4

This book is a translation of the original German edition „Facharztwissen

Viszeralchirurgie“ by Billmann, Franck, published by Springer-Verlag
GmbH, DE in 2021. The translation was done with the help of artificial
intelligence (machine translation by the service DeepL.com). A subsequent
human revision was done primarily in terms of content, so that the book
will read stylistically differently from a conventional translation. Springer
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Preface
“First comes the word, second comes the medicine, and last comes the
knife”1—solid knowledge and a structured knowledge of general and
visceral surgery are obligatory prerequisites, especially for doctors in
advanced training, fellows and future specialists in general and visceral
surgery.
Medical expertise in visceral surgery and bystanding subjects has seen a
continuous and rapid increase in knowledge in recent years. The increasing
specialization in surgery has led the editors to the present concept of a short
compendium, which allows the reader to access the current up-to-date
knowledge summarized by specialists in a condensed and clear manner.
Our book aims to provide the readers with a clear understanding of the
main aspects of general and visceral surgery. In a condensed, bullet point
form, the current and actual knowledge of visceral and general surgery is
summarized in this book, to which numerous renowned specialists have
contributed in its 16 chapters.
For learning residents and fellows, special emphasis is placed on
detailed and precise recommendations concerning the diagnostic procedure
and the indication for therapy. The surgical procedure is described step by
step in a compact and comprehensive form. New technologies and
evidence-based treatment strategies are taken into account and brief
overviews facilitate the targeted memorization of the most important facts.
This new, completely revised second edition takes into account the rapid
development of our field on the one hand and the high response to the book
on the other.
For the preparation of the specialist examination in general surgery or
visceral surgery, the book is therefore an ideal companion as well as a
reference in everyday clinical practice.
Tobias Keck
Franck Billmann
Lübeck, Germany
Heidelberg, Germany
March 2021
Abbreviations
γGT γ-Glutamyl transferase
18
F-FDG-PET 18 F-fluorodeoxyglucose positron emission tomography
5-ASA 5-aminosalicylic acid
5-FU 5-Fluorouracil
5-HIAA test 5-Hydroxyindolacetic acid test
99 m
Tc-MIBI 99 m Technetium methoxyisobutyl isonitrile
a. a. From anocutaneous line
AAA Abdominal aortic aneurysm
AAGBI Association of Anesthetists of Great Britain and Ireland
Ab Antibody
ABBA Axillo-bilateral-breast approach
ABCDE Airway, breathing, circulation, disability, exposure sequence
ACDC Acute cholecystitis: early versus delayed cholecystectomy (study)
ACE Angiotensin-converting enzyme
ACS Abdominal compartment syndrome
ACTH Adrenocorticotropic hormone
AD Autosomal dominant
AEG Adenocarcinoma of the esophagogastral junction, adenocarcinoma
of the esogastric junction
AIDS Acquired immunodeficiency syndrome
AIS Abbreviated injury scale
AJCC American Joint Committee on Cancer
AKS Abdominal compartment syndrome
ALM Adenoma like mass
ALT Atypical lipomatous tumor
AMI Acute mesenteric ischemia
AMS Superior mesenteric artery
ANCA Antineutrophil cytoplasmic antibody
AP Alkaline phosphatase
APC Adenomatous colonic polyposis, adenomatous polyposis coli
APP Abdominal perfusion pressure
Approx. Approximatively
ASA American Society of Anesthesiologists
ASCA Anti-Saccharomyces cerevisiae antibody
ASS Acetylsalicylic acid
AT II Angiotensin II
ATA American Thyroid Association
ATLS Advanced trauma life support
ATN Acute tubular necrosis
AVPU Scheme Alert, voice, pain, unresponsive scheme
AWMF Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen
Fachgesellschaften
BC Before christ
BDA Berufsverband Deutscher Anästhesisten
BG Blood glucose
BIS Bispectral index
BMI Body mass index
BPD Biliopancreatic diversion
BPD/DS Biliopancreatic diversion with duodenal switch
BS Blood sugar
BSA Body surface area
BSR Blood sedimentation rate
BUN Blood urea nitrogen
BW Body weight
CAD Coronary artery disease
CAEK Surgical Working Group Endocrinology
CAM-ICU Confusion Assessment Method/Intensive Care Unit
CAPD Continuous ambulatory peritoneal dialysis
CAr Cardiac arrhythmia
CASH Chemotherapy-Associated Steato Hepatitis
CaSR Calcium-sensing receptor
CASTLE Carcinoma with thymus-like differentiation
CC Score Completeness of cytoreduction score
CCC (or CC) Cholangiocarcinoma
CCDS Color-coded Doppler sonography
CCE (also CHE) Cholecystectomy
CCIS Cleveland clinic incontinence score
CDD Classification of diverticular disease
CDUS Color Doppler ultrasound
CEA Carcinoembryonic antigen, carcinoembryonal antigen
CEEA Circular end-to-end anastomosis
CHD Coronary heart disease
CIBD Chronic inflammatory bowel disease
CLL Chronic lymphocytic leukemia
CM Contrast medium
CME Complete mesocolic excision
CMI Chronic mesenteric ischemia
CMS Circulation, motor function, sensory function
CMV Cytomegalovirus
CNS Central nervous system
CO Cardiac output
COPD Chronic obstructive pulmonary disease
CRC Colorectal cancer
CRH Corticotropin-releasing hormone
CRM Circumferential resection margin
CRP C-reactive protein
CRS
Cytoreductive surgery
CSC Chronic sclerosing cholangitis
CT Computer tomography
CUSA Cavitron ultrasonic surgical aspiration
CVC Central venous catheter
CVD Cardiovascular disease
CVI Chronic venous insufficiency
CVL Central venous line
CVP Central venous pressure
DALM Dysplasia-associated lesion or mass
DD Differential diagnosis
DES Drug eluting stent
DGF Delayed graft function
DJ Double J catheter
DOACs Direct-acting oral anticoagulants
DPAM Disseminated peritoneal adenomucinosis
DPPHR Duodenum preserving pancreas head resection
DSO German foundation for organ transplantation
Dx Diagnosis
E.g. Exempli gratia (for example)
EBV Ebstein–Barr virus
ECOG Eastern Cooperative Oncology Group
EDTA Ethylenediaminetetraacetic acid
EEG Electroencephalogram
EGAPP Evaluation of genomic applications in practice and prevention
EGD Esophagogastroduodenoscopy
EGFR Epidermal-growth-factor receptor
EMR Endoscopic mucosal resection
EndoVAC Endoscopic vacuum therapy
ENETS
European Neuro-Endocrine Tumor Society
EPCAM Epithelial cell adhesion molecule
ERC Endoscopic retrograde cholangiography
ERCP Endoscopic retrograde cholangiopancreaticography
ERD Erosive reflux esophagitis, erosive reflux disease
ESBL Extended-spectrum beta-lactamase
ESC European Society of Cardiology
ESD Endoscopic submucosal dissection
ESDR End-stage renal disease
ESMO European Society for Medical Oncology
ESP Eurotransplant senior program
ESR Erythrocyte sedimentation rate
ET Eurotransplant
etc. et cetera
ETKAS Eurotransplant kidney allocation system
EUS Endoscopic ultrasound
FAMMM Syndrome Familial atypical multiple birthmark melanoma
syndrome
FAP Familial adenomatous polyposis
FAST Focused assessment with sonography in trauma
FB Finger breadth
FHH Familial hypocalciuric hypercalcemia
Fig. Figure
FISH Fluorescence in situ hybridization
FLR Functional liver volume after resection
FMTC Familial medullary thyroid cancer
FNA Fine needle aspiration cytology
FNCLCC Fédération Nationale des Centres de Lutte Contre le Cancer
FNH Focal nodular hyperplasia
FNMTC Familial non-medullary thyroid cancer
FOBT Fecal occult blood test
FPTC Familial papillary thyroid cancer
Fri French (= Charriere)
fT3, fT4 Free triiodothyronine (fT3), free thyroxine (fT4)
FTC Follicular thyroid carcinoma, follicular thyroid cancer
G6PD Glucose-6-phosphate dehydrogenase
GC General condition
GERD Gastroesophageal reflux disease
GFR Glomerular filtration rate
GI tract Gastrointestinal tract
GIA Gastrointestinal anastomosis
GIST Gastrointestinal stromal tumors
GLP-1 Glucagon-like peptide 1
GM-CSF Granulocyte macrophage colony-stimulating factor
GOT Glutamate oxaloacetate transaminase = aspartate aminotransferase,
AST or ASAT
GPT Glutamate pyruvate transaminase = Alanine aminotransferase, ALT
or ALAT
HAL Hemorrhoidal artery ligation
HBV Hepatitis B virus
HCC Hepatocellular carcinoma
HCV Hepatitis C virus
HD High definition
HDi Hemodialysis
HIPEC Hyperthermic intraperitoneal chemotherapy
HIT Heparin-induced thrombocytopenia
HIV Human immunodeficiency virus
HLA Human leucocyte antigen
HNF-1alpha Hepatocyte nuclear factor 1alpha
HNPCC Hereditary non-polyposis colorectal cancer
HP Helicobacter pylori
HPF High power field
HPT-JT Hyperparathyroidism-jaw tumor syndrome
HPV Human papillomaviruses
HSV Herpes simplex virus
HTK Histidine tryptophan ketoglutarate
HU High urgency
HUS Hemolytic uremic syndrome
IAD Intra-abdominal pressure
IAH Intra-abdominal hypertension
IAP Intra-abdominal pressure
IBD Inflammatory bowel disease, chronic inflammatory bowel disease
IC Incontinence index
ICS Intercostal space
ICU Intensive care unit
ICV Ileocecal valve
IDC Indwelling urinary catheter
IDDM Insulin-dependent diabetes mellitus
IEN Intraepithelial neoplasia
IFN Interferon
IH Inguinal hernia
ILP Isolated limb perfusion
IMC Intermediate care
INR International normalized ratio
IORT Intraoperative radiotherapy
IPMN Intraductal papillary-mucinous neoplasia
IPOM Intraperitoneal onlay mesh
IPST Intraperitoneal stoma mesh
IRE Irreversible electroporation
ISGLS
International Study Group of Liver Surgery
ITP Idiopathic thrombocytopenic purpura
KCl Potassium chloride
KLLN Killin
KT Kidney transplantation
KTx Kidney transplantation
LAD Lymphadenectomy
LAGB Laparoscopic adjustable gastric banding
LAR Low anterior resection
LARR Low anterior rectal resection
LDH Lactate dehydrogenase
LDL Low density lipoprotein
LDP Lloyd Davis position, modified Lloyd Davis position
LIFT Ligation of intersphincteric fistula tract
Lig. Ligament
LMWH Low molecular weight heparin
LN Lymph nodes
LRFS Local relapse-free survival
MALS Median arcuate ligament syndrome
MALT Lymphoid tissue of the mucosa-associated type, mucosa-
associated lymphoid tissue
MAO Monoaminooxidase
MAP Mean arterial pressure
MCN Mucinous cystadenoma, mucinous cystic neoplasm
MCP Metoclopramide
MELD Model for end-stage liver disease
MEN 1 Multiple endocrine neoplasia type 1
MET Metabolic equivalent of task
MFH Myxoid fibrous histiocytoma
Min. Minute(s)
MIVAP Minimally invasive video-assisted parathyroidectomy
MIVAT Minimally invasive video-assisted thyroidectomy
MMP Mismatch probability
MMR Mismatch repair
Mod. Modified
MODY Maturity onset diabetes of the young
MPNST Malignant peripheral nerve sheath tumor
MRCP Magnetic resonance cholangiopancreatography
MRI Magnetic resonance imaging
MRSA Methicillin-resistant Staphylococcus aureus
MRSE Methicillin-resistant Staphylococcus epidermidis
MSH Melanocyte-stimulating hormone
MSI Microsatellite instability
MTC Medullary thyroid cancer
NAFLD Non-alcoholic fatty liver disease
NASH Non-alcoholic fatty liver hepatitis, non-alcoholic steatohepatitis
NCCN National comprehensive cancer network
NCH/NSY Neurosurgery
NEN Neuroendocrine neoplasia
NERD Non-erosive reflux disease
NET Neuroendocrine tumor
NF-NET Non-Functional NET
NIBPM Non-invasive blood pressure measurement
NMTC Non-medullary thyroid cancer
NOA/NOACs New oral anticoagulants
NOMI Non-occlusive mesenteric ischemia
NOTES Natural orifice transluminal endoscopic surgery
NRS Numerical rating scale
NSAID Non-steroidal anti-inflammatory drugs
NSHPT
Neonatal severe hyperparathyroidism
Nu-DESC Nursing delirium screening scale
NYHA New York Heart Association
OAD Oral antidiabetics
ODS Obstructive defecation syndrome
OGTT Oral glucose tolerance test
OPSI Overwhelming post-splenectomy infection
OSAS Obstructive sleep apnea syndrome
OTSC Over-the-scope-clip
Pa O2 Arterial oxygen partial pressure
PACU Post-anesthesia care unit
PAD Peripheral artery disease
p-ANCA Perinuclear anti-neutrophil cytoplasmic antibody, perinuclear
cytoplasmic antibody
PAOD Peripheral arterial occlusive disease
PAP Perioperative antibiotic prophylaxis
PBC Primary biliary cirrhosis
PCI Peritoneal cancer index
PCR Polymerase chain reaction
PCT Procalcitonin
PDC Peridural catheter
PDGFRA Platelet-derived growth factor receptor α
PDS Polydioxanone
PE Physical exam
PEEP Positive end-expiratory pressure
PEI Percutaneous Ethanol Injection
PET Positron emission tomography
PG Parathyroid gland
PHLF Posthepatectomy liver failure
pHPT Primary hyperparathyroidism
PICCO Pulse index continuous cardiac output
PIPAC Pressurized intraperitoneal aerosol chemotherapy
PMCA Peritoneal mucinous carcinomatosis
PME Partial mesorectal excision
PNE Percutaneous nerve evaluation
pNET Neuroendocrine tumor of the pancreas
PNET Primitive neuroectodermal tumor
POCD Postoperative cognitive deficit
POEM Peroral endoscopic myotomy
POMC Proopiomelanocortin
PONV Postoperative nausea and vomiting
POPF Postoperative pancreatic fistula
PostR Postoperative radiotherapy
PPH Post-pancreatectomy hemorrhage
PPI Proton pump inhibitor
PPNAD Primary pigmented nodular adrenocortical disease
PPPD Pylorus-preserving pancreaticoduodenectomy (pancreatic head
resection)
PRA Panel reactive antibodies
PRBCs Packed red blood cells
PrR Preoperative radiotherapy
PSA Prostate-specific antigen
PSC Primary sclerosing cholangitis
PSDSS Peritoneal surface disease severity score
PT Prothrombin time
PTA Percutaneous transluminal angioplasty
PTC Papillary thyroid cancer
PTCD/PTBD Percutaneous transhepatic cholangiodrainage/Percutaneous
transhepatic biliary drainage
PTEN Phosphatase and tensin homolog
PTH Parathormone
PTHC Percutaneous transhepatic cholangiography
PTHrP Parathyroid hormone-related peptide
PTT Partial thromboplastin time, partial thromboplastin time
PTU Propylthiouracil
PUMP Preperitoneal umbilical hernia mesh plasty
R status Residual status
RAMPS Radical antegrade modular pancreatosplenectomy
RCR Revised cardiac risk
RET gene Rearranged-during-transfection gene
RFA/RFTA Radiofrequency ablation/radiofrequency thermoablation
RPS Retroperitoneal sarcoma
RR Recovery room
RYGB Roux-en-Y Gastric Bypass
SADI-S Single anastomosis duodeno-ileal bypass with sleeve gastrectomy
SB-NET Small bowel neuroendocrine tumor
SBO Small bowel obstruction
SCM Sternocleidomastoid muscle
SCN Serous cystadenoma, serous cystic neoplasm
SDH Succinate dehydrogenase
Sect. Section
SEER Surveillance, epidemiology, and end results program
SETTLE Spindle epithelial tumor with thymus-like differentiation
sFLR Standardized functional liver volume, standardized future liver
remnant
SG Sleeve gastrectomy
sHPT Secondary hyperparathyroidism
SIBO Small intestinal bacterial overgrowth
SIRS Systemic inflammatory response syndrome
SIRT Selective internal radiotherapy
SLAF Subcutaneous linea alba fasciotomy
SMA Smooth muscle actin
SNM Sacral nerve modulation
SNS Sacral nerve stimulation
SPECT Single photon emission computed tomography
SPN Solid pseudopapillary neoplasia
SpO2 Pulsoximetric oxygen saturation
SPPT Solid pseudopapillary neoplasia, solid pseudopapillary tumor (of
the pancreas)
SSI Infection of a surgical site, surgical site infection
STARR Stapled transanal rectal resection
STIKO Ständige Impfkommission (Robert Koch Institut)
STS Soft tissue sarcoma
SUDD Symptomatic uncomplicated diverticular disease
SUV Standardized uptake value
T3 Triiodothyronine
T4 Thyroxine
TACE Transarterial chemoembolization
TAMIS Transanal minimally invasive surgery
TAPP Transabdominal preperitoneal plasty
TaTME Transanal Total Mesorectal Excision
TEM/TEO Transanal endoscopic microsurgery/surgery
TEP Total extraperitoneal plasty
Tg Thyroglobulin
TgAb Thyroglobulin antibody
tHPT Tertiary hyperparathyroidism
Thyroid Thyroid gland
TLV Total liver volume
TME Total mesorectal excision
TNF Tumor necrosis factor
TNM Tumor node metastasis (classification)
TPG/GTA German transplant act
TPHA Treponema pallidum hemagglutination assay, test for infection
with the causative agent of syphilis (lues)
TPIAT Total pancreatectomy with autologous islet cell transplantation
TPO-MAb = TPO-Ab = MAb Thyroid peroxidase antibodies =
microsomal antibodies
TRAb TSH receptor autoantibodies
TRF Thyrotropin = TSH-releasing factor
TRH Thyrotropin-releasing hormone
TSH Thyroid-stimulating hormone
TTR amyloidosis Transthyretin amyloidosis
TTR Transthyretin
UCSF University of California San Francisco
UFH Unfractionated heparin
UGI Upper gastrointestinal
UGIT Upper gastrointestinal tract
UICC Union for International Cancer Control
US Ultrasound, sonography
USD US Dollar
VAS Visual analogue scale
VATS Video-assisted thoracoscopy
VBG (VBP) Vertical banded gastroplasty (Vertical banded Gastro plasty)
VRAM Vertical rectus abdominis myocutaneous flap
VRE Vancomycin-resistant enterococci
VZV Varicella-Zoster virus
WBC White blood cell count
WDTC Well-differentiated thyroid cancer
WHO World Health Organization
WSACS World Society of Abdominal Compartment Syndrome
Contents
1 Esophagus, Stomach and Duodenum
Torben Glatz and Jens Höppner
1.​1 Anatomy and Physiology
1.​1.​1 Esophagus
1.​1.​2 Stomach
1.​1.​3 Duodenum
1.​2 Leading Symptoms and Diagnosis
1.​2.​1 Leading Symptoms
1.​2.​2 Diagnosis
1.​2.​3 Therapeutic Principles
1.​3 Benign Diseases of the Esophagus
1.​3.​1 Diverticular Diseases of the Esophagus
1.​3.​2 Achalasia
1.​3.​3 Esophageal Perforation
1.​3.​4 Hiatal Hernias
1.​3.​5 Gastroesophageal​Reflux Disease (GERD)
1.​3.​6 Guidelines
1.​4 Malignant Diseases of the Esophagus
1.​4.​1 Overview
1.​4.​2 Esophageal Carcinoma (Including AEG)
1.​5 Benign Diseases of the Stomach
1.​5.​1 Gastroduodenal Ulcer Disease
1.​5.​2 Guidelines
1.​6 Malignant Diseases of the Stomach
1.​6.​1 Gastric Adenocarcinoma
 1.​6.​2 Gastrointestinal​Stromal Tumours (GIST)
1.​6.​3 Guidelines
1.​7 Diseases of the Duodenum
1.​7.​1 Diverticular Disease of the Duodenum
1.​7.​2 Duodenal Cancer
1.​7.​3 Guidelines
References
2 Small Intestine and Appendix
Didier Mutter
2.​1 Anatomy of the Small Intestine
2.​1.​1 Measured Values
2.​1.​2 Limits
2.​1.​3 Wall Structure of the Small Intestine
2.​1.​4 Circulation
2.​1.​5 Innervation
2.​1.​6 Small Intestine Functions
2.​2 Diseases of the Small Intestine
2.​2.​1 Clinical Presentation
2.​2.​2 Imaging
2.​2.​3 Crohn’s Disease
2.​2.​4 Small Intestinal Neoplasms
2.​2.​5 Other Diseases of the Small Intestine
2.​2.​6 Treatment Strategies
2.​3 Vermiform Appendix
2.​3.​1 Anatomy of the Vermiform Appendix
2.​4 Diseases of the Appendix
 2.​4.​1 Appendicitis Vermiformis
2.​4.​2 Malignant Diseases
References
3 Colon
Oliver Thomusch
3.​1 Anatomy and Physiology
3.​1.​1 Definition and Limits
3.​1.​2 Tasks
3.​1.​3 Location and Classification
3.​1.​4 Measured Values
3.​1.​5 Characteristic Features of the Colon
3.​1.​6 Blood Supply and Drainage
3.​2 Benign Diseases of the Colon
3.​2.​1 Diverticulosis and Diverticulitis
3.​2.​2 Colonic Polyps
3.​2.​3 Ulcerative Colitis
3.​2.​4 Chronic Constipation
3.​2.​5 Guidelines
3.​3 Colon Cancer and Hereditary CRC Syndromes
3.​3.​1 Colon Carcinoma
3.​3.​2 HNPCC (Hereditary Non-polyposis Colorectal Cancer):​
Lynch Syndrome
3.​3.​3 Other Hereditary CRC Syndromes
3.​3.​4 Guidelines
Reference
4 Rectum
Jens Wannenmacher and Stefan Willis
 4.​1 Anatomy and Physiology
4.​1.​1 Definition, Location and Structure
4.​1.​2 Anatomy and Embryology
4.​1.​3 Physiology
4.​2 Benign Diseases
4.​2.​1 Benign Neoplasms/​Malformations
4.​2.​2 Rectal Prolapse
4.​3 Malignant Diseases
4.​3.​1 Histological Tumour Entities
4.​3.​2 Rectal Cancer
4.​3.​3 Guidelines
Further Reading
5 Anorectum
Jens Wannenmacher and Stefan Willis
5.​1 Anatomy and Physiology
5.​1.​1 Anatomy
5.​1.​2 Physiology
5.​2 Benign Diseases
5.​2.​1 Hemorrhoidal Disease
5.​2.​2 Anal Vein Thrombosis
5.​2.​3 Anal Fissure
5.​2.​4 Anorectal Abscess
5.​2.​5 Anorectal Fistulas
5.​2.​6 Pilonidal Sinus
5.​2.​7 Fecal Incontinence
5.​2.​8 Anorectal Voiding Dysfunction (Outlet Constipation)
 5.​3 Malignant Disease:​Anal Carcinoma
5.​3.​1 Definition
5.​3.​2 Epidemiology
5.​3.​3 Aetiology
5.​3.​4 Symptomatology
5.​3.​5 Diagnosis
5.​3.​6 Differential Diagnosis
5.​3.​7 Therapy
5.​4 Guidelines
Suggested Reading
6 Endocrine Organs
Franck Billmann, Courtney Elisabeth Gibson and Robert Udelsman
6.​1 Anatomy and Physiology of the Thyroid Gland
6.​1.​1 Embryology and Anatomy
6.​1.​2 Physiology
6.​2 Diseases of the Thyroid Gland
6.​2.​1 Epidemiology
6.​2.​2 General Methods of Investigation
6.​2.​3 Basics of Surgical Therapy, Complications and
Postoperative Care
6.​2.​4 Benign Thyroid Diseases
6.​2.​5 Malignant Thyroid Diseases
6.​2.​6 Workup of a Solitary or Dominant Thyroid Nodule
6.​2.​7 Guidelines
6.​3 Familial Malignant Syndromes of the Thyroid Gland
6.​3.​1 Introduction (.​Table 6.​14)
6.​3.​2 Hereditary Medullary Thyroid Carcinoma
 6.​3.​3 Familial Papillary Thyroid Carcinoma (FPTC)
6.​3.​4 Rare Genetic Syndromes Associated with Thyroid Cancer
6.​3.​5 Guidelines
6.​4 Anatomy and Physiology of the Parathyroid Gland
6.​4.​1 Anatomy
6.​4.​2 Physiology (◘ Fig.​6.​4)
6.​5 Diseases of the Parathyroid Gland
6.​5.​1 Benign Parathyroid Diseases
6.​5.​2 Parathyroid Cancer
6.​5.​3 Guidelines
6.​6 Anatomy and Physiology of the Adrenal Gland
6.​6.​1 Embryology
6.​6.​2 Anatomy
6.​6.​3 Physiology
6.​7 Diseases of the Adrenal gland
6.​7.​1 Primary Hyperaldosteroni​sm (Conn Syndrome)
6.​7.​2 Cortisol-Producing Adrenal Adenoma
6.​7.​3 Pheochromocytoma​
6.​7.​4 Adrenocortical Carcinoma
6.​7.​5 Adrenal Incidentaloma
6.​7.​6 Adrenal Metastases
6.​7.​7 Principles of Adrenal Surgery
6.​7.​8 Guidelines
References
7 Bariatric and Metabolic Surgery
Michel Gagner and Franck Billmann
7.​1 Epidemiology
7.​1.​1 Incidence
7.​1.​2 Health Economic Consequences
7.​2 Pathophysiology and Principle of Action of Bariatric Surgery
7.​2.​1 Pathophysiology
7.​2.​2 Working Principle of Bariatric Surgery
7.​3 Clinical Evidence
7.​3.​1 Bariatric Surgery:​Laparoscopic Versus Open
7.​3.​2 Bariatric Surgery:​Prospective Controlled Studies
7.​3.​3 Metabolic Consequences of Bariatric Surgery
7.​3.​4 Mortality:​Bariatric Surgery Versus Drug Therapies
7.​4 Specific Current Bariatric Interventions
7.​4.​1 Roux-Y Gastric Bypass “Gastric Bypass”
7.​4.​2 Banded Gastric Bypass
7.​4.​3 Laparoscopic Adjustable Gastric Banding (LAGB)
7.​4.​4 Biliopancreatic Diversion (BPD)
7.​4.​5 Biliopancreatic Diversion with Duodenal Switch (BPD/​DS)
7.​4.​6 Gastric Sleeve Resection
7.​5 Complications
7.​5.​1 Mortality
7.​5.​2 Gastrointestinal​Complications
7.​5.​3 Other Complications
7.​6 Historical Interventions and Interventions in the Context of
Studies
7.​6.​1 Historical Interventions
7.​6.​2 Interventions in the Context of Studies
 7.​7 Metabolic Surgery
7.​7.​1 Definition
7.​7.​2 Scientific Basis
7.​8 S3 Guidelines (February 2018)
7.​8.​1 Quality Assurance
7.​8.​2 Diagnosis and Evaluation
7.​8.​3 Indication
7.​8.​4 Choice of Procedure
7.​8.​5 Technical Aspects and Complications
7.​8.​6 Aftercare
7.​8.​7 Guidelines
References
8 Liver, Gallbladder and Bile Ducts
Katrin Hoffmann and Peter Schemmer
8.​1 Anatomy and Physiology of the Liver
8.​1.​1 Definitions
8.​1.​2 Macroscopic and Microscopic Anatomy
8.​1.​3 Tasks of the Liver and Functional Liver Volume
8.​1.​4 Location
8.​1.​5 Measured Values
8.​1.​6 Blood Supply and Drainage
8.​1.​7 Terminology of Liver Resections (◘ Fig.​8.​1)
8.​2 Diseases of the Liver
8.​2.​1 Benign Diseases
8.​2.​2 Malignant Diseases of the Liver
8.​2.​3 Technique of Liver Resection
 8.​3 Liver Transplantation
8.​3.​1 General and Legal Basis
8.​3.​2 Evaluation and Follow-Up of Liver Function
8.​3.​3 Indications for Liver Transplantation:​Relevant
Underlying Diseases in Adults
8.​3.​4 Contraindication​s for Liver Transplantation
8.​3.​5 Surgical Principles
8.​4 Anatomy and Physiology of the Gallbladder and Bile Ducts
8.​4.​1 Gallbladder (Vesica Biliaris)
8.​4.​2 Bile Ducts
8.​4.​3 Blood Supply and Drainage of the Gallbladder and Bile
Ducts
8.​5 Diseases of the Gallbladder and Bile Ducts
8.​5.​1 Benign Diseases of the Gallbladder
8.​5.​2 Benign Diseases of the Bile Ducts
8.​5.​3 Gallbladder Carcinoma
References
9 Pancreas
Kim C. Honselmann and Tobias Keck
9.​1 Anatomy and Physiology
9.​1.​1 Definition, Location and Structure
9.​1.​2 Anatomy and Embryology
9.​1.​3 Physiology
9.​2 Benign Diseases
9.​2.​1 Acute Pancreatitis
9.​2.​2 Chronic Pancreatitis
9.​2.​3 Guidelines
 9.​3 Malignant Diseases
9.​3.​1 Pancreatic Carcinoma
9.​3.​2 Guidelines
9.​4 Cystic Neoplasms
9.​4.​1 Intraductal Papillary Mucinous Neoplasia (IPMN)
9.​4.​2 Serous Cystic Neoplasms (SCN)
9.​4.​3 Mucinous Cystic Neoplasia (MCN)
9.​4.​4 Solid Pseudopapillary Neoplasia (SPN)
9.​4.​5 Guidelines
9.​5 Endocrine Neoplasms
9.​5.​1 Definition
9.​5.​2 Epidemiology
9.​5.​3 Symptoms
9.​5.​4 Diagnosis
9.​5.​5 Therapy
9.​5.​6 Guidelines
References
10 Kidney Transplantation
Bernd Jänigen, Franck Billmann and Przemyslaw Pisarski
10.​1 Introduction
10.​1.​1 Legal Framework
10.​1.​2 Structure in Germany
10.​2 Indication for Transplantation and Preparation of the
Recipient
10.​2.​1 Indication for Transplantation
10.​2.​2 Recipient Preparation
10.​2.​3 Registration in the Waiting List
 10.​3 Deceased Organ Donation
10.​3.​1 Organ Donation and Donor Selection
10.​3.​2 Organ Allocation
10.​3.​3 Organ Retrieval
10.​4 Living Kidney Donation
10.​4.​1 Prerequisites
10.​4.​2 Legal Limits in Germany
10.​4.​3 Advantages of Living Kidney Donation
10.​4.​4 Donor Evaluation [Preparation and Diagnosis]
10.​4.​5 Donor Operation
10.​4.​6 Risks and Complications
10.​4.​7 Donor Aftercare
10.​5 Kidney Transplantation
10.​5.​1 Back-Table Preparation of the Kidney
10.​5.​2 Surgical Technique of Transplantation
10.​5.​3 En Bloc Renal Transplantation
10.​5.​4 Surgical Complications
10.​6 Postoperative Treatment
10.​6.​1 Inpatient Stay
10.​6.​2 Immunosuppressio​n
10.​6.​3 Organ Rejection
10.​6.​4 Infections
10.​6.​5 Aftercare
10.​7 Results
11 Spleen
Therezia Bokor-Billmann and Franck Billmann
 11.​1 Spleen:​Generalities
11.​1.​1 Embryology and Developmental Disorders
11.​1.​2 Anatomy
11.​1.​3 Physiology
11.​2 Spleen Diseases
11.​2.​1 Benign Haematological Diseases
11.​2.​2 Other Benign Diseases
11.​2.​3 Malignant Diseases
11.​2.​4 Spleen Trauma
11.​2.​5 Post-splenectomy Morbidity
References
12 Peritoneum
Jörg Pelz
12.​1 Anatomy and Physiology of the Peritoneum
12.​1.​1 General Anatomy
12.​1.​2 Physiology (Tasks) of the Peritoneum
12.​2 Benign Diseases of the Peritoneum
12.​2.​1 Peritonitis
12.​2.​2 Peritoneal Adhesions
12.​3 Pseudomyxoma Peritonei
12.​3.​1 Definition
12.​3.​2 Classification
12.​3.​3 Aetiology
12.​3.​4 Clinical Presentation
12.​3.​5 Therapy
12.​3.​6 Prognosis
 12.​4 Malignant Diseases of the Peritoneum
12.​4.​1 Mesothelioma
12.​4.​2 Peritoneal Carcinomatosis
12.​5 Cytoreductive Surgery (CRS) and HIPEC
12.​5.​1 Curative CRS and HIPEC
12.​5.​2 Prophylactic/​Adjuvant CRS and HIPEC
12.​5.​3 Palliative Therapeutic Concepts for Ascites
12.​5.​4 Complications
12.​5.​5 Results
12.​5.​6 PIPAC (Pressurized Intraperitoneal Aerosol
Chemotherapy)
12.​5.​7 Guidelines
References
13 Hernia
Jens Otto, Thorsten Lindenau and Karsten Junge
13.​1 Anatomy and Classification
13.​1.​1 Hernia Anatomy
13.​1.​2 Epidemiology
13.​1.​3 Pathogenesis and Classification
13.​1.​4 Hernia-Specific Complications
13.​2 General Diagnosis and Therapy Principles
13.​2.​1 Diagnosis
13.​2.​2 Therapy Principles
13.​3 Incisional Hernia
13.​3.​1 Definition
13.​3.​2 Incidence
13.​3.​3 Aetiology
 13.​3.​4 Clinical Presentation
13.​3.​5 Diagnosis
13.​3.​6 Surgical Therapy
13.​3.​7 Guidelines
13.​4 Inguinal and Femoral Hernia
13.​4.​1 Anatomy, Definition and Classification
13.​4.​2 Epidemiology
13.​4.​3 Pathophysiology
13.​4.​4 Clinical Presentation
13.​4.​5 Diagnostic Procedures
13.​4.​6 Therapeutic Principles
13.​4.​7 Guidelines
13.​5 Umbilical and Epigastric Hernia
13.​5.​1 Umbilical Hernia
13.​5.​2 Epigastric Hernia
13.​5.​3 Clinical presentation
13.​5.​4 Diagnosis
13.​5.​5 Therapy
13.​5.​6 Guideline
13.​5.​7 Differential Diagnosis:​Rectus Diastasis
13.​6 Parastomal Hernia
13.​6.​1 Definition
13.​6.​2 Epidemiology
13.​6.​3 Risk Factors and Prevention
13.​6.​4 Clinical Presentation
13.​6.​5 Relevant Complications
 13.​6.​6 Therapy
13.​6.​7 Guidelines
13.​7 Other Rare Hernias
13.​7.​1 Spieghel’s Hernia (Hernia Lineae Semilunaris)
13.​7.​2 Hernia Obturatoria
13.​7.​3 Hernia Ischiadica
13.​7.​4 Hernia Perinealis
13.​7.​5 Lumbar Hernia
References
14 Gastrointestinal​Stromal Tumors and Sarcomas
Daniel Oertli, Holger Bannasch, Athanasios Tampakis,
Christoph Kettelhack and Tobias Keck
14.​1 Gastrointestinal​Stromal Tumours (GIST)
14.​1.​1 Definition
14.​1.​2 Epidemiology and Tumour Localisation
14.​1.​3 Clinical Presentation
14.​1.​4 Pathology
14.​1.​5 Diagnosis
14.​1.​6 Therapy
14.​1.​7 Guidelines
14.​2 Soft Tissue Tumours of the Extremities
14.​2.​1 General:​Classification
14.​2.​2 Clinical Presentation
14.​2.​3 Diagnosis
14.​2.​4 Classification
14.​2.​5 Prognosis
14.​2.​6 Therapeutic Principles
 14.​2.​7 Guidelines
14.​3 Retroperitoneal Sarcomas
14.​3.​1 Epidemiology and Prognosis
14.​3.​2 Pathology
14.​3.​3 Classification
14.​3.​4 Molecular Genetics
14.​3.​5 Clinical Presentation
14.​3.​6 Diagnosis
14.​3.​7 Therapy
14.​3.​8 Management of Recurrences
14.​3.​9 Follow up
14.​3.​10 Guidelines
References
15 Perioperative Medicine
Maren Rudat and Sebastian Stehr
15.​1 Preoperative Phase
15.​1.​1 Risk Stratification
15.​1.​2 Laboratory and Blood Products
15.​1.​3 Additional Investigations
15.​1.​4 Perioperative Anticoagulation
15.​1.​5 Medication Management
15.​1.​6 Information from the Anaesthetist’s Point of View
15.​2 Intraoperative Phase
15.​2.​1 Intraoperative Monitoring According to AAGBI and BDA
Guidelines
15.​2.​2 Volume Management
15.​2.​3 Hemodynamics
 15.​2.​4 Heat Retention
15.​2.​5 Perioperative Antibiotic Therapy
15.​3 Postoperative Phase
15.​3.​1 Analgesia
15.​3.​2 Postoperative Nausea and Vomiting (PONV)
15.​3.​3 Delirium/​Postoperative Cognitive Deficit (POCD)
15.​3.​4 Recovery Room (PACU)
15.​3.​5 Intensive Care Unit (ICU)/​Intermediate Care (IMC)
15.​4 Fast Track Surgery
15.​4.​1 Definition
15.​4.​2 Preoperative Management
15.​4.​3 Intraoperative Management
15.​4.​4 Postoperative Management
15.​4.​5 Guidelines
Reference
16 Emergency Surgery
Benjamin Weixler, Raoul A. Droeser, Robert Mechera,
Christian A. Nebiker, Debora Nowakowski, Heidi Misteli and
Henry Hoffmann
16.​1 Polytrauma:​Abdominal Trauma
16.​1.​1 Anatomy of the Abdomen
16.​1.​2 Injury Mechanisms (Aetiology and Pathophysiology)
16.​1.​3 Management and Diagnosis
16.​1.​4 Therapeutic Procedure
16.​1.​5 Guidelines
16.​2 Ileus/​Obstruction
16.​2.​1 Definition—Classification
 16.​2.​2 Epidemiology
16.​2.​3 Pathophysiology
16.​2.​4 Diagnosis
16.​2.​5 Mechanical Ileus (Mechanical Obstruction)
16.​2.​6 Paralytic Ileus/​Functional Obstruction
16.​2.​7 Therapy
16.​2.​8 Specific Therapy for Certain Types of Ileus
16.​3 Abdominal Compartment Syndrome
16.​3.​1 Definitions
16.​3.​2 Aetiology
16.​3.​3 Clinical presentation
16.​3.​4 Diagnosis
16.​3.​5 Therapy
16.​3.​6 Guidelines
16.​4 Intestinal Ischemia
16.​4.​1 General
16.​4.​2 Acute Intestinal Ischaemia
16.​4.​3 Chronic Mesenteric Ischaemia (CMI)
16.​4.​4 Guidelines
Contributors
Holger Bannasch
Department of Plastic, Hand and Aesthetic Surgery, Schwarzwald-Baar-
Hospital Villingen-Schwenningen, Villingen-Schwenningen, Germany

Franck Billmann
Department of General, Visceral and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Germany

Therezia Bokor-Billmann
Department of Dermatology, University Hospital Heidelberg, Heidelberg,
Germany

Raoul A. Droeser
Clarunis—Universitäres Bauchzentrum Basel, University Hospital Basel,
Basel, Switzerland

Michel Gagner
Sacré Coeur Hospital, Clinique Michel Gagner, QC, Canada

Courtney Elisabeth Gibson

Department of Endocrine Surgery, Smilow Cancer Hospital at Yale, New
Haven, CT, USA

Torben Glatz
Department of General, Visceral and Vascular Surgery, Marien Hospital
Herne, University Hospital Ruhr-University Bochum, Herne, Germany

Henry Hoffmann
Center for Hernia Surgery and Proctology, ZweiChirurgen GmbH, Basel,
Switzerland

Katrin Hoffmann
Department of General, Visceral and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Deutschland

Kim C. Honselmann
Department of Surgery, University Hospital Schleswig-Holstein, Luebeck,
Germany

Jens Höppner
Department of Surgery, University Hospital Schleswig-Holstein, Luebeck,
Germany

Bernd Jänigen
Department of General, Visceral and Transplant Surgery, University
Hospital Freiburg, Freiburg, Germany

Karsten Junge
Department of General, Visceral and Minimally-invasive Surgery, Rhein-
Maas Hospital, Würselen, Germany

Tobias Keck
Department of Surgery, University Medical Center Schleswig-Holstein,
Luebeck, Germany

Christoph Kettelhack
Clarunis—Universitäres Bauchzentrum Basel, University Hospital Basel,
Basel, Switzerland

Thorsten Lindenau
Department of General, Visceral and Minimally-invasive Surgery, Rhein-
Maas Hospital, Würselen, Germany

Robert Mechera
Clarunis—Universitäres Bauchzentrum Basel, University Hospital Basel,
Basel, Switzerland

Heidi Misteli
Department of Surgery, Hospital Uster, Uster, Switzerland

Didier Mutter
Department of Digestive and Endocrine Surgery, NHC, Pôle Hépato-
Digestif, University Hospital Strasbourg, Strasbourg, France
Christian A. Nebiker
Department of General Surgery, Hospital Aarau, Aarau, Switzerland

Debora Nowakowski
Department of Surgery, Hospital Baselland, Basel, Switzerland

Daniel Oertli
Department of General Surgery, University Hospital Basel, Basel,
Switzerland

Jens Otto
Department of General, Visceral and Minimally-invasive Surgery, Rhein-
Maas Hospital, Würselen, Germany

Jörg Pelz
Department of General, Visceral and Oncological Surgery, St. Bernward
Hospital, Hildesheim Hospital, Hildesheim, Germany

Przemyslaw Pisarski
Department of General, Visceral and Transplant Surgery, University
Hospital Freiburg, Freiburg, Germany

Maren Rudat
Interdisciplinary Intensive Care Unit, Sana Kliniken Ostholstein, Eutin
Hospital, Eutin, Germany

Peter Schemmer
Department of General, Visceral and Transplant Surgery, University
Hospital Graz, Graz, Austria

Sebastian Stehr
Department of Anesthesiology and Intensive Care Medicine, University
Hospital Leipzig, Leipzig, Germany

Athanasios Tampakis
Clarunis—Universitäres Bauchzentrum Basel, University Hospital Basel,
Basel, Switzerland
Oliver Thomusch
Department of General, Visceral and Transplant Surgery, University
Hospital Freiburg, Freiburg, Germany

Robert Udelsman
Miami Cancer Institute, Miami, FL, USA

Jens Wannenmacher
Surgical Department A, Ludwigshafen Hospital, Ludwigshafen, Germany

Benjamin Weixler
Department of General and Visceral Surgery, Campus Benjamin Franklin,
Charité—University Hospital Berlin, Berlin, Deutschland

Stefan Willis
Surgical Department A, Ludwigshafen Hospital, Ludwigshafen, Germany
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_1

1. Esophagus, Stomach and Duodenum

Torben Glatz1 and Jens Höppner2
(1) Department of General, Visceral and Vascular Surgery, Marien
Hospital Herne, University Hospital Ruhr-University Bochum, Herne,
Germany
(2) Department of Surgery, University Hospital Schleswig-Holstein,
Lübeck, Germany

Torben Glatz (Corresponding author)

Email: [email protected]

Jens Höppner
Email: [email protected]

1.1 Anatomy and Physiology

1.1.1 Esophagus
– Cervical, thoracic and abdominal esophagus (total length approx. 25–28
cm)
– Physiological esophageal constrictions at the upper esophageal sphincter,
tracheal bifurcation and lower esophageal sphincter
– Transition from the hypopharynx with striated, voluntarily innervated
musculature to the esophagus with smooth musculature
– Histologically lined with squamous epithelium
– Transport function for food pulp and saliva

1.1.2 Stomach
– The cardia, fundus, corpus and antrum form the stomach (◘ Fig. 1.1)
– Sphincter muscle at the stomach outlet (pylorus)
– Histologically lined with cylindrical epithelium
– Blood supply via arcade from right and left gastric artery (small
curvature), arcade from left and right gastroomental artery (large
curvature) as well as short gastric vessels
– Reservoir function for food pulp
– Production of pepsin and hydrochloric acid for digestion
– Production of intrinsic factor (vitamin B12 absorption in the terminal
ileum)

Fig. 1.1 Classification of the stomach according to external shape. (Mod. according to von Lanz and
Wachsmuth 2004)

1.1.3 Duodenum
– Superior part, descending part, inferior part, ascending part of the
duodenum (pars I–IV duodeni)
– Common orifice of bile duct and pancreatic duct at the major duodenal
papilla (papilla Vateri) in the descending part, sometimes accessory
pancreatic duct with separate orifice into the duodenum (minor duodenal
papilla)
– Histologically lined with cylindrical epithelium
– Blood supply via gastroduodenal artery as well as branches of the
superior mesenteric artery (sup. and inf. pancreatoduodenal artery, Rio-
Branko arcade to gastroduodenal artery)
– Mixture of food pulp with bile and pancreatic secretions

1.2 Leading Symptoms and Diagnosis

1.2.1 Leading Symptoms
– Different for esophagus and stomach/duodenum

Esophagus
– Swallowing disorders/dysphagia
– Regurgitation of food
– Retrosternal pain
– Weight loss

Stomach
– Upper abdominal pain
– Regurgitation
– Weight loss

Upper GI Bleeding
– Vomiting blood (hematemesis)/tarry stools (hematochezia)/anemia

1.2.2 Diagnosis
Endoscopy
– Esophagogastroduodenoscopy (EGD)
– Endosonography for staging examination of neoplasms
– Manometry for the diagnosis of the movement disorders of the
esophageal musculature
– 24 h-pH-metry, impedance measurement for the diagnosis of
gastroesophageal reflux disease

Radiology/Nuclear Medicine
– Contrast esophagogram
– Computed tomography chest/abdomen
– MRI and PET-CT for special indications
– Gastric emptying scintigraphy

1.2.3 Therapeutic Principles

– Conservative
– Endoscopic
– Surgical

1.3 Benign Diseases of the Esophagus

Summary
– Diverticular disease:
– Zenker’s diverticulum
– Midesophageal diverticulum
– Epiphrenic diverticulum
– Achalasia:
– Endoscopic and surgical therapy
– Gastroesophageal reflux disease (GERD)/hiatal hernias
– Axial hiatal hernia
– Paraesophageal hiatal hernia
– Gastroesophageal reflux disease—Diagnosis
– Gastroesophageal reflux disease—pharmacological and surgical
therapy
– Esophageal perforations

1.3.1 Diverticular Diseases of the Esophagus

Etiology
Zenker’s Diverticulum and Epiphrenic Diverticulum (Pulsion
Diverticulum)
– Due to a mismatch between increased intraluminal pressure and
anatomical muscle gap; preferentially at predilection sites above the
esophageal sphincter
– Pseudodiverticulum (diverticular sac consists only of mucosa and
submucosa)
Midesophageal Diverticulum (Traction Diverticulum)
– Diverticulum includes all wall layers of the esophagus
– Due to traction on the esophagus from outside, e.g. due to residual
embryonic tissue connections between trachea and esophagus or also in
the context of inflammatory processes in the mediastinum
– Located mostly in the middle esophagus
– Overall very rare

Forms
Zenker’s Diverticulum (Hypopharynx)
– Most frequent esophageal diverticulum (incidence 2:100,000/year)
– Age of manifestation: 70–80 years
– Predominantly men affected
– Location: Killian’s triangle (between cricopharyngeal muscle and inferior
constrictor of the pharynx)
– Increased tone of the cricopharyngeal muscle and impaired relaxation of
the upper esophageal sphincter
Killian-Jamison Diverticulum
– Pulsion diverticulum
– Origin immediately below the upper sphincter
– Localization ventrolaterally under the cricropharyngeal muscle or
– Localization dorsal through the Laimer triangle
– Mostly small and asymptomatic diverticula
Epiphrenic Diverticulum
– Pulsion diverticulum
– Localized up to 10 cm above the Z-line
– Much rarer than Zenker’s diverticulum
– Development due to increased intraluminal pressure
– Usually associated with achalasia or diffuse esophageal spasm

Symptoms
– Dysphagia
– Regurgitation of undigested food
– Foetor ex ore
– Chronic cough and aspiration of food debris
– Recurrent pneumonias
– Lump feeling in the throat
– Cervical borborygmi (pathognomonic in Zenker diverticulum)
– Retrosternal pain and heartburn (epiphrenic diverticula)

Therapy
Zenker-Diverikel
– Cervical diverticular resection and myotomy of the cricopharyngeal
muscle
– Cervical diverticulopexy and myotomy of the cricopharyngeal muscle
– Endoscopic interventional transoral splitting (stapler, laser, needle knife)
Epiphrenic Diverticulum
Caution
Treatment of the underlying esophageal motility disorder required.
– Laparoscopic diverticulotomy and myotomy of the lower esophageal
sphincter (+/− fundoplication)
– Transthoracic diverticulotomy and myotomy of the lower esophageal
sphincter

1.3.2 Achalasia
– Incidence = 1/100,000 population/year

Etiology
Pathogenesis
– Degeneration of the myenteric plexus
– Lack of relaxation of the lower esophageal sphincter
Forms
Primary Achalasia
– Etiology unknown
Secondary Achalasia
– Chagas disease
– Gastric Cancer
– Esophageal Cancer

Symptoms
– Dysphagia
– Regurgitation of food
– Retrosternal pain
– Aspiration

Complications
– Aspiration pneumonia
– Esophageal cancer (increased incidence in achalasia)

Staging
– Stage I: Hypermotile form
– Stage II: Hypomotile form
– Stage III: Amotile form

Diagnosis
Esophagogastroduodenoscopy (EGD)
– Exclusion of malignancy and benign stenosis
Manometry
– Pressure measurement in the esophagus via probe
– Slow retraction of the probe during the swallowing act and digital
recording and evaluation of peristalsis
– Highest sensitivity for the diagnosis of achalasia
– Findings: Combination of hypermotility/hypomotility/peristalsis and lack
of relaxation of the lower esophageal sphincter
X-Ray (Esophagogram)/Computed Tomography
– Classic “champagne glass” shape of the esophagus with prestenotic
dilatation

Therapy
Medical Therapy
Principle
– Drug-induced relaxation of the smooth muscle fibers of the lower
esophageal sphincter
Preparations
– Calcium channel blockers (e.g. nifedipine)
– Long-acting nitrates
– Phosphodiesterase-5 inhibitor (sildenafil)

Drug Therapy
– Best results with nifedipine (10–30 mg) sublingually approx. 30 min
before a meal (leads to a relaxation of the lower oesophageal
sphincter lasting approx. 60 min).
– Overall, the effect of drug therapy is usually not satisfactory:
persistence of symptoms and side effects of the drugs (headache,
hypotension, etc.).

Endoscopic Therapy
Pneumatic Dilatation
– Dilatation with special balloon under fluoroscopy or under endoscopic
guidance
– Disruption of the muscle fibers of the lower esophageal sphincter
– Multiple applications are often necessary, but long-term alleviation of
symptoms is possible in up to 50–80% of patients
– Risk of esophageal perforation due to dilatation
Endoscopic Injection of Botulinum Toxin
– Low risk procedure
– High initial response rate (>75%)
– Frequent early recurrences (50%)
Peroral Endoscopic Myotomy (POEM)
– Endoscopic alternative to surgical myotomy
– Procedure:
– Endoscopic incision of the mucosa, then submucosal tunnelling and
long-stretch myotomy by diathermy
– In the short-term follow-up results comparable with the surgical
procedure
– Centre-based expertise
Surgical Therapy
Surgical Therapy Options
– Myotomy of the lower esophageal sphincter possible via laparoscopy,
laparotomy or thoracotomy (Vaezi et al. 2013) or robotically-assisted
– Gold standard: Laparoscopic Heller myotomy + fundoplication
– For stage III: discuss esophagectomy with gastric tube reconstruction
Results After Myotomy
– Partial or complete relief of symptoms in 90% of patients
– 30% of the patients develop reflux symptoms postoperatively, therefore
an additional fundoplication is mandatory

Surgical Procedure
Laparoscopic Heller Myotomy
– Supine position with spread legs
– Insertion of the camera trocar, insertion of the pneumoperitoneum;
operation may be robotically assisted
– Exposure of the esophageal hiatus by means of a liver retractor and
positioning of the patient
– Ventral mobilization of the esophagus into the lower mediastinum
– Identification and protection of the vagus nerve
 – Longitudinal severing of the muscle fibres of the lower oesophageal
sphincter including cardia while sparing the mucosal tube
– Covering the defect with an anterior fundoplication (► Sect. 1.3.5), or
Nissen fundoplication

1.3.3 Esophageal Perforation

Summary
– Different etiologies (malignant, spontaneous, iatrogenic)
– Life-threatening disease
– Therapy today mostly endoscopic (stent or endoluminal vacuum
therapy EndoVAC)
– Surgical therapy (primary suture + fundoplication or muscle flap) as
effective therapy option

Etiology
– Iatrogenic:
– As a result of endoscopic procedures (EGD, ERCP, endosonography)
– Complication of cardiothoracic surgery
– Gastric tube
– Malignant:
– Tumor perforation due to esophageal cancer
– Spontaneous:
– Boerhaave Syndrome
– Often as a result of sudden vomiting
– Lower/middle third esophageal rupture

Symptoms
– Acute chest pain:
– Differential Diagnosis:
– Myocardial Infarction
– Pulmonary Embolism
 – Aortic dissection
– Frequently misdiagnosed
– Hematemesis
– Dyspnea
– Fever
– Complications:
– Mediastinitis
– Pleural effusion/empyema
– Pneumothorax
– Septic shock

Diagnosis
Endoscopy (EGD)
– Localization of the perforation
– Assessment of the size of the perforation
Computed Tomography of the chest
– Oral and intravenous contrast agent
– Confirmation of transmural perforation
– Detection of pleural empyema/mediastinal abscess

Therapy
– Closure of the perforation
– Endoluminal vacuum therapy
– Drainage of the contaminated cavities (mediastinum, pleura)
Surgical Therapy
– Primary suture of the esophageal perforation
– Additional coverage by means of fundoplication or muscle flap
– Insertion of mediastinal drains
– Insertion of chest drain(s)
– Video-assisted thoracoscopy (VATS) for pleural empyema
– Ultima Ratio: Esophageal resection and reconstruction via gastric tube or
diversion
Endoscopic Therapy
– Actually Gold standard
– Less invasive and comparably effective
– Endoscopic insertion of a partially coated metal stent
– Endoscopic vacuum therapy-EndoVAC
– Over-the-scope clip (OTSC) only for small and recent perforations
Conservative Therapy
– Indicated only in palliative situation or very old perforation without
sepsis
– Transcutaneous or endoluminal drainage of the contaminated cavity
Prognostic Factors
– Delayed therapy (>48 h) unfavourable
– Septic shock at the time of therapy
– Spontaneous esophageal perforation (Boerhaave syndrome; Connelly et
al. 2013)
– Size and localization of the perforation not prognostically relevant

1.3.4 Hiatal Hernias

Etiology
– Acquired pathology in the majority of patients
– Risk factors:
– Overweight
– Pregnancy
– Connective tissue aging

Types of Hiatal Hernias (◘ Fig. 1.2)

Cardiofundal Malposition
– Mildest type
– Often incidental finding
Fig. 1.2 Types of hiatal hernia. (a) Axial sliding hernia. (b) Paraesophageal hernia. (c) Mixed
Hernia. (From von Lanz and Wachsmuth 2004)

Axial Sliding Hernia

– 90% of all hernias
– Intrathoracic position of the gastric cardia
Paraesophageal Hernia
– Lower esophageal sphincter intraabdominal
– Partial or complete intrathoracic stomach (upside-down stomach)
Mixed Forms
– Rarely, additional herniation of omentum, small or large intestine

Symptoms
Axial Hernias
– Often asymptomatic (90%)
– But predisposition for gastroesophageal reflux with insufficient function
of the lower esophageal sphincter
Paraesophageal Hernias
– Initially often asymptomatic as well
– Possibly postprandial unspecific abdominal or thoracic complaints
– Complications:
– Dysphagia
– Incarceration
– Ulceration
– Chronic anemia
– Dyspnea

Therapy
Symptomatic Therapy
– Proton-pump inhibitors
– See below: Therapy of reflux disease (► Sect. 1.3.5)
Surgical Therapy
– Strategy: Reduction of the hernia, anterior and/or posterior hiatoplasty,
fundoplication or gastropexy
– Indication:
– In axial hernia only in case of symptomatic reflux (GERD, 24 h pH-
metry, volume reflux, metaplasia)
– Indication for elective surgical intervention for paraesophageal hernia
– Emergency indication for incarcerated hernias (usually
paraesophageal)
Reinforcement of hiatoplasty by reinforcement with non-resorbable or
bioresorbable mesh possible, benefit not proven. Risk of injury of
esophagus or severe complications.

1.3.5 Gastroesophageal Reflux Disease (GERD)

Summary
 – GERD subsumes different disease entities
– Prevalence: 15% of the western population
– Multifactorial pathophysiology
– Therapy initially conservative with proton pump inhibitors (PPI)
– Laparoscopic fundoplication as an effective therapeutic option in case
of failure of drug therapy or patient preference

Definition
– “GERD” = “gastroesophageal reflux disease”
– The term subsumes the following entities:
– Erosive reflux esophagitis of various degrees of severity (ERD)
– Non-erosive reflux disease (NERD)
– Hypersensitive esophagus
– Extraesophageal manifestations
– Complications of GERD
– Functional reflux complaints

Etiology
Demographics
– Prevalence: about 15% with increasing incidence
– Approximately 50% of patients with GERD do not have endoscopically
definable lesions (NERD)
– Approx. 5% of GERD patients develop Barrett’s esophagus = intestinal
metaplasia of the epithelium in the (distal) esophagus
Pathophysiology and Risk Factors
– Pathophysiology:
– Primary GERD: Unclear dysfunction of the lower esophageal
sphincter
– Secondary GERD: in the context of other diseases or as a consequence
of surgical treatment (esophageal cancer, post-heller myotomy)
– Predisposing factors (due to dysfunction of the lower esophageal
sphincter):
– Pregnancy
 – Obesity
– Hiatal Hernia
– Nutritional factors
– Predisposing factors (due to irritating reflux):
– Overproduction of gastric acid, e.g. due to Helicobacter pylori
– Alkaline reflux (e.g. bile reflux after gastrectomy)
– Alcohol, coffee, nicotine, various drugs affect both the lower
esophageal sphincter and gastric content
– Frequently increased symptoms postprandially and due to bending or
pressing

Symptoms
– Chief complaint = heartburn
– Other symptoms:
– Diffuse epigastric pain
– Retrosternal pain
– Belching
– Volume reflux with regurgitation of food residues
– Dysphagia
– Irritative cough, hoarseness

Diagnosis
Esophagogastroduodenoscopy (EGD)
– Detection of erosive lesions
– Classification according to the Los-Angeles classification (◘ Table 1.1)
– Often no correlation between intensity of complaints and endoscopic
findings
– Exclusion of complication:
– Stenosis
– Barrett’s Esophagus
– Dysplasia
– Ulceration
– Esophageal Cancer
Table 1.1 Los-Angeles classification of gastroesophageal reflux disease
Stage Findings
A Erosions <5 mm
B Erosions >5 mm
C Confluent erosions <75% of circumference
D Confluent erosions >75% of circumference

24 h pH-Metry/Impedance Measurement
– Quantification of gastroesophageal reflux via probe
– Prior discontinuation of PPI
– Highest sensitivity and specificity for the detection of GERD
With pH-metry only acid reflux is detected, with impedance
measurement: detection of any type of reflux.
Manometry
– Relevant for the exclusion of motility disorders, especially before
surgical therapy (fundoplication, magnetic sphincter augmentation)
– Hypomotility of the distal esophagus often associated with long-lasting
GERD
– Details ► Sect. 1.3.2

Therapy
Conservative Therapy
– Changes in “life style”: weight loss, avoidance of noxious substances,
sleeping with the upper body elevated, discontinuation of triggering
medications
– Medical: Proton pump inhibitors (PPI)
– Objective: Symptom control and healing of existing erosions
– Therapy failure of PPI: always detailed diagnosis with endoscopy and
pH-metry/impedance measurement
– Asymptomatic erosive reflux esophagitis: therapy indicated
Surgical Therapy
– Indication:
– Long-term need for therapy
– Inadequate symptom control with PPI
 – Volume reflux
– Side effects of drug therapy
– Patient preference
– Operation of choice = laparoscopic fundoplication:
– 360°—Nissen, 270° posterior—Toupet
– Always with hiatoplasty (if necessary reduction of a hiatal hernia)
– Results:
– Success rate approx. 85% with thorough patient selection
– Lower success rate for re-operation (re-fundoplication)
Even after surgical treatment, endoscopic controls are
recommended for pre-existing Barrett’s esophagus.

Surgical Procedure
Laparoscopic Fundoplication 360° According to Nissen
– Supine split-legs position (french position)
– Insertion of the camera trocar, insertion of the pneumoperitoneum;
robot-assisted operation possible
– Exposure of the esophageal hiatus by means of a liver retractor and
positioning of the patient
– Visualisation of the left and right diaphragmatic crus
– Mobilization of the esophagus into the lower mediastinum, with
creation retroesophageal window
– Identification of the vagal nerve
– Partial mobilization of the gastric fundus (division short gastric
vessels)
– Calibration of the esophagus with large lumen gastric tube (bougie)
– Posterior hiatoplasty with non-absorbable suture material
– Retroesophageal pulling of the fundus
– Creation of the wrap: suture of the retroesophageal fundus anterior to
additional part of fundus with 2–3 non-absorbable sutures
– Fixation of the wrap to the esophagus with distal suture

1.3.6 Guidelines
AWMF guideline registry: gastroesophageal reflux disease (German
Society of Gastroenterology, Digestive and Metabolic Diseases, AWMF),
2014, AWMF registration number: 021/013—valid until May 31, 2019
currently under revision (7/2020).

1.4 Malignant Diseases of the Esophagus

1.4.1 Overview
Esophageal Cancer
– Squamous cell carcinoma
– Adenocarcinoma
– Adenosquamous carcinomas, undifferentiated carcinomas

Adenocarcinoma of the Gastroesophageal Junction (AEG)

– AEG 1, AEG 2 and AEG 3 with esophageal infiltration are classified as
esophageal carcinoma according to UICC TNM 8th version (2017).
Differentiation AEG 1–3: according to the epicenter of the tumor, not the
upper margin.

1.4.2 Esophageal Carcinoma (Including AEG)

Summary
– Squamous cell carcinoma vs. adenocarcinoma: different etiology and
tumor biology
– Leading symptoms: Dysphagia, weight loss, hematemesis
– Pretherapeutic staging: EGD/endosonography/CT (cTNM + histology)
– Neoadjuvant therapy for T3 or any T with N+: Neoadjuvant
radiochemotherapy (adenocarcinoma and squamous cell carcinoma) or
perioperative chemotherapy for adenocarcinoma
– Operative standard: Thoracoabdominal esophagectomy with 2-field
lymphadenectomy and advancement of stomach into right chest and
esophagogastric anastomosis
– High rate of postoperative complications after esophagectomy
– Minimally invasive laparoscopic and robotic-assisted procedures with
potential benefits in terms of postoperative morbidity
 – Minimally invasive abdominal gastric mobilisation/advancement
(hybrid OP) with evidence-based (Ib) advantages
– In case of functional or oncological inoperability definitive
radiochemotherapy or systemic chemotherapy, if necessary palliative
insertion of esophageal stents

Definition
– All epithelial malignancies between upper and lower esophageal
sphincter
– Adenocarcinomas of the gastroesophageal junction with infiltration of
the esophagus are defined as esophageal carcinoma (UICC TNM 8)

Types
Adenocarcinoma
– 95% in the distal esophagus
Adenocarcinoma of the Gastroesophageal Junction (AEG)
– Definition: All adenocarcinomas with tumor center 5 cm proximal to 5
cm distal to the gastroesophageal junction. Definition by tumour
epicentre, not upper margin (UICC 8th version)
– Classification according to Siewert:
– AEG 1: Tumour centre 1–5 cm proximal to the gastro-esophageal
junction
– AEG 2: Tumour centre from 1 cm proximal to 2 cm distal to the
gastro-esophageal junction
– AEG 3: Tumour centre 2–5 cm distal to the gastro-esophageal junction
Squamous Cell Carcinoma
– May occur throughout the esophagus
Adenosquamous Carcinomas, Undifferentiated Carcinomas
– Rare entities

Epidemiology and Etiology

Occurrence
– Approx. 6000 new cases in Germany/year
– Significant increase in the incidence of adenocarcinoma in Europe and
the USA
– 80% men, 20% women
Risk Factors
Squamous Cell Carcinoma
– Nicotine abuse
– Alcohol abuse
– Achalasia
– History of radiation therapy in the head and neck region
Adenocarcinoma
– Gastroesophageal reflux disease
– Barrett’s Esophagus
– Nicotine abuse
– Achalasia

Tumor Spread
Continuous Spread
– Intramural
– Direct organ infiltration (pericardium, pleura, aorta)
Lymphogenous Spread
– Lymph node levels: cervical, mediastinal and abdominal
Hematogenous Spread
– Hepatic: via portal vein
– Pulmonary, osseous or cerebral: via vena cava or liver

Classification
UICC/AJCC TNM 8 Classification (2017)
– T (Tumor)
– TX Tumor cannot be assessed
– T0 No primary tumor detectable
 – Tis High-grade dysplasia (malignant cells above the basement
membrane)
– T1a Infiltration of the lamina propria and muscularis mucosa
– T1b Infiltration of the tela submucosa (further subcategories)
– T2 Infiltration of the tunica muscularis
– T3 Infiltration of the adventitia
– T4a Infiltration of pleura, pericardium, peritoneum, azygos vein or
diaphragm
– T4b Infiltration of other organs, such as aorta, vertebral body or
trachea
– N (Lymph nodes)
– NX Regional lymph nodes cannot be assessed
– N0 No metastases in the lymph nodes
– N1 Metastases in 1–2 regional lymph nodes
– N2 Metastases in 3–6 regional lymph nodes
– N3 Metastases in 7 or more regional lymph nodes
– M (Metastases)
– M0 No Distant Metastasis
– M1 Distant Metastasis(es)
UICC Stages According to the TNM Classification 8th Version (2017)

Stage 0 Tis N0 M0
Stage I T1 N0 M0
Stage IIa T1 N1 M0
Stage IIb T2 N0 M0
Stage III T2 N1 M0
T3–4a N0–1 M0
Stage IVa T1–4a N2 M0
T4b N0–2 M0
T1–4 N3 M0
Any T N3 M0
Stage IVb Any T Any N M1

Symptoms
– Weight loss
– Retrosternal pain
– Hematemesis/melena/anemia

Diagnosis
Esophagogastroduodenoscopy (EGD)
– Biopsy
– Confirmation of tumor diagnosis
– Localization of the tumor
Endosonography
– Infiltration depth
– Assessment of T- and N-stage
Thoracic CT, Abdominal CT
– Assessment of primary tumor and lymph node involvement
– Distant metastases
Bronchoscopy
– For squamous cell carcinoma to exclude second carcinoma
– If the tumor is closely located to the central airways (tracheal infiltration,
bronchial infiltration)
Panendoscopy
– In the case of squamous cell carcinoma to exclude second carcinoma in
the ENT area
PET-CT, MRI Abdomen
– Not required for primary diagnosis
– Only to exclude metastases in rare and special indications
– Helpful in recurrence diagnosis

Therapy
Indication
– Therapeutic approach according to guideline (AWMF 021/023OL:
12/2018) depending on preoperative staging (◘ Figs. 1.3 and 1.4)
– Surgical therapy in specialized centers with high case load
– In early stages (T1a) consider endoscopic local therapy
– In case of planned two-cavity procedure, consider general operability,
especially cardiac and pulmonary comorbidity
– In case of inoperability, severe comorbidity or patient preference:
definitive radiochemotherapy

Fig. 1.3 Treatment algorithm for functionally operable and oncologically resectable squamous cell
carcinoma of the esophagus. (From Guideline Program in Oncology 2018; courtesy)
Fig. 1.4 Treatment algorithm for functionally operable and oncologically resectable
adenocarcinomas of the esophagus and gastroesophageal junction. (From Guideline Program in
Oncology 2018; courtesy)

Multimodal Therapy
Principles
– Improved local and systemic tumor control
– Indication usually for tumor stage T3/4 and/or positive lymph node status
– Down staging of primary inoperable tumors
– Increased R0 resection rates
– Reduced recurrence rate
– Prolonged overall survival
– After neoadjuvant therapy: re-staging (endoscopy and CT)
– Surgery: usually at time interval of 4–6 weeks after neoadjuvant therapy
Neoadjuvant Radiochemotherapy
– For squamous cell carcinoma and adenocarcinoma
– 36–54 Gy radiation dose with simultaneous chemotherapy
– Currently different protocols (e.g. CROSS protocol: 41.4 Gy divided
into 23 single doses of 1.8 Gy each plus chemotherapy with
carboplatin and paclitacel)
Perioperative Chemotherapy
– Alternative treatment protocol for adenocarcinoma of the
gastroesophageal junction
– Protocol analogous to multimodal therapy for gastric carcinoma (► Sect.
1.6)
– Advantages over neoadjuvant radiochemotherapy possibly due to
improved systemic tumor control and reduced perioperative morbidity
Additive/Palliative Therapy
Principles
– Endoscopic stent insertion
– Installation of percutaneous endoscopic gastrostomy or catheter
jejunostomy for enteral feeding
– Palliative radiochemotherapy
– Palliative chemotherapy
Strategy
– In case of locally inoperable tumor or functionally inoperable patient:
definitive radiochemotherapy (long-term survival >10–35% in stage
II/III)
– In case of R1/R2 resection and lack of possibility for surgical resection:
postoperative radiochemotherapy
– In case of recurrence or tumor persistence after definitive
radiochemotherapy: salvage esophagectomy may be necessary (caution:
increased postoperative morbidity)
– In metastatic adenocarcinoma: palliative chemotherapy
– In metastatic squamous cell carcinoma: palliative chemotherapy
– In case of pronounced dysphagia and weight loss: endoscopic
implantation of a self-expanding metal stent recommended, also possible
prior to neoadjuvant therapy
Operative Therapy Principles
Local Endoscopic Interventional Procedures
– Indication:
– If there is evidence of high-grade intraepithelial neoplasia or mucosal
carcinoma (<2 cm, no lymphatic invasion L0, no venous invasion V0,
no ulceration, grading G1/G2) in Barrett’s esophagus
– In case of lymphatic or blood vessel infiltration, poor degree of
differentiation (≥G3), submucosal infiltration or tumor remnant at the
basal resection margin: indication for esophageal resection
– Disadvantages:
– No reliable assessment of the lymph node status
– No certainty of R-status for extended resections in piece-meal
technique
– High risk of stenosis after (circular) resection of extensive findings
– Principle and Endoscopic Procedure:
– Endoscopic resection depending on the extent and localization of the
tumor
– In case of Barrett’s mucosa additional thermoablation of the complete
area
– Endoscopic mucosal resection (EMR)
– Endoscopic submucosal dissection (ESD)
Esophagectomy
– Principles of resection:
– For AEG 3: transhiatal extended gastrectomy (► Sect. 1.6)
– For AEG 2 alternatively:
– Transhiatal extended gastrectomy or
– Esophagectomy
– For tumors with massive infiltration on the stomach:
esophagogastrectomy
– For AEG 1: always abdominothoracic esophagectomy
 – Standard procedure = Ivor-Lewis esophagectomy (see “Operative
Procedure”):
– Mobilization and resection of the esophagus via right thoracotomy
or thoracoscopy with mediastinal en bloc lymphadenectomy
– Dissection of the esophagus at the level of the azygos arch or in the
upper thoracic aperture
– Lymphadenectomy in the abdominal compartment and gastric
mobilization and advancement via upper abdominal laparotomy or
laparoscopy
– Alternatively, thoracic and abdominal parts can each be minimally
invasive
– Hybrid laparoscopic/thoracotomic or laparotomic/thoracoscopic
procedure widely used
– Complete laparoscopic and thoracoscopic (if necessary, with
robotic-assisted technique)
– Potential reduction of pulmonary complications
– Less blood loss, faster recovery
– For squamous cell carcinoma: resection of the esophagus into the
upper thoracic aperture with cervical lymphadenectomy if necessary
– One-stage resection-reconstruction as a standard procedure
– Two-stage resection-reconstruction: with temporary cervical diversion
of the esophagus and gastric blind closure, interval reconstruction in
septic patients after (tumor) perforation
– Principles of Reconstruction:
– Preferred Reconstruction via gastric mobilization, advancement and
intrathoracic anastomosis
– Alternatively cervical anastomosis via separate left cervical incision
– Reconstruction with colonic interposition for tumor infiltration of the
stomach
– Reconstruction in the posterior mediastinum, alternatively retrosternal
– Postoperative complications:
– High rate of perioperative complications (morbidity up to 70%)
– Pulmonary complications (pneumonia, pleural effusion,
pneumothorax)
 – Anastomotic leak (therapy by stent insertion or ENDOVAC, surgical
revision)
– Chyle Leak due to injury of the thoracic duct
– Delayed gastric emptying
– Wound infections, post-operative bleeding, etc.
– Cardiac complications (high rate of arrhythmias, pericardial effusion)
– Perioperative management:
– Preoperative respiratory therapy, exercise and nutrition program
– Treatment of patients in specialized centers
– Optimized anaesthetic and intensive care management
– Peridural catheter for postoperative analgesia
– Postoperative nutrition via catheter jejunostomy, alternatively
parenteral nutrition
– Aspiration prophylaxis, if necessary scheduled postoperative
bronchoscopies

Surgical Procedure
Ivor Lewis Esophagectomy
– Preoperative colonoscopy: if colon interposition necessary!
– Anaesthetic preparation: Peridural catheter, central venous catheter,
continuous arterial blood pressure measurement, double lumen
endotracheal tube
– Positioning of the patient in a semi-lateral position, right thoracic
elevation
– Alternative: Intraoperative repositioning Left lateral position →
Supine position
– Right thoracotomy
– Division of the azygos vein
– Mobilization of the esophagus including the periesophageal lymphatic
and fat tissue and the peribronchial lymph nodes. Caution: thoracic
duct!
– Upper abdominal median laparotomy
– Mobilization of the stomach while sparing the gastroepiploic arcade
– Division of the left gastric artery and short gastric vessels
– Abdominal lymphadenectomy (hepatic artery, coeliac trunk, splenic
artery)
 – Resection of the esophagus and the proximal stomach
– Mobilization of the stomach (stapler-resection)
– Transhiatal advancement of the stomach into the posterior
mediastinum
– End-to-side anastomosis using a circular stapler
– Insertion of chest tubes
– Alternatively, minimally invasive procedure

Prognostic Factors
– Postoperative staging (UICC)
– Lymph node ratio (quotient of affected and removed lymph nodes)
– Lymphatic/venous invasion
– Response to neoadjuvant therapy (clinical and histopathological
regression)
– R status (residual status)

Follow-Up
Purpose
– Symptom-oriented follow-up
– Diagnosis and treatment of functional disorders (recurrence or benign
complications of treatment)
– Nutritional medical follow-up, additional nutrition if necessary
– Early detection of potentially curable local recurrences
Implementation
– After successful endoscopic therapy of a high-grade intraepithelial
neoplasia or an early carcinoma, regular control endoscopies (after 3
months, then every 6 months for 2 years and thereafter annually)
– After oesophagectomy, no predefined scheme, e.g. history, physical
examination and computed tomography of abdomen/thorax every 6
months

1.5 Benign Diseases of the Stomach

1.5.1 Gastroduodenal Ulcer Disease
 Summary
– Mostly due to Helicobacter pylori positive gastritis
– Non-specific symptoms or upper abdominal pain
– Complications: Hemorrhage, penetration, perforation, stenosis,
neoplasia
– Differential diagnosis: gastric cancer
– Conservative therapy of the underlying pathology + proton-pump
inhibitors
– Endoscopic therapeutic options for complications: Injection or clipping
of bleedings, dilation of stenoses
– Surgical interventions for complicated ulcer disease:
– Surgical hemostasis in case of endoscopically uncontrollable
bleeding
– Excision and suturing for perforation
– Distal gastric resection for perforation or stenosis

Etiology
Appearance
– Incidence approx. 200/100,000
– Ratio men:women = 3:1
– Localization mostly at the small curvature, antrum and in first part of
duodenum
Risk Factors
– Chronic gastritis due to Helicobacter pylori
– Genetic predisposition (blood group 0, HLA B-5)
– Use of NSAIDs (ASS, diclofenac, ibuprofen): increased risk especially in
combination with glucocorticosteroids
– Smoking
– Zollinger-Ellison syndrome (gastrinoma)
– Hypercalcemia, usually with parathyroid adenoma
– Acute stress ulcer: risk factor independent etiology (after polytrauma,
long intensive care stay, major operations, etc.)

Symptoms
– Epigastric pain
– Fasting pain: for duodenal ulcer
– Postprandial pain: for gastric ulcer
– Upper gastrointestinal bleeding
– Perforation with rapid onset (chemical) peritonitis. Caution: masked
symptoms with occult perforation

Diagnosis
Endoscopy (EGD)
– Confirmation of diagnosis and biopsy to exclude malignancy
– With conservative therapy always re-endoscopy to record complete
healing of the ulcer (DD cancer!)
Radiology
– Computed tomography with oral contrast medium: only indicated to
exclude penetration or perforation
Further Diagnosis
– Diagnosis of Helicobacter pylori
– Determination of gastrin, calcium and parathormone to exclude a
hormonal cause

Complications
– Bleeding
– Perforation/penetration
– Stenosis
– Neoplasia

Therapy
Conservative Therapy
– For Helicobacter-positive gastritis: eradication with proton-pump
inhibitor and clarithromycin + amoxicillin (French triple therapy) or
metronidazole (Italian triple therapy) for 7 days
– Avoidance of noxious substances (NSAR, smoking, coffee, alcohol,
stress)
– Proton pump inhibitors
Interventional Therapy
– In case of ulcer bleeding: bleeding control by injection with
adrenalin/histoacryl or clipping
– In case of endoscopically uncontrollable ulcer bleeding or high risk of
recurrent bleeding after primary successful endoscopic hemostasis:
Interventional angiography with endovascular hemostasis (coiling of
gastroduodenal artery)
– In case of stenosis: endoscopic dilation (bougie) possible
Surgical Therapy
– In gastrinoma or parathyroid adenoma: surgical therapy of the underlying
pathology (► Chaps. 6 and 9).
– Surgical therapy of ulcer complications:
– Ulcer perforation: if possible excision and primary suture of the ulcer,
otherwise distal gastric resection
– Gastric stenosis: distal gastric resection or gastroenterostomy, if
malignant cause of stenosis can be ruled out
– 2/3 gastric resection (Billroth I with gastroduodenostomy or Bilroth II
with gastrojejunostomy) and the vagotomy procedures for the treatment
of ulcer disease: obsolete today due to effective conservative treatment
options—PPI, HP (Helicobacter pylori) eradication.
– In case of ulcer bleeding in the first part of duodenum: extra- and
intraluminal (duodenum) closure of the gastroduodenal artery.

Surgical Procedure
Distal Gastric Resection Analogous to Billroth II
– Anesthesiology preparation: peridural catheter, central venous
catheter, continuous arterial blood pressure measurement
– Supine position
– Upper abdominal median laparotomy, alternatively upper abdominal
transverse laparotomy
– Distal gastric mobilization of the stomach
 – Resection of approx. 2/3 of the distal stomach by transection of the
postpyloric duodenum
– Closure of the duodenal stump with stapler, if necessary additional
sutures
– Reconstruction using a small bowel loop (Y-Roux technique or
classical omega reconstruction)

1.5.2 Guidelines
AWMF Guideline Register: Helicobacter pylori and gastroduodenal ulcer
disease (German Society of Gastroenterology, Digestive and Metabolic
Diseases, AWMF), 2014, AWMF registration number: 021/001, ► http://​
www.​awmf.​org/​leitlinien.​html

1.6 Malignant Diseases of the Stomach

1.6.1 Gastric Adenocarcinoma
Summary
– Frequent tumor worldwide, decreasing incidence in the western
population
– Intestinal or diffuse histological differentiation according to Lauren
– Early lymphogenic, hematogenic and peritoneal spreading
– Tumour stage is often advanced at the time of diagnosis
– For T3/4 or N+: Multimodal therapy concept consisting of
perioperative chemotherapy and surgical resection
– Therapeutic standard: Total gastrectomy with D2 lymphadenectomy
– For distal tumors: Subtotal gastrectomy possible

Definition
– All tumors of the gastric antrum, corpus and cardia with distance >5 cm
from the Z-line
– AEG 3 tumors without esophageal infiltration (UICC TNM 7, 2010)
– Tumors of the gastroesophageal junction with esophageal infiltration are
classified as esophageal cancers (UICC TNM 7, 2010)

Forms
– According to histology (Lauren classification)
– Intestinal type
– Diffuse type (signet ring cells)
– According to localization
– Antrum
– Corpus/Fundus
– Subcardial (AEG 3)

Epidemiology and Etiology

Occurrence
– Approx. 15,000 new cases in Germany per year
– Predominantly men (ratio men:women = 3:2)
– Older patients
– Incidence varies considerably from region to region (increased incidence
mainly in Asia, but also in South America, Southern and Eastern Europe)
– In the western population, decreasing incidence of gastric cancer, but
increasing incidence of gastroesophageal junction tumors
Risk Factors
– Helicobacter pylori
– Age
– Low socioeconomic status
– Nicotine and alcohol abuse
– Family history
– Previous gastric surgery
– Pernicious anaemia
– Nutritional and environmental factors

Tumor Spread
– Continuous
– Intramural
– Direct organ infiltration (spleen, pancreas, colon, duodenum)
– Lymphogenic (◘ Fig. 1.5)
– Early lymphogenic metastasis
– Lymph node compartments D1–D4
– Hematogenic
– Hepatic via portal vein
– Pulmonary, osseous, cerebral
– Peritoneal carcinomatosis
– Often early peritoneal seeding
– Ovarian metastasis: “Krukenberg tumor”
Fig. 1.5 Regional and distant lymph nodes of the stomach. Lymph node compartment D1: 1–6,
lymph node compartment D2: 7–11, lymph node compartment D3: 12–14, lymph node compartment
D4: 15–16. (According to Siewert et al. 2010)

Classification
TNM 7 Classification (2010)
– T (Tumor)
– T0 No primary tumor detectable
 – Tis carcinoma in situ
– T1a Infiltration of the lamina propria or muscularis mucosae
– T1b Infiltration of the tunica submucosa
– T2 Infiltration of the muscularis propria
– T3 Infiltration of the subserosa
– T4a Penetration of the visceral peritoneum without infiltration of
adjacent organs
– T4b Penetration of the visceral peritoneum with infiltration of
adjacent organs (pancreas, spleen, liver)
– N (Node)
– N0 No metastases in the lymph nodes
– N1 Metastases in 1–2 regional lymph nodes
– N2 Metastases in 3–6 regional lymph nodes
– N3a Metastases in 7–15 regional lymph nodes
– N3b Metastases in 16 or more regional lymph nodes
– M (Metastasis)
– M0 No distant metastases
– M1 distant metastasis(s)
UICC Stages According to the TNM Classification

Stage 0 Tis N0 M0
Stage Ia T1 N0 M0
Stage Ib T1 N0 M0
T2 N0 M0
Stage IIa T1 N2 M0
T2 N1 M0
T3 N0 M0
Stage IIb T1 N3 M0
T2 N2 M0
T3 N1 M0
T4a N0 M0
Stage IIIa T2 N3 M0
T3 N2 M0
Stage 0 Tis N0 M0
T4a N1 M0
Stage IIIb T3 N3 M0
T4a N2 M0
T4b N0/1 M0
Stage IIIc T4a N3 M0
T4b N2/3 M0
Stage IV Each T Each N M1

Symptoms
– Often unspecific
– Inappetence/weight loss
– Nonspecific upper abdominal pain
– Recurrent vomiting
– Hematemesis/melena/anaemia

Diagnosis
Esophagogastroduodenoscopy/Endosonography
– Biopsy
– Confirmation of tumor diagnosis
– Localization of the tumor
– Infiltration depth
– Assessment of T- and N-stage
Thoracic CT, Abdominal CT
– Assessment of the primary tumor and lymph node involvement
– Distant metastases
Diagnostic Laparoscopy
– Staging of peritoneal carcinomatosis
– Biopsy if necessary
– Laparoscopy is an important diagnostic step prior to preoperative therapy
PET-CT, MRI Abdomen, Bone Scintigraphy
– Not required for primary diagnosis
– Only to exclude metastases in rare and special indications
– Helpful in recurrence diagnosis

Therapy
Indication
– Therapeutic procedure according to the guideline depending on the
preoperative staging (◘ Table 1.2 and ◘ Fig. 1.6)
– Consider endoscopic mucosal resection at T1a stage
– Curative therapy for localized and locally advanced tumors
– In case of inoperability, severe comorbidity or patient request palliative
chemotherapy
– Palliative gastrectomy for limited metastasis in the young and/or low
comorbid patient
– Palliative gastrectomy for tumor hemorrhage and tumor perforation
Table 1.2 Therapeutic strategy for gastric cancer depending on preoperative staging; T1 and T2 see
Fig. 1.6

Tumor Therapy
stage
T2 N0 M0 Primary resection
T3/4 N0 Perioperative chemotherapy + resection
M0
Tx N+ M0
Tx Nx M1 Palliative therapy: palliative gastrectomy, gastroenterostomy, palliative chemotherapy
Consider resection + peritonectomy + HIPEC for limited peritoneal carcinomatosis and
good response to perioperative chemotherapy

HIPEC hyperthermic intraperitoneal chemoperfusion

Fig. 1.6 Guideline criteria and expanded criteria for early gastric cancer. (From Guideline Program
in Oncology 2019; used with permission)

Multimodal Therapy
Principles
– Improved local and systemic tumor control
– Prognostic improvement in locally advanced tumors
– Indication usually for tumor stage T3/4 and/or positive lymph node status
– Down staging of inoperable tumors
– Increased R0 resection rates
– Reduced recurrence rate
– Prolonged overall survival
– After neoadjuvant therapy: re-staging (endoscopy, computed
tomography)
– Surgery at intervals of 4–6 weeks after neoadjuvant therapy
Perioperative Chemotherapy
– Combination of pre- and postoperative chemotherapy
– Various platinum-based chemotherapy protocols:
– ECF (epirubicin, cisplatin and 5-fluorouracil)
– ECX (epirubicin, cisplatin, capecitabine)
– EOX (epirubicin, oxaliplatin, capecitabine)
– FLOT (docetaxel, folic acid, 5-fluorouracil, oxaliplatin)
 – DCF (docetaxel, cisplatin, 5-fluorouracil)
– Cisplatin + 5-fluorouracil
Adjuvant Radiochemotherapy
– Widely used in the USA, in Europe only for limited lymphadenectomy
(≤D1) or R1/2 resection according to interdisciplinary board
recommendation.
– Consider in lymph node positive patients without preoperative
chemotherapy even after D2 lymphadenectomy.
Additive/Palliative Therapy
Principles
– Palliative gastrectomy for bleeding tumors or limited metastatic tumors
with obstructive symptoms
– Gastroenterostomy for stenosis and inoperable tumor
– Palliative chemotherapy
– Definitive radiochemotherapy
Strategy
– In case of R1/R2 resection and no possibility of further surgical
resection: adjuvant radiochemotherapy
– In case of locally inoperable tumor or distant metastasis: palliative
chemotherapy
– Palliative gastrectomy only for bleeding tumors after exhaustion of
endoscopic and angiographic methods
– Bypass procedure: for clinically manifest gastric outlet stenosis
Operative Therapy Principles
– Local endoscopic interventional procedures:
– Indication:
– Intraepithelial neoplasms of any size as well as early gastric cancers
that meet all four of the following criteria should be resected
endoscopically en-bloc: <2 cm in diameter, not ulcerated, mucosal
carcinoma, intestinal type/grade of differentiation good to moderate
“(G1/G2)”
 – Early gastric cancers with a maximum of one “extended criterion”
can be endoscopically resected curatively. Extended criteria are;
differentiated mucosal carcinoma (G1/2) without ulceration and
size >2 cm; differentiated mucosal carcinoma with ulceration <3
cm; well-differentiated carcinoma with submucosal invasion <500
μm and size <3 cm; undifferentiated mucosal carcinoma <2 cm in
diameter (provided there is no biopsy evidence of tumor cells <1
m)
– Indication for gastric (partial) resection plus lymphadenectomy in
the presence of risk criteria or residual tumor at the basal resection
margin
– Disadvantages:
– No reliable assessment of the lymph node status
– No assessment of R-status for extended resections in piece-meal
technique
– Principle and endoscopic procedure:
– Endoscopic resection depending on extension and localization
– Endoscopic mucosal resection (EMR)
– Endoscopic submucosal dissection (ESD)
– Goal: En-bloc resection
– Gastrectomy/Gastric Resection:
– Principles of resection:
– A safe resection distance of 5 cm for the intestinal type and 8 cm for
the diffuse type should be achieved
– Gastrectomy with lymphadenectomy of the compartments D1 and
D2 is standard
– For distal tumors, subtotal (4/5) gastrectomy is sufficient, leaving a
small proximal gastric remnant
– In case of subcardial gastric cancer (AEG 3) a transhiatal extended
gastrectomy including the distal esophagus is necessary
– In case of limited peritoneal carcinomatosis, gastrectomy with local
peritonectomy and, if necessary, hyperthermic intraoperative
chemotherapy can be performed with curative intention
– Laparoscopic and robotic-assisted surgery can be performed in a
specialized center
 – Principles of Reconstruction:
– No clear recommendation, procedure depending on the experience
of the surgeon
– Classical Roux-en-Y reconstruction
– Different techniques of jejunum pouch reconstruction, e.g. J-pouch
(possible quality of life advantage)
– Postoperative complications:
– Duodenal Stump Insufficiency
– Anastomotic leak of the esophagojejunostomy
– Pulmonary and cardiac complications
– Pancreatic fistula after D2 lymphadenectomy
– Cachexia

Surgical Procedure
Gastrectomy
– Anaesthesiological preparation: Peridural catheter, central venous
catheter, continuous arterial blood pressure measurement
– Supine position
– Upper abdominal median laparotomy, alternatively upper abdominal
transverse laparotomy
– Incision of gastrocolic ligament and opening of lesser sac (great
omentum resected en-bloc with gastric specimen)
– Transection of the gastro-splenic ligament and short gastric vessels
near the spleen
– Division of the gastroomental artery close to the pancreatic head
– Transection of the right gastric artery
– Division of the postpyloric duodenum and closure of the duodenal
stump
– D2 lymphadenectomy including the lymph nodes around the hepatic,
celiac and splenic arteries, and division of left gastric artery at its
origin near the coeliac trunk
– Division of the vagal nerve at the abdominal esophagus
– Dissection/Mobilisation of the abdominal esophagus
– Roux-en-Y Reconstruction using a small bowel loop (Jejunum)
Prognosis
Prognostic Factors
– Postoperative stage (UICC TNM)
– Lymph node ratio (quotient of affected and removed lymph nodes)
– Lymphatic/venous invasion
– Response to neoadjuvant therapy (clinical and histopathological
regression according to Becker et al. 2011)
– R status (residual status)

Follow-Up
Goals
– Symptom-oriented follow-up
– Rule out functional disorders as a result of recurrence or as benign
complications of treatment
– Nutritional medical follow-up, additional nutrition if necessary
– Early detection of potentially curable local recurrences
– Early detection of distant metastases
Implementation
– After successful endoscopic therapy of a high-grade intraepithelial
neoplasia or an early cancer, regular control endoscopies (every 3 months
in the first year, every 6 months in the second year, thereafter annually)
– After gastrectomy, so-called symptom-oriented follow-up without a
predefined scheme, e.g. anamnesis, physical examination and computer
tomography of abdomen/thorax every 6 months

1.6.2 Gastrointestinal Stromal Tumours (GIST)

► Chapter 14

1.6.3 Guidelines
Guideline program oncology (German Cancer Society, German Cancer Aid,
AWMF): “Gastric carcinoma”—Diagnostics and therapy of
adenocarcinomas of the stomach and esophagogastric junction, long version
2.0, 8/2019, AWMF registration number: 032-009OL
1.7 Diseases of the Duodenum
Summary
– Duodenal diverticulum: Common finding usually not requiring any
treatment
– Duodenal ulcer: ► Sect. 1.5
– Duodenal cancer
– Rare tumor disease
– FAP (familial adenomatous polyposis)/duodenal polyps as risk
factors

1.7.1 Diverticular Disease of the Duodenum

Incidence
– Approximately 10–20%

Types
– Duodenal diverticula are mostly found near pancreas head
– Rarely intraluminal or intramural duodenal diverticula, as congenital
malformations, originating from a mucosal duplication

Symptoms
– Mostly incidental finding during ERCP
– Mostly asymptomatic and without need for therapy

Therapy
– Very rarely duodenal diverticula require surgery

Complications
– Rarely upper GI bleeding and perforation.
– Very rarely obstruction of bile duct (jaundice) and pancreatic duct
(pancreatitis) due to compression.

1.7.2 Duodenal Cancer

Etiology and Tumor Manifestation
Appearance
– Rare tumor disease
– Duodenal adenoma as a precancerous condition
– Frequently in the context of hereditary tumor syndromes: FAP, HNPCC
(“hereditary non-polyposis colorectal cancer”), Peutz-Jeghers syndrome,
Gardner syndrome
– Other risk factors: Crohn’s disease, celiac disease
– Up to 90% of patients with FAP develop duodenal polyps; lifetime risk
of duodenal cancer is 3–4%
Symptoms
– Often asymptomatic
– Hematemesis/anaemia
– Stenosis/inappetence/weight loss/vomiting
– Obstructive jaundice or pancreatitis if infiltration of the duodenal papilla

Diagnosis and Therapy

Diagnosis
– Endoscopic diagnosis with biopsy: always + immediately in case of
suspected tumor
– In case of tumor detection: CT abdomen/thorax and if necessary
endosonography for reliable staging
Endoscopic Therapy
– Duodenal polyps are removed endoscopically analogous to colon polyps
– In the case of larger, flat polyps, consider ablation using the piece-meal
technique with additional thermal ablation of the affected area, if
necessary
– For duodenal polyposis, determine Spigelman score (polyp number,
polyp size, histologic type, grading of intraepithelial neoplasia): Stage I–
IV
– In stage I–III regular endoscopic controls
– In stage IV, consider surgical therapy
Surgical Therapy
– Surgical excision with transverse closure of the excision site: for
adenomas that cannot be removed by endoscopy
– Transduodenal papillary excision with re-insertion of the main pancreatic
duct and bile duct: in case of papillary adenomas
– Pancreas sparing duodenectomy (caution: morbidity): for benign tumors
(e.g. duodenal polyposis)
– Radical oncological resection (pylorus preserving
pancreatoduodenectomy or Whipple operation): in the case of duodenal
cancer
Multimodal Therapy
– No recommendations due to conflicting data
– No neoadjuvant therapy
– Adjuvant therapy analogous to the recommendations for colon cancer (►
Chap. 3)
– For ampullary cancer survival benefit with adjuvant chemotherapy with 5
FU/leukovorin or gemcitabine (ESPAC-3) after R0 resection
Palliative Therapy
– Surgical gastroenterostomy: as a bypass procedure in symptomatic
patients with inoperable tumors
– Endoscopic biliary stent/prosthesis insertion or surgical palliative
biliodigestive anastomosis: in the case of obstructive jaundice
– Palliative chemotherapy: analogous to colon cancer (► Chap. 3),
consider palliative radiochemotherapy if necessary
Prognosis
– 5-year survival rate: approx. 30%
– For N0, M0, R0 = 50–70% 5-year survival

1.7.3 Guidelines
Guideline program oncology (German Cancer Society, German Cancer Aid,
AWMF): S3 guideline colorectal carcinoma, long version 2.1, 2019, AWMF
registration number: 021-007OL, ► http://​leitlinienprogra​mmonkologie.​de/​
Leitlinien.​7.​0.​html
 Oncology guideline program (German Cancer Society, German Cancer
Aid, AWMF): Diagnostics and therapy of squamous cell carcinomas and
adenocarcinomas of the esophagus, long version 2.0, 2018, AWMF
registration number: 021/023OL, ► http://​leitlinienprogra​mmonkologie.​de/​
Leitlinien.​7.​0.​html

References
Becker K, Langer R, Reim D, Novotny A, Zum Meyer Buschenfelde C, Engel J, Friess H, Hofler H
(2011) Significance of histopathological tumor regression after neoadjuvant chemotherapy in gastric
adenocarcinomas: a summary of 480 cases. Ann Surg 253:934–939. https://​doi.​org/​10.​1097/​SLA.​
0b013e318216f449​
[Crossref][PubMed]

Connelly CL, Lamb PJ, Paterson-Brown S (2013) Outcomes following Boerhaave’s syndrome. Ann
R Coll Surg Engl 95:557–560. https://​doi.​org/​10.​1308/​003588413X136299​60049199
[Crossref][PubMed][PubMedCentral]

Siewert JR, Lordick F, Stein HJ (2010) Ösophaguskarzinom. In: Siewert JR, Rothmund M,
Schumpelick V (eds) Praxis der Viszeralchirurgie Onkologische Chirurgie, vol 3. Springer,
Heidelberg, pp S713–S734
[Crossref]

Vaezi MF, Pandolfino JE, Vela MF (2013) ACG clinical guideline: diagnosis and management of
achalasia. Am J Gastroenterol 108:1238–1249; quiz 1250. https://​doi.​org/​10.​1038/​ajg.​2013.​196
[Crossref][PubMed]

von Lanz T, Wachsmuth W (2004) Praktische Anatomie. Bauch. Springer, Berlin

[Crossref]

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_2

2. Small Intestine and Appendix

Didier Mutter1
(1) Department of Digestive and Endocrine Surgery, NHC, Pôle Hépato-
Digestif, University Hospital Strasbourg, Strasbourg, France

Didier Mutter
Email: [email protected]

2.1 Anatomy of the Small Intestine

Key Points
– Longest part of the gastrointestinal tract
– Difficult endoluminal access
– Surgical exploration: one of the diagnostic modalities
– New diagnostic techniques available: non-invasive assessment by
double balloon endoscopy, imaging techniques (CT/MRI), capsule
endoscopy

2.1.1 Measured Values

– Length = 270–290 cm (from pylorus to cecum)
– Duodenum = approx. 20–25 cm
– Jejunum = approx. 100–110 cm
– Ileum = approx. 150–160 cm
– Diameter = 1.8–2.5 cm
– Surface:
– Small intestine tube (cylinder) = 0.69 m2 (for a 5 m long small
intestine)
– Functional resorptive surface = 120–900 m2 (depending on author)

2.1.2 Limits
– Duodenum: from pylorus to duodenojejunal flexure
– Jejunum: Oral border = from duodenojejunal flexure (Treitz ligament)
– Ileum: No clear boundary between jejunum and ileum; ileum to ileocecal
junction

2.1.3 Wall Structure of the Small Intestine

Four layers (from the outside to the inside):
– Serosa:
– Consisting of visceral peritoneum
– Coating of jejunoileum + anterior surface of duodenum
– Muscularis propria:
– Smooth muscles
– Fine longitudinal outer + thicker circular inner layer
– Plexus myentericus: between the two layers
– Submucosa:
– Fibroelastic connective tissue
– Includes vessels + nerves (Meissner’s plexus)
– Mucosa:
– Muscularis mucosae + lamina propria + epithelial cell layer
– Epithelial cell layer = goblet cells, Paneth cells, enterocytes and
enteroendocrine cells

Surface Multiplication (► Sect. 2.1.1)

– Plicae circulares (Kerck ring folds): Transverse folds of the mucosa
(prominent in the distal duodenum + jejunum) lead to an increase in
surface area from 0.69 to 1 m2
– Villi: surface multiplication factor up to 30-fold
– Microvilli: surface multiplication factor = 30

2.1.4 Circulation
Arterial Blood Flow
– Excluding superior mesenteric artery
– Exception = Proximal duodenum through branches of the truncus
coeliacus
– Division pattern of the superior mesenteric artery:
– Specific branches for pancreas
– Specific branches for distal duodenum
– Specific branches for small intestine
– Specific branches for ascending and transverse colon
– Collateral system = vascular arcades of the mesentery
– For the jejunum: long vasa recta of one or two arcades
– For the ileum: short vasa recta of 4–5 arcades (ileum: better blood
circulation)

Venous Drainage
– Superior mesenteric vein
– Drainage (with V. splenica) in V. portae hepatis (behind the neck of the
pancreas)

Lymphatic Drainage
– From the mucosa through the small intestine wall
– Draining of mesenteric lymph nodes
– Main drainage pathway of fats into the bloodstream
– Immunological role + role in the distribution of cells in the case of
malignant intestinal neoplasms

Mesenteric Base
– Fixed to the posterior abdominal wall
– From the left view of LWK 2, oblique to the right and caudal to the right
sacroiliac joint
2.1.5 Innervation
– Innervation of the small intestine = autonomic nervous system

Parasympathetic Component
– Fibres of the vagus nerve
– Function: influence on secretion, motor function + all phases of intestinal
activity

Sympathetic Component
– Nerve ganglia: Collected in the plexus around the superior mesenteric
artery.
– Function: vascular contractility, intestinal secretion and motor function
and pain sensation

2.1.6 Small Intestine Functions

Digestion and Nutrient Absorption
– Small intestine: main role in absorption of nutrients + water +
electrolytes + minerals
– Peristalsis = intestinal contractions from oral to aboral 1–2 cm/s
– Main function: transport of the chyme through the intestine
– Motor pattern different between digestive phase and sobriety

Endocrinological Function
– Small intestine = largest endocrine organ in the body
– Products: Hormones + Peptides
– Paracrine + autocrine functions + neurotransmitter function

Immunological Function
– Antigen processing, humoral and cellular immunity
– Lymphoid tissue: in the Peyer’s plaques, the lamina propria +
intraepithelial lymphocytes

2.2 Diseases of the Small Intestine

2.2.1 Clinical Presentation
Key Points
– Small intestine diseases = broad spectrum
– Most frequent small bowel disease = small bowel ileus after previous
surgery
– Exploratory laparoscopy/laparotomy = often the optimal solution:
– Free preparation of the intestine
– Resection or bypass
– Well accepted: Hardly any postoperative restrictions, resection
mostly limited
– Imaging: key role in diagnosis + optimal decision making in treatment
of small bowel disease

General
– Mostly unspecific Clinical Presentation
– Broad spectrum of clinical signs: From simple chronic pain to acute
peritonitis
– Clinical picture depends on the etiological underlying disease:
– Inflammatory bowel disease (Crohn’s disease) = most common small
bowel lesion
– Neoplastic lesions
– Small bowel obstruction (in the context of adhesions) (► Sect. 2.2.5)
– Other rare pathologies (e.g. Meckel’s diverticulum)

Inflammatory Bowel Disease

Development
– Onset: Often insidious; rarely also acute
– Medical history: Slow + protracted
– Alternatively symptomatic phases (abdominal pain + diarrhoea) and
asymptomatic phases
– Progressive increase in symptomatic phases: More frequent, longer and
with more pronounced symptomatology
Symptoms
– Inflammatory symptoms of the gastrointestinal tract
– Typical triad: Chronic recurrent episodes of diarrhoea + abdominal pain
+ weight loss
– Possible symptoms:
– Pain in the right lower abdomen (differential diagnosis: appendicitis)
– Hematochezia: blood in the stool
– Urge to stool
– Abdominal cramps and abdominal pain
– Feeling of incomplete evacuation
– Constipation (up to ileus)
– General Symptomatology:
– Fever
– Loss of Appetite
– Weight loss
– Fatigue
– Night sweats
– Menstrual irregularities
– Extraintestinal manifestations (30% of patients):
– Manifestation: GI (gastrointestinal) symptoms, dependent or
independent
– Skin lesions: Erythema nodosum and pyoderma gangraenosum…
– Arthritis/arthralgias
– Uveitis and iritis
– Hepatitis and pericholangitis
– Aphthous stomatitis
– Amyloidosis
– Pancreatitis
– Nephrotic syndrome
Complications
– Main complications = constipation + perforation
– Constipation to the point of ileus:
– Etiology = chronic fibrosing lesions, lumen obstruction (partial to
complete)
– Perforation:
– Free (rare) vs. covered
– Abscesses: Localized, formation in relation to the perforations…
– Fistulas:
– Etiology = adhesions due to inflammation
– Abnormal connection between two adjacent organs
– From the small intestine: to the small intestine, urinary bladder,
vagina, stomach, skin
– Generalized peritonitis = rare
– Perianal lesions (fissure, fistula, stricture, abscess): For anal/rectal
involvement
– Infestation of esophagus or stomach possible
– Malignant neoplasms of the small and large intestine: Crohn’s disease =
predisposition

Neoplastic Intestinal Diseases

– Variable onset of disease
Symptoms
– Early symptoms: mostly non-specific, over months to years
– Dyspepsia
– Anorexia
– Malaise
– Dull abdominal pain
– Pain: most frequent symptom (often due to obstruction, partly due to
intussusception)
– Intestinal bleeding: most frequent symptom
(haematochezia/haematemesis)
– Obstruction/Ileus: In 15–35% of patients due to tumor infiltration and
adhesions.
– Palpable mass: In 10–20% of patients.
– Perforation: up to 10% of patients (especially in sarcomas/lymphomas)
GIST (Gastrointestinal Stromal Tumors)/Carcinoid Tumors
(► Chapter 14)
– Special separate tumor entity
– Malignant carcinoid syndrome = rare (10% of cases)
– Hemodynamic manifestations: Flushing, asthma
– Cardiac manifestations: Cardiac lesions
– Intestinal manifestations: Diarrhea, hepatomegaly
– Specific markers:
– Elevated urine markers: 5-hydroxyindolacetic acid (5-HIAA, 24 h
measurement)
– Chromogranin A in serum (marker of neuroendocrine tumors)
– Metastases: Clinical presentation = as in other neoplastic diseases

2.2.2 Imaging
– Modalities:
– Radiological imaging
– Endoscopic imaging
– Indications:
– Atypical symptoms
– Complications (e.g. bleeding, obstruction)

Radiological Imaging
Conventional Abdominal Radiograph
– Obsolete, no longer primarily indicated (also due to radiation exposure)
– Allows exclusion of ileus; no information about etiology
– Ileus sign:
– Dilated loops of small intestine (with/without colonic dilatation)
– Multiple air-liquid levels
– Localization of the ileal height (proximal vs. distal)
CT Examination with Contrast Medium
– Gold standard for v. a. small bowel disease/ileus
– Pros:
– Localization of the affected segments
– Identification of the etiology (extra- or intraluminal lesion)
– Identification of complications: Ileus, intestinal ischemia (pneumatosis
intestinalis, “portal venous gas”), intestinal necrosis
– Staging examination (TNM) in the case of a malignant tumour
– Key examination for extraluminal lesions
Colon Contrast Enema
– Obsolete due to lack of meaningfulness
– Contraindicated due to risk of perforation and when surgery is indicated
CT Enterography/MRI Enterography
– High sensitivity and specificity in the diagnosis of small intestinal
diseases (especially chronic inflammatory bowel diseases)
– Principle:
– Small bowel distension (by oral intake of 1–2 L preparation 1 h before
examination)
– i.v. contrast medium
Abdominal Sonography
– Not much use in small bowel Diagnosiss
– Exception: intestinal ultrasound with contrast medium (chronic
inflammatory bowel diseases)

Endoscopic Imaging
Colonoscopy/Esophagogastroduodenoscopy (EGD)
– Very useful for CIBD (chronic inflammatory bowel disease):
– Visualization of aphtous ulcerations
– Cobblestone mucosa pattern
– Discontinuity of the segments concerned
– In the setting of atypical/malignant lesions: Ideal for biopsy confirmation
– For the treatment of proximal/distal bleeding
Double Balloon Endoscopy
– Access to most of the small intestine possible
– Biopsy of a lesion possible (especially after radiological imaging)
Capsule Endoscopy
– Currently standard method of examination of the small intestinal mucosa
– Application only after exclusion of a stenosis/obstruction (CT)
– Special software for image analysis (automated analysis of anomalies)
– New generation of capsules: Integrated biopsy system

2.2.3 Crohn’s Disease

Pathophysiology
– Benign lesion of the small intestine (mostly small intestine and colon)
– Chronic transmural inflammation of the intestinal tract (affected
segments: all from mouth to anus possible)
– Unknown etiology
– Pathogenesis: two hypotheses:
– Primary dysregulation of the mucosal immune system: excessive
immunological response against normal microflora; as consequences:
– Alteration in intestinal microflora or disrupted epithelial barrier
function: pathologic response of the normal mucosal immune system
– Triggering/Favoring Factors:
– Infections (Mycobacterium paratuberculosis)
– Immunological reactions (humoral and cellular)
– Genetic defects (IBD1 locus)
– Environmental factors
– Dietary factors
– Smoking

Diagnosis
– Clinical suspicion
– Laboratory chemistry: orienting, serological markers: perinuclear
antineutrophil cytoplasmic antibodies (p-ANCA) and anti-
Saccharomyces cerevisiae antibodies (ASCA)
– Atypical p-ANCA: in patients with IBD, especially ulcerative colitis
– Atypical p-ANCA + ASCA for the differential diagnosis of ulcerative
colitis (p-ANCA +) vs. Crohn’s disease (ASCA +)
– Atypical p-ANCA + ASCA prognostic for the development of IBD
– Imaging: to confirm the diagnosis by
– CT: typical = transmural bowel wall thickening; visualization of
extraintestinal complications
– Endoscopy: typical = aphtous ulcerations with granulations;
surrounding mucosa normal; biopsy: granulomas with Langerhans
giant cells; systematic exploration of the ileum

Differential Diagnosis (= Other Inflammatory Bowel Diseases)

– Infectious intestinal diseases (Yersinia, Campylobacter, Salmonella,
Shigella, Tuberculosis, Amoebiasis)
– Acute appendicitis
– For CIBD: Spontaneous symptom relief
– If symptoms during surgery: no resection, no biopsy (except emergency
indications: abscess, perforation)

2.2.4 Small Intestinal Neoplasms

Epidemiology
– Rare + insidious tumors = diagnosis difficult
– Necessity high level of suspicion
Small bowel = 80% of GI tract, 90% of mucosal surface; small bowel
tumors = rare (1–2% of GI malignancies)
– Often late diagnosis (advanced stage) = poor prognosis

Diagnosis
– Diagnosis through combined imaging modalities
– Flexible endoscopy: For lesions in the duodenum + terminal ileum
– Double balloon endoscopy: For the middle part of the small intestine
– Capsule endoscopy: contraindicated for malignant lesions with strictures
– CT:
– For the detection of extraluminal gastrointestinal stromal tumors
(GIST)
– For staging malignant tumors (mesenteric lymph nodes, liver
involvement, abdominal wall infiltration)
– Very sensitive (90% diagnostic certainty)
– Somatostatin receptor scintigraphy: higher sensitivity in the localization
and extension balance of these tumors
– Intestinal MRI is increasingly used
– Despite imaging modalities, diagnosis mostly during elective or
emergency surgery (e.g. carcinoid tumors)

Histological Classification
– GIST = most frequent tumour in the small intestine (mostly
asymptomatic)
– Adenomas = most frequent benign tumor in autopsy series
– Benign tumors = majority of small bowel neoplasms (mostly
asymptomatic + incidental findings)
Adenomas
– 15% of all tumours of the small intestine
– Incidence in ileum, jejunum, duodenum = 50%, 30%, 20%
– Symptoms (if symptomatic, but usually autopsy findings): obstruction,
bleeding
– Classification:
– True adenomas
– Villous adenomas: Mostly in the duodenum, possibly associated with
FAP (familial adenomatous polyposis); possible malignancy
– Brunner’s gland adenomas: hyperplastic lesions in the proximal
duodenum; can cause peptic ulcer; non-malignant = endoscopic
treatment
Hamartomas
– Part of the Peutz-Jeghers syndrome
– Heritable dominant pattern with high penetrance
– Mucocutaneous melanotic pigmentation + gastrointestinal polyps
– Small lesions (1–2 mm), brown-black, in the circumoral region of the
face, oral mucosa, forearms, palm, plantar, fingers and perianal region
– Complete jejunum + ileum affected; more rarely rectum, colon, stomach
– Clinical Presentation: abdominal colic (intermittent intussusception);
bleeding rare
Hemangiomas
– Submucosal vascular proliferation
– Mostly in the jejunum
– 3–4% of benign tumours of the small intestine; multiple in 60% of cases
– Possibly part of Osler-Weber-Rendu disease or Turner syndrome
– Symptoms: Often bleeding
– Treatment: Limited resection sufficient
Gastrointestinal Stromal Tumors (GIST)
– Most frequent mesenchymal tumors of the GI tract
– Pathogenesis:
– GIST cell: development of precursor cell of Cajal cell (myenteric
plexus)
– Activating mutation of KIT protein kinase receptor (CD117) or
platelet-derived growth factor receptor α (PDGFRA)
– Expression of CD117 and CD34
– Benign/malignant GIST = 3–4/1
– Localization: Throughout the GI tract; more common: stomach, small
intestine
– Symptoms:
– Intramural growth: obstruction (ileus)
– Extramural growth: larger mass, bleeding
– Risk of recurrence: mitotic index >2/50 “high-power fields” = increased
local recurrence/metastasis risk
– Malignant GIST:
– 20% of malignant tumours of the small intestine
– More frequent in the jejunum and ileum
– Mostly >5 cm diameter at diagnosis
– Origin = Muscularis propria: extramural growth
– Symptoms = obstruction, hemorrhage, perforation (due to
hemorrhagic necrosis)
– Metastasis: Hematogenous: Liver, lung, bone; lymphatic = rare
– Prognosis dependent on:
– Tumor size
– Mitotic index
– Invasion of the lamina propria
Adenocarcinomas
– 50% of malignant tumours of the small intestine
– Localization: Mostly duodenum + proximal jejunum
– Risk factors:
– FAP
– “Hereditary non-polyposis colorectal cancer” (HNPCC)
– Peutz-Jeghers syndrome
– Crohn’s disease
– Gluten Intolerance Enteropathies
– Biliary diversions
– Smoking
– Alcohol consumption (>80 g/dL ethanol)
– Consumption of red meat or food preserved in salt
– Prognosis dependent on:
– Disease stage
– Time of diagnosis: Usually late
Lymphomas
– Manifestation: Primary lesion or part of a systemic disease
– Lesion often in the ileum
– Often associated with celiac disease of immunodeficiency status
– Lesions usually large (>5 cm) with infiltration of the intestinal wall
– Symptoms:
– Pain
– Weight loss
– Nausea, vomiting
– Changes in bowel habits
– Complications:
– Perforation: Frequent (25% of cases)
– Fever = sign of systemic involvement
Small Intestine NET (Carcinoid Tumors)
Terminology: Small bowel NET = international consensus, instead of
carcinoid tumor of the small bowel (Small Bowel NET, SBNET)
– Pathophysiology:
– Carcinoid = part of the neuroendocrine tumors (NET)
– Tumor cells (= multipotent cells) originate from enterochromaffin
cells (Lieberkühn crypts of the small intestine)
– Tumor cells can produce substances (depending on the cells’ site of
origin): Serotonin, P-substance etc.
– Size growth = very slow
– After serosa invasion: severe desmoplastic reaction, mesenteric
fibrosis, intestinal kinking and intermittent obstruction
– Tumor location (in decreasing frequency):
– Appendix (most frequent localization) 45%
– Small intestine (second most common location, especially the last 60
cm of the ileum): Ileum 28%
– Rectum 16%
– Carcinoids mostly multicentric in the small intestine
– Often coexistence with another malignancy of a different type (colon
adenocarcinoma) or with multiple endocrine neoplasia type 1 (MEN1)
– Malignancy:
– Carcinoids of the ileum/jejunum = higher malignancy than carcinoids
of the appendix
 – Malignancy potential associated with: Tumor location, tumor size,
invasion, growth type.
– Carcinoids <1 cm: 2% are metastatic
– Carcinoids of 1, 2 or >2 cm: metastases in 50%, 80% and 90% of
cases
– Forecast:
– Carcinoid = best prognosis of all malignant tumors of the small
intestine
– 5-year survival = 65% (in patients with regional disease), 35% (in
patients with distant metastasis)
– Clinical Presentation:
– 70–80% of patients = asymptomatic; carcinoid = incidental finding
– Obstruction: In connection with intussusception due to tumor
– Carcinoid syndrome:
– Only a small percentage of patients
– Clinical Presentation: Episodic attacks of cutaneous flushing,
bronchospasm, diarrhea, and vasomotor collapse
– Treatment:
– Extended metastasis: palliative resection (because of slow-growing
tumors)
– NET of the midgut (high recurrence rate): Long follow-up, at least 7
years
Metastatic Lesions
– More common than primary tumors
– Mostly from intra-abdominal primary tumors
– Small bowel involvement:
– Through direct extension
– Due to peritoneal metastasis
– Metastases from extra-abdominal tumors rare (breast cancer, bronchial
cancer, skin melanomas)

2.2.5 Other Diseases of the Small Intestine

Diverticula and Meckel’s Diverticula
– Diverticulum of the small intestine = frequent occurrence
– Rarely symptomatic = usually no indication for surgery
– True diverticulum (congenital): Diverticulum consisting of all wall layers
Duodenum Diverticulum
– Second most frequent diverticular localization after colon
– Mostly periampullary (2-cm radius around the ampulla Vateri)
– Mostly originating from medial duodenal wall
– Mostly asymptomatic; diagnosis during endoscopy or imaging
– Complications:
– Occlusion of the choledochal duct/pancreatic duct
– Bleeding
– Perforation
– Blind Loop Syndrome
– Treatment:
– Asymptomatic/random findings: No treatment
– Surgical treatment: necessary in less than 5% of cases
Jejunum and Ileum Diverticula
– Rare, usually false diverticula
– Mostly multiple, protruding from mesenteric side of intestine
– Symptomatology (mostly chronic):
– Unclear abdominal pain
– Malabsorption
– Functional pseudoobstruction
– Low-grade bleeding
– Complications (rare):
– Diverticulitis
– Perforation
– Abscess
– Bleeding
– Obstruction/Ileus
– Treatment:
– If asymptomatic/random findings: No treatment
– In case of complication: resection + primary anastomosis
Meckel’s Diverticulum
– Most frequent congenital small intestine anomaly
– Localized antimesenteric side of the ileum, 45–60 cm proximal to the
ileocecal valve
– Mostly incidental finding
– Pathophysiology
– Origin = incomplete occlusion of the omphalomesenteric duct
– Cells of the omphaloenteric duct = pluripotent; Meckel’s diverticulum
often with heterotopic tissue: gastric, colonic, pancreatic mucosa
– Clinical Presentation:
– Bleeding (most common form of presentation)
– Obstruction/Ileus
– Volvulus or intussusception
– Incarceration
– Diverticulitis
– Treatment (symptomatic Meckel’s diverticulum) = surgery (usually
laparoscopic):
– Meckel’s diverticulum resection: transverse stapler resection
– Small bowel segment resection of the diverticulum-bearing segment

Ulcerations and Fistulas

Ulcerations
– Rarely
– Most often associated with Crohn’s disease, typhoid fever, tuberculosis,
lymphoma, lesions of gastrinoma
– Drug-induced ulcerations: Coated KCl tablets, corticosteroids, NSAIDs
(nonsteroidal anti-inflammatory drugs, ulceration usually in the ileum)
– Treatment (if necessary) = small bowel segment resection + anastomosis
Enterocutaneous Fistulas
– Etiology:
– Mostly iatrogenic
– Neighbour abscesses
– Traumas
– Rarely spontaneous (then in the context of Crohn’s disease)
– Risk Factors/Predisposition:
– Radiation in the anamnesis
– Intestinal obstruction
– CIBD
– Mesenteric vascular disease
– Intraabdominal sepsis
– Clinical Presentation:
– Generalized peritonitis: Rare
– Classification: In terms of localization and output volume (high vs.
low output)
– High-output fistula, if output ≥500 mL/24 h
– Proximal fistulas: More serious problem due to higher output,
electrolyte loss, malabsorption (distal segment eliminated)
– Poor prognostic factors (= no spontaneous healing):
– High-Output
– Severe interruption of intestinal continuity (>50% of circumference)
– Active CIBD
– Malignant disease
– Radiation enteritis
– Distal obstruction
– Undrained abscess
– Short fistula tract (<2.5 cm)
– Epithelialization of the fistula tract
– Treatment:
– Somatostatin: rapid reduction of output + shorter healing time of the
fistula
– Surgery: If no spontaneous healing
Small Bowel Obstruction/Ileus
– Most common disease of the small intestine
Etiology
– Postoperative adhesions (60%)
– Malignant diseases
– Crohn’s disease
– Hernia
Classification of Obstruction/Ileus
– Partial vs. complete
– Simple vs. Strangulated (Trapped)
Clinical Presentation
– Abdominal pain: colicky, intermittent
– High ileus: Briefly persistent + bilious vomiting
– Distal ileus: progressive pain, persistent for days + abdominal
distension
– Nausea, vomiting
– Diarrhoea or constipation
Complications
– Necrosis
– Perforation

Caution
Signs of necrosis/perforation are fever and tachycardia.

Diagnosis
– CT abdomen:
– Sensitivity = 90–96%, Specificity = 96%
– Very effective in evaluation of ileus + diagnosis of tissue damage
– Ideal in assessing which patient can be treated conservatively vs.
surgically
 – Effective in strangulation detection
– Effective detection of strangulation/complication by CT of the abdomen.
– Classic guiding paradigm of “never let the sun rise and set on an ileus”
no longer appropriate
– CT diagnosis also enable conservative treatment
Treatment
– Highly dependent on Clinical Presentation + etiologies (◘ Table 2.1)
– Depending on the CT findings
– Basic indication for surgery: in case of persistence of pain for hours
(laparotomy vs. laparoscopy)
Table 2.1 Therapeutic strategies for small bowel obstruction (ileus)

Etiology Type Management

Adhesions Partial SBO Non-operative treatment over 24–48 h
Intraluminal contrast medium examinations
Surgery
Complete and symptomatic Surgery
SBO
Neoplasia Primary Resection
Secondary Resection, bypass or stoma
Crohn’s disease Initial presentation Bowel resection
Perforation, phlegmon Bowel resection
Multiple strictures Bowel resection, stricturoplasty
Gallstone ileus Enterotomy
Radiation enteritis Bypass or resection
Meckel- Meckel’s or bowel resection
Divertickel
Invagination Spontaneous Reduction
Tumorous Resection
Bezoare Enterotomy, extraction
Fragmentation/propulsion into the caeca
NSAID stricture Bowel resection, stricturoplasty, balloon
dilatation
SBO small bowel obstruction

2.2.6 Treatment Strategies

Drug Therapy
– Must always be considered
Surgery = mostly overtherapy, unnecessary bowel resection + surgical
complications.
Crohn’s Disease
– Medicinal + surgical treatment = palliative
– Therapeutic objective: alleviation of acute exacerbation + alleviation of
complications
– Drug therapy: induction + maintenance of remission
– Aminosalicylates
– Corticosteroids
– Immunosuppressive drugs
– Antibiotics
– Anti-TNF antibody
Malignant Lesions
– Adjuvant radiotherapy/chemotherapy: Best survival rates
– Curative resection only in 50% of patients
– Metastases in 1/3 of cases already at the time of surgery
– 5-year overall survival = 25%
GIST/Small Bowel NET (Carcinoid)
– GIST: Targeted therapies must always be considered:
– Targeted therapy to specific molecules, e.g. imatinib with effect on
KIT protein and PDGFRA protein
– Small bowel NET/carcinoids:
– Long-acting somatostatin analogues (octreotide)
– Effective against symptoms; no proven action on tumor inhibition
– Also as palliative treatment for disseminated lesions
Surgical Treatment
General Principles
Segmental Small Bowel Resection + Anastomosis
– Treatment of choice in most cases
– Benign lesions: Limited resection (short segment of small bowel +
limited division of mesentery).
– Malignant lesions: Oncologic resection (with control of vessels at their
origin + lymphadenectomy + free small bowel resection margins)
Laparoscopic Resection
– Mostly for GIST

Surgical Procedure
Standard Procedure: Laparoscopic GIST Resection
– Lesion must be presentable laparoscopically
– Transillumination (diaphanoscopy) to visualize the vascular supply of
the segment to be resected
– Incision of the mesentery, control of the vessels by ligatures or vessel
sealing systems: ultrasound dissectors (Sonicision, Covidien), high-
frequency thermal fusion devices (LigaSure, Covidien)
– Cutting through the intestine using a 60-mm linear stapler (e.g.
EndoGIA, Covidien)
– Isoperistaltic side-to-side anastomosis to restore intestinal continuity:
antimesenteric opening of the two intestinal segments, anastomosis
using a linear stapler via this incision, closure of the intestinal incision

Caution
Short bowel syndrome = risk in small bowel resection due to resection
of healthy tissue
Therefore, always weigh well the indication for resection

Bypass Procedures
– In selected cases of ileus/obstruction
Treatment of CIBD (E.g. Crohn’s Disease)
Indications for Surgical Treatment
– Obstruction/Ileus
– Perforation
– Fistula or abscess
– Bleeding
– Complications affecting adjacent tissues
Strategy
– Preoperative imaging: essential for the clarification of multiple lesions
– Treat the affected bowel segment specifically
– Limit to one short bowel segment (recurrent resection of long segments =
no better outcome + risk of short bowel syndrome)
– Obstruction/Ileus: Mostly partial/temporary
– Drug therapy indicated
– In targeted cases: Endoscopic dilatation
– If surgery is necessary: Segmental resection + primary anastomosis
– Intraoperatively: always careful exploration (macroscopy + palpation) of
the entire peritoneal cavity (to exclude secondary lesions)
– In case of obstruction by strictures:
– Stricturoplasty = longitudinal incision of the fibrotic tissue
(preservation of the mucosa) + transverse closure
– Indications for this technique:
– Multiple stricture areas in long segments
– For patients who have already undergone resection
– If stenosis due to fibrosis: no acute inflammation
– In generalized peritonitis: external enterostomy indicated
Treatment of Benign Lesions
– Potentially malignant lesions: Resection like malignant lesions
– Symptomatic benign lesions: Endoscopic destruction/mucosal resection
– Segmental resection: laparotomy/laparoscopy; possibility of
intraoperative identification of the lesion
– Always complete small bowel exploration to exclude other lesions
– Treatment of complications (obstruction/bleeding): Surgery
Treatment of Malignant Lesions
– Malignant tumors: obligatory oncological resection + regional
lymphadenectomy
– Carcinoid tumors: treatment depends on tumor size + localization +
presence of metastases:
– Tumor <1 cm without local lymph nodes = segmental small bowel
resection
– Tumor >1 cm, multiple or regional LK metastases = oncological
resection (wide bowel resection + mesentery)
– Involvement of the terminal ileum = hemicolectomy on the right side
– Cholecystectomy indicated: Because of lifelong somatostatin analogue
treatment in most patients…
– Metastases = surgery in the sense of debulking (symptom relief)

2.3 Vermiform Appendix

2.3.1 Anatomy of the Vermiform Appendix
Normal Anatomy
– Base:
– Localized at convergence of the long taeniae (inferior surface) of the
caeca
– Anatomical relationship allows localization during surgery
– Tip: Most often retrocecal in the peritoneal space

Localization Variations (According to Wakeley and Testut &

Latarjet)
– Retrocecal (65%)
– Pelvin (31%)
– Subcaecal (2%)
– Preileal (1%)
– Rare variations (1%)
The different localizations form the origin of the myriad of symptoms in
acute appendicitis.
Circulation and Lymphatic Drainage
– A. appendicularis: branch of A. ileocolica
– Lymphatic drainage to the anterior ileocolic lymph nodes

Caution
Because of the prevention of postoperative bleeding, it is essential to
control the appendicular artery during appendectomy (need to know
the anatomy).

Histological Features
– Mucosa: goblet cells (distributed in mucosa): Mucus production
– Submucosa: Lymph follicle = important defence function (early stages of
development)

2.4 Diseases of the Appendix

2.4.1 Appendicitis Vermiformis
Key Points
– One of the most common acute digestive diseases in children/adults
– Treatment (appendicitis and complications) as a therapeutic challenge,
depending on:
– Clinical Presentation
– Biochemistry
– Imaging
– Current standard = laparoscopic appendectomy
– Alternative treatment (for uncomplicated appendicitis) = conservative
antibiotic treatment
– Complications: Abscess/perforation

Physiopathology
– Etiology of appendicitis = appendiceal stump obstruction
– Obstruction by stool, lymphoid hyperplasia, food debris (fibers),
parasites, neoplasms.
– Obstruction:
– Bacterial overgrowth + mucus accumulation
– Intraluminal distension
– Increase in wall pressure
– Loss of the epithelial mucosal barrier
– Perforation (after about 48 h after onset of symptoms) +
abscess/peritonitis

Symptoms
Initial Symptoms
– Periumbilical pain = visceral pain (due to luminal distension)
– Nausea + Vomitus
Progressive Symptoms (Due to Inflammation of the Surrounding
Structures)
– Localized pain in the right lower quadrant
– Possible vomiting
– Fever: parallel with leukocytosis + CRP elevation
Other possible symptoms: urological symptoms, diarrhoea, paralytic
ileus, functional intestinal obstruction.

Clinical Presentation: Biochemistry

Clinical Presentation
– Local pain at McBurney point (possibly with defensive tension)
– Dunphy’s sign: pain in the right lower abdomen during coughing
– Rovsing’s sign: pain in the right lower abdomen on retrograde palpation
of the colon
– Blumberg sign (= release pain): Pain in the right lower abdomen after
release of pressure in the left lower abdomen
– Obturator sign: pain in the right lower abdomen on internal rotation of
the hip = sign of pelvic appendicitis
– Iliopsoas sign: pain in the right lower abdomen with extension of the
right hip = sign of retrocecal appendicitis
– With perforated appendicitis: pronounced intense pain + diffuse
contracture
Biochemistry
– Mostly leukocytosis >11.5 × 103/mm3
– Elevated CRP
In the absence of one of these two signs appendicitis is unlikely. Here,
surveillance should be continued (for prophylaxis of unnecessary surgery).

Imaging
– Necessary to ensure diagnosis (prevention of unnecessary surgery)
– Necessary in case of an uncertain diagnosis
Ultrasound (US)
– Sensitivity = approx. 85%; specificity >90%
– Signs of acute appendicitis:
– Anteroposterior diameter of the appendix ≥7 mm
– Thick-walled appendix
– Noncompressible luminal structure
– Cocard sign: Target in the transverse section of the appendix
– Appendicolite
Computer Tomography (CT)
– Standard imaging in acute appendicitis
– Sensitivity = 90%, specificity = 80%
– Negative appendectomy after CT = rate < 10%
– No increase in perforation rate
Diagnostic Laparoscopy
– In case of uncertain diagnosis
– Direct examination of the appendix + peritoneal cavity (other diseases)
– Indications: Primarily in young women with questionable US/CT
findings

Differential Diagnosis
Operative Differential Diagnosis
– Invagination
– Meckel’s diverticulitis
Non-Operative Differential Diagnosis
– Acute gastroenteritis
– Mesenteric lymphadenitis
– CIBD
– Constipation
– Functional pain
– Pyelonephritis
– Colitis
– Diverticulitis
– Ileus
– Tumour of the GI tract
Gynaecological Differential Diagnosis
– Tuboovarian abscess
– Torsion of the ovary
– Ruptured ovarian cyst
– Ectopic pregnancy
– Gynecological tumors (uterus, tube, ovary)
The large number of differential diagnoses (mostly nonoperative)
increases the importance of preoperative imaging.

Surgical Treatment Modalities

Treatment Strategy
Early Surgical Appendectomy
– In most cases of acute appendicitis
Antibiotic Treatment
– Perioperative antibiotic prophylaxis indicated: second generation
cephalosporins (cover aerobic + anaerobic contamination)
– Systematic antibiotic treatment not recommended (no influence on
postoperative complications)
– Non-perforated appendicitis: perioperative single dose, no postoperative
antibiotics to reduce postoperative wound infection/intra-abdominal
abscesses
– Perforated/gangrenous appendicitis: postoperative intravenous antibiotics
until patient is afebrile (at least 5 days)
Laparoscopic Appendectomy
Minimally Invasive (Laparoscopic) Appendectomy = Currently the
Gold Standard

Surgical Procedure
Laparoscopic Appendectomy
– Positioning as for open appendectomy (supine position, legs together,
right arm abducted 90°, left arm along the body)
– Surgeon + assistant to the left of the patient; monitor to the right of the
patient
– Standard instrumentation: Laparoscopy tray: 0° or 30° laparoscope
with HD camera, lap scissors, atraumatic fenestrated graspers,
monopolar and bipolar coagulation grasper, clip applicator, irrigation
aspirator, Röder loops (e.g. Surgitie ligating loop, Covidien),
endoscopic salvage bag; possibly stacker (e.g. EndoGIA linear
staplers, Covidien), suture material. Trocars: One 10- to 12-mm trocar
(optics), and two 5-mm trocars (working trocar)
– 10-mm optic trocar placed subumbilically through open access; two 5-
mm trocars suprapubically and laterally of the left rectus abdominis
muscle under visual control
– Patient in Trendelenburg position + turned to the left side
– First step = exploration of the peritoneal space
– Confirmation of the diagnosis + exclusion of differential diagnoses,
especially Meckel’s diverticulum, adnexa
– Second step = dissection:
– Adequate presentation of the appendix (consequences of taenia of
the caecum) + mobilization (adhesiolysis)
– Elevation of the appendix + transection of the mesenteriolum
(bipolar forceps, bipolar scissors) until adequate visualization of
the appendix base
– Control of the appendicular artery:
 – Monopolar/bipolar coagulation, vessel sealing devices (e.g.,
LigaSure, Covidien), stapler, suture of the artery (no technique
comparatively better)
– Third step: Setting down the appendix
– Prior to this, the base is treated with 2–3 Röder loops (e.g. Surgitie
Ligating Loop, Covidien)
– Setting down of the appendix; in case of very inflamed/necrotic
stump: stapler (staple suture device) with possibly distal part of the
caecum (gangrenous appendicitis, pronounced inflammation of
caecal base, abscess, perforation, peritonitis)
– Fourth step: extraction of the appendix
Using a salvage bag to prevent contamination of the abdominal wall

Controversy with Normal Appearing Appendix at Laparoscopy

– Leave appendix vs. appendectomy
– Always complete exploration of the abdominal cavity to exclude
differential diagnoses (e.g. Meckel’s diverticulum, Crohn’s disease,
mesenteric lymphadenopathies, pelvic disease, abscesses, ovarian
torsion, hernias)
– Current position: After exclusion of differential diagnoses =
appendectomy
– Arguments for appendectomy (expert opinion):
– Infection of the mucosa often inapparent in early phase
– Risk for re-operation > Risk for removal of a normal appendix
– Since 1894 standard = open appendectomy (McBurney incision)
– For about 20 years standard = minimally invasive appendectomy (also
for complicated appendicitis)

Advantages of the Minimally Invasive Procedure

– Less postoperative pain, shorter hospital stay, rapid recovery, low
complication rate, lower readmission rate, better quality of life
– For perforated appendicitis: fewer wound infections
– Diagnostic appendectomy: Useful in cases of uncertain diagnosis
 – Conversion laparoscopic open: Very rare

Resection Technique
Retrospective study (Mutter and Marescaux 2013): Consecutive series with
262 patients:
– Resection technique
– Endoscopic ligation: 207 cases (79%)
– Stapler appendectomy: 55 cases (21%)
– Indication for Stapler appendectomy given by the surgeon:
– Severe inflammation: 38 cases (69%)
– Questionable viability of the appendage base 14 cases (25.5%)
– Necrosis of the appendix base 3 cases (5.5%)
Evidence-Based Approach
– Inverting the appendiceal stump into the caecum: No evidence of benefit
– Need for bipolar coagulation of the mucosa of the appendiceal stump
(prevention of abscess by secretion); risk of local necrosis by monopolar
electric current and possible postoperative fistula
– Irrigation: no evidence of benefit; risk of spreading germs (Douglas
abscess); “suction only strategy” recommended
– Fascia of the 10 mm trocar must be adapted
Open Appendectomy
McBurney Incision
– McBurney incision = conventional approach
– Allows easy access to the appendix
– Limitations:
– Complete abdominal exploration impossible
– Impossible adnexal exploration
– Mostly oversized incision (does not correspond to the theoretical ideal
incision)
Median Laparotomy
– Indications:
– If McBurney is insufficient for adequate exploration or if the appendix
is very inflamed
– In exceptional cases of serious intra-abdominal complications (need
for preoperative imaging)
– Some of these cases can be treated with medication, interventions or
conservatively

Surgical Procedure
Open Appendectomy
– McBurney incision: oblique incision in the right lower quadrant of the
abdomen
– Distraction of muscles (prevention of postoperative hernias)
– Opening of the peritoneum
– Localization of the appendix (follow taenia of the caecum) +
advancement in front of the abdominal wall; minimization of the risk
of rupture by careful manipulation of the inflamed tissue
– Severing of the mesenteriolum between clamps + ligation
– Skeletonization of the appendix base + ligation using absorbable
sutures
– Deposition of the appendix after clamping
– Abdominal wall closure; no drainage recommended

Drug Therapy
– Indicated in two situations:
– Uncomplicated appendicitis—only (CT evidence)
– Severe complications of appendicitis supportive
Uncomplicated Appendicitis
– Surgical therapy = still standard for complicated appendicitis
– Evidence-based:
– Effectiveness in the treatment of uncomplicated appendicitis:
antibiotic = operative
 – Need for adequate CT diagnosis: markers of uncomplicated
appendicitis
– Duration of antibiosis (e.g. amoxicillin + clavulanic acid) = 14–21
days
– Antibiotic therapy of uncomplicated appendicitis: supported by studies
(Vons et al. 2011; Spirt 2010; Varadhan et al. 2012)
Severe Complications (Depicted by Imaging)
– Perforation: 23–73% of cases
– Perforation with abscess: 10–13% of cases
– Aim of drug therapy = to prevent major/difficult surgical procedures
Treatment Strategy
– Abscesses >5 cm: Interventionally guided drainage
– Abscesses <5 cm: Antibiotic treatment (treatment of the acute phase) +
appendectomy after 6–8 weeks

Caution
Periappendicular abscess:
– Surgery: increased risk of bleeding, wound infection, fistula,
adhesions
– Perioperative appendiceal abscess: non-operative treatment
(reduction of complications)

2.4.2 Malignant Diseases

– Primary tumors of the appendix = Rare
– Usually only diagnosed postoperatively (in cases of appendicitis) in the
pathological examination
– Most common: mucinous tumors and carcinoid tumors of the appendix

Mucocele of the Appendix

Pathophysiology
– Appendiceal lumen obstruction with intraluminal accumulation of
mucus: appendiceal distension and mucocele
– Histological classification (Histology appendiceal mucosa):
– Benign epithelium with retention cyst
– Hyperplasia/low-grade atypia = low-grade mucinous appendiceal
neoplasia
– Malignant = mucinous adenocarcinoma
Epidemiology
– Simple/hyperplastic mucoceles (acellular mucus) = 5–25% of cases
– Mucinous cystadenoma (63–84% of cases): Appendix neoplasia with
dysplastic epithelium (analogous to colonic polyps)
– Mucinous adenocarcinoma (11–20% of cases): High-grade cell dysplasia
and invasion of muscularis mucosae + stromal invasion
Clinical Presentation
– Mostly unspecific
– Most frequently: Clinical Presentation of acute appendicitis (► Sect. 2.4)
– Possibly palpable tumor
– Asymptomatic patients = 25–50%
Diagnosis
Tumor Marker (Preoperative)
– CEA (“carcinoembryonic antigen”): possible indication of malignancy
Sonography
– Encapsulated cystic lesion in the lower right quadrant
– Liquid content with different echogenicity (mucus density)
– Multiple echogenic layers in the dilated appendix = pathognomonic
CT Abdomen
– Cystic mass with thin low-density wall, direct communication to the
caecum
– Linear/spotted calcifications of the wall = typical for mucocele of the
appendix
– No calcifications of the wall in appendiceal abscesses
Colonoscopy
– Soft erythematous mass with central ulceration (= protrusion of the
appendicular ostium)
– To exclude synchronous neoplastic lesions of the colon (in up to 20% of
cases)
Treatment
– Surgical therapy (strategy analogous to conventional appendectomy)
– Extent of resection: Depending on histology + extent of disease
Retention Cysts
– Resulting in: chronic obstruction of the appendiceal lumen
– Mucosa: Flat cuboidal epithelium
– Surgical extent = Simple appendectomy sufficient
Appendix Mucoceles
– Appendiceal mucoceles <2 cm without intraoperative rupture = benign
– Appendiceal mucoceles >2 cm = neoplastic
– Operation Extent:
– Appendectomy: resect appendiceal mesentery (for histological
examination of lymph nodes) + prevent rupture (in case of
manipulation/extraction)
– Right hemicolectomy only in case of infiltration of the residual limb
Ruptured Mucocele (= Pseudomyxoma Peritonei/Mucinous
Carcinomatosis)
– Rupture = displacement of epithelial cells + mucus in the peritoneal
space
– Clinical Presentation:
– Appendicitis signs
– Increased abdominal girth
– Ovarian mass
– Inguinal hernia
– Treatment: CRS (cytoreductive surgery) + HIPEC (hyperthermic
intraperitoneal chemotherapy)
Forecast
Prognostic Factors
– Extent of the peritoneal tumor conglomerate
– Histological grade of the tumor
Course of the Disease
– In most patients: Dissemination of tumor cells in the abdomen at the time
of diagnosis
– Most of these neoplasms are noninvasive
– Metastases = rare; locoregional recurrence = frequent (ileus)
Survival
– Without treatment: Very poor prognosis (no chance of cure + very limited
survival)
– With aggressive CRS + HIPEC: 5-year survival = 50–96% in selected
patient groups (if no distant metastases + complete cytoreduction)
– CRS + HIPEC must be performed early in the disease history

Carcinoid Tumors: Neuroendocrine Tumors of the Appendix

– Carcinoid tumors of the appendix = part of the carcinoids of the midgut
(common embryological origin)
– Terminology: carcinoids of the appendix = well-differentiated
neuroendocrine tumors (NET) of the appendix
– Histology: NET = enterochromaffin cells (expression of S-100)
– NET = malignant tumours with benign behaviour pattern
Epidemiology
– Appendix-NET = 5% of all intestinal carcinoids
– Mostly in patients around 40 years of age
– Appendiceal carcinoids <1 cm + not localized in appendiceal base = 90%
– Appendectomy = sufficient treatment
– Mostly retrospective postoperative diagnosis
Diagnosis
– Depending on the presence of a Clinical Presentation
Non-functional NET
– Slow growing
– Mostly years until first symptoms and diagnosis
– Mostly diagnosis in the context of surgery (pathology)
Functional NET
– Carcinoid syndrome: hormonal production = symptoms + dosable
products
– Marker:
– 5-HIAA (degradation product of serotonin) test in urine
– Chromogranin A
– Serotonin
Environment Diagnosis
– As with other NET: possibility of other gastrointestinal genitourinary
tumors
– Colonoscopy: to exclude other colon tumors
– Other screening examination depending on age and other risk factors

Treatment (According to Recommendations of the American

National Cancer Institute and ENETS)
– ◘ Figure 2.1
– NET <1 cm, not in appendix base
– Appendectomy
– NET >2 cm
– Hemicolectomy right + ileocecal lymphadenectomy (risk of
metastases)
– 1 cm < NET < 2 cm
– Treatment controversial
– Hemicolectomy on the right:
– When infiltration of the mesoappendix
– If R1 at the resection margin
– If N+ (lymph node metastases)
 – In case of high proliferation activity (high Ki67 index), high mitotic
index, angioinvasion
– If mixed histology (goblet cell carcinoid)

Fig. 2.1 Treatment algorithm of NETs of the vermiform appendix according to the recommendations
of the American National Cancer Institute and the ENETS* R0 = tumor-free resection margins**
very limited evidence*** risk factors are: V1 (histologic vascular invasion)—L1 (histologic
lymphatic vascular invasion)—G2 grading—>3 mm infiltration or infiltration of the mesoappendix

Goblet Cell Carcinoid or Adenocarcinoid

– Rare variant with mixed endocrine and exocrine properties
– Associated with poor prognosis

Aftercare
– Monitoring in patients with elevated chromogranin A (indicator for
extended resection)
– Survival:
– Excellent for locoregional tumors
– Tumours with distant metastases: 10-year survival = 30%

Noncarcinoid Tumors of the Appendix

– Appendix = possible location of all intestinal tumors
– Rare tumor entities
Classification According to World Health Organisation (WHO) (◘ Table
2.2)
– Epithelial tumors
– Non-epithelial tumors
Table 2.2 Histological WHO classification of appendiceal tumours

Epithelial tumors Non-epithelial tumors

Adenoma Neuroma
Tubular Lipoma
Villös Leiomyoma
Tubulovillous Gastrointestinal stromal tumor
Serrated Leiomyosarcoma
Kaposi’s sarcoma
Other
Carcinoma Malignant lymphoma
Adenocarcinoma
Mucinous adenocarcinoma
Signet ring cell carcinoma
Small cell carcinoma
Non-differentiated carcinoma
Carcinoid (well differentiated neuroendocrine neoplasia) Secondary tumors
Tubular carcinoid Hyperplastic (metaplastic) polyp
Goblet cell carcinoid (mucinous carcinoid)
Mixed carcinoid adenocarcinoma
Other

Overview: Appendix Adenocarcinoma

 – Rare: 0.08% of all carcinomas
– Mucinous appendiceal adenocarcinoma = most frequent subtype (better
prognosis after resection)
Clinical Presentation
– Appendicitis in the elderly patient
– Mucocele
Treatment
– Hemicolectomy right = standard procedure
– Drug treatment/chemotherapy for specific diseases (lymphomas etc.)

References
Spirt MJ (2010) Complicated intra-abdominal infections: a focus on appendicitis and diverticulitis.
Postgrad Med 122:39–51
[Crossref][PubMed]

Varadhan KK, Neal KR, Lobo DN (2012) Safety and efficacy of antibiotics compared with
appendicectomy for treatment of uncomplicated acute appendicitis: meta-analysis of randomised
controlled trials. BMJ 5:344

Vons C, Barry C, Maitre S, Pautrat K, Leconte M, Costaglioli B, Karoui M, Alves A, Dousset B,

Valleur P, Falissard B, Franco D (2011) Amoxicillin plus clavulanic acid versus appendectomy for
treatment of acute uncomplicated appendicitis: an open-label, non-inferiority, randomized controlled
trial. Lancet 377:1573–1579
[Crossref][PubMed]

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F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_3

3. Colon
Oliver Thomusch1
(1) Department of General and Visceral Surgery, University Hospital Freiburg,
Freiburg, Germany

Oliver Thomusch
Email: [email protected]

3.1 Anatomy and Physiology

3.1.1 Definition and Limits
– Definition
– Colon = Intestinum crassum, part of the gastrointestinal tract
– Distally from the small intestine, proximal from the rectum
– Limits
– Proximal = ileocecal valve (= Bauhin); border between terminal ileum and
colon
– Distal = indistinct; transition zone between sigmoid colon and rectum =
transition of the taenia into closed longitudinal muscles of the rectum

3.1.2 Tasks
– Transport of chyme
– Thickening of chyme
– Active Na+ resorption (with diffusion of H2O)
– Absorption capacity = up to 5 L/day (9 L in total in the whole gastrointestinal
tract)
– Active secretion of K+, Cl−, HCO3−
– Further development of chyme
– Breakdown of the cellulose content by bacterial colonisation of the colon (1010
bacteria/g faeces)
– Resorption of 40–50% of the fibres

3.1.3 Location and Classification

– Five colonic segments from proximal to distal:
– Caecum (= appendix, coecum) with appendix vermiformis (= appendix)
– Ascending colon
– Transverse colon
– Descending colon
– Colon sigmoideum (= sigma)
– Position of the colon in relation to the peritoenum
– Caecum (distal part) + appendix: Intraperitoneal
– Caecum (proximal part): Secondary retroperitoneal
– Ascending colon: Secondary retroperitoneal
– Transverse colon: Intraperitoneal
– Descending colon: Secondary retroperitoneal
– Sigmoid colon: Intraperitoneal

3.1.4 Measured Values

– Colon length = 1/4 of the length of the intestine (in adults 140–160 cm)
– Lumen of the colon varies in size: in adults
– Caecum = approx. 6.5 cm (maximum width approx. 9 cm)
– Colon ascendens = approx. 5.5 cm
– Transverse colon approx. 5 cm
– Descending colon = approx. 4 cm
– Sigmoid colon = approx. 5.5 cm

3.1.5 Characteristic Features of the Colon

– Taeniae coli
– 0.5–1 cm wide light bands = shirred outer longitudinal muscles of the colon
– Beginning at cecum-appendix junction to sigmoid colon
– Three Taenia: libera, mesocolica, omentalis
– Haustra coli
– Puffed sleeve-like protrusions of the colon between plicae semilunares coli
– Plicae semilunares coli
– Crescent-shaped mucosal folds of the colon
– Appendices epiploicae
– Small sac-like protrusions of the colonic serosa filled with fatty tissue from tela
subserosa
– Located near the Taeniae libera and mesocolica

3.1.6 Blood Supply and Drainage

Arteries
– Superior mesenteric artery (ileocolic artery + right colic artery + middle colic
artery): Caecum + appendix + ascending colon + transverse colon
– Inferior mesenteric artery (left colic artery + sigmoid artery): left half of
transverse colon + descending colon + sigmoid colon
– Riolan anastomosis = anastomoses between two watershed areas: Between middle
colic artery and left colic artery (Ramus ascendens), supply area superior and
inferior mesenteric arteries

Veins
– Veins parallel to the arteries = portal vein/Henle loop

Lymphatic Drainage (◘ Fig. 3.1)

– Along the associated arteries
– Paracolic at the small blood supply vessels (arcades)
– Intermediate: Along the main vessels
– Central: In the area of the aorta
Fig. 3.1 Lymphatic drainage of the colon. 1 Ileocolic artery, 2 Right colic artery, 3 Middle colic artery, 4 Left
colic artery, 5 Sigmoid arteries, 6 Inferior mesenteric artery

Caution
Lymphatics along the marginal arteries = LN (lymph node) metastases to
proximal and distal possible: safety distance to the proximal and distal resection
border = 10 cm

3.2 Benign Diseases of the Colon

3.2.1 Diverticulosis and Diverticulitis
Key Points
– Acquired benign disease of the colon; incidence increasing with age
 – Complications: Diverticulitis, hemorrhage, abscess, perforation, stenosis…
– Therapy: primarily conservative; surgery: in case of complications/recurrent
diverticulitis

Definitions
Colon Diverticulum
– Acquired protrusion of the intestinal wall
– Diverticulum: Protrusion of the entire intestinal wall
– Pseudodiverticulum: Protrusion of the mucosa + submucosa through muscle-weak
gaps of the colon wall
Diverticular Disease
– Occurrence of symptoms/complications in the context of diverticulosis
Diverticulitis (= Pathological)
– Peridiverticulitis: inflammatory process originating by the colonic diverticulum
– Pericolitis: spread to the intestinal wall (= focal pericolitis)

Epidemiology
– Prevalence of diverticulosis:
– Increases with age
– Under 40 years = rare
– 60 years = approx. 30%
– 85 years = approx. 65%
– Men:Women = 1:1
– Diverticular disease: clinical symptomatic = 10–25% of diverticular carriers
(complications in 5%)
– Diverticulitis: incidence = 80–126/100,000 population/year

Etiology/Pathogenesis
– Multifactorial
– Increased intraluminal pressure
– Weakness of the intestinal wall: mostly passage of the vessels
Sigmoid colon = high pressure zone = increased intraluminal pressure; 90% of
diverticula in the sigmoid.

Risk Factors
– Higher prevalence at older ages
– Genetic predisposition (e.g. Marfan syndrome, Ehlers-Danlos syndrome,
polycystic kidney disease)
– Dietary fiber deficiency
– Higher body weight (BMI >30 kg/m2)
– Recurrence rate after acute diverticulitis: depending on severity (between 2% and
35%) (= guideline)
– Complicated diverticulitis:
– Relevant mortality (0–13%)
– Special risk under immunosuppression (8–24%)
Increased prevalence of colorectal cancer in diverticulosis is not proven.

Complications
– Diverticulitis (see above)
– Development due to stool retention = bacterial growth in the diverticulum =
inflammation
– Initial lesion before abscess, perforation
– Diverticular bleeding
– In 5% of diverticula carriers (= hematochezia)
– Independently of inflammation
– Risk = age, nonsteroidal anti-inflammatory drugs, right-sided diverticula
(Asian patients)
– Abscess and/or fistula formation
– Severe complications
– Covered perforation/open perforation with peritonitis
– Stenosis

Symptoms
– Diverticulosis = asymptomatic
– Diverticulitis:
– Left lower abdominal pain (abrupt onset, rapidly progressive)
– Possibly pressure-painful roller (palpatory)
– Fever, nausea, vomiting, dysuria
– Change in bowel movements, possibly blood in the stool
– Diverticular bleeding
– Painless peranal bleeding
– Sustained/Intermittent
– Spontaneous healing (80% of cases)
 – High recurrence rate

Classifications
– Classification of diverticular disease according to the German S2k guideline of the
AWMF (Classification of diverticular disease, CDD) (◘ Table 3.1)
– Classification according to Hinchey (for perforated sigmoid diverticulitis) (◘
Table 3.2)
– Classification according to Hansen and Stock (for sequence: diverticulosis,
diverticulitis, complications) (◘ Table 3.3)
Table 3.1 Classification of diverticulitis/diverticular disease (CDD)

Type 0 Asymptomatic diverticulosis

Incidental finding; asymptomatic
No disease
Type 1 Acute uncomplicated diverticular disease/diverticulitis
Type Diverticulitis/diverticular disease without Symptoms related to the diverticula
1a environmental reaction
Inflammatory signs (laboratory): optional
Typical sectional imaging
Type Diverticulitis with phlegmonous bypass reaction Inflammatory signs (laboratory): obligatory
1b
Sectional imaging: phlegmonous diverticulitis
Type 2 Acute complicated diverticulitis
as 1b, additionally:
Type Microabscess Covered perforation, small abscess (≤1 cm);
2a minimal paracolic air
Type Macroabscess Para- or mesocolic abscess (>1 cm)
2b
Type Free perforation Free perforation, free air/liquid
2c
Generalized peritonitis
Type Purulent peritonitis
2c1
Type Fecal peritonitis
2c2
Type 3 Chronic diverticular disease
Recurrent or persistent symptomatic diverticular disease
Type Symptomatic uncomplicated diverticular disease Typical clinical presentation
3a (SUDD)
Inflammatory signs (laboratory): optional
Type Recurrent diverticulitis without complications Signs of inflammation (laboratory) present
3b
Cross-sectional imaging: typical
Type Recurrent diverticulitis with complications Detection of stenoses, fistulas, conglomerate
3c
Type 4 Diverticular bleeding Detection of the source of bleeding

Table 3.2 Classification of perforated sigmoid diverticulitis according to Hinchey

Division Definition
Hinchey I Local pericolic abscess after perforation into the mesocolon
Hinchey II Distant abscess
IIA Circumscribed, drainable distant abscess
IIb Diffuse abscess with fistula formation
Hinchey III Purulent peritonitis
Hinchey IV Fecal peritonitis

Table 3.3 Classification according to Hansen and Stock

Division Definition
Stage 0 Asymptomatic diverticulosis
Stage 1 Diverticulitis without intestinal wall overflow, clinically unspecific complaints, inconspicuous CT
findings
Stage 2a Phlegmonous form, on CT extension into the pericolic fat tissue
Stage 2b Spread of inflammation to adjacent organs by covered perforation, extraluminal gas inclusions in CT
or abscess formation
Stage 2c Free perforation, clinical signs of acute abdomen and evidence of free air
Stage 3 Chronic recurrent, development of intestinal wall fibrosis and luminal narrowing

Diagnosis
Medical History (Medication, Tobacco Consumption)
Clinical Examination
– Abdomen: palpation, auscultation, digital-rectal examination
– See below Symptoms
– Measurement of body temperature
Laboratory Tests
– Leucocytosis
– CRP elevation, accelerated blood sedimentation
– Urine analysis
– In sepsis: elevated procalcitonin
Diagnostic Imaging
– Ultrasound examination:
– Bowel wall thickening
 – Dom sign: Hypo-echoic lesion, eccentric next to the intestinal wall (=
inflammatory diverticulum)
– Abscess, fatty tissue compression
– Cross-sectional imaging: CT with contrast agent = standard: confirmation of
diagnosis + exclusion of complications
– Colonoscopy:
– No colonoscopy to confirm the diagnosis of acute diverticulitis (risk of
perforation!)
– Important role in lower GI (gastrointestinal) bleeding and/or to exclude tumor
– Colonoscopy after conservatively treated diverticulitis and planned elective
sigmoid resection: (Usually after 4–6 weeks, to exclude other relevant
pathologies

Caution
Colonoscopy in acute inflammatory situation = high risk of perforation.

Therapy
Prophylaxis of Diverticulitis
Primary Prophylaxis
– Regular physical activity
– High fiber diet
– Preservation of normal weight
Secondary Prophylaxis
– Insufficient data = no general recommendations possible
– Prophylaxis of recurrent diverticular disease:
– Nutrition
– Lifestyle
– Physical activity
– Medications (mesalazine, probiotics, rifaximin)
Conservative Therapy
Asymptomatic Diverticulosis
– Primary prophylaxis (see above)
– Acute uncomplicated diverticulitis without risk factors for a complicated course
– Close clinical and laboratory control
– Low recurrence rate
– No indication for surgery
 – Antibiotic therapy:
– No acceleration of healing
– No prevention of complications/recurrences
– Exception = in the case of necessary immunosuppressive drug therapy after
transplantation, collagenoses etc.
Complicated Diverticulitis
– Inpatient treatment
– If necessary, parenteral fluid substitution in case of insufficient oral fluid intake
– Oral intake of food if necessary (depending on clinical situation)
– Parenteral antibiotic therapy
– For retroperitoneal/paracolic abscesses = interventional drainage + control
Surgical Therapy
Surgery Indications
– Emergency surgery:
– CDD Type 2c
– Evidence of freely perforated sigmoid diverticulitis with clinical or radiological
signs of peritonitis
– Failure of conservative therapy in complicated diverticulitis (acute abdomen,
sepsis)
– Diverticular hemorrhage with circulatory effect or persistent Hb effect that
cannot be controlled by interventions
– Elective surgery in the inflammation-free interval (>3–4 weeks):
– Recurrent diverticulitis with structural changes and complications CDD type 3c
(fistula formation, stenosis, unclear dignity)
– After successfully treated complicated diverticulitis CDD type 2b
(macroperforation, abscess)
– Clinically uncomplicated symptomatic diverticular disease CDD type 3a or
chronic recurrent diverticulitis CDD type 3b
– Recurrent, localized, clinically relevant diverticular bleeding
– Diverticular hemorrhage: endoscopic hemostasis or angiography with
embolization not possible
– Surgery is not indicated:
– Asymptomatic diverticulitis CDD type 0
– Acute uncomplicated diverticulitis CDD type 1
– In the interval after successful conservative therapy of complicated sigmoid
diverticulitis with microabscess CDD type 2a
 – Self-limiting or interventional successfully treated diverticular bleeding
Surgical Strategy
– Objective = removal of the diverticulum (diverticulitis)-bearing intestinal segment
– Laparoscopic (or laparoscopic-assisted) vs. open resection: Laparoscopic = fewer
local complications, wound infections, intra-abdominal abscesses, postoperative
ileus and fascial dehiscence
– Standard procedure = sigmoid resection + primary continuity restoration (if
necessary with protective ileostoma) also in case of perforated sigmoid
diverticulitis
– Laparoscopic peritoneal lavage + drainage, without resection: if necessary also for
Hinchey III
– Sigmoid resection with primary anastomosis superior to Hartmann’s procedure in
the haemodynamically stable and immunocompetent patient under 85 years of age
(Lambrichts et al. (2019) Lancet 4(8), 599–610)

Caution
Continuity restoration after Hartmann resection (discontinuity resection with
rectal blind closure and terminal stoma) occurs in only about 50% of patients
and is associated with substantial morbidity (44%) and lethality (5%).

– Technical aspects:
– Proximal resection margin: In any case proximal to the chronically or acutely
inflammatory altered wall sections in the healthy intestine
– Distal resection margin: In the upper rectum (better blood circulation) distal of
the high pressure zone
– Stapler vs. manual suture anastomosis = equivalent
– Preservation of the inferior mesenteric artery recommended = avoidance of
damage to the sacral plexus (= tubular sigmoid resection preferred)
– In septic/instable patients with difficult mobilization of the left flexure =
Hartmann’s procedure

3.2.2 Colonic Polyps

Definition
– Growths of different genesis into the lumen of the colon

Epidemiology
– Accumulation with increasing age
– Men > Women
– Localization: >50% in the rectum
Classification (◘ Table 3.4)
– Histological classification = behaviour/precancerous lesions
Table 3.4 Classification of colonic polyps

Designation Definition
Adenoma Epithelial neoplasia (precancerous lesion) with a tendency to degeneration
Hyperplastic polyp Small benign mucosal change, low tendency to degeneration
Inflammatory polyp Small benign mucosal change, without degenerative tendency (associated with
chronic inflammatory bowel disease)
Familial adenomatous Obligate precancerous lesion; mutation of the APC gene (autosomal dominant);
polyposis (FAP) risk of degeneration = 100%
Hamartoma Atypical differentiation of germinal tissue (mutation) = polyposis with a tendency
to degeneration (e.g. Peutz-Jeghers syndrome; Cowden syndrome)
Serrated polyp Epithelial neoplasia; adenoma with high malignant potency

Symptoms
– Mostly incidental finding (= asymptomatic)
– Peranal mucus discharge: With large polyps
– Bleeding
– Complications:
– Degeneration (adenoma-carcinoma sequence)
– Obstruction
– Invagination
– Prolapse

Diagnosis
– Digital-rectal examination
– Rectoscopy/complete colonoscopy with biopsy/ablation
– Colon contrast imaging, CT colonography (rare, obsolete)

Therapy
Endoscopic Therapy
– If possible, always endoscopic
– Ablation of the polyp (thermal snare, forceps) in sano, goal = clean-colon
– Endoscopic mucosal resection (EMR)
– Submucosal resection/dissection (SMR/SMD)

FAP (Familial Adenomatous Polyposis)

– First colonoscopy obligatory at the age of 10 years, then annually
– If adenomas are detected = proctocolectomy indicated between onset of puberty
up to the age of 20 years
– Followed by annual pouchoscopy
– Human genetic counselling (diagnosis in the family)
Guideline: Polypectomy
Implementation
– Documentation of the localization
– Polyp >5 mm: complete resection by loop ablation
– Polyp ≤5 mm: complete resection with forceps or snare
– Endoscopic mucosal resection
– Endoscopic full-thickness resection
– Histology obligatory:
– Statement on the completeness of the removal
– In case of carcinoma detection necessary: pT (in case of sessile polyps the sm
invasion measurement in μm), grading, L-, R-classification (local complete
removal in depth and to the side).
– pT1 carcinomas: “low risk” = G1, G2, L0/ “high risk” = G3, G4, L1
Postpolypectomy Strategy
– High-risk pT1 carcinoma (even if R0 ablation) = oncological resection
– Low-risk pT1 carcinoma incompletely ablated = complete endoscopic/local
surgical removal
– If R0 situation not achievable or doubt about pT1 situation = oncological surgical
resection
Follow-up
– Low-risk pT1 carcinoma after complete endoscopic R0 ablation = endoscopy after
6 months, complete colonoscopy after 3 years
– After removal of small, single, non-neoplastic polyps = no need for follow-up =
control colonoscopy after 10 years
– Complete ablation of neoplastic polyps
– Time of control colonoscopy depending on number, size and histology
– In case of 1–2 adenomas <1 cm without higher-grade intraepithelial neoplasia
after 5–10 years
Surgical Therapy
– For large polyp with a large base
– For non-ablatable polyp
– In case of carcinoma detection, after polypectomy
– Technique:
– Exploration, colotomy, ablation
– Colonic segment resection
– Transanal full wall excision
If carcinoma is detected in the histology, oncological resection of the colon
segment bearing the polyp is essential (► Sect. 3.3).
Follow-Up Care After Colonoscopic Ablation
– Depending on the histology
– Control colonoscopy:
– After ablation of 1–2 adenomas with low-grade intraepithelial neoplasia: after 5–
10 years
– After ablation of >3 adenomas or villous parts or high-grade neoplasia: After 3
years
– Sessile adenomas or questionable in-toto removal: After 2–6 months

3.2.3 Ulcerative Colitis

Key Points
– Chronic inflammatory bowel disease confined to the colon and rectum,
continuous affection of the mucosa
– Risk of formation of DALM (“dysplasia associated lesion or mass”) → colon
carcinoma
– Cure through restorative proctocolectomy

Definition
– Inflammatory bowel disease
– Mucosa + submucosa of the colon and rectum affected
– Continuous spreading of the lesions = ulcerations
– Autoimmunity in the pathogenetic background = genetic predisposition + specific
triggers (stress, infection)

Epidemiology
– Incidence: 3.0–3.9 per 100,000 population
– Prevalence: 160–250 per 100,000 population
– Age peak at 16–25 years
– Women > Men

Etiology
Etiopathogenesis
– Not fully clarified
– Autoimmune pathogenesis: genetic predisposition + specific triggers (stress,
infection)
– Positive family history, currently more than 160 known gene loci
– Other factors: diet, psychosomatic causes, nicotine, intestinal microbiome
Course
– Onset of inflammation: In the rectum
– Spread in oral direction, restricted exclusively to rectal and colonic mucosa
– Acute phase: red edematous mucosa, contact bleeding, microscopy: granulocytic
crypt abscesses
– Chronic phase: mucosa destruction with loss of fold relief = pseudopolyps;
microscopy: lymphocytic histiocytic infiltration

Clinical Presentation
Intestinal Manifestations
– Bloody-mucous diarrhea = leading symptom
– Abdominal discomfort: Pain, tenesmus
– Systemic signs of infection (e.g. reduced general condition, fever)
Extraintestinal Manifestations (15–20%)
– Erythema nodosum
– Aphtae, pyoderma gangraenosum
– Episcleritis, uveitis
– Peripheral and axial arthritis (ankylosing spondylitis)
– Primary sclerosing cholangitis (PSC), increased risk for development of chronic
sclerosing cholangitis (CSC)
Course
– Acute-fulminant (5%): Sudden onset of illness (diarrhea, septic temperatures,
septic shock); complications: Toxic megacolon; lethality approx. 30%
– Chronic-Continuous (10%): Without complete remission
– Chronic-recurrent (85%): Recurrent exacerbations; periods of complete
remissions
Complications
– Massive bleeding
– Toxic megacolon
– Growth disorder
– Backwash ileitis (in up to 10% of patients spread to the ileum DD Crohn’s
disease)

Caution
Risk of colon cancer development due to ulcerative colitis!

Diagnosis
Anamnesis
– Type and onset of symptoms, food intolerances, medications, etc.
– Stool anamnesis
Complete Physical Examination
– Digital-rectal examination (blood detection)
– Extraintestinal manifestations (especially skin)
Lab
– Inflammatory status (leukocytosis, blood sedimentation rate, CRP, α2-globuline)
– Hemoglobin, iron balance (exclusion of bleeding)
– Kidney function
– Transaminases, cholestasis parameters (bilirubin, alkaline phosphatase, γ-
glutamyltransferase) in primary sclerosing cholangitis
– p-ANCA (antineutrophil cytoplasmic antibodies): 60–70% of cases
– Calprotectin/Lactoferrin in stool: progression parameter in any inflammatory
bowel disease)
– Exclusion of intestinal infection: e.g. Clostridium difficile, CMV
(cytomegalovirus), travel history
– Stool diagnosis
Imaging
– Colon double contrast enema:
– Loss of the mucosal relief = “bicycle tube”
– Pseudopolyps
– Sonography: Thickened colonic mucosa
– Hydro-MRI
Endoscopy
– Rectoscopy, ileocolonoscopy
– Biopsies of all intestinal sections
– Danger of perforation in case of inflammation
Endoscopic Classification
– Proctitis (limited to rectum)
– Left-sided colitis (to left flexure)
– Extensive colitis

Differential Diagnosis
– Crohn’s disease
– Diverticulitis
– Infectious colitis
– Ischemic colitis
– Drug-toxic colitis
– Colon Cancer
– Irritable Bowel Syndrome

Therapy
Conservative-Medical Therapy
Long-term remission maintenance therapy should be given to all patients after
successful relapse therapy
Uncomplicated Ulcerative Colitis
Proctitis
– Mesalazine ≥1000 mg/day as suppository
– Plus topical steroids (budenoside-rectal foam) or additional oral administration of
mesalazine, if necessary
Left-Sided Colitis
– Rectal mesalazine as an enema or foam (≥1 g/day) in combination with oral
mesalazine-releasing preparations (≥3 g/day)
– If necessary, systemic steroid therapy 0.5–1 mg/kg body weight/day prednisolone
equivalent

Cancer Prevention
– Significantly increased risk of cancer = colonoscopy annually in patients with
ulcerative colitis (after 8 years of disease)
– Risk reduction: Aminosalicylate long-term therapy
– In case of high-grade IEN (intraepithelial neoplasia) = proctocolectomy

Complicated/Severe Ulcerative Colitis

– Inpatient treatment, interdisciplinary
– Thrombosis prophylaxis
– Parenteral fluid and electrolyte balance
– No motility inhibiting drugs
– Systemic steroid therapy, e.g. 1 mg/kg body weight/day prednisolone equivalent
– In case of contraindication for system. Steroid therapy, Infliximab, Ciclosporin A
or Tacrolimus can be used
– In case of insufficient clinical efficacy of steroids, these can be supplemented with
TNF antibodies, tofacitinib, or with ciclosporin A or tacrolimus. In the case of
infliximab, combination therapy with a thiopurine should preferably be used
– Surgical proctocolectomy
– Definition of severe colitis = criteria of Truelove and Witts:
– More than six bloody diarrhea per day
– Fever
– Tachycardia
– Anemia
– BSR >30 mm/h
– Always interdisciplinary therapy
Time-Adapted Approach
– Time points for response to therapy, onset of remission, time point for
discontinuation of medication in remission (◘ Table 3.5)
Table 3.5 Time-adapted approach, time points for response to therapy, onset of remission, time point for
discontinuation of medication in remission

Drug Response after Remission after Time of weaning

5-aminosalicylic acid 2–4 weeks 8–12 weeks After 2 years
Budenoside 2 weeks 8–10 weeks (After 6–12 months)
Systemic steroids 1 week 4 weeks No permanent therapy
Anti-TNF-α 1st–2nd gift 8 weeks After 2 years
Azathioprine, 6-mercaptopurine, methotrexate 8 weeks 12–16 weeks After >3.5 years
Calcineurin inhibitors 5–7 days 3 months? After 6–12 months

TNF tumour necrosis factor

Infliximab and ciclosporin are comparable as salvage therapy in acute severe

steroid-insensitive ulcerative colitis.

Caution
– Before anti-TNF-α therapy: exclude latent tuberculosis!
– Before immunosuppressive therapy in chronic inflammatory bowel disease
patients with a negative VZV (varicella-zoster virus) history
(chickenpox/herpes zoster) or negative VZV serology, perform vaccination:
– HPV (human papillomavirus) vaccination in girls and young women
– Pneumococcal vaccination
Surgical Therapy
Surgery Indications
– Free or covered perforation
– Therapy refractory bleeding
– Drug-therapy refractory relapse
– Conservative-therapy refractory course
– Colon stenosis (of unclear dignity)
– Suspicion or detection of carcinoma, DALM
Important: Intraepithelial neoplasia (IEN) (WHO criteria) → continence-
preserving proctocolectomy
– Histopathologically graded (low/high grade)
– In flat, non-inflamed mucosa
– Secondary assessment by reference pathologists
– DALM (inflammatory bowel disease-associated): Dysplasia-associated lesion or
mass
– ALM: “adenoma like mass”
Standard Surgery: Restorative Proctocolectomy
– Laparoscopic or conventional open surgery
– If necessary, staged surgery: e.g. 3-stage procedure
– Subtotal colectomy with terminal ileostomy
– Residual proctocolectomy (with ileoanal pouch anastomosis) + double barrel
ileostomy
– Reversal of Ileostomy
– In case of ileoanal pouch = leave not longer than 2 cm rectal mucosa, if
necessary secondary transanal mucosectomy
– Contraindications:
– Severe sphincter insufficiency (check sphincter function, e.g. enema)
– Perianal fistula
– Age >60 years (relative CI)

Surgical Procedure
 Restorative Proctocolectomy
– Transabdominal total colon and rectum resection (comparable to FAP
Procedure—► Sect. 3.3.3)
– Peranal exposure of the rectal stump (Parks retractor)
– Injection of the mucosa above the dentate line
– Dissection the mucosa cranially
– Transanal/transabdominal transection of the rectal wall (with/without
preservation of a rectal cuff)
– Mobilization of the ileum = tension-free anastomosis
– Reservoir formation: Formation of a 15-cm ileum J-pouch with stapling suture
device (GIA 90 mm), via antimesenteric incision in the ileum loop
– Peranal anastomosis: machine/hand anastomosis
– Hand anastomosis: pull-through of the reservoir through rectal cuff + pouch-
anal anastomosis (single stitch suture, all-layer)
– Protective double barrel loop ileostomy

Follow-Up
– Ileostomy reversal (after 2–3 months): Only after checking the reservoir tightness
(pouchoscopy + CM imaging) + continence check (e.g. enema).
– Pouchoscopy: annually = exclusion of cancer or pouchitis
Alternative Procedure
– In case of cancer: surgery according to oncological criteria
– Turnbull procedure (creation of ileostoma and colostoma) for toxic megacolon
– Double barrel ileostomy
– Two colonic fistulas (transverse colon + sigmoid colon)
– Lethality = 2–5% vs. 30% for subtotal colectomy
– Subtotal colectomy
– Emergency surgery
– Blind closure of the rectum (Hartmann operation)
– Interval proctocolectomy
– High lethality
Preventive Care (Cancer Prophylaxis)
– Indication
– Ulcerative pancolitis that has been present for >8 years
– Left-sided colitis persisting for more than 15 years
– Synchronous primary sclerosing cholangitis (PSC)
 – If the rectum is left in place or if there is a terminal ileostomy with rectal stump
– Complete colonoscopy with step biopsies
– At least four biopsies every 10 cm
– Annually
– Primary prevention of colorectal carcinoma (CRC) = aminosalicylates

3.2.4 Chronic Constipation

In Short
– Rule out laxative abuse
– Neuronal pathologies: usually very early manifestation
– Rule out rectocele

Definition
– Subjectively unsatisfactory (<3 bowel evacuation per week or ≥2 leading
symptoms of constipation: heavy straining, lumpy or hard stool, subjectively
incomplete defecation, subjective obstruction, manual maneuvers to facilitate
defecation)
– For at least 3 months

Epidemiology
– Western countries: incidence = approx. 15%
– Women > Men
– Age-associated: Increases with age

Etiology
– Low-fiber diet: association, but no causal relationship
– Reduced fluid intake
– Lack of exercise
– Neuromuscular factors: enteric neuropathy: Cajal cells, myopathy: intestinal
smooth muscle
– Diseases that can lead to secondary constipation (◘ Table 3.6)
– Medications with constipation potency (◘ Table 3.7)
Table 3.6 Diseases that can lead to secondary constipation

Endocrinopathies Diabetes mellitus

Hypothyroidism
Hyperparathyroidism
MEN 1 and MEN 2
Neurological diseases Parkinson’s disease
Multiple sclerosis
Apoplexy
Paraplegic Syndrome
Paraneoplastic intestinal
Neuropathies
Psychiatric diseases Depression
Somatization disorder
Other diseases Ovarian carcinoid
Scleroderma
Amyloidosis
Myotonic dystrophy
Obstructive/Stenosing
Intestinal disorders

MEN Multiple endocrine neoplasia

Table 3.7 Drugs with constipation potency

Drug group Drugs

Analgesics Opiates
Antacids Aluminium hydroxide, calcium carbonate
Antidepressants Tricyclics (imipramine, clomipramine, amitriptyline, dibenzepine), tetracyclics
(anticholinergics) (maprotiline, mianserine)
Antiepileptic drugs Carbamazepine
Antihypertensives β-blockers (e.g. atenolol), calcium antagonists (e.g. verapamil), clonidine
Anti-Parkinson’s medication Anticholinergics (e.g., biperiden), amantadine, bromocriptine
Antiemetics 5-HT3 antagonists (e.g. ondansetron)
Antitussives Preparations containing codeine
Chemotherapeutics Vincristine, vinblastine
Diuretics Thiazides, sulfonamides
Iron supplements Iron(II) and iron(III) salts
H2 blocker Cimetidine, Famotidine, Ranitidine

Lipid-lowering agent Ion exchangers (e.g. colestipol, colestyramine)

Neuroleptics Phenothiazines (e.g. chlorpromazine), thioxanthenes, butyrophenones,
dibenzodiazepine (clozapine)
X-ray contrast agent Barium salts
Spasmolytics Butylscopolamine, trospium chloride

Diagnosis
Anamnesis
– Defecation disorder
– Medication
Physical Examination
– Rectal digital examination
– Gynaecological examination if necessary
Further Diagnosis
– Abdominal Ultrasound
– Colonoscopy after the age of 55
– Anorectal manometry
– MRI Defecography
– Colonic transit time

Therapy
Step-By-Step Therapy (◘ Fig. 3.2)
– First stage: General recommendations = high-fibre diet, if necessary addition of
psyllium husks, wheat bran
– Second stage:
– Suppositories and clysms, plentiful fluid intake, adequate exercise, refraining
from suppressing the urge to defecate
– First choice: macrogol (osmotic laxative), bisacodyl, narium picosulfate
(stimulate colonic motility and water secretion)
– Second choice: sugars e.g. lactulose, anthraquinones
– Third Stage:
– Prucalopride: e.g. Resolor®: prokinetic serotonin (5 HT4) receptor agonist =
promotion of intestinal motility
– Lubiprostone: e.g. Amitiza®: direct chloride channel activator = increase water
and chloride secretion
– Linaclotide: e.g. Constella®. Agonist = guanylate cyclase agonist = increase
water and chloride secretion
– Fourth stage: Combinations of stages 1–3 (after special diagnosis)
– Fifth Stage: Sacral nerve stimulation
Fig. 3.2 Therapeutic algorithm for chronic constipation. 1st choice Approved for constipation in women if
laxatives are ineffective or intolerant. 2nd choice Available through international pharmacies, linaclotide
approved for obstipation-predominant IBS

Surgery
– Rarely indicated, after careful consideration most likely subtotal colectomy (80–
90% improvement)
– Estimation of the potential effect: Temporary ileostoma or permanent ileostoma
on patient’s request
– Alternative: Antegrade irrigation via appendix or caecal stoma

3.2.5 Guidelines
AWMF guideline: S2k guideline diverticular disease/diverticulitis, register number
021/20. Currently under revision, planned completion 31.07.2021
AWMF guideline colorectal cancer January 2019, registration number
021/007OL.
S3 guideline ulcerative colitis 8/19, AWMF registration number 021/009
3.3 Colon Cancer and Hereditary CRC Syndromes
3.3.1 Colon Carcinoma
In Short
– Adenoma-carcinoma sequence: screening colonoscopy
– Standard procedure: Surgery with adjuvant chemotherapy (from T3/N+)

Definition
– Epithelial malignancy of the colon (between the caecum and rectosigmoid
junction)
– Upper limit (level) of rectum (rigid rectoscopy) = 16 cm from ano (in Europe)

Epidemiology
– Second most common tumor in western industrialized nations
– Incidence in Germany = 80/100,000 inhabitants per year
– Men = Women
– Multiple synchronous colorectal carcinomas = 2–5%
– From 50 years of age: doubling of incidence and mortality per decade of life

Etiology and Pathogenesis

– Interaction of genetic factors and environmental influences
Risk Categories
– Sporadic: approximately 70%, acquired somatic mutation associated with:
– Higher age (>40 years)
– Tobacco consumption, alcohol consumption
– Risk-increasing diseases: Colorectal adenomas, chronic inflammatory bowel
diseases (► Sect. 3.2.3 Ulcerative colitis), ureterosigmoideostomy, carcinomas of
other organs (mamma, uterus, ovary, urinary bladder)
– Familial: approx. 20–30%, polymorphisms and gene loci with lower penetrance
– Hereditary: approx. 5%, hereditary mutation with high penetrance (► Sect. 3.3.2
Hereditary CRC syndromes)
Protective Factors
– High-fiber, low-fat, low-meat diet
– Fast stool passage
– Aminosalicylates
– Vitamin C, folic acid
Pathogenesis
– Adenoma-carcinoma sequence (90%): Due to increasing mutations over years
– De novo carcinomas (10%): Without adenoma manifestation (e.g. ulcerative
colitis)
– Hereditary forms: Germline mutations already existing = carcinoma at a young
age

Classification
TNM Classification (2017)
– T (tumor)
– Tx Primary tumor not assessable
– T0 No evidence of primary tumor
– Tis carcinoma in situ: intraepithelial or invasion of the lamina propria
– T1 Invasion of the submucosa
– T2 Invasion of the muscularis propria
– T3 invasion of the subserosa, or pericolic fat tissue
– T4a Perforation of the visceral peritoneum
– T4b Invasion of adjacent organs
– N (lymph nodes)
– N0 No regional lymph node metastases
– N1a 1 affected lymph node
– N1b 2–3 affected lymph nodes
– N1c Tumour nodule in the pericolic fat tissue
– N2a 4–6 affected lymph nodes
– N2b More than 6 affected lymph nodes
– M (metastases)
– M0 No distant metastases
– M1a Metastases in another organ
– M1b Metastases in more than one other organ
UICC Staging of Colorectal Cancer

UICC stage T (tumor) N (lymph nodes) M (metastases)

0 Tis N0 M0
I T1, T2 N0 M0
IIA T3 N0 M0
UICC stage T (tumor) N (lymph nodes) M (metastases)
IIB T4a N0 M0
IIC T4b N0 M0
III Each T N1, N2 M0
IIIA T1, T2 N1a M0
T1 N2a M0
IIIB T3, T4a N1 M0
T2, T3 N2a M0
T1, T2 N2b M0
IIIC T4a N2a M0
T3, T4b N2b M0
T4b N1, N2 M0
IVA Each T Each N M1a
IVB Each T Each N M1b

Histological Grading
– G1: Well differentiated
– G2: Moderately differentiated
– G3: Poorly differentiated (e.g. mucinous)
– G4: Undifferentiated (e.g. small cell, signet ring cell)
– V0/V1: Vein intrusion present/absent
– L0/L1: Intrusion into lymphatic vessels present/absent
– Pn0/Pn1: Perineural sheath infiltration present/absent

Symptoms
– Mostly uncharacteristic features
– Blood in the stool
– Change in bowel habits
– B-symptoms (fever, night sweats, weight loss)
– Performance drop, fatigue
– Tumor Anemia
– Rare abdominal pain
Complications
– Ileus
– Tumor perforation
– Fistulas
– Relevant bleeding
Diagnosis
Standard Investigations
– Anamnesis
– Stool habits, body weight, blood in the stool, pain
– Family history: for risk assessment of hereditary colorectal cancer (◘ Tables
3.8, 3.9, and 3.10)
– Clinical examination of the abdomen
– Digital rectal examination
– FOBT (Fecal Occult Blood Test = Haemoccult®)
– Lab
– Blood count, electrolytes, kidney function, coagulation status
– Complete colonoscopy

Table 3.8 Amsterdam I criteriaa

1 CRC was diagnosed in at least 3 relatives

2 One of you should be a first degree relative of the other two
3 At least 2 consecutive generations are affected
4 At least 1 CRC was diagnosed before the age of 50 years
5 Familial adenomatous polyposis (FAP) was excluded
6 CRC are verified by histopathological examination

CRC colorectal carcinoma

a
Families must meet all criteria

Table 3.9 Amsterdam II criteriaa (risk assessment of hereditary colon carcinoma)

1 Lynch syndrome-associated carcinoma has been diagnosed in at least 3 relativesb

2 One of you should be a first degree relative of the other two

3 At least 2 consecutive generations are affected
4 At least 1 tumor was diagnosed before the age of 50 years
5 Familial adenomatous polyposis (FAP) was excluded
6 Tumors are verified by histopathological examination

a
Families must meet all criteria
b
Colorectal tumor or tumor of the endometrium, small intestine, ureter, or renal
pelvis
Table 3.10 Revised Bethesda guidelines (risk assessment of hereditary colon carcinomaa)

1 CRC before the age of 50

2 Presence of synchronous, metachronous CRC (or Lynch syndrome)-associated tumorsb, regardless of age

3 CRC with MSI-H histologyc diagnosed in patients <60 years of age

4 CRC diagnosed in patients with one or more first degree relatives with Lynch syndrome-associated tumor,
one of whose carcinomas was diagnosed before age 50 years
5 CRC diagnosed in a patient with 2 or more first or second degree relatives with Lynch syndrome-associated
tumor, regardless of age

a
MSI investigation required if only one criterion is met
b
Endometrial, gastric, ovarian, pancreatic, biliary, small bowel, brain tumors
(usually glioblastoma in Turcot syndrome), seborrheic gland adenomas, and
keratoankanthomas in Muir-Torre syndrome, hepatobiliary carcinomas, transitional
cell carcinomas of the renal pelvis or ureter
c
Presence of tumor-infiltrating lymph nodes, Crohn’s disease-like lymphocytic
infiltration, mucinous/seal-ring differentiation, or medullary growth pattern
Guideline-Based Preoperative Diagnostic of Tumor Staging
– Digital-rectal examination
– Complete colonoscopy + biopsy
– Tumor not passable = colonoscopy 3–6 months postoperatively or intraoperatively
– Pneumocolon CT if necessary
– Abdominal ultrasound (especially liver)
– Chest X-ray in 2 planes
– CEA (carcinoembryonic antigen) determination
– Useful in individual cases: spiral CT or MRI abdomen, spiral Chest CT
Colorectal Cancer Screening (in the Asymptomatic Population)
– Colonoscopy = standard procedure
– From the age of 50
– If the findings are unremarkable = repetition after 10 years
– Alternative: Sigmoidoscopy every 5 years + yearly FOBT (Guajak procedure)
– FOBT = consisting of 3 test letters with 2 order fields each for 3 consecutive
stools
– Positive FOBT test = colonoscopy check!

Caution
In first degree relatives of patients with CRC or colorectal adenomas, a
complete colonoscopy should be performed before the age of 50 years
approximately 10 years before the age of onset of the cancer in the index
patient, latest at the age of 50 years.
Guideline: Polypectomy
– ► Section 3.2.2

Therapy
Treatment Strategy
– Colon cancer = indication for surgery
– Always aim for R0 resection
– Contraindication to surgery:
– General inoperability of the patient
– Inoperability of the tumor (R0 not achievable): Diffuse peritoneal
carcinomatosis with distant metastases, infiltration of the great vessels
Surgical Therapy
Principles of Surgical Therapy
– Oncological resection principles
– Laparoscopic vs. open: equivalent if oncologic principles are adhered to
– Extent of resection (◘ Fig. 3.3) depending on resection of the supplying vessels
and the lymphatic drainage areas
– Right Hemicolectomy:
– Indication: cancer of the caecum, ascending colon; for cancer of the right
flexure = extended right hemicolectomy
– Complications: Injury to the branches of the superior mesenteric artery, injury
to the right ureter, injury to the duodenum, tearing of the pancreatic head veins
(loop of Henle)
– CME = complete mesocolic resection
– Left Hemicolectomy:
– Indication: cancer of the descending colon, of the proximal sigmoid; in case of
cancer of the left flexure = extended left hemicolectomy
– Complications: Injury to the spleen, hemorrhage from splenocolic ligament,
injury to the left ureter
– Colon transversum resection
– Indication: Cancer of the middle of the transverse colon; for tumors close to the
flexure = hemicolectomy
– Complications: Insufficient anastomotic perfusion = anastomotic leakage
– Colon sigmoideum resection
– Indication: Cancers of the middle/distal sigmoid colon
– Complications: Injury to the left ureter, inadequate anastomotic perfusion =
anastomotic leakage
Fig. 3.3 a–d Extent of resection of various colon cancers. a Carcinoma of the appendix, caecum and ascending
colon. Right hemicolectomy and lymphadenectomy. b Carcinoma of the transverse colon. Resection of the
transverse colon including the flexurae coli dextra and sinistra and lymphadenectomy. c Carcinoma of the
descending colon. Resection of the distal half of the transverse colon, descending colon, sigmoid colon, and
lymphadenectomy. d Carcinoma of the sigmoid colon. Resection of the distal descending colon, sigmoid colon,
proximal rectum, and lymphadenectomy

Surgical Procedure
Right Hemicolectomy
– Longitudinal laparotomy vs. upper abdominal transverse laparotomy vs.
laparoscopic approach
– Complete mesocolic excision (CME)
– Exploration, marking (e.g. vessel loops) of the colon at the level of the
resection margins (proximal margin = 10–20 cm of the Bauhin valve)
– Mobilization of the caecum and ascending colon; exposure of the right ureter;
detachment of the colon/mesocolon from Gerota’s fascia
– Mobilization of the right flexure (transection of the hepatocolic and
duodenocolic ligaments)
– Transection of gastrocolic ligament for distal resection border
– Transection of the great omentum at the level of the distal resection margin;
omentum remains en bloc on the specimen
– Transection of the mesentery (with mesenteric vessels) between ligatures
– Ligation of the ileocolic vessels close to superior mesenteric vein and colic
arteries and the right branch of the colic artery and vein close to Henle’s loop
(CME)
– Remove the bowel at the level of the resection margins
– Side-to-side ileotransversostomy
– Closure of the mesenteric gap

Surgical Procedure
Left Hemicolectomy
– Longitudinal laparotomy vs. laparoscopic approach
– Complete mesocolic excision (CME)
– Exploration, marking of the colon at the level of the resection margins
(proximal: depending on tumor location; distal: above the peritoneal fold)
– Incision of the white line (Toldt) and mobilization of the descending colon +
sigmoid; exposure of the left ureter
– Dissection of the left mesocolon from Gerota’s fascia medially
– Transection of the great omentum at the level of the proximal resection margin;
omentum remains en bloc on the specimen
– Mobilization of the left colonic flexure (transection of the splenocolic and
phrenocolic ligaments)
– Severing the mesentery between ligatures
– Transection of the inferior mesenteric vein at the inferior border of the pancreas
(lateral to lig. Treitz), transection of the inferior mesenteric artery centrally
– Pay attention to the course of the parasympathetic nerves
– Remove the bowel at the level of the resection margins
– Transversorectostomy (usually circular end to end anastomosis)
– Closure of the mesenteric gap

Surgical Procedure
Colon Transversum Resection
– Longitudinal laparotomy vs. upper abdominal transverse laparotomy vs.
laparoscopic approach
– Complete mesocolic excision (CME)
– Exploration, marking of the colon at the level of the resection margins
– Transection of the gastrocolic ligament
– Mobilization of the right colonic flexure and the ascending colon
– Mobilization of the left colonic flexure
– Radicular resection of the A. and V. colica media
– Transection of the transverse mesocolon at the lower border of the pancreas;
including lymphadenectomy
– Transection of the mesentery between ligatures
– Remove the bowel at the level of the resection margins
– Ascendodescendostomy as end-end anastomosis
– Closure of the mesenteric gap

Surgical Procedure
Oncological Colon Sigmoideum Resection
– Median lower abdominal laparotomy vs. laparoscopic approach
– Complete mesocolic excision (CME)
– Exploration, marking of the colon at the level of the resection margins
(proximal: transition descending colon—sigmoid; distal: rectosigmoid
transition)
– Incision of the white line (Toldt) and mobilization of the descending colon +
sigmoid; exposure of the left ureter
– Dissection/Mobilization of the mesosigmoid from Gerota fascia medially
– Exposure and resection of the inferior mesenteric artery (preservation/non-
preservation of the left colic artery); check of the blood supply of the proximal
end of the intestine
– Transection of the inferior mesenteric vein at the inferior border of the pancreas
 – Medial incision of the mesenteric peritoneum at the insertion along the aorta
and blunt detachment from the retroperitoneum; Attention: protection of the
autonomic nerves
– Resection of the superior rectal artery
– Transection of the mesosigma between ligatures
– Mobilization of the proximal rectum
– Incision of the pelvic floor peritoneum
– Exposure of the rectum, dorsally in the Waldeyer space, then ventrally and
laterally (paraproctia).
– Transection of the upper mesorectum up to the level of the distal resection
border (No Coning = thinning of the distal mesorectum)
– Resection of tumor bearing colon segment
– Circular end-to-end descendorectostomy (usually mechanically with transanal
CEEA (“circular end-to-end anastomosis”) stapler)

Postoperative Complications
– Suture insufficiency = anastomotic insufficiency
– With peritonitis: relaparotomy, lavage, Hartmann resection or resection +
anastomosis, creation of protective ileostoma
– Abscess: drainage (possibly CT-guided), irrigation, if necessary creation of
protective ileostoma
– Fecal fistula without peritonitis (infraperitoneal): stoma creation + Endo-VAC-
application until cleaning of the cavity, waiting for spontaneous healing.
– Postoperative bleeding
– Mechanical ileus due to adhesive small bowel obstruction = relaparotomy +
adhesiolysis
– Hernia
– Cancer Recurrence
Principles for Specific Situations
– Multivisceral resection:
– In case of adherence of the tumor to adjacent organs = en bloc multivisceral
resection
– Caution: Biopsies should be strictly avoided = risk of tumor cell dissemination
(spillage)
– Carcinoma in FAP: Restorative proctocolectomy with small bowel pouch + lymph
node dissection according to the location of the carcinoma
– Carcinoma in HNPCC:
– Proceed in the same way as for sporadic CRC
– If necessary subtotal colectomy + prophylactic hysterectomy +
salpingoovarectomy at the time of abdominal surgery
– Carcinoma in ulcerative colitis: restorative proctocolectomy + systematic
oncologic lymphadenectomy with CME
Principles in Metastatic Colon Cancer
– Liver metastases
– If R0 resection for liver and all other lesions possible = liver resection
– Neoadjuvant systemic chemotherapy if necessary
– Pulmonary metastases
– If R0 resection possible = resection
– For synchronous liver and lung metastases → resection of liver metastases first
– Peritoneal carcinomatosis
– If R0 (CC-0) resection possible = cytoreductive surgery (CRS) + peritonectomy
+ hyperthermic intraperitoneal chemotherapy (HIPEC) indicated
Adjuvant Chemotherapy
Caution
– Prerequisite is the oncological R0 resection!
– Indication based on histology with TNM classification (pN0 classification
possible if at least 12 regional lymph nodes in specimen) (see above)

Indications
– Stage III (UICC)
– Stage II (UICC) (with microsatellites instability) or with risk factors:
– pT4 tumor, tumor perforation/rupture
– Emergency Operation
– Number of examined lymph nodes too low
– Adjuvant chemotherapy may be considered after R0 resection of synchronous or
metachronous liver metastases
Contraindications
– Poor general condition
– Uncontrolled infection
– Liver cirrhosis Child B/Child C
– Severe coronary heart diseases (CHD); heart failure: NYHA (New York Heart
Association) III/IV
– Preterminal/terminal renal failure
– Limited bone marrow insufficiency
Standard Chemotherapy = FOLFOX (5-FU/Folinic Acid/Oxaliplatin)
– Protocol Examples:
– FOLFOX4: folinic acid, 5-FU (5-fluorouracil), oxaliplatin every 2 weeks for 12
cycles
– Guideline states that patients over 70 years of age should not receive
oxaliplatin-containing therapy
– In case of contraindication to oxaliplatin-containing regimens = monotherapy
with fluoropyrimidines: oral 5-FU prodrug capecitabine, 8 cycles of 3 weeks
each
– In R0-resected stage III colon cancer, additional administration of cetuximab
does not add benefit to FOLFOX even in KRAS wild type
No age restriction for adjuvant chemotherapy (general contraindications to be
considered) = patients ≥75 years of age in stage III have survival benefit from
adjuvant chemotherapy; oxaliplatin provides little additional benefit.
Palliative Chemotherapy
– Stage IV: Indicated for primary irresectability, independently of metastasis-related
symptoms
– For example, FOLFOX, FOLFIRI, bevacizumab, cetuximab…
– Regorafenib (small molecule multikinase inhibitor) = survival benefit in
metastatic colorectal cancer after failure of all standard therapies

Oncologic Follow-up
– Stage I: Not indicated
– Stage II and III: Follow-up indicated after R0 resection
– Principles of oncologic follow-up: ◘ Table 3.11
Table 3.11 Programmed follow-up for colon cancer UICC II and III (S3 Guidelines Colorectal Carcinoma)

Investigation Months
3 6 9 12 15 18 21 24 36 48 60
Medical history, physical examination, CEA x x x x x x x
Colonoscopy xa xb xb

Abdominal Sonographyc x x x x x x x
Investigation Months
3 6 9 12 15 18 21 24 36 48 60

Sigmoidoscopy (rectoscopy)d x x x x

Spiral CTe x

Chest X-ray (no consensus)

a
If a complete preoperative colonoscopy has not been performed
b
If the findings are unremarkable (no adenoma, no carcinoma) next colonoscopy
after 5 years
c
A meta-analysis showed an advantage for an imaging procedure to detect liver
metastases in follow-up. For this reason, application of the simplest and less
expensive procedure
d
Only for rectal cancer without adjuvant or neoadjuvant radiochemotherapy
e
Only for rectal cancer 3 months after completion of tumor-specific therapy (surgery
or radiation/chemotherapy) as initial findings

Prognosis
– Cumulative 5-year survival rate = 60%
– 5-year survival rate by UICC stage:
– UICC I = approx. 70–100%
– UICC II = approx. 60–91%
– UICC III = approx. 44–60%
– UICC IV = approx. 3–7% (without therapy)

3.3.2 HNPCC (Hereditary Non-polyposis Colorectal Cancer): Lynch

Syndrome
Key Points
– Hereditary disease associated with colorectal cancer
– Also associated with other cancers (including endometrial cancer)
– Defect in mismatch repair genes

Definition
– HNPCC = Lynch syndrome
– Most frequent form of hereditary colorectal cancer
– Autosomal-dominant inheritance, no 100% penetrance

Epidemiology
– Approx. 1–3% of all CRC patients
– 2% of all endometrial cancers
– Most common form of hereditary CRC
– Lynch syndrome: Compared to sporadic CRC
– Younger patient age
– Better prognosis
– Much lower metastatic tendency: synchronous CRC 18%, metachronous CRC
30% after 10 years, 50% after 15 years, right-sided CRC 60%
– Lifetime risk of CRC (up to 75 years) (◘ Table 3.12)
Table 3.12 Cumulative lifetime risks in patients with Lynch syndrome

Cancer Lynch syndrome (%) General population (%)

CRC male 54–74 5
CRC female 30–52 5
Endometrium 28–60 2
Ovary 6–7 1
Stomach 6–9 <1
Small intestine 3–4 <1
Pancreas <1–4 1
Hepatobiliary 1 Rarely
Urinary tract 3–8 Rarely
Brain 2–3 <1
Seborrhoeic skin tumour/keratoankanthoma 1–9 Rarely

Etiology
– HNPCC: “hereditary non-polyposis colorectal cancer” (introduced in 1985)
– Bethesda/Amsterdam criteria for the diagnosis of HNPCC
– Lynch syndrome: mutation identified
– Mismatch repair (MMR) gene: mutation (MSH2, MLH1, MSH6, PMS2)
– Malignancies in LS (Lynch syndrome) patients: Due to somatic mutation of the
second gene = microsatellite instability (MSI)
– Lynch I: CRC only
– Lynch II: CRC + cancer of the genitourinary tract
– Muir-Torre syndrome: Lynch syndrome + sebaceous gland cancers or
keratoacanthomas

Diagnosis
Anamnesis
– Amsterdam criteria I + II (◘ Tables 3.8 and 3.9)
– Bethesda criteria (◘ Table 3.10)
Test for Mismatch Repair Defect
– If Bethesda criteria met
– By PCR, much cheaper = immunohistochemistry
– Histology
– In biopsy of CRC, MSI can be identified with almost 100% sensitivity and
specificity
– Increased incidence of mucinous carcinomas, signet ring carcinomas, medullary
carcinomas

Caution
A significant proportion of loss of MLH1 expression is the result of promoter
methylation (BRAF V600 mutation) and not an MMR defect

Prevention
– Monitoring of Lynch syndrome mutation carriers (◘ Table 3.13)
– Complete colonoscopy: annually from the age of 25, in any case 5 years before
the lowest age of onset of the disease in the family
– Females at risk: From the age of 25 annual gynaecological examination +
transvaginal US
– If there is a positive family history of gastric cancer: annual EGD from the age of
25
– Upper abdominal Ultrasound annually
Table 3.13 Recommendations for surveillance of Lynch syndrome mutation carriers by the German S3 guideline
(compared with the recommendations of the Mallorca group (European branch of InSIGHT, ► http://​www.​
mallorca-group.​eu), EGAPP (► https://​www.​egappreviews.​org/​recommendations/​Lynch.​htm), and NCCN (►
https://​www.​nccn.​org)

Colonoscopy Lower Gastroscopy Lower Abdominal Lower Gynecology Lower Other

interval age interval age ultrasound age Interval age
(years) limit (years) limit interval limit (years) limit
(years)
S3 1 25a 1b 25 1 25 1 25 Genetic
guideline counseling
at the age
of 18
Mallorca 1–2 20–25 1–2b 30–35 1 30–35 1–2, TVU, 30–35 Urinalysis
Group aspiration and
biopsy… cytology if
there is a
family
history of
urinary
 Colonoscopy Lower Gastroscopy Lower Abdominal Lower Gynecology Lower Other
interval age interval age ultrasound age Interval age
(years) limit (years) limit interval limit (years) limit
(years)
tract
cancer.
EGAPP 1–2 20–25 1–2, TVU, 30–35 Genetic
endometrial counselling
biopsy…
NCCN 1–2 20– 1–2, TVU 30–35
25c or
endometrial
aspirate

TVU transvaginal ultrasound

a
Same age or at least 5 years younger than the youngest age at diagnosis in the
family
b
When cancer runs in the family
c
Same age or 10 years younger than youngest age at diagnosis in the family

Surgical Therapy
Despite regular monitoring, the relative risk of developing a tumor is 5.8 times
higher compared to a mutation-negative cohort.
– Oncological resection: According to the standard rules for CRC
– Extended resection: e.g. subtotal colectomy + ileosigmoidostomy = justified in
individual cases
– If necessary prophylactic hysterectomy + salpingo-oophorectomy at the time of
abdominal surgery

3.3.3 Other Hereditary CRC Syndromes

Familial Adenomatous Polyposis (FAP)
Definition
– Obligate precancerous lesion
– Risk of cancer = almost 100% from the age of 15 onwards
– About 1% of all CRC
– Other extracolic manifestations
Etiology
– Mutation APC gene
– Autosomal dominant inheritance (75% of cases)
– New mutation (25% of cases)
Tumour Spectrum
– Duodenal and papillary adenomas
– Gastric Adenomas
– Abdominal and extraabdominal desmoid tumors
– Thyroid cancers
– Malignant CNS tumours (mostly medulloblastomas)
– Hepatoblastomas
– Osteomas, epidermoid cysts, pigmentary abnormalities of the retina
Prevention
– From the age of 10, after human genetic counselling predictive genetic diagnosis
– If mutation confirmed:
– Rectosigmoidoscopy annually from the age of 10 at the latest
– If adenomas are detected = complete colonoscopy
– Until proctocolectomy annual repetition of complete colonoscopy
– Esophagogastroscopy (EGD) with inspection of the papilla region: At the latest
from the age of 30 every 3 years, if necessary annually in case of changes
– Extracolic manifestations: Annual ultrasound of the abdomen, from the age of 10
onwards annual ultrasound of the thyroid gland
Therapy
– Sphincter-preserving proctocolectomy (► Sect. 3.2.3)
Follow-Up
– Pouchoscopy yearly
– If preserved rectal stump = rectoscopy every 4 months

Hamartomatous Polyposis Syndromes

Definition
– Peutz-Jeghers Syndrome
– Juvenile polyposis coli
– Cowden syndrome: PTEN (“phosphatase and tensin homolog”) gene
Prophylaxis
– No general recommendations due to sparse evidence available
Diagnosis and Therapy
– See above (CRC)
– No general recommendations due to sparse evidence available
3.3.4 Guidelines
S3 Guideline Colorectal Carcinoma 1/2019, AWMF Register Number 021/007/OL
Schmiegel W, Reinacher-Schick A, Arnold D, Graeven U, Heinemann V,
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© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_4

4. Rectum
Jens Wannenmacher1 and Stefan Willis1
(1) Surgical Department A, Ludwigshafen Hospital, Ludwigshafen,
Germany

Jens Wannenmacher
Email: [email protected]

Stefan Willis (Corresponding author)

Email: [email protected]

4.1 Anatomy and Physiology

S. Willis

4.1.1 Definition, Location and Structure

Definition
– Part of the large intestine between sigmoid colon and anus

Location
– In the pelvis with close relationship to neighbouring organs—therefore
special features in diagnosis and therapy

Limits
– Boundary between colon and rectum defined differently: in Germany at
16 cm and in the USA at 12 cm (from anocutaneous line (a.a.) measured
with rigid rectoscope (caution literature comparison)
– According to the Union for International Cancer Control (UICC) division
into 3 parts:
– Upper third 12–16 cm a.a. (= intraperitoneal portion)
– Middle third 6–12 cm a.a.
– Lower third <6 cm a.a.

4.1.2 Anatomy and Embryology

Topographic Anatomy
– Curved course along the sacrum and the coccygeal bone to the levator
funnel
– Dorsal retroperitoneal position up to the promontory, ventral variable
peritoneal envelope (excavatio rectouterina in females or rectovesicalis
in males)
– Extraperitoneal envelopment of the rectum by the mesorectum with the
lymphovascular pathways
– Circumferential boundary of the mesorectum by the visceral pelvic
fascia, which turns into the parietal pelvic fascia at the level of the pelvic
floor, in between avascular separating layer
– Denonvillier’s fascia = ventral part of the parietal pelvic fascia (covering
of vagina or seminal vesicle and prostate together with associated vessels
and nerves)
– Waldeyer’s fascia = dorsal part of the parietal pelvic fascia (covering the
presacral venous plexus and the vegetative hypogastric and pelvic
nerves)
– Inferior part of the rectum without mesorectal and fascial envelope

Blood Supply and Drainage

– Arterial Supply:
– From cranial = superior rectal artery (end section of the inferior
mesenteric artery, course: dorsal in the mesorectum)
– From caudal: inferior rectal artery (from A. iliaca interna),
occasionally inconstant middle rectal artery
– Venous drainage:
– 2/3 oral: drainage in superior rectal vein to inferior mesenteric vein
and portal vein
– 1/3 aboral: drainage in middle rectal veins + inferior rectal veins to
internal iliac vein and inferior vena cava
– Lymphatic drainage: bidirectional
– Cranial along the superior rectal artery to paraaortal nodes and vessels
– Distally along the internal iliac artery
– No lymphatic vessel arcades near the intestinal wall

Caution
Caudal portion = intramural lymphatic drainage (due to missing
mesorectum)

Innervation
– Through autonomic nerves:
– Sympathetic innervation:
– inferior mesenteric trunc ventral to the aorta at the level of the
outlet of the inferior mesenteric artery, becomes the superior
hypogastric plexus presacral at the level of the promontory, than
divides into right and left hypogastric nerves, which course dorsal
to the mesorectum
Damage of the sympathetic portion leads to retrograde ejaculation.
– Parasympathetic innervation:
– pelvic splanchnic nerves unite with the sympathetic hypogastric
nerves at the level of the seminal vesicles and form the inferior
hypogastric plexus (= pelvic plexus).
– Further course along the lateral border of the prostate gland
Damage of the parasympathetic part leads to erectile dysfunction or
disturbance of lubrication and bladder emptying disorder.

4.1.3 Physiology
– Direct continuity with sigmoid colon, but different function
Special Functions of the Rectum
– Defecation: action of defecation
– Continence: ability to retain stool; controlled voiding
– anorectal continence apparatus

4.2 Benign Diseases

S. Willis

4.2.1 Benign Neoplasms/Malformations

Polyps/Adenomas (Chap. 3)
– Mostly asymptomatic
– Symptoms:
– Mostly incidental findings during screening endoscopy
– Bleeding
– Mucus production
– Passage obstruction
– Histology obligatory

Caution
Adenoma = facultative precancerous lesion

– Therapy:
– Endoscopic ablation with snare, EMR (endoscopic mucosal resection)
or submucosal dissection
– Surgical therapy by means of transanal resection or TEM (transanal
endoscopic microsurgery) possible
– Avoid peacemeal resections (histology, topographic assignment for R1
resection)

Schwannomas, Leiomyomas, Angiomyomas

– Very rare
– Diagnosis occasionally by CT- or EUS (endoscopic ultrasound)-guided
puncture
– Therapy: Enucleation in healthy tissue is sufficient
Hirschsprung’s Disease
– Congenital intestinal aganglionosis
– Pathophysiology:
– Aganglionic segment starting from linea dentata, reaching to different
degrees proximally, dilated intestine above it
– Mostly disease of childhood
– Diagnosis: by deep rectal biopsy
– Therapy: Resection of the aganglionic segment and the adjacent
dysfunctional dilated section

Infiltrating Endometriosis
– Unclear pathogenesis, mostly young women
– Symptoms:
– Unspecific, cyclically occurring complaints
– Pelvic pain, rectal bleeding, constipation, diarrhoea
– Therapy:
– Primarily conservative/symptomatic therapy
– Recurrence rate: up to 60%
– Resection required in individual cases
– Interdisciplinary approach at specialised centres

4.2.2 Rectal Prolapse

Key Points
– Etiology unclear
– Mainly older, female patients affected
– Diagnosis by inspection
– Often simultaneous constipation + incontinence
– Choice of procedure dependent on: size of the prolapse + comorbidity
of the patient
– Perineal procedures: Lower morbidity but worse long-term outcomes
– Abdominal procedures: Better results, but higher risk

Definition, Classification, Differential Diagnosis, Epidemiology

Definition
– Protrusion of the entire rectal wall outwards through the anal sphincter
apparatus
Classification
– Grade 1: Internal, partial prolapse (intussusception)
– Grade 2: Internal prolapse extending to the anocutaneous line
– Grade 3: External solid wall prolapse
First-degree prolapse can also be detected in a high percentage of
healthy individuals; findings requiring treatment only when symptoms
occur.
Differential Diagnoses
– Anal prolapse (► Sect. 5.​2.​1 Haemorrhoids)
– Mucosal prolapse alone
Epidemiology
– Age peak: children around 3 years and older women
– Incidence = 1% in the over-65s
– Combination with bladder, vagina and/or uterus prolapse = frequent

Etiology and Pathogenesis

– Variable expression of morphological/functional changes:
– Abnormally deep rectovesicale/rectovaginale space (“Cul de sac”)
– Atone pelvic floor and sphincter muscles
– Diastasis of the pelvic diaphragm (levator ani muscle)
– Mobile mesorectum with lack of lateral + dorsal fixation
– Pudendal nerve neuralgia
– Causal pathogenesis vs. secondary phenomenon = unclear
– Often associated with:
– Functional disorders (e.g. excessive pressing during defecation)
– Structural changes (e.g. hysterectomy, post anal atresia in children)

Symptoms and Diagnosis

Symptoms
– Common concomitant symptomatology:
– Symptoms of constipation (up to 65%)
– Symptoms of incontinence (up to 90%)
– Rectal prolapse = clinical diagnosis
Diagnosis
– Inspection
– Rectal digital examination = crucial!
– Spontaneously reducible in the initial stage
– If more severe: Manual reduction required
– Dynamic pelvic floor MRI: Helpful for evaluation of the ventral
compartments and detection of an enterocele
– Rectoscopy/colonoscopy: to exclude endoluminal concomitant diseases
– Anal manometry not required
Therapy
– Therapy goals
– Permanent removal of the prolapse
– Restoration of adequate function
– Strategy: Choice of procedure depends on:
– Prolapse size
– Comorbidity of patients
Non-surgical Therapy
– Preoperative exhaustion of conservative options (stool regulation, pelvic
floor exercises, biofeedback if necessary)
– Possible combination of all procedures with:
– Sacral nerve neuromodulation/stimulation (SNS, stable good
continence improvement)
– Sphincteroplasty/levatoroplasty (poor long-term results)
Surgical Therapy
– Therapy principles
– Resection, fixation or plication of the redundant bowel
– Abdominal or perineal/transanal procedure
– Strategy
– No significant risks = laparoscopic resection rectopexy (= best
functional long-term result)
– In moderate prolapse without constipation: Current preference for
laparoscopic ventral rectopexy
– In case of high risk for abdominal surgery: perineal procedures
No clear recommendations based on evidence-based randomized trials.
Perineal and Transanal Procedures
– Wrapping procedure of the anus
– Techniques: Thiersch ring, subcutaneous placement of foreign
material.
– Results: unsatisfactory + partly considerable complication rates =
obsolete
– Rehn-Delorme operation/procedure
– Principle:
– Transanal mucosal resection + suprasphincteric plication of the
prolapsed rectum
– Possible in analgosedation
– Results:
– Low morbidity and mortality, mean recurrence rate approx. 20%
after 2 years
– In many cases improvement of continence
– Altemeier Rectal resection
– Principle:
– Perineal rectum resection with reanastomosis ± pouching at the
level of the dentate line
 – Circular resection of the prolapse possible using staple suture
devices Transtar®
– Results:
– Recurrence rate lower than after Rehn-Delorme (5–15%),
immanent risk of anastomotic insufficiency with pelvic sepsis (4%)
– Significant improvement in constipation, frequent worsening of
continence (urge incontinence, stool smearing)

Surgical Procedure
Rehn-Delorme Procedure
– preoperative colonic irrigation
– General anaesthesia, spinal anaesthesia
– Lithotomy position (Lloyd-Davis position), single-shot antibiotics
with metronidazole i.v.
– Sphincter dilation, maximum eventration of the prolapse with 2
clamps
– Injection of the submucosa with diluted adrenaline saline solution
(better separation of the layers)
– Incision of the mucosa 1 cm orally of the dentate line and circular
dissection and resection of the mucosa cylinder of the entire prolapse.
– Accordion-like folding and reduction of the intestinal tube by 4–5
mattress sutures
– Reanastomosis of the mucosa

Abdominal Procedures
– Rectopexy without resection
– Principle:
– Dorsal suture rectopexy
– Laparoscopic ventral mesh rectopexy (d’Hoore), posterior mesh
fixation (Ripstein / Wells) largely abandoned
– Avoidance of lateral mobilization leads to improved postoperative
function
– Use of foreign materials (alloplastic meshes) with risk of erosion,
fistula and stenosis formation
 – Results:
– Recurrence rates around 10%
– Significant variation in functional outcomes
– Loop formation and kinking of the redundant sigmoid: marked
increase in constipation
– Resection rectopexy
– Principle:
– Stable stretching and fixation of the rectum + removal of the
redundant sigmoid + usually suture rectopexy at the promontory
– Due to anastomosis, alloplastic material is usually not used
– Results:
– Recurrence rate = 2–8%
– Improvement of constipation in more than 50% of patients
– Improvement of continence in 60–90% of patients
Today’s standard = laparoscopic procedure (additional advantages)

Surgical Procedure
Laparoscopic (Resection) Rectopexy
– General anesthesia, lithotomy position (Lloyd-Davis), perioperative
antibiosis
– Trocar placement (see laparoscopic sigmoid resection)
– Lateral mobilization of the sigmoid, visualization of the left ureter
– Entering the vessel-free dorsal layer at the level of the promontory,
preparation down to the pelvic floor
– Incision of the peritoneum at the anterior fold, anterior dissection up
to the upper third of the vagina or up to the seminal vesicles
– Caution: Do not cut the lateral ligaments!
– If resection: Tubular transection of the mesosigmoid with preservation
of the superior rectal artery, transection of the rectum above the
promontory with a stapler, Pfannenstiel incision, resection of the
bowel at the descendosigmoid junction, double-stapling anastomosis
– Fixation of the rectum to the presacral fascia at the level of the
promontory by non-absorbable simple interrupted sutures close to the
 midline (caution: injury to the presacral venous plexus or pelvic
nerves)
– reconstruction of the anterior rectovaginal peritoneum by continuous
suture

4.3 Malignant Diseases

S. Willis and J. Wannenmacher

Key Points: Rectal Cancer

– Most common malignancy of the rectum
– Locoregional risk of recurrence: Higher than for colon cancer (due to
lymphatic spread pathways, narrowness of the pelvis)
– Operative standard = en bloc resection of the tumor with regional
vascularization. Systematic pathoanatomical examination on perirectal
tumor spread
– Continence-preserving surgery (approx. 85% of rectal carcinomas):
Through better understanding of continence mechanisms + optimized
surgical technique.
– Current Standards:
– Tumors of the upper third of the rectum: Proximal partial mesorectal
excision + reconstruction by end-to-end descendorectostomy
– Tumors of the middle and distal third of the rectum: Total mesorectal
excision obligatory + side-to-end anastomosis/colonic pouch-anal
anastomosis
– Local excision (transanal or by TEM): for limited to small and
histologically favorable uT1 tumors
– For extraperitoneally located T3/4 tumors: Neoadjuvant therapy
approaches > postoperative radiochemotherapy

4.3.1 Histological Tumour Entities

– Rectal carcinoma = most frequent malignancy of the rectum
– GIST (Chap. 14)
– Mesenchymal submucosal tumor
 – High malignancy potential at >5 cm and/or >5 mitoses per 50 HPF
(high power field = microscopy field)
– Aim for complete surgical removal
– if necessary, follow-up treatment with Imatinib
– Neuroendocrine carcinoma/carcinoid
– Rectum = most frequent localization in the intestine
– Increasing incidence
– Malignancy potential: depending on the degree of differentiation (G1–
G3)
– Tumours <2 cm mostly benign: local resection sufficient
– Oncological radical resection for tumours >2 cm + proven malignancy
– Simultaneous cholecystectomy if planned therapy with somatostatin
analogues
– Lymphomas, sarcomas = rarities

4.3.2 Rectal Cancer

Definition
– All epithelial malignancies from the linea dentata to 16 cm ab ano
measured with the rigid rectoscope

Forms/Classification
– By growth:
– Exophytic polypous
– Endophytic ulcerative
– Diffusely infiltrating
– By histological cell type:
– Mostly adenocarcinomas
– Rare adenosquamous carcinomas
– By differentiation:
– Low grade
– High grade

Epidemiology and Etiology

– s. Colon cancer
– More than 50% involve the rectum

Tumor Spread
– Continuous
– Intramural
– Direct organ infiltration
– Discontinuous
– Tumor satellites in the mesorectum outside lymph nodes
– At a distance of up to 4 cm from the tumor
– Lymphogenous
– Mesorectal and para-aortic lymph nodes
– Rarely iliac lymph nodes
– Hematogenous
– Into the liver via portal vein
– Into the lungs (rare) in distal tumors via the vena cava

Classification
Classification According to Mason (Clinical Staging)
– After palpation
– CS I Mucosa displaceable
– CS II Intestinal wall displaceable
– CS III Intestinal wall partially fixed
– CS IV Intestinal wall fixed
– CS V Disseminated disease
TNM Classification (2017)
– T (tumor)
– T0 No infiltration
– T1 Infiltration of the submucosal layer
– T2 Infiltration of the muscularis propria
– T3 Infiltration of the subserosa
 – T4a Infiltration of the visceral peritoneum
– T4b Infiltration of other organs/structures
– N (lymph nodes)
– N0 No metastases in the lymph nodes
– N1 Metastases in 1–3 regional lymph nodes
– N2a Metastases in 4–6 regional lymph nodes
– N2b Metastases in >6 regional lymph nodes
– M (metastases)
– M0 No distant metastases
– M1 distant metastases

Derivation of UICC Stages from TNM Classification

Stage I T1, T2 N0 M0
Stage II T3, T4 N0 M0
Stage III Each T N1, N2 M0
Stage IV Each T Each N M1

Symptoms
– Section 3.3 Colon cancer

Diagnosis
Rectal Digital Examination
– Assessment of the tumor location
– Infiltration depth and sphincter function
Rigid Rectoscopy
– Biopsy
– Exact localization (distance from anocutaneous line)
Colonoscopy
– Exclusion of second tumor
Endorectal Ultrasound
– Infiltration depth
– Crucial for the evaluation of T1 tumors
– Limited for the assessment of lymph node involvement
MRI Pelvis
– Distance to circumferential resection margin (CRM)
– Infiltration depth
– Crucial for local staging of T2 to T4 tumors
– Limited for evaluation Lymph node involvement
Thoracic CT, Abdominal CT
– Exclusion of distant metastases; sonography abdomen and Chest X-ray
alternatively possible, but less sensitive
PET-CT
– Not required for primary diagnosis
– Helpful in recurrence diagnosis

Therapy
Indication
– Therapeutic procedure according to guidelines depending on
preoperative staging (◘ Table 4.1)
– Increasing trend towards neoadjuvant therapy depending on the distance
of the tumor from the mesorectal fascia, currently evaluation in trials:
distance to CRM <1 mm: neoadjuvant therapy; distance to CRM ≥1 mm:
primary resection.
– Optimal procedure for cancer in the upper third of the rectum = unclear =
neoadjuvant therapy and surgery vs. treatment as in sigmoid cancer
(primary surgery ± adjuvant chemotherapy)
– Increased morbidity in emergency surgery for ileus:
– In case of ileus: relief by insertion of a double-barrel transverse
colostomy or endoscopic insertion of a fully covered metal stent,
followed by definitive therapy according to the guidelines
Table 4.1 Therapy strategy depending on preoperative staging

Tumor stage Therapy

T1 “low risk” Local excision
T1 “high risk”, T2 Primary resection
N0 M0
T3/4 N0 M0 Neoadjuvant therapy, followed by resection + adjuvant chemotherapy
Tx N+ M0
Tx Nx M1 Radical resection of tumor and metastases ± adjuvant chemotherapy
Primary tumor resection followed by additive chemotherapy and
metastasectomy or vice versa
Palliative therapy

Neoadjuvant Therapy
– Neoadjuvant radiotherapy: Significant reduction of local recurrence rate
in locally advanced tumor stages from 27 to 11%.
– Adjuvant radiotherapy: positive effect after optimal surgery smallerwhen
compared with neoadjuvant, but still present
Long-Term Radiochemotherapy (Preferred in Germany and USA)
– Target:
– Downstaging + Downsizing
– Increase in the rate of sphincter-preserving surgery
– Implementation:
– Conventional fractionated radiotherapy with 45–50 Gy (28 single
doses of 1.8 Gy each) + concomitant chemotherapy with 5-FU and
folinic acid (5-fluorouracil) or capecitabine over a period of 6 weeks
– Operation 6–8 weeks later
– In recent meta-analyses, higher remission rates after 10–12 weeks (but
contradictory RCT from France)
– Postoperative: Adjuvant chemotherapy
Short-Term Therapy (Preferred in the Netherlands, Poland and
Scandinavia)
– No tumor reduction
– In the lower third of the rectum: less effective than long-term
radiochemotherapy
– Implementation:
– Exclusively radiotherapy with 25 Gy distributed over 5 individual
doses
– Operation in the immediate aftermath
– Comparable results with regard to the oncological outcome—in recent
studies, delayed surgery with subsequent tumor reduction is also
possible
– Problems of Neoadjuvant Therapy as a Whole:
– Preoperative overstaging in 18% of UICC-II/-III classified patients,
especially correct detection of lymph node status (see above)
– Long-term side effects possible: sphincter weakness, potency
disorders, secondary cancers
– No significant effect on survival rate, therefore generally not indicated
in the metastatic stage
– Neoadjuvant chemotherapy without radiotherapy or intensified
neoadjuvant chemotherapy with prior or subsequent radiotherapy (=
“total neoadjuvant therapy”): Currently the subject of trials (RAPIDO)
– Complete remission after neoadjuvant therapy: radical surgery
generally indicated due to remaining vital tumor cells, “watch and
wait” = individually possible, preferably only in studies

Adjuvant Therapy
Modalities
– As adjuvant chemotherapy after long-term neoadjuvant therapy (see
above)
– As combined radiochemotherapy after R0 resection and not-performed
neoadjuvant therapy in stages II and III
– After R1 resection, tumor perforation or intraoperative tumor rupture also
indicated in stage I
Results
– Lower local recurrence rate and higher morbidity than after neoadjuvant
therapy
– Benefit of adjuvant chemotherapy in the context of long-term
neoadjuvant therapy = controversial
– No benefit from intensification of chemotherapy

Additive/Palliative Therapy
Principles
– Individual approach depending on tumor location, extent of metastasis
and general condition of the patient
– Distant metastasis = prognostic in non-stenosing/non-bleeding tumor
with extensive metastasis
– Benefit of primary tumor resection before chemotherapy = unclear
Strategy
– In stenosing cancer and multimorbid patients:
– Use of a double-barrel stoma or insertion of a flexible metal stent
– With or without subsequent chemotherapy
– In patients in good general condition and potentially resectable
metastases = curative approach:
– Primary resection of the primary tumor, if necessary additive
chemotherapy and subsequent resection of the metastases
– Alternatively primary resection of the metastases with subsequent
resection of the primary tumor
– Depending on the localization, thermoablation instead of or in
combination with resection of metastases
– Additive chemotherapy not longer than max. 5 cycles; also in case of
complete radiological response: Metastasectomy obligatory (in 30%
still vital tumor cells detectable).
– Up to 30% long-term survival after R0 resection of primary tumor and
metastases
– Benefit of additional “pseudoadjuvant” chemotherapy after R0
resection of primary tumor and metastases = not proven
– Benefit of “pseudoneoadjuvant” chemotherapy before resection of
primary resectable metastases = controversial
– Palliative chemotherapy with as few side effects as possible (e.g. 5-FU,
capecitabine), additive chemotherapy with as good a response as possible
(e.g. FOLFOX/FOLFIRI ± EGFR/VEGF antibodies)
Operative Therapy Principles
Local Limited Procedures
– Indication:
– For malignant, non-invasive polyps
– For carcinomas with early infiltration of the submucosa, maximum T1
sm 1–2, maximum size 3 cm without other negative predictors (G1–2,
R0, L0, V0, Pn0).
– Disadvantages:
– No assessment of lymph node status possible, but under these
conditions low risk of metastasis (approx. 2%)
– Increased risk of local recurrence compared to anterior resection
(approx. 10%)
– Principle:
– Surgical rectal full wall excision
– Endoscopic resection (endoscopic mucosal resection, submucosal
dissection)
– Avoid peacemeal resection
– Surgical procedure:
– Transanal full wall excision (lower third of the rectum)
– Transanal endoscopic microsurgery: TEM/TEO = transanal
endoscopic surgery, TAMIS (“transanal minimally invasive surgery”);
middle and upper third of the rectum
In multimorbid patients, locally limited rectal resection is permissible as
an individual therapy after appropriate patient information, even in the case
of locally advanced tumours (exception).

Surgical Procedure
Local Limited Rectal Resection
– Bowel preparation helpful
– General or locoregional anaesthesia
– Positioning with tumor at floor level
– Safety distance 1 cm
 – Transanal resection: exposition of tumor using anal spreader,
placement of holding sutures, pulling the tumor caudally, excision
with electrocautery, transverse suture closure
– Transanal microsurgery/endoscopy: insertion of the instrumentation,
marking of the resection line with electrocautery, dissection of the
rectal wall with electric knife, transverse suture closure
– Rapid opening of the suture in case of suspicion of pararectal
infection

Rectal Resection
– Principles of resection:
– Removal of the rectum + en bloc removal of the locoregional
lymphatic drainage area
– Preparation along the anatomical enveloping fasciae (see above)
– For tumors in the upper third: Anterior rectal resection with partial
mesorectal excision (PME)
– For tumors in the middle and lower third: low anterior rectal resection
with total mesorectal excision (TME)
– Radicular ligation of the inferior mesenteric artery and vein, no
prognostic difference between truncal ligation and preservation of the
left colic artery
– Protection of the autonomic nerves (see above) essential
– Maintain sufficient distal clearance margin:
– Anterior resection with PME: 5 cm
– Low anterior resection with TME for high-grade tumors: 2–3 cm
– Low anterior resection with TME for low-grade tumors: 1 cm
– After neoadjuvant therapy and negative frozen section: At least 0.5
cm
– En bloc resection of tumor-adherent organs (multivisceral
resection)
– Laparoscopic surgery is oncologically equivalent in suitable
patients (less favourable results possibly in low-located rectal
carcinoma (ALACART, ACOSOC Trial)—value of robot-assisted
procedures in lower conversion rate in men with narrow pelvis
(ROLARR Trial))
– Principles of Reconstruction:
– Reconstruction depending on the extent of resection:
– PME: End-to-end anastomosis (residual rectal pouch available)
– TME: colon-J-pouch-anal anastomosis, alternatively in case of
narrow pelvis or voluminous mesentery coloplasty-pouch-anal
anastomosis or side-to-end anastomosis (= reduction of stool
frequency and imperative urge to defecate)
– Anastomosis:
– Double-stapling technology
– For very distally located tumors: intersphincteric resection with
coloanal hand suture
– Ta TME (transanal TME) developed as a transanal adjunct to TME
in obese men with low-seated tumors—possible advantages in
clarity but higher incidence in urethral lesions.
– Protective stoma after low anterior resection
– Background:
– Insufficiency rate increases distally (up to 30%), therefore optional
after PME
– Does not prevent the insufficiency, but significantly reduced
inflammatory reaction in the pelvis
– Double-barrel ileostomy with less prolapse and lower complication
rate than reverse transversostomy
– Double-barrel transversostoma with less postoperative fluid loss
(preferred in elderly patients with renal insufficiency)
Preoperative bowel irrigation and marking of the stoma position (lying,
standing and sitting) are important.

Surgical Procedure
Open Low Anterior Rectal Resection
– General anesthesia, lithotomy (Lloyd-Davis) positioning, peridural
catheter
– Median laparotomy, exploration of the abdomen
– Lateral mobilization of the descending colon, exposure of the left
ureter
– Mobilization of the left colonic flexure from lateral to medial
– transection of the inferior mesenteric artery approx. 1 cm preaortic,
transection of the inferior mesenteric vein at the lower edge of the
pancreas
– Radicular transection of the mesentery, transection of the colon at the
descendosigmoidal junction
– Start of TME dorsally, sharp dissection between mesorectum and
Waldeyer’s fascia, sparing the hypogastric nerves down to the pelvic
floor
– Anterior dissection along the Denonvillier’s fascia, protection of
seminal vesicles and prostate or vagina
– Transection of lateral bridges along the hypogastric nerves, circular
preparation of the rectum at the pelvic floor
– “Rectal washout”, transection of rectum with a linear stapler
– Colon J-pouch: limb length 5–6 cm, coloplasty pouch: 6–8 cm
incision ventrally between the taeniae coli with transverse closure,
side-to-end anastomosis: stump with 2–3 cm length
– Transanal double stapling anastomosis, protective stomy
– Eventually placement of a suprapubic bladder catheter in men

Surgical Procedure
Laparoscopic Low Anterior Rectal Resection
– Lithotomy (Lloyd-Davis) positioning, vacuum mattress, shoulder
supports
– Pneumoperitoneum, insertion of the trocars
– Preliminary transection of the vessels, mobilization of the descending
colon and the left flexure from medial to lateral (caution: vegetative
nerves and pancreatic tail)
– TME as for open resection
– Distal transection with angled stacker, several magazines may be
required
– Retrieval of the specimen through widening of the incision in the left
lower abdomen or suprasymphyseal Pfannenstiel incision
– Reconstruction and anastomosis as for open resection
Abdominoperineal Rectal Extirpation
– Indication:
– For tumors infiltrating the sphincter/anal canal
– If the distal clearance margin is not sufficient (see above)
– Principles:
– For deep-seated T1/2 tumors: classical abdominoperineal extirpation
leaving the lateral levator muscles intact
– In advanced tumor stages: Cylindrical rectal extirpation including the
levator musculature, coverage by pedicled myocutaneous flap
(VRAM, bilateral gluteal shift flap)
– Results:
– Higher local recurrence rate than after sphincter-preserving surgery
– Conventional and laparoscopic procedure = oncologically equivalent

Surgical Procedure
Abdominoperineal Rectal Extirpation
– Insertion of a transurethral bladder catheter
– Abdominal part:
– Mobilization of the left colonic flexure not required
– TME as in anterior resection with sphincter preservation in
classical extirpation, preparation only up to the levator attachment
in cylindrical extirpation
– Creation of a terminal descendostoma, prevention of a parastomal
hernia by mesh augmentation or extraperitoneal drainage
– Insertion of an omental patch into the sacral cavity
– Perineal part:
– Preparation for classical extirpation in lithotomy (Lloyd-Davis)
position, for cylindrical extirpation: knee-chest position if
necessary (better overview for large tumors)
– Suture (closure) and circular dissection of anus
– Transection of the ischiorectal fat
– Transection of the anococcygeal ligament or coccygeal resection
– Transection or resection of the levator ani muscle
 – Ventral release of the specimen (caution: urethral injury)
– Layered wound closure or flap plastic reconstruction

Prognosis
Prognostic Factors
– Depth of infiltration into the intestinal wall
– Presence of lymph node and distant metastases
– Tumor cell differentiation
5-Year Survival Rates
– 5-year survival rate = on average 40–60% (most frequent finding = stage
III)
– 5-year survival rates by UICC stage:
– Stage I = approx. 80–100%
– Stage II = approx. 60–80
– Stage III = approx. 30–60
– Stage IV = approx. 0–57%
– 5-year survival rate in stage IV dependent on:
– Lymph node status
– Number and size of metastases
– CEA level (tumor marker >200 μg/L unfavorable)
– Disease-free interval (<12 months unfavorable)

Follow-up
Targets
– Early detection of potentially curable local recurrences (up to 25%)
– Early detection of distant metastases (up to 25%)
– Early detection of metachronous second tumors (up to 10%)
Time Intervals
– Every 6 months:
– Anamnesis
– Physical examination
– CEA determination
 – Abdominal Ultrasound
– After 1 and 5 years:
– Colonoscopy
– Exception: If no preoperative complete colonoscopy due to e.g.
stenosis: colonoscopy within the first 6 months postoperatively
– On a yearly basis:
– Thoracic X-ray = optional
Special Features
– Stage I after radical resection (very low risk): Colonoscopy only
recommended
– After local resection (due to increased risk of local recurrence):
Endoscopic controls after 6, 24 and 60 months recommended
– CT, MRI and PET-CT = suitable for detecting recurrences; not
recommended in routine follow-up due to insufficient evidence
– No age limit for follow-up
– No follow-up after palliative therapy

4.3.3 Guidelines
Guideline program oncology (German Cancer Society, German Cancer Aid,
AWMF): S3 guideline colorectal carcinoma, long version 2.1, 2019, AWMF
registration number: 021-007OL, ► https://​www.​leitlinienprogra​mm-
onkologie.​de/​fileadmin/​user_​upload/​Downloads/​Leitlinien/​Kolorektales_​
Karzinom/​Version_​2/​LL_​KRK_​Langversion_​2.​1.​pdf

Further Reading
Alkatout I, Egberts JH, Mettler L et al (2015) Interdisziplinäre Diagnostik und Therapie der tief
infiltrierenden Endometriose. Zentralbl Chir 140:630–638. https://​doi.​org/​10.​1055/​s-0034-1383272
[Crossref]

Braun J, Kasperk M, Saklak M, Ulmer F, Willis S (2011) Gutartige Erkrankungen von Dickdarm und
Rektum. In: Siewert JR, Rothmund M, Schumpelick V (Hrsg) Praxis der Viszeralchirurgie,
Gastroenterologische Chirurgie, 3 Aufl. Springer, Heidelberg, pp S527–S584

Church JM, Raudkivi PJ, Hill GL (1987) The surgical anatomy of the rectum—a review with
particular relevance to the hazards of rectal mobilisation. Int J Color Dis 2(3):158–166
[Crossref]
Fleshman J, Branda M, Sargent DJ et al (2015) Effect of laparoscopic-assisted resection vs open
resection of stage II or III rectal cancer on pathologic outcomes: the ACOSOG Z6051 randomized
clinical trial. JAMA 314(13):1346–1355. https://​doi.​org/​10.​1001/​jama.​2015.​10529
[Crossref][PubMed][PubMedCentral]

Fritzmann J, Weitz J (2013) Rektumkarzinom—Teil 1: Präoperative Diagnostik. Allgemein

Viszeralchirurgie 7:59–69
[Crossref]

Horisberger K, Kienle P (2013) Rektumkarzinom—Teil 2: Operationstechnik. Allgemein

Viszeralchirurgie 7:163–181
[Crossref]

Jayne D, Pigazzi A, Marshall H et al (2017) Effect of robotic-assisted vs conventional laparoscopic

surgery on risk of conversion to open laparotomy among patients undergoing resection for rectal
cancer: the ROLARR randomized clinical trial. JAMA 318(16):1569–1580. https://​doi.​org/​10.​1001/​
jama.​2017.​7219
[Crossref][PubMed][PubMedCentral]

Lefevre JH, Mineur L, Kotti S et al (2016) Effect of interval (7 or 11 weeks) between neoadjuvant
radiochemotherapy and surgery on complete pathologic response in rectal cancer: a multicenter,
randomized, controlled trial (GRECCAR-6). J Clin Oncol 34(31):3773–3780
[Crossref][PubMed]

Lindsey I, Guy RJ, Warren BF, Mortensen NJ (2000) Anatomy of Denonvilliers’ fascia and pelvic
nerves, impotence, and implications for the colorectal surgeon. Br J Surg 87(10):1288–1299
[Crossref][PubMed]

Luo C, Plank AW, Merrie AE, Plank LD, Bissett IP, Saramanayake CB (2010) Systematic review on
ventral rectopexy for rectal prolapse and intussusception. Dis Colon Rectum 12:504–512

Matzel KE, Heuer S, Zhang W (2008) Rektumprolaps. Chirurg 79:444–451

[Crossref][PubMed]

Pox CP (2014) Rektumkarzinom Teil 3: multimodale Therapie und Nachsorge. Allgemein

Viszeralchirurgie 8:287–295
[Crossref]

Ramage JK (2008) Consensus guidelines for the management of patients with digestive
neuroendocrine tumours: well-differentiated colon and rectum tumour/carcinoma.
Neuroendocrinology 87:31–39
[Crossref][PubMed]

Standring S (2009) Gray’s anatomy. The anatomical basis of clinical practice. 40 Aufl. Churchill
Livingstone, Elsevier, London

Stevenson AR, Solomon MJ, Lumley JW et al (2015) Effect of laparoscopic-assisted resection vs

open resection on pathological outcomes in rectal cancer: the ALaCaRT randomized clinical trial.
JAMA 314(13):1356–1363. https://​doi.​org/​10.​1001/​jama.​2015.​12009
[Crossref][PubMed]
Ulrich A (2019) Chirurgir des Rektumkarzinoms UpDate 2019. Dtsch Arztebl 116(23–24):4

Willis S, Schumpelick V (2010) Rektumkarzinom. In: Siewert JR, Rothmund M, Schumpelick V

(Hrsg) Praxis der Viszeralchirurgie Onkologische Chirurgie, 3 Aufl. Springer, Heidelberg, pp S713–
S734

Wittekind C (2017) TNM—Klassifikation maligner Tumoren, 8 Aufl. WILEY-VCH, Weinheim, pp

S98–S100. isbn:978-3-527-34280-8

Suggested Reading
Bahadoer RR, Dijkstra EA, van Etten B et al (2021) Short-course radiotherapy followed by
chemotherapy before total mesorectal excision (TME) versus preoperative chemoradiotherapy, TME,
and optional adjuvant chemotherapy in locally advanced rectal cancer (RAPIDO): a randomised,
open-label, phase 3 trial. Lancet Oncol 22:29–42

Kreis ME, Ruppert R, Ptok H, Strassburg J, Brosi P, Lewin A, Schön MR, Sauer J, Junginger T,
Merkel S, Hermanek P, OCUM Study Group (2016) Use of preoperative magnetic resonance
Imaging to select patients with rectal cancer for neoadjuvant chemoradiation—interim analysis of the
German OCUM Trial (NCT01325649). J Gastrointest Surg 20(1):25–32

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_5

5. Anorectum
Jens Wannenmacher1 and Stefan Willis1
(1) Surgical Department A, Ludwigshafen Hospital, Ludwigshafen, Germany

Jens Wannenmacher
Email: [email protected]

Stefan Willis (Corresponding author)

Email: [email protected]

Key Points
– Complex anatomy and physiology of the anorectum: variety of proctological
diseases
– Treatments as different as etiologies
– Defecation/Stool Continence: Complex Interaction of the Large Intestine
Rectum Pelvic Floor: Different Therapeutic Approaches Required for Fecal
Incontinence/Outlet Obstipation

5.1 Anatomy and Physiology

5.1.1 Anatomy
– Anatomy of anal canal: approx. 0.5 cm distal to the linea anocutanea to dentate
line (pectinate line)
– Length = approx. 3–4 cm
– 10–25 crypts (excretory ducts of the proctodeal glands) at the level of the dentate
line
– Hemorrhoidal plexus (Corpus cavernosum recti): subepithelial venous plexus at
the upper edge of the anal canal
– Epithelial lining: Tripartite
– Distal to the linea anocutanea: keratinizing squamous epithelium
– Anal canal: Non-keratinizing squamous epithelium (anoderm)
– Proximal to the dentate line: transitional cell area (cylindrical epithelium)
– Musculature: Smooth + striated
– Internal anal sphincter: smooth muscle; continuation of the ring muscle of the
rectum; innervation: autonomous via stretch receptors in rectal wall
– External anal sphincter: transversely striated muscle; encloses the internal
sphincter like a cylinder; pars subcutanea, superficialis, profunda; innervation:
somatic = pudendal plexus
– Puborectal muscle (M. puborectalis): transversely striated muscle; cranial of
the external sphincter, corresponds to the lowest part of the levator ani muscle;
embraces the rectum from the dorsal side (= in the form of a loop); innervation:
somatic = pudendal plexus
– Blood supply: inferior rectal artery, middle rectal artery

5.1.2 Physiology
– Continence:
– Internal anal sphincter: Involuntary continuous contraction (resting pressure)
– External anal sphincter: voluntary contraction on demand (pinch pressure);
contraction prevents relaxation of the internal sphincter (= voluntary inhibition
reflex)
– Puborectal muscle: voluntary contraction = maintenance and reduction of the
anorectal angle
– Hemorrhoidal plexus: venous outflow of the “haemorrhoids”; contraction of
the internal anal sphincter = partial throttling of the outflow; erectile tissue
function = intraluminal sealing of the anal canal = fine continence
– Rectum: variable reservoir function (compliance); retrograde stool transport
possible through segmental contractions
– Defecation:
– Stool-filled ampoule = stimulation of the stretch receptors
– Stimulation = contraction of the detrusor recti
– Relaxation of the internal anal sphincter ani: rectoanal inhibition reflex
– By abdominal pressure + voluntary relaxation of the external anal sphincter
and puborectal muscles (enlargement of the anorectal angle) = defecation.

5.2 Benign Diseases

5.2.1 Hemorrhoidal Disease
Key Points
 – Typical localisation: 3 (left lateral), 7 (right posterior) and 11 (right anterior)
o’clock in lithotomy (Lloyd-Davis) position
– Hemorrhoids = physiological: important role in fine continence
– Hemorrhoidal disease = at the appearance of symptoms
– first and second degree hemorrhoids = conservative/interventional therapy
– third and fourth degree hemorrhoids = surgical therapy

Definition
– Normal anal cushions (pads): Usually at 3 o’clock, 7 o’clock and 11 o’clock in
Lloyd-Davis position
– By placing these pads next to each other = precise closure of the anal canal = fine
continence; anal cushions contribute about 15% of resting anal pressure
– Haemorrhoidal disease = in the case of hypertrophy and/or the appearance of
symptoms =

Classification
– First-degree hemorrhoid:
– Only proctologically visible protrusion
– Second-degree hemorrhoid:
– Cushion prolapse during pressing
– Spontaneous reduction
– Third-degree hemorrhoid:
– Cushion prolapse during pressing
– Manual reduction necessary (no spontaneous reduction)
– Fourth-degree hemorrhoid:
– Cushion permanently prolapsed
– Irreducible or reducible, with immediate reprolapse
– Subdivision possible into:
– Grade 4a: reducible under anaesthesia
– Grade 4b: Not reducible under anaesthesia
– Anal prolapse: fourth degree circular hemorrhoids ± mucosal prolapse, radial
folding (does not correspond to the circular folding seen in rectal prolapse; ►
Sect. 4.​2)

Epidemiology
– No exact data on prevalence and incidence
– One of the most common diseases in western industrialised countries (70% of
people suffer from hemorrhoids at least once in their lives)
– Men: Women = 2: 1
– Frequency peak: 45–65 years

Etiology
– Main Causes:
– Genetic factors
– defecation disorder
– Stool consistency
– Mechanism:
– Disturbed venous outflow = swelling of the hemorrhoidal plexus = in the
course hypertrophy of the tissue
– Hypertrophy of the tissue = among other things loosening of the ligamentous
connections = lack of retraction of the cushions after defecation
– Causal factors (among others):
– Increased intra-abdominal pressure (e.g. heavy pushing, pregnancy)
– Chronic sphincter hypertrophy
– Increased resting tone with inadequate relaxation
– Fecal impaction in chronic constipation
– Also chronic diarrhea tendency: No sufficient stretching and relaxation of the
anal canal = traumatization of the still bulging anal cushion.

Symptoms
– Transanal bleeding = cardinal symptom:
– Mostly bright red blood during or after defecation
– Often bleeding from the congested vessels of the covering mucosa, not from
hemorrhoid itself
– Extensive bleeding can lead to hemorrhagic shock
– Pain:
– Not typical, as not located in the sensitively supplied area of the anal canal
– Painful in incarcerated haemorrhoidal prolapse and thrombus formation
– Other symptoms:
– Smearing and oozing due to disturbance of fine continence
– Irritative-toxic anal eczema with pruritus ani: Resulting from oozing
Diagnosis
– Anamnesis
– Inspection, rectal digital examination
– Procto/rectoscopy
– Colonoscopy (exclusion of other diseases, especially colorectal carcinoma)

Differential Diagnosis
– Sentinel Pile (marisc): flap-like folds of skin on the external anal ring which do
not fill up during pressing
– Anal vein thrombosis (► Sect. 5.2.2)
– Fissures (► Sect. 5.2.3)
– Rectal prolapse (► Sect. 4.​2 Benign diseases of the rectum)
– Anal fibromas: benign fibromas lined with squamous epithelium; length up to 4
cm (fibroma pendulans)
– Malignant and benign tumors

Therapy
Basic Therapy
– Change of defecation behaviour + adjustment of diet = avoidance of strong
pressing and especially post pressing (= high-fibre diet)
– Drug therapy (hemorrhoidalia): Only symptomatic, no long-term therapy with
ointments containing cortisone
– Treatment of secondary changes (e.g. irritative-toxic anal eczema with e.g. zinc
paste)
Conservative and Interventional/Semioperative Therapy
– For hemorrhoids grade 1 and 2
Sclerotherapy (Blond or Blanchard or Bensaude procedures)
– Blond: submucosal injection of e.g. polidocanol or quinine into the hemorrhoidal
tissue
– Blanchard or Bensaude: Injection of e.g. phenol-almond solution into the area of
the afferent hemorrhoidal arteries.
– Principle: Fixation of the convolutes above the linea dentata by inflammation and
scarring
– Complications:
– Bleeding
– Allergic reactions
– Rectal Necrosis
Infrared Coagulation
– Principle: Infrared rays = superficial tissue necrosis above the linea dentata =
scarring + fixation of hemorrhoids
Rubber Ring Ligation (According to Barron)
– Principle: Ligation of the convolutes via an applicator = necrosis of the tissue
with scarring
– Complications:
– Post-operative bleeding up to 14 days after intervention (caution:
anticoagulated patients!)
– Severe pain with application below the linea dentata
– Allergic reactions
– Rectal Necrosis
– Abscesses
– Urinary retention
Doppler-Guided Hemorrhoidal Artery Ligation (HAL)
– Principle: Localization + ligation of the haemorrhoidal arteries via a special
proctoscope with built-in Doppler transducer = shirring + scarring
Recto-Anal Repair
– Principle: localization + ligation of the hemorrhoidal arteries + additional
continuous shirring of the haemorrhoidal convolutes
– Successful even without (expensive) Doppler support
Surgical Therapy
– For hemorrhoids ≥ grade 3 = surgical therapy
Milligan-Morgan Hemorrhoidectomy
– = gold standard

Surgical Procedure
Milligan Morgan Operation
– General anesthesia, spinal anesthesia, saddle block
– Lithotomy position, single-shot antibiotics with metronidazole i.v.
– Sphincter dilatation, insertion of the spreader
– Start with the largest hemorrhoid
– Placement of 2 sharp clamps on anocutaneous line and hemorrhoid
 – Injection of the anocutaneous tissue with e.g. diluted adrenaline saline solution
(better separation from the internal sphincter)
– Arch-shaped incision of the perianal skin and wedge-shaped incision of the
anoderm on both sides in the direction of the vascular pedicle
– If present, co-resection of the sentinel piles (mariscs)
– Dissection of the hemorrhoid with scissors or diathermy
– Resection of the base of hemorrhoid and suture with absorbable suture
– Same procedure at the other two positions
– Hemostasis, if necessary insertion of a tamponade
– Ensure that the anoderm bridges are wide enough

Ferguson Hemorrhoidectomy
– Principle: Resection as in Milligan-Morgan-OP, additionally continuous closure
of the anoderm defect except for a small drainage field
– Pros:
– Less pain
– Better bleeding control
– Anatomically correct reconstruction of the anal canal
– Disadvantage: Higher stenosis rate
Subanodermal Resection (Parks Procedure)
– Principle: Y-shaped incision of the anoderm, subanodermal/submucosal resection
of the hemorrhoid, reconstruction of the anoderm
– Advantage: Low anoderm loss (especially if extensive findings)
– Disadvantage: Quite complex surgical technique
Stapler Hemorrhoidopexy (Longo Procedure)
– Principle: Circular resection of the rectal mucosa by means of a stapler at the
point of attachment of the hemorrhoids = pexie of the haemorrhoids at their place
of origin
– Pros:
– No violation of the sensitive anoderm
– Less postoperative pain
– Anatomically correct reconstruction of the anal canal
– Reduction of mucosal prolapse
– Disadvantages:
– Significantly higher costs (stapler device)
– No histological workup of the hemorrhoids
 – Significant stretching of the anal canal

Surgical Procedure
Stapler Hemorrhoidopexy
– General anaesthesia, spinal anaesthesia or saddle block
– Single-shot antibiosis optional
– Sphincter dilation, insertion of the transparent speculum, fixation with single
stitch sutures
– Starting at 12 o’clock in Lloy-Davis, placement of a mucosal, purse string
suture with monofilament suture 2–3 cm above the linea dentata
– Inserting the fully opened stapler, placing the pressure plate above the purse
string suture, closing the same
– Pulling the two suture ends through openings on the stapler head and knotting
a tab
– Under continuous pull on the sutures, closure of the stapler
– Caution: In female patients, check that the posterior vaginal wall is not traped
in the device!
– Firing and removal of the stapler after 1 minute compression time
– Checking for blood dryness, arterial bleedings should be stitched
– Insertion of a tamponade if necessary

Therapy Strategy for Grade 4 Hemorrhoids

– In the acute stage: First cooling, analgesia and local therapy to reduce the oedema
(if possible)
– Plastic Reconstructive Procedures (Fansler-Arnold):
– Resection of haemorrhoids + reconstruction of the anal canal with transposition
flap
– Operatively demanding, time-consuming, high complication rate
– Combined procedures: e.g. Ferguson’s procedure for the most extensive findings
+ hemorrhoidal artery ligation with recto-anal repair for the smaller findings
– Two-stage approach
– For hemorrhoids that can be reduced under anesthesia: In experienced hands
Longo’s stapler hemorrhoidopexy possible (with increased recurrence rate)
– For all resecting procedures: Keep anoderm loss as low as possible

Grade-Adapted Therapy
– In all stages: High-fibre diet, fluid intake, correct defecation as flanking measures.
– Grade 1: Sclerotherapy, infrared coagulation, if necessary HAL
– Grade 2: Rubber band ligation, HAL, if necessary sclerotherapy, recto-anal repair
– Grade 3: Closed/open hemorrhoidectomy, stapler haemorrhoidopexy
– Grade 4: Closed/open hemorrhoidectomy, stapler hemorrhoidopexy if necessary,
two-stage procedure if necessary
Complications
– Postoperative pain (less common with stapler hemorrhoidopexy than with
resecting procedures)
– Bleeding
– Urinary retention
– Sentinel piles (Mariscs)
– Recurrence
– Anal Fissure
– Fecal Incontinence
– Anal stenosis (especially with extensive resections of the anoderm = Whitehead
anus)
– Recurrence (approx. 15%)
– Most severe complications (rectovaginal fistula, Fournier’s gangrene, pelvic
sepsis) = very rare

5.2.2 Anal Vein Thrombosis

Key Points
– Blood clots of the subcutaneous and subanodermal veins of the external anal
region
– Incision and expression (in very painful patients = immediate relief from
symptoms); disadvantage = high recurrence rate
– Therapy of choice for pronounced findings = total excision

Definition
– Acute thrombosis of the subanodermal and subcutaneous veins of the inferior
hemorrhoidal plexus
– Singular or pearled (chambered)
– Size: Up to plum size
“External hemorrhoid” is an Incorrect denomination.

Epidemiology
– Approx. 5% of proctological patients
– Men: Women = 2: 1
Etiology
– Exact etiology is not clear
– Often, however, no cause can be identified

Triggering Factors
– Thermal influences (cold, muggy weather)
– Physical exertion (e.g. jogging, cycling)
– Intra-abdominal pressure (e.g. pressing, defecation, pregnancy)
– Nutritive factors (alcohol, hot spices)
– Mechanical factors (proctological surgery, anal intercourse)
– Diarrhea
– Enlarged hemorrhoidal cushions (connection to the succutaneous venous plexus =
slowing of blood flow)

Symptoms
– Usually abrupt pain in the anal area with swelling
– Infrequent moderate dull ache, occasionally on touch only
– Other symptoms:
– Itchy
– Stitch
– Burning

Diagnosis
– Analogous to the diagnosis of hemorrhoidal disease (► Sect. 5.2.1)

Differential Diagnosis
– Thrombosed hemorrhoids
– Mariscs
– Abscesses
– Anal Fibroids
– Melanoma(!)
– Anal (marginal) carcinoma

Therapy
Conservative Therapy
– In patients with few symptoms and chronic findings:
– Application of antiphlogistic local therapeutics
– Administration of systemic acting non-steroidal anti-inflammatory drugs
Surgical Therapy
– Incision, drainage, expression of the thrombus under local anesthesia:
– Suitable for small findings
– Mostly immediate relief from complaints
– Disadvantage: High recurrence rate
– Complete excision under anesthesia:
– Indicated for larger findings
– Advantages: Low recurrence rate + histological examination possible
– Disadvantage: Larger wound area
Complications
– Rare in open wound healing
– Recurrences: the more complete the excision, the rarer

5.2.3 Anal Fissure

Key Points
– Elongated, painful ulceration in the anal canal, usually at 6 o’clock in Lloyd-
Davis position
– Acute anal fissure: anaesthetic creams, sphincter stretching, sphincter relaxing
drugs
– In case of unsuccessful therapy or chronicity with formation of outpost fold and
hypertrophic anal papilla: excision (Gabriel procedure)
– In case of post-operatively non-healing fissures and high tonus: if necessary
lateral sphincterotomy

Definition
– Elongated ulceration of the anal canal, about 90% at 6 o’clock in Lloyd-Davis
position
– Transition from acute to chronic fissure often fluent

Acute Anal Fissure

– Superficial, elongated defect of the anoderm with sharply defined edges
– Bloody or greasy wound bed

Chronic Anal Fissure

– Rough, raised rim wall
– Sclerosed internal fibers at the base of the fissure
– Cranially hypertrophied anal papilla, caudally “outpost fold” (Marisc)

Epidemiology
– Approx. 15% of proctological patients
– Men: Women = 1: 1

Etiology
Main Cause = Heavy Pressing
– Strong pressing during hard defecation → tearing of the anoderm → reactive
cramping of the musculature due to pain → difficult defecation, reduced
perfusion → poor healing tendency = vitious circle
Other Causes
– Anal fistula
– Manipulation
– Cryptitis
– Diarrhea
– Chronic spincter spasm
– Chronic inflammatory bowel diseases
– Hemorrhoidal disease (cofactor)
Symptoms
– Acute pain: onset with defecation, possibly lasting for hours
– Proctogenic constipation: due to fear of pain recurrence
– Light red blood accumulation
– Secretion, smearing of stool
– Dyscontinence
– Pencil thin stool
Diagnosis
– Corresponds to the diagnosis of hemorrhoidal disease (► Sect. 5.2.1)
– If impossible due to pain = injecting the fissure with a local anaesthetic
– If, despite anaesthesia, impossible and there is doubt about the diagnosis =
proctological examination under anaesthesia to exclude e.g. an abscess
Differential Diagnosis
– Fissure in Crohn’s disease
– Anal lues, AIDS-associated lesions
– Anal Carcinoma
– Intersphincteric abscesses
– Rhagades

Therapy
Conservative Therapy
– Primary conservative therapy for acute anal fissure
– Stool regulation, sufficient drinking quantity, creams
– Sphincter stretching:
– Daily self-stretching of the sphincter with anal dilator
– If necessary, subsequent insertion of an anal tampon
– Anaesthetic creams, occasionally local anaesthesia required
– Effective, but currently largely abandoned in favor of drug therapy
– Nitroglycerin:
– Application of 0.2% nitroglycerine ointment = reduction of sphincter tone +
improvement of blood circulation
– 3 times daily for 8 weeks into the entire anal canal
– Systemic side effects common (especially headache)
– Calcium antagonists:
– 0.2% nifedepine or 2% diltiazem ointment = reduction of sphincter tone +
improvement of blood flow
– Intraanal use not required
– Systemic side effects rare
– Higher recurrence rate
– Botulinum toxin:
– Injection of botulinum toxin = muscle paralysis for up to 3 months = support of
the healing process
– Temporary incontinence up to 5%
– High costs
Surgical Therapy
– Indication: Chronic anal fissures
– Sphincter stretching and fissurectomy (Gabriel procedure) = standard in German-
speaking countries

Surgical Procedure
Gabriel Procedure
– General anesthesia, spinal anesthesia, saddle block
 – Lithotomy (Lloyd Davis) position
– Cautious sphincter dilatation, insertion of the spreader
– Caution: Sphincter stretching (Lord technique) (8 fingers) = obsolete
– Exclusion of fistula, probing of the crypts at the level of the anal papilla
– Excision of outpost fold, fissure and hypertrophic anal papilla en bloc with
protection of the sphincter with diathermy or scissors
– Targeted transection of remaining, sclerosed sphincter fibers
– Waiver of sphincterotomy
– Triangular drainage, wider towards the outside
– Insertion of a tamponade if necessary
– Lateral sphincterotomy
– Widespread in the Anglo-American area
– Transection of the lower two thirds of the internal sphincter at 3 o’clock
Lloyd-Davis position with protection of the anoderm
– Reduced tone as a prerequisite for fissure healing
– Disadvantage: Considerable risk of (late) incontinence
– Eisenhammer fissurectomy:
– With additional deep sphincterotomy in the area of the fissure
Obsolete because of the risk of formation of a keyhole defect with disturbance of
fine continence

Stage-Appropriate Therapy
– High-fibre diet, fluid intake, correct defecation as accompanying measures
– Conservative:
– Anal stretchers, anesthetic creams
– Nitroglycerin
– Calcium antagonists
– Botulinum toxin
– Operative therapy:
– Sphincter stretching and Gabriel fissurectomy
– Eventually lateral sphincterotomy

Complications
– Systemic side effects (nitroglycerin, Botox)
– abscess-, fistula formation
– Incontinence (Botox, lateral sphincterotomy)
– Recurrence

5.2.4 Anorectal Abscess

Key Points
– Classification depends on the localization
– Origin mostly in cryptoglandular tissue
– Anorectal abscess = emergency indication
– Therapy = surgical

Definition
– Classification of the anorectal (periproctitic) abscess: According to the
localization
– Classification:
– Subanodermal abscess
– Intersphincteric abscess
– Ischioanal abscess
– Supralevator/pelvic abscess

Epidemiology
– Incidence = approx. 2–3 per 10,000 inhabitants/year
– Frequency peaks between 30 and 50 years of age
– Men: Women = 3: 1

Etiology
Cryptoglandular Origin
– >90% of the cases
– obstruction of the proctodeal glands (e.g. stool) = cryptitis = secondary abscess =
abscess spread along avascular planes (path of least resistance)
Rarer causes
– Inflammatory bowel diseases, especially Crohn’s disease
– Rectal perforation (e.g. swallowed toothpicks, anal foreign bodies)
– Diagnosis or surgical manipulation
– Tuberculosis

Caution
Gravity abscess in e.g. appendicitis, pyelonephritis or sigmoid diverticulitis
Symptoms
– Mostly acute painful swelling, possibly reddening in the anal area
– With advanced findings: Fever, general ill feeling, sepsis…

Caution
Supralevatorial abscess: Often dull pain in the pelvis, back pain, externally
inconspicuous.

Diagnosis
– Inspection:
– Usually sufficient
– Further invasive examinations usually not tolerated by the patient
– Supralevator abscess:
– Exception
– Fluctuation and circumscribed tenderness during rectal examination
– In unclear cases:
– Rectoscopy + endosonography (if tolerated by the patient)
– CT/MRI if necessary
– In case of doubt = proctological examination/endosonography under
anaesthesia
Differential Diagnosis
– Carbuncle
– Infected atheroma
– Acne inversa
– Anal fissure, anal vein thrombosis
– Pilonidal sinus
– Neoplasia

Therapy
Conservative Therapy
– Not purposeful
– Even with spontaneously perforated abscesses = usually no sufficient drainage
Surgical Therapy
– Therapy of the anorectal abscess = operative
– Subanodermal, ischioanal, and most intersphincteric abscesses = generous
excision.
– For high intersphincteric and supralevatoric abscesses = transrectal discharge
(avoidance of fistula formation)

Surgical Procedure
Abscess Excision
– General anesthesia, spinal anesthesia, saddle block
– Lithotomy (Lloyd Davids) position
– Antibiotics for extensive phlegmon or sepsis
– Procto-/rectoscopy, if necessary endosonography
– Longitudinal oval excision of the abscess with sparing of the sphincter
– If a fistula can be visualized without any problems, thread placed in fitula
(seton procedure) or split (fistulotomy) if it is deeply seated (experience of the
surgeon!)
– Caution: Never force a fistula presentation in an acute situation (via falsa)
– Tamponade insertion
– Specimen to pathology
– Proctoscopy to exclude fistula disease after a few weeks

Complications
– Sepsis, Fournier’s gangrene with delayed treatment
– Recurrence, especially if external drainage field is too small
– Sphincter damage
– Fistula formation as a late consequence

5.2.5 Anorectal Fistulas

Key Points
– Classification according to course (path)
– Anal fistula = chronic stage, anorectal abscess = acute stage of the same usually
cryptoglandular disease
– Therapy = surgical
– Goodsall rule: Above a line between 9 and 3 o’clock Lloyd-Davis position the
fistulas run in a straight line (inner and outer ostium on a same radial line),
below in an arc (inner ostium mostly at 6 o’clock in Lloyd Davis position)

Definition and Classification

Definition
– Connection of the anal canal with the skin surface lined with granulation tissue
Classification of Anorectal Fistulas (According to Course)
– Subanodermal fistula
– Intersphincteric fistula
– Transsphincteric fistula
– Suprasphincteric fistula
– Extrasphincteric fistula
Epidemiology
– Incidence = approx. 2 per 10,000 inhabitants/year
– Frequency peak: between 30 and 50 years of age
– Men: Women = 3: 1
Etiology
– Anorectal fistula = chronic form
– Anorectal abscess = acute form of the same disease
Cryptoglandular Origin
– >90% of the cases
– Proctodeal gland obstruction (e.g. faecal) → cryptitis → secondary abscess →
abscess spread along avascular plane (path of least resistance) → perianal
perforation (spontaneous or surgical): Connection between anal canal and body
surface (skin)
Rarer Forms of Fistula
– Atypical fistulas in Crohn’s disease
– Ischiorectal fistulas
– Rectovaginal fistulas
– Superficial fistulas in acne inversa
– Congenital fistulas
Symptoms
– Putrid, occasionally feculent secretion
– Recurrent, usually spontaneously perforating abscesses
– In the case of prolonged progression: Reduction of continence performance
Diagnosis
– Rectal digital examination
– Careful atraumatic probing of the fistula tract
– If the fistula is already thread-reinforced (seton procedure), have the patient
pinched = good estimation of the fistula’ course possible
– Procto/rectoscopy
– Endosonography, if necessary MRI (fistula course, exclusion of fuchsbau)
– Colonoscopy: mainly for Crohn’s disease
Differential Diagnosis
– Differentiation of fistulas of cryptoglandular origin from other forms of fistulas
(see above)
– Anal Fissure
– pilonidal sinus

Therapy
Conservative Therapy
– When excising a periproctitic abscess = do not force fistula presentation, since
spontaneous healing is possible
– Established fistula = indication for surgery (no spontaneous healing + high
probability of recurrent abscesses)
– Malignant degeneration possible (fistula carcinoma)
Surgical Therapy
– Thread drainage (seton procedure):
– Principle: Insertion of a non-absorbable suture/plastic vessel loop (“Vessel
loop” = more comfortable for patient)
– Therapeutic approaches: Healing of the acute inflammation, fibrosis of the
fistula tract, two-stage definitive treatment.
– In cryptoglandular fistulas: Spontaneous healing not to be expected
(occasionally successful with Crohn’s disease fistulas under drug therapy).
– Permanent solution for pre-damaged sphincter (caution: rarely malignant
degeneration)

Caution
“Cutting seton” should no longer be used due to high risk of incontinence.

– Fistula Splitting/Cleavage:
– Subanodermal, intersphincteric or deep transsphincteric fistula = splitting over
probe + open wound treatment
– Cure rates = up to almost 100%
– Individually different sphincter weakening after splitting (pre-damaged
sphincter, narrower ventral sphincter in women, etc.)
– In no case cut more than 1/3 of the sphincter mass.
– Fibrin Glue:
– Principle: curettage of the fistula tract + filling with fibrin glue
– Long-term healing rates = only 20%
– Possible therapy option in the case of a clearly damaged sphincter, since the
sphincter muscle is not affected.
– Fistula Plug:
– Principle: Occlusion of the fistula tract with porcine submucosa/biocompatible
synthetic polymer plug.
– Cure rates = 20–28%
– Indication: Similar to fibrin glue
– LIFT(“ligation of intersphincteric fistula tract”)-OP:
– Relatively new procedure
– Cure rates = 50–80%
– Low incontinence rate, as the sphincter apparatus is only slightly affected by
the gentle surgical technique.
– In the case of very scarred findings, visualization of the fistula tract in the
(narrow) intersphincteric space is often difficult.

Surgical Procedure
LIFT-OP
– General anesthesia, spinal anesthesia, saddle block
– Lithotomy (Lloyd Davis) position
– Perioperative antibiotic therapy optional
– Incision anocutaneous line above the (thread-armed) fistula
– Dissection of the intersphincteric space and visualization of the
intersphincteric fistula tract
– Suture ligature of the duct at the junction with the internal sphincter muscle
– Severing or excision of the duct
– Curettage of the external duct, if necessary drainage field extension
– Suture ligature of the duct at the junction with the external sphincter muscle
– Adaptive suture
Plastic fistula closure by flap:
– Principle: Fistula excision, muscle suture and covering of the internal ostium
by mucosa-submucosa flap, rectum full wall flap (advancement flap) or
anodermal flap
– Studies on cure rate = very inhomogeneous (46–95%)
– Relatively demanding surgical technique
 – Incontinence rate is low, as the sphincter apparatus is only slightly affected in
this procedure
Fistula excision with primary sphincter reconstruction:
– Principle: splitting of the sphincter, complete excision of the fistula tract,
reconstruction of the sphincter
– Relatively good cure rates of 60–80%
– Relatively high risk of continence disturbance, especially in case of dehiscence
of the sphincter suture (4–32%)
– Sophisticated surgical technique = prerequisite = great experience of the
surgeon

Stage-Appropriate Therapy
– Superficial fistula, good continence performance:
– Fistula splitting
– High fistula or limited continence performance:
– Plastic reconstruction
– Poor continence performance, hidden situs:
– Consider occluding procedures (in case of high recurrence rate)
– Consider lifelong thread drainage (rarely malignant degeneration possible)

Complications
– Septic complications
– Recurrence
– Incontinence
– Malignant degeneration with long course

5.2.6 Pilonidal Sinus

Key Points
– Frequent disease, which mostly affects young men
– Acute or chronic inflammation, mostly of the coccyx region
– Caution: The acute abscessing form is a surgical emergency
– Complete excision with open wound treatment is the most frequently performed
operation

Definition
– Inflammation of the subcutaneous tissue, mostly of the coccyx region
– In the sinus: granulation tissue, cell detritus, hair
– Different clinical forms:
– Asymptomatic form
– Acute abscessed form
– Chronic form

Epidemiology
– Frequency = 48/100,000 inhabitants (Germany, 2012)
– Frequency peaks between the tenth and 30th year of life
– Men: Women = 2.5–3: 1

Etiology
Risk Factors
– Genetic disposition possible
– Strong hairiness
– Profuse perspiration
– Deep anal fold
– Sitting activity
Pathophysiology
– Rubbing movements of the buttocks → penetration of broken hairs with their
ends close to the roots into the skin → skin scales act as barbs → migration of the
hair into the subcutaneous fat → foreign body granuloma → abscess possible

Symptoms
Asymptomatic Form
– Irritationless primary openings
– Incidental finding
Acute Abscessed Form
– Swelling
– Redness
– Pain
– eventually Perforation
– If it progresses = fever, sepsis…
Chronic Form
– Purulent secretion
Diagnosis
– As a rule, the clinical examination is sufficient
Differential Diagnosis
– Carbuncle
– Anorectal Abscess
– Anorectal Fistula

Therapy
Conservative Therapy
– Instillation of 80% phenol solution (not approved in Germany due to toxicity)
– Epilation by means of shaving (also as recurrence prophylaxis) = not useful
Surgical Therapy
– Radical en bloc excision with open wound treatment
– Most frequently used surgical procedure
– Recurrence rate = 2–13%
– Disadvantage: Often healing time of several weeks with corresponding
incapacity to work

Surgical Procedure
En Bloc Excision of the Pilonidal Sinus
– General anaesthesia, for very small findings local anaesthesia
– Prone position
– Antibiotics for extensive phlegmon or sepsis
– Staining of the ducts with e.g. methylene blue solution
– Whetstone-shaped excision of the complete findings with all lateral passages
with diathermy, if necessary down to the sacral fascia
– Bevelling of incision edges
– Tamponade insertion
– Plastic procedures:
– Defect coverage after excision by flap plastic of different types (e.g.
Karydakis, Cleft-Lift, Limberg, see below)
– Also referred to as “off-midline procedures” because of the lateral
displacement of the wound
– Significantly shorter healing time
– Number of “real” recurrences comparable with open wound treatment
– Disadvantage: Number of abscesses, dehiscences, wound healing disorders
relatively high (up to 45%), if not performed in a completely infection-free
 area

Surgical Procedure
Karydakis Flap/Cleft-Lift Procedure
– Symmetrical, elliptical excision of the sinus
– Mobilization of a subcutaneous flap of the opposite side
– Three-layer wound closure outside the midline

Surgical Procedure
Limberg Plastic
– Rhombic excision of the sinus
– Mobilization of rhombic-shaped flap down to the gluteal fascia
– Pivoting and tension-free sewing in of the flap
– Sparing excision of the pori, curettage of the fistula tracts
– Only for locally limited findings
– Procedures with unclear data:
– Fibrin instillation
– Autologous stem cells
– Laser therapy
– Not recommended:
– Excision with marsupialization of the wound edges
– Midline suture excision

Stage-Adapted Therapy
– Asymptomatic form:
– Prophylactic surgery does not seem necessary
– Acute abscessing form (emergency):
– Definitive radical excision, or
– Abscess relief and definitive therapy with plastic covering if necessary in
the infection-free interval
– Chronic form:
– Definitive radical excision, if necessary with plastic covering
 – In the case of locally limited findings, a semi-interventional procedure can
be considered

Complications
– Sepsis if delayed therapy
– Recurrence
– Long healing process (with open wound treatment)
– Abscess, wound healing disorder (with plastic covering)
– Malignant degeneration (rare)
– Cosmetically unsightly scarring

5.2.7 Fecal Incontinence

– In this chapter, only the surgical therapy methods are discussed in detail.

Key Points
– Frequent clinical picture with a high number of unreported cases
– Often multifactorial
– Therapy of fecal incontinence usually conservative at first

Definition
– Loss of intestinal contents at the wrong time or in the wrong place

Clinical Classification
– Grade I: Inability to hold air (winds) in a controlled manner
– Grade II: Inability to retain liquid stools
– Grade III: inability to retain normally formed stools

Score Classification
– Better, as quality of life is also taken into account
– For example, Cleveland Clinic Score (◘ Table 5.1), Jorge/Wexner Score

Table 5.1 Cleveland Clinic Incontinence Score (CCIS)a

Air Liquid stool Formed stool Using incontinence pads

Occasionally 1 4 7 1
>1/week 2 5 8 2
Daily 3 6 9 3
a
Incontinence index (IC): 0 perfect continence, 1–7 good continence, 8–14 moderate
incontinence, 15–20 severe incontinence, >20 complete incontinence

Epidemiology
– Prevalence = up to 3% of the population (depending on the study); up to 30% of
patients in nursing homes
– Prevalence increases with age
– Women more frequently affected overall

Etiology
Pathophysiology
– Mostly multifactorial
– For example, delivery lesion (defect) at a young age → atrophy of the sphincter
with age → constipation tendency → prolapse formation with chronic nerve
damage → combined incontinence
Risk Factors
– Congenital (anomaly, spina bifida, Hirschsprung’s disease)
– Neurological (e.g. multiple sclerosis, Parkinson’s disease)
– Autonomic nervous system (diabetes mellitus)
– Gastrointestinal diseases
– Tumors
– Procedures in the pelvis: low anterior rectal resection, irradiation
– Sphincter injuries (delivery, trauma, surgery)
– Rectal voiding disorders (overflow incontinence, prolapse)
– Increasing age
Symptoms
– Uncontrolled continence (air and stool)
– Stool smearing (“soiling”)
– Often anal eczema
Diagnosis
– Anamnesis: Essential!
– Inspection
– Eczema, stool stains on the anus?
– Let the patient push: Prolapse?
– Palpation
– Tonus
 – Palpable dent
– Rectocele, prolapse?
– Procto/rectoscopy
– Tumor exclusion
– Changes in the anal canal, internal prolapse?
– Holding tests
– If the clysma/enema can be held for minutes without any problems, the
diagnosis must be questioned
– Endosonography: sphincter damage?
– Manometry
– Colonoscopy
– In the presence of inflammatory bowel disease
– To exclude tumor disease
– In individual cases: MRI, (MR) defecography, neurological examination

Therapy
Conservative Therapy
– Use of aids (e.g. anal tampons, pads)
– Pelvic floor training
– Biofeedback Training
– Electrostimulation
– Tibial nerve stimulation
– Medicinal stool regulation
– Anal irrigation
Surgical Therapy
– Sphincter reconstruction:
– Butt-on-put or overlapping suture of a (fresh) sphincter defect
– Also possible for older defects
– Result: Initially mostly good, in the course of the years = often deterioration of
continence performance (especially with concomitant neurogenic disorder)
– Disadvantages: Relatively high rate of postoperative complications (wound
healing disorder, suture dehiscence) = deterioration of preoperative continence
performance
– Muscle tightening (“pre-/post-anal repair”, “total pelvic floor repair”):
– Constriction of the anal canal without severing the muscle
– Result: Initial success rates = 60–80%; in the 5-year course = approx. 25%
– Sacral nerve modulation (SNM) formerly sacral nerve stimulation (SNS):
– Principle: Peripheral stimulation of the sacral spinal nerves; stimulation takes
place far away from the target organ = iatrogenic damage to the sphincter is
therefore excluded
– Indication now also extended to higher grade sphincter defects
– Result: Success rate = 58% over the longer term
– 2 surgical steps: PNE (percutaneous nerve evaluation) testing and permanent
sacral nerve modulation (SNM, SNS); high predictive value (90%) regarding
therapy success due to test stimulation
– Postoperative: 2-week phase with external stimulation, then 2-week phase
without stimulation
– Keeping a stool diary by the patient
– If symptoms improve >50% in the stimulation phase = indication for
permanent stimulation

Surgical Procedure
PNE Testing and SNM
– General anaesthesia (no muscle relaxation medication), for patients insensitive
to pain also local anaesthesia
– Prone position
– Single-shot antibiosis with cephalosporin
– Marking of the sacral foramina S2–S4 under fluoroscopy or using anatomical
landmarks
– Puncturing the foramina at a 60-degree angle with a needle electrode
– Contraction of the sphincter after application of current to the electrode
indicates correct position
– Selection of the ideal foramen (uniform contraction, low threshold, low
involvement of the lower extremity)
– Insertion of the foramen electrode using the modified Seldinger technique
– Subcutaneous placement of the foramen electrode, position control, connection
with stimulator

Surgical Procedure
If Indication for Permanent Stimulation
– Complete removal of the percutaneous extension
– Creation of a pacemaker pocket gluteal outside the seating area
– Attaching and connecting the generator aggregate
– Sinking of the aggregate
– Skin closure
 – Dynamic Graciloplasty (gracilis muscle transposition):
– Principle: The pedunculated, mobilized gracilis muscle is wrapped around
the anal canal; permanent stimulation via neurostimulator = long-term
transformation of type 2 fibers into enduring type 1 fibers
– Result: Continence rate = 50–83%.
– Demanding, complex operation, complication rate = 50%
– Artificial sphincter:
– Principle: Closure sleeve around the anal canal; control pump in the scrotum
or labium
– Result: High continence rate (up to 95% for solid and liquid stools)
– Demanding procedure with high complication rate > 50% (infections)
– Magnetic sphincter:
– Principle: Band with several magnets, which is placed around the anal canal,
similar to a bracelet; when at rest the magnets stick together = closure of the
anal canal; when pressed = the magnets move apart = opening of the anal
canal.
– Relatively new procedure, conclusive studies are lacking
– Definitive stoma placement:
– If all conservative and surgical measures fail
– If the patient suffers psychological stress: stoma (= controlled incontinence)
= significant improvement in quality of life
– Other procedures:
– “bulking agents”: ultrasound-guided instillation of silicone or similar into
the intersphincteric space
Questionable indication in case of minor symptoms and limited defect

Complications
– Social isolation, depression with delayed diagnosis
– Recurrence
– Wound healing disorder
– Implant infections with consecutive removal
– Rectal perforation, rectal necrosis, sepsis…
– Allergic reactions (“bulking agents”)
– Deterioration of continence performance due to surgery

5.2.8 Anorectal Voiding Dysfunction (Outlet Constipation)

 Key Points
– Frequent clinical picture with a high number of unreported cases
– Often multifactorial
– Therapy usually conservative at first or, if clearly possible, elimination of the
underlying cause
– As a rule, combination therapy is necessary after surgical removal of the cause
(stool regulation, nutritional counseling, exercise, etc.)
– Difference Outlet-Obstipation vs. Slow-Transit-Obstipation

Definition
Definition
– Chronic constipation = if at least 2 of the following criteria are present in 3
months of the last 12 months (ROM III criteria):
– Strong pressing
– Hard stool
– sensation of incomplete emptying
– Subjective obstruction
– Manual support for defecation in >25% of defecations
– <3 stools per week
Classification
– In scores possible (e.g. Cleveland Clinic Score for chronic constipation; ◘ Table
5.2)

Table 5.2 Cleveland clinic constipation scorea

Bowel Difficulty: Completeness: Pain: Time: Aid: Failure: History: Points

movement painful feeling of abdominal minutes type of unsuccessful duration of
frequency emptying incomplete pain of toilet aid emptying constipation
emptying use per attempts (years)
trial per 24 h
1–2 Never Never Never Less Without Never 0 0
times/day than 5 help
2 Rarely Rarely Rarely 5–10 Stimulant 1–3 1–5 1
times/week laxatives
1 Sometimes Sometimes Sometimes 10–20 Digital 3–6 5–10 2
time/week aid or
enema
Less than 1 Mostly Mostly Mostly 20–30 6–9 10–20 3
time/week
Less than 1 Always Always Always More More than 9 More than 4
time/month than 30 20
a
Minimum score = 0, maximum score = 30, constipation = score > 15
Epidemiology (Of All Forms of Constipation)
– Incidence: increasing with age, 30% of those over 60 years of age
– Men: Women = 1: 3
Etiology
– Pelvic floor dyssynergy
– Anismus: involuntary, spontaneous contraction
– Symptomatic rectocele
– Intussusception: invagination of excess rectum
– Compression of the rectum due to enterocele/sigmoidocele
– Anal Stenosis
– Anal Fissure
– stenosing tumor
Symptoms
– Strong pressing during defecation
– sensation of incomplete emptying
– Fractionated emptying of small amounts of stool
– Digital clearing out
– Dull pressure pain in the pelvis
Diagnosis
– Anamnesis
– Concomitant diseases
– In particular, keeping a stool diary
– Inspection
– Palpation
– Sphincter tone
– Rectocele, prolapse
– Stenosing process
– Dyssynergy
– Procto/rectoscopy
– Tumor exclusion
– Changes in the anal canal
– Internal prolapse
– Manometry not obligatory
– Balloon Expulsion Test: Ability of the patient to evacuate a water-filled balloon
inserted into the rectum.
– Colonoscopy: In case of suspected inflammatory bowel disease/tumour disease
– Hinton test: differentiation from slow transit obtipation
– (MR) Defecography:
– (Internal) prolapse
– Rectocele, enterocele
– Intussusception
– Pelvic floor dyssynergy
– In individual cases: MRI, CT, neurological examination

Therapy
Surgical Therapy
– Depends on underlying cause
– Pelvic floor dyssynergy:
– Biofeedback Training
– Pelvic floor exercises under professional guidance
– Anismus (involuntary, spontaneous contraction):
– Biofeedback Training
– Botulinum toxin injection
– Symptomatic rectocele:
– Posterior colporrhaphy
– Transanal shirring with mucosal resection

Caution
Rectoceles = also frequent in healthy women, only in 10–20% of the obstipated
patients the symptoms are due to it

– Intussusception (invagination of rectum excess):

– STARR surgery (“stapled trans anal rectal resection”): Circular transanal rectal
full wall resection = circular anastomosis + “straightening” of the rectum
– Transtar® surgery: larger resection possible (► Sect. 4.​2.​2 Rectal prolapse)
The term ODS (obstructive defecation syndrome) is often used synonymously
for the symptom triad intussusception, rectocele and voiding dysfunction.
– Compression of the rectum by enterocele/sigmoidocele:
– Elevation of the pelvic floor + (resection) rectopexy
– Anal stenosis: surgical correction
– Anal fissure: ► Sect. 5.2.3
– Stenosing tumor: Depending on the dignity
– Resection
– Stoma creation, if necessary radiochemotherapy
Complications
– Depending on the chosen therapy method

5.3 Malignant Disease: Anal Carcinoma

Key Points
– Anal canal carcinoma and anal margin carcinoma
– Anal canal carcinoma:
– Surgical therapy of anal canal carcinoma limited to very small
findings/recurrences
– Standard = Radiochemotherapy
– Anal marginal carcinomas (therapy analogous to skin tumors): Primary surgical
treatment

5.3.1 Definition
Anal Carcinoma
– Anal canal = from anocutaneous line to anorectal junction (anoderm + transitional
zone)
– Histology:
– Unkeratinized squamous epithelium 85%
– Remainder = adenocarcinomas

Classification: TNM Classification

– T (tumor)
– T1 Tumor <2 cm
– T2c Tumor >2 cm < 5 cm
– T3 Tumor >5 cm
– T4 Infiltration of adjacent organs
– N (lymph nodes)
– N0 No regional lymph node metastases
 – N1 Metastases in regional lymph nodes
– N2 Metastases unilateral inguinal or iliacal LN
– N3 Metastases bilaterally perirectal, inguinal or iliacal LN
– M (metastases)
– M0 No distant metastases
– M1 distant metastases present

Anal Margin Carcinoma

– Anal margin = from linea anocutanea to 5 cm distal thereof
– Histology: keratinizing squamous epithelium

5.3.2 Epidemiology
– Incidence = 0.5–1.5/100,000 population per year
– Men: Women = 1: 1.5
– Peak incidence: 50–70 years of age

5.3.3 Aetiology
– Known risk factors:
– Infections with human papilloma virus (HPV)
– HIV infections
– Immunosupression
– Anal sex
– Long-term therapy with corticosteroids
– Smoking

5.3.4 Symptomatology
– Blood accumulation, mucous discharges
– Pain
– Itchy skin
– Stool irregularities
– Continence disorders
– Enlarged inguinal lymph nodes

5.3.5 Diagnosis
– Clinical examination
– Proctoscopy, rectoscopy, endosonography
– Colonoscopy: To exclude a second carcinoma
– Sonography/MRI pelvis + groin (lymph node metastases?)
– Exclusion of distant metastases
– Biopsy, in the case of small findings also excision biopsy (= simultaneously
therapeutic!)

5.3.6 Differential Diagnosis

– Hemorrhoids, Mariscs
– Anal fistula, anal fissure
– Anal Fibroids
– Rectal Cancer
– Melanoma, anal marginal carcinoma, basal cell carcinoma, fistula carcinoma
– Dermatological diseases

5.3.7 Therapy
Conservative Therapy
– Combined radiochemotherapy = therapy of choice for advanced findings
– External radiation including the inguinal lymph nodes
– Total dose: Up to 60 Gy
– 5-FU (fluorouracil) continuous infusion: weeks 1 and 5
– Mitomycin as radiosensitizer
– 5-year survival depending on stage = up to 80%

Surgical Therapy
– Anal canal carcinoma:
– In case of findings <1 cm after exclusion of sphincter infiltration and lymph
node metastases and in case of good differentiation = complete excision with
safety margin possible
– In case of recurrence/contraindication to radiotherapy = cylindrical
abdominoperineal rectum extirpation, if necessary as multivisceral resection.
– In case of stenosing tumor = protective stoma creation before planned
radiotherapy
– Anal margin carcinoma:
– Therapy of anal margin carcinoma = therapy of skin tumours
– Radical excision with a safety margin of 1 cm
– In case of lymph node involvement: lymph node dissection
– Radiatiotherapy if necessary
– Photodynamic therapy if necessary

Complications
 – After radiotherapy:
– Incontinence
– (Refractory) Proctitis
– Diarrhea
– Recurrence
– Post-op:
– Wound healing disorders
– Bladder emptying disorders
– Impotence
– Stoma problems (prolapse, parastomal hernia, stenosis)
– Recurrence

5.4 Guidelines
German Society for Neurogastroenterology, German Society for Digestive and
Metabolic Diseases, DGIM, CACP, DGAV, DGK, Deutsche Reizdarmselbsthilfe e.V.
(2013) S2k-Leitlinie Chronische Obstipation: Definition, Pathophysiologie,
Diagnostik und Therapie. AWMF registration number 021/019, AWMF online: ►
http://​www.​awmf.​org/​uploads/​tx_​szleitlinien/​021-019l_​S2k_​Chronische_​
Obstipation_​2013-06_​01.​pdf
German Society for Coloproctology, BCD, DGAV, CACP, DGVS, DDG
(6/2020) S3 Guideline: Sinus pilonidalis. AWMF registration number 081/009,
AWMF online: ► http://​www.​awmf.​org/​leitlinien/​detail/​ll/​081-009.​html
Ommer A, Herold A, Berg E, Farke S, Fürst A, Hetzer F, Köhler A, Post S,
Ruppert R, Sailer M, Schiedeck T, Strittmatter B, Lenhard BH, Bader W, Gschwend
JE, Krammer H, Stange E (2011) S3 guideline: cryptoglandular anal fistulas.
Coloproctol 33:295–324
Ommer A, Herold A, Berg E, Farke S, Fürst A, Hetzer F, Köhler A, Post S,
Ruppert R, Sailer M, Schiedeck T, Strittmatter B, Lenhard BH, Bader W, Gschwend
JE, Krammer H, Stange H (2011) S3 guideline: anal abscess. Coloproctol 33:378–
392

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incontinence: is initial success sustained over time? Color Dis 15:1499–1503
[Crossref]
Bock S, Wolff K, Marti L, Schmied BM, Hetzer FH (2013) Long-term outcome after transanal rectal resection in
patients with obstructed defecation syndrom. Dis Colon Rectum 56:246–252
[Crossref][PubMed]

Gingert C, Hetzer FH (2014) Stuhlinkontinenz. Coloproctol 36:125–137

[Crossref]

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Kohortenstudie. Chirurg 75:160–167
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Heitland W (2012) Perianale Fistel und Analfissur. Chirurg 83:1033–1039

[Crossref][PubMed]

Herold A, Joos A, Bussen D (2012) Operationen beim Hämorrhoidalleiden. Chirurg 83:1040–1048

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Hirschburger M, Schwandner T, Hecker A, Kierer W, Weinel R, Padberg W (2014) Fistulectomy with primary
sphincter reconstruction in the treatment of high transsphincteric anal fistulas. Int J Color Dis 29:247–252
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Joos AK, Herold A (2010) Hämorrhoidalleiden. Gastroenterologe 5:326–335

[Crossref]

Karakayali F, Karagulle E, Karabulut Z, Oksuz E, Moray G, Haberal M (2009) Unroofing and marsupialization
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[Crossref]

Milligan ET, Morgan CN, Lond LE (1937) Surgical anatomy of the anal canal, and the operation treatement of
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[Crossref]

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[Crossref]

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for obstructed defecation: results of the German STARR registry. Langenbecks Arch Surg 395:505–513
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Stelzner F (1984) Die Ursache des Pilonidalsinus und der Pyodermia fistulans sinifica. Langenbecks Arch Chir
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plug for anal fistula of cryptoglandular origin. Color Dis 15:1510–1514
[Crossref]

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review of the clinical effectiveness of neuromodulation in the treatement of faecal incontinence. Br J Surg
100:1430–1447
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[Crossref]

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OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_6

6. Endocrine Organs
Franck Billmann1 , Courtney Elisabeth Gibson2 and Robert Udelsman3
(1) Department of General, Visceral and Transplant Surgery, University Hospital
Heidelberg, Heidelberg, Germany
(2) Department of Endocrine Surgery, Smilow Cancer Hospital at Yale, New
Haven, CT, USA
(3) Miami Cancer Institute, Miami, FL, USA

Franck Billmann (Corresponding author)

Email: [email protected]

Courtney Elisabeth Gibson

Email: [email protected]

Robert Udelsman
Email: [email protected]

6.1 Anatomy and Physiology of the Thyroid Gland

F. Billmann

Key Points
Thyroid gland (Thyroid):
– Cervical endocrine gland (normal weight 15–20 g)
– Blood supply via 4 anastomosing arteries
– Closely related to: recurrent laryngeal nerve, parathyroid glands
– Regulatory circuit: TRH (thyrotropin-releasing hormone)—TSH (thyroid-
stimulating hormone)—T3 (triiodothyronine), T4 (thyroxine)—TRH/TSH

6.1.1 Embryology and Anatomy

– Large cervical endocrine gland
Macroscopy
– Two lateral lobes connected by isthmus
– Pyramidal lobe: Cranial process from isthmus; inconstant (50% of cases)
– Total adult weight = 15–20 g

Microscopy
– Thyroid follicle = structural and functional unit
– follicular epithelium = source of thyroid hormones
– C cells: Release of calcitonin (= regulation of the blood calcium level; ► Sect.
6.1.2)

Localization
– Isthmus at the level of the 2nd–4th tracheal cartilage
– Lateral lobes adjacent to the cricoid and thyroid cartilage

Blood Supply
– 4 arteries + inconstant vessels
– Superior thyroid artery: Paired, from external carotid artery
– Inferior thyroid artery: Paired, from thyrocervical trunk (subclavian artery)
– A. thyreoidea ima: Unpaired from aorta or truncus brachiocephalicus;
inconsistent
– Venous drainage: Variable; drainage into internal jugular vein + brachiocephalic
vein

Surgical-Relevant Anatomical Relationships

– Superior laryngeal nerve: from the inferior ganglion of the vagus nerve;
divides at the level of the hyoid bone into the external branch (pharyngeal
musculature) and internal branche (plica vocalis)
– Recurren laryngeal nerve: branch of the vagus nerve; course between trachea
and esophagus, directly behind the thyroid gland; division into anterior
branche (for Mm. vocalis) and posterior branche
– Parathyroid glands: dorsal to the thyroid gland; variable location (► Sect.
6.4)
– Lymph node groups (◘ Table 6.1 and ◘ Fig. 6.1)
Table 6.1 Classifications of locoregional thyroid lymph nodes (LN)
Compartment US classification UICC classification Japanese
classification (Robbins et al. 2008) (Wittekind et al. 2003) classification
(Dralle et al. 1994) (Qubain et al.
2002)
Compartment 1 Without side assignment: Without side assignment: LN Without side
(1a cervicocentral right, Level 1 (submental, groups 1 and 2 (submental, assignment:
1b cervicocentral left) submandibular), Level 6 submandibular), and 8 Regional LN
(central), Level 7 (central (central) groups 1–4
caudal)
Compartment 2 Without side assignment: Without side assignment: LK Without side
(cervicolateral right) Level 2A, 2B (cranial groups 2, 3 (cranial jugular), assignment:
Compartment 3 jugular), Level 4 (caudal 4 (middle jugular), 5 (caudal Regional LN
(cervicolateral left) jugular), Level 5A, 5B jugular), 6 (dorsal lateral), groups 5–7
(lateral jugular) and 7 (lateral
supraclavicular).
Compartment 4 (4a upper – – –
infrabrachiocephalic
mediastinum right, 4b
left)
Fig. 6.1 a–d Classifications of locoregional thyroid lymph nodes. Comparison between a compartment
classification according to Dralle, b US classification according to Robbins, c UICC/TNM classification
and d Japanese classification
Development
– During development, displacement of thyroid caudally with formation of
thyroglossal duct.
– regression of the thyroglossal duct in the course (possible peristence =
pyramidal lobe)

6.1.2 Physiology
Thyroid Hormones (Thyroxine, T4, and Triiodothyronine, T3)
– Production and release by follicular epithelial cells
– Active form = T3
– Function (on almost all body cells):
– Metabolism increase
– Important role in growth + development of the nervous system

Control Loop (Negative Feedback)

– Goal = accurate control of T3, T4
– Control of thyroid by hypothalamus + hypophysis (pituitary gland): mediators
(TRF and TSH)

Calcitonin
– Produced and released by C cells
– Physiological antagonist of parathyroid hormone (PTH; ► Sect. 6.4
Parathyroid)
– Calcium regulation

6.2 Diseases of the Thyroid Gland

F. Billmann

6.2.1 Epidemiology
Goiter and Multinodular Goiter
– Prevalence: 33.1% of the working population
– Incidence of thyroid nodules:
– 23.2% of patients with goiter (approx. 20 million people in Germany)
– Age-dependent
Hyperthyroidism (Thyroid Autonomy)
– Prevalence: subclinical hyperthyroidism = 1.8%, manifest hyperthyroidism =
0.4%.
– Etiology:
– Graves’ disease: leading cause in regions without iodine deficiency
– toxic nodular goiter/toxic adenoma: In regions with iodine deficiency
– Iodine-induced hyperthyroidism (acute high iodine intake): Increasingly rare
– Amiodarone (high iodine) induced hyperthyroidism: 30–40% of amiodarone
treated patients

Autoimmune Diseases
– Prevalence: No precise data
– possibly associated with autoimmune diseases of other organs
– Autoimmune thyroiditis: women:men = 10:1
– Graves’ disease:
– 5–20 cases/100,000 inhabitants per year
– Women:Men = 6–8:1

Thyroid Cancer
– Most common endocrine tumor
– Prevalence: men = 4/100,000 per year; women = 8.7/100,000 per year
– Women:Men = 2:1
– Germany: 5000 new cases/year
– Mortality declining (by 40% in recent years)

6.2.2 General Methods of Investigation

Key Points
– Standard examinations: clinical examination + laboratory thyroid function
tests (TSH) + ultrasound (sonography)
– FNA (fine needle aspiration cytology): To exclude malignancy
– Scintgraphy, CT, MRT: Only for special questions

Clinical Examination
– Medical history: signs of hyper- or hypothyroidism (◘ Table 6.2)
– Inspection, palpation (position, size, consistency, swallowing displacement of
the thyroid), auscultation (blood flow)
– Exclusion: globus sensation, voice change, stridor, dysphagia.
– Search for cervical lymph nodes
Table 6.2 Comparison of symptoms of hyperthyroidism and hypothyroidism

Symptoms Hyperthyroidism Hypothyroidism

General Rapid fatigue Fatigue
Anorexia
Cycle Irregularity
Weight loss Weight gain
Weakness Muscle weakness
Heat intolerance Hypothermia
Increase appetite Hypercholesterolemia
Cardiovascular Tachycardia Bradycardia
Hypotension
Neurological Nervousness/restlessness
Insomnia
Hyperreflexia Hyporeflexia
Tremor
Apathy Adynamics
Depression Depression
Confusion
Deep hoarse voice
Gastrointestinal Diarrhea
Polydipsia
Constipation Constipation
Pulmonology Dyspnea
Musculoskeletal Muscular atrophy
Muscle cramps
Dermatological Sweating Hair loss
Facial Edema
Pale yellowish skin coloration

Laboratory Thyroid Function Tests

Basic Diagnosis (For Each Patient)
– TSH: Examination of thyroid function (see below: Overview of normal levels)
– Euthyroid
 – Hyperthyroidism (subclinical/manifest)
– Hypothyroidism (subclinical/manifest)
Specific Diagnosis (For Further Clarification)
– Free thyroid hormones (fT3, fT4)
– TRH test
– Thyroglobulin (Tg)
– Antibodies (in autoimmune thyroiditis): TRAb (TSH receptor autoantibodies),
TgAb (thyroglobulin antibodies), TPO-MAb (thyroid peroxidase antibodies,
microsomal antibodies)
– Calcitonin

Normal Levels of Thyroid Metabolism

– Serum TSH = 0.3–6 mU/L
– Serum fT3 = 3–9 pmol/L (2–6 ng/L)
– Serum fT4 = 9–29 pmol/L (7–23 ng/L)
– Serum calcitonin <2.8 pmol/L (<10 ng/dL)

Imaging Studies
Ultrasound
– Orienting study
– High resolution linear transducers (7.5–18 MHz)
– Display of nodules from 0.5–1 mm
– Special Techniques:
– Color-coded duplex sonography: vascularization of the thyroid nodules
– Elastography: degree of hardness of the thyroid/a nodule
– Contrast-enhanced sonography (under evaluation)
– “Acoustic Radiation Force Impulse-Imaging” (under evaluation)

Imaging Studies
– Ultrasound = key examination
– Cross-sectional imaging (CT/MRT) = environmental diagnosis + therapy
planning
– CT, MRI, nuclear medicine procedures: Only for further clarification

Computer Tomography (CT)

– Assessment of the thyroid environment (trachea, esophagus, vessels)
– Good retrosternal/intrathoracic assessment
– Detection of metastases (lymph nodes from 1 cm, lung +++)

Contrast Medium Containing Iodine

– In case of manifest autonomy/hyperthyroidism only approved in case of vital
indication
– Radioiodine therapy through this administration for several months impossible
Magnetic Resonance Imaging (MRI)
– Mostly for surgery planning
– Good sensitivity with regard to compression/infiltration of adjacent structures
– Detection of abnormal lymph nodes
– Postoperative tumor follow-up: differentiation of scar vs. tumor recurrence
Nuclear Medicine Diagnosis
– Use of radioactively labelled substances (involved in the metabolism of
Thyroid) (99mTc-pertechnetate scintigraphy)
– Exploration of the function of the thyroid/nodules (semiquantitative)
– Relatively poor image resolution
– Differentiation between cold and hot nodules (possible causes: ◘ Table 6.3)
– Specific investigations: for specific questions
– 131I-scintigraphy (whole body scintigraphy)
– 18F-fluorodeoxyglucose positron emission tomography (18 F-FDG-PET)
(whole-body tomography, possibly CT-coupled)
124
– I-Positron Emission Tomography
– “Medullary Thyroid Carcinoma” section: 111 indium-pentetreotide; 99 Tc-
Tyr3-octreotide scintigraphy; 68 Ga-DOTATOC; 68Ga-DOTATATE-PET; 18F-
DOPA-PET; 18F-FDG-PET.
Table 6.3 Possible causes of cold and hot nodules

Cold nodules Hot nodules

Cancer Compensated toxic thyroid adenoma
Cyst Decompensated toxic thyroid adenoma
Hemorrhage
Nonstoring adenoma
Regressive change
Focal inflammations
– Well-differentiated iodine-storing metastases = usually only low FDG
enrichment
– De-differentiated metastases no longer storing iodine = intensive FDG
enrichment

Fine Needle Aspiration Cytology (FNA)

– Objective = selection of lesions suspicious of malignancy to avoid unnecessary
surgery
– Accuracy: Depending on the experience of the examiner/pathologist
– Inexpensive, easy to perform, low complications
Principle
– Contraindication = haemorrhagic diathesis
– Local anesthesia
– Puncture with disposable cannula (25–23 G)
– Ultrasonic control, monitoring
– Transfer of the punctate onto slides, air drying, staining
Interpretation of the Cytological Findings
– Quality control
– Interpretation: current consensus between Italian/UK Royal College of
Pathologists/Bethesda classifications (◘ Table 6.4)
– Diagnostic reliability (see malignancy rate ◘ Table 6.4)
Table 6.4 Interpretation of fine needle aspiration cytology (FNA; comparison (consensus) Italian/UK Royal
College of Pathologists/Bethesda classifications)

Italian Bethesda UK Royal Cytology category Malignancy Therapy

system System College of risk recommendation
Pathologists
System
TIR 1 I Thy 1 Non-diagnostic or 1–4% Repeat FNA under
insufficient test sonographic controlc
materiala
TIR 1C Ic Thy 1c Non-diagnostic with dependent on Repeat FNA under
cystic fluid clinical sonographic controlc
presentation
TIR 2 II Thy 2/Thy 2c Benign 0–3% Annual control: clinical
examination+TSH +
sonography
Renewed FNA if size
progression
Italian Bethesda UK Royal Cytology category Malignancy Therapy
system System College of risk recommendation
Pathologists
System
TIR 3A III Thy 3a Atypia of undetermined 5–15% Repeat FNA (3–6
significance or months)
follicular lesion of Surgery for persistent
undetermined A/FLUS, with frozen
significanceb (A/FLUS) section examination
TIR 3B IV Thy 3f Follicular or Hurtle cell 15–30% Surgery due to high risk
neoplasiab of malignancy
Frozen section no further
benefit
TIR 4 V Thy 4 Suspicious for 60–75% Surgery due to high risk
Malignancyb of malignancy with
frozen section
TIR 5 VI Thy 5 Malignant 97–99% Surgery with definitive
thyroidectomy
Preoperative sonography
to exclude lymph node
metastases (neck
dissection)

a
Non-diagnostic or inadequate if quality criteria not met: At least 6 groups, each
group with at least 10 follicular cells, at least 2 aspirates for each nodule examined
b
Categories III, IV and V are collectively referred to as “intermediate” and require
repeat FNA (III) or surgical exploration (IV and V)
c
After renewed non-diagnostic FNA, surgery should be performed (risk of
malignancy = 8%)
Diagnostic Strategy
– Geographic variation in the prevalence of thyroid nodules = different strategies
– USA: FNA = primary diagnostic procedure (with clinical examination and
sonography)
– Germany: FNA = additional method as part of a differentiated approach
– Clinical history-oriented procedure (► Sect. 6.2.6 Workup of a solitary thyroid
nodule)

6.2.3 Basics of Surgical Therapy, Complications and Postoperative

Care
Preoperative Measures
– Control/achievement of a euthyroid metabolic state
– Laboratory chemistry parameters: Blood count, electrolytes, coagulation,
eventually blood type and Packed red blood cells (PRBCs) if large retrosternal
goitre
– Calcitonin: For the early detection of medullary thyroid cancer
– Cervical ultrasound: complementary to the clinical examination
– FNA: For suspicious nodules >1.0 cm
– Preoperative laryngoscopy: examination of vocal cord function
– Imaging procedures for mechanical impairments (CT, MRI)
– chest X-ray
– Patient education
– Marking of the skin incision directly preoperatively

Basics of Surgical Therapy

Key Points
– Gold standard = open hemithyroidectomy/thyroidectomy (◘ Table 6.5)
– Caution:
– Protection of the parathyroid glands
– Visualisation of the recurrent laryngeal nerve (= necessity of a dry
operation field) to avoid injury
– Minimally invasive techniques: Cosmetic benefits only
– Lymphadenectomy: Compartmental/regional only
– Postoperative Complications (informed consent):
– Bleeding
– Recurrent laryngeal nerve lesion
– Hypoparathyroidism
– Rarely thyrotoxic crisis, tracheomalacia
– Quality Criteria: Magnifying loupes/microsurgical technique.
Table 6.5 Clinical factors in favour of/against initial total thyroidectomy
In favor of total thyroidectomy 1. Planned radioiodine therapy due to known (or suspicion of)
differentiated thyroid cancer:
a. Malignant FNA with lesion >4 cm
b. Relevant extrathyroidal extension on US or intraop.
c. Clinical, intraop. or ultrasound signs of LN metastases
d. Known distant metastases
e. Abnormal result of the molecular examination
2. Medullary thyroid cancer
3. Bilateral thyroid disease:
a. Euthyroid/toxic goiter
b. Graves’ disease
c. Contralateral dominant nodule
d. Radiotherapy in anamnesis
e. Familial predisposition syndrome
f. Indication of contralateral parathyroidectomy
4. Struma ovarii
Controversial/no consensus 1. Known or suspected unilateral differentiated thyroid cancer 1–
4 cm with low-risk signs on ultrasound
2. Index lesion under known thyroid hormone therapy
3. Unilateral differentiated thyroid cancer with need for
Tg/ultrasound surveillance
4. Unilateral lesion in complex medical situation
5. Unilateral lesion and patient preference for total thyroidectomy.
In favor of hemithyroidectomy with 1. Unilateral papillary thyroid microcarcinoma low-risk on
isthmus resection ultrasound
2. Unilateral lesion with inconspicuous molecular examination
3. Unilateral goiter

Open Surgical Technique (= Procedure of Choice)

– Obligatory compromise: finding-oriented/preservation of
function/minimization of complications (recurrent laryngeal nerve,
parathyroid glands)
– Standard technique = extracapsular lobectomy with isthmus resection
– Subtotal resection = inadequate (higher risk of injury to the recurrent
laryngeal nerve and parathyroid glands).
– Ideal: Operation in centres with adequate expertise
– Rules:
– Good exposure (= excellent visualization of the recurrent laryngeal nerve
+ parathyroid glands)
 – Systematic identification of the anatomical structures + careful dissection
– Preoperative informed consent (operation, alternative procedures, possible
complications)
– Preoperative confirmation of the euthyroid metabolic state

Surgical Procedure
Open Hemithyroidectomy/Thyroidectomy
– General anaesthesia (only rarely locoregional anaesthesia possible)
– Cervical spine extension, roll or vacuum mattress under the shoulders
– Access: 4–5 cm Kocher collar incision, in skin fold approx. 1 finger width
above the jugulum (preoperative marking)
– Transection of the platysma muscle, formation of a subplatysmal flap which
is retracted cranially (holding suture)
– Incision of the linea alba, lateral retraction of the strap muscles (infrahyoid
muscles)
– Preparation only on the side of the nodule(s)
– Neuromonitoring of the vagus nerve before resection
– Finding the right plane on the thyroid capsule (crucial)
– Dissection of the upper thyroid pole; visualization of the upper pole vessels:
transection + ligation (close to the capsule); mobilization of the upper pole
– Mobilization to lateral + caudal; transection of the Kocher veins;
mobilization of the lower pole
– Medial retraction of the thyroid and central preparation
– Exposure of the inferior thyroid artery and the recurrent laryngeal nerve;
neuromonitoring prior to resection; exposure of the inferior thyroid artery
(close to thyroid capsule)
– Identification of both parathyroid glands and assessment of blood flow; if
insufficient blood flow: parathyroidectomy and autotransplantation into the
sternocleidomastoid muscle
– Complete mobilization of the SD lobe while sparing the recurrent laryngeal
nerve
– Subtle hemostasis; close to the nerve, PDS (polydioxanone)-6/0 sutures (no
electrocoagulation)
– Final neuromonitoring of the recurrent laryngeal nerve and the vagus nerve
after resection
– Thyroidectomy: Only justified if neuromonitoring is unremarkable on the
primary side; analogous procedure on the opposite side
 – Suture of the strap muscles; suture of the platysma muscle; continuous
subcutaneous suture; skin closure (suture, glue)

Minimally Invasive Surgical Techniques

– Only in centres with adequate expertise
– Purely aesthetic benefits (evidence-based)
– New complications (vascular injury, nerve injury) = critical use of these
techniques.
– 3 groups of procedures:
– Purely endoscopic procedures (collar, prethoracic, axillary, perimammillary
or supramandibular approach)
– Open video-assisted procedures (MIVAT)
– Open procedures with minimum incision length
– Indications, relative and absolute contraindications (◘ Table 6.6)
Table 6.6 Indications, relative and absolute contraindications for MI (minimally invasive) techniques

Indications Absolute contraindications Relative contraindications

Nodule, diameter < 3 cm History of cervical surgery History of neck irradiation
Thyroid volume < 20 mL Large goiter Hyperthyroidism
Benign nodule Locally advanced cancer Thyroiditis
Low-risk papillary carcinoma Lymph node metastases

Surgical Procedure
Minimally Invasive Video-Assisted Thyroidectomy (MIVAT) According to
Miccoli
– General anesthesia
– No hyperextension of the cervical spine
– Access: 1–2 cm transverse skin incision, in jugular fossa (preoperative
marking)
– transection of the platysma muscle and formation of a subplatysmal flap
– Incision of the linea alba (3 cm) and retraction of the strap muscles to the
lateral side, medial retraction of the thyroid
– After exposure of the thyroid: Further preparation videoscopically-assisted
(30°, 5-mm endoscope)
– Mobilization and resection of the thyroid: Following the rules of open
surgery
 – supply of the vessels: ligation impossible, therefore electrosurgery (bipolar
vessel sealing devices, ultrasound dissection devices) or stapling devices
– Conversion to open surgery always possible
– Continuous subcutaneous suture; skin closure (suture, glue)

Surgical Procedure
Complete Endoscopic Thyroidectomy According to Gagner
– General anesthesia
– Moderate extension of the cervical spine
– Access: 5 mm transverse neck incision, above the jugulum (preoperative
marking)
– Opening of the cervical fascia; preparation below the platysma
– Insertion of 5-mm trocar into the subplastysmal space; CO2 insufflation (10
mmHg)
– Dissection along the anteromedial border of the sternocleidomastoid muscle
(SCM), using a 0° endoscope via the 5 mm trocar; then use a 30° endoscope
as soon as sufficient space is created.
– 3 additional working trocars: 3-mm trocar on the midline, 3-mm trocar on
the ipsilateral SCM, 5-mm trocar on the anterior border of the SCM
– Opening of the linea alba; retraction of the sternohyoideus and
sternothyroideus muscles medially
– Mobilization of the thyroid lobe
– Sealing/Transection of the Kocher veins (clip, ultrasound)
– Identification and dissection of the parathyroid glands and the laryngeal
recurrent nerve
– Identification and Sealing/Transection of the inferior thyroid artery (clip,
ultrasound)
– Isolation of the upper pole vessels and Sealing/Transection of the same (clip,
ultrasound)
– Isolation of the lower pole vessels and Sealing/Transection of the same (clip,
ultrasound)
– Transection of the Berry ligament and isthmus; extraction of the specimen
– Continuous subcutaneous suture; skin closure (suture, glue)

Cervical Lymphadenectomy (LAD) for Thyroid Cancer

Surgical Anatomy and Classification Systems
– ► Section 6.1, ◘ Fig. 6.1 and ◘ Table 6.1
Pathophysiology
– Lymphadenectomy for thyroid cancer (limited to the neck)
– = curative intervention: resection of the LN (lymph node) metastases
– = preventive intervention: prevention of LN metastases
– Lymphadenectomy: the most important curative treatment modality for
locoregional LN metastases
– Indication + extent = depending on tumor biology
– Tumour type (papillary, follicular, low-differentiated, undifferentiated,
medullary)
– Tumor extension (intrathyroidal vs. extrathyroidal; locoregional vs. distant
metastases)
– Currently no consensus due to lack of studies: prophylactic vs. therapeutic LAD
– Adjuvant therapy modalities (= no replacement of surgery):
– Radioiodine therapy (for differentiated thyroid cancers)
– External radiation (for undifferentiated cancers or locally advanced
differentiated cancers)
Sentinel Node Biopsy Technique
– Only in the context of studies
– Not routine use because of: High variability of lymphatic drainage/Frequent
multiple primary tumours.
Selective LAD (“Berry Picking”)
– Contraindicated
– In case of locoregional recurrence: after already performed compartment-
oriented LAD
Compartment-Oriented LAD
– Classification + definition of cervical lymph node dissections (◘ Table 6.7)
– Standard procedure for LN-positive thyroid cancer
– Surgical strategy: In case of preoperatively confirmed locally advanced thyroid
cancer:
– Centripedal tactics
– Centrifugal tactics
– Mediastinal LAD (sternotomy): Only in case of confirmed LN metastasis.
Table 6.7 Forms of cervical lymphadenectomies
Forms of neck Resection extension
dissection
Radical neck Removal of the lymph node groups 1–5 including the sternocleidomastoid muscle,
dissection internal jugular vein and vagus nerve
Modified radical Removal of lymph node groups 1–5 leaving at least one of the following structures:
neck dissection sternocleidomastoid muscle, internal jugular vein and vagus nerve
Selective neck Removal of groups of cervical lymph nodes, leaving at least one group intact.
dissection Classically, one distinguishes:
Central neck dissection: removal of the lymph nodes of groups 1a and 1b according
to Dralle (6 according to Robbins)
Lateral neck dissection: removal of the lymph nodes of groups 2 and 3 according to
Dralle (2–5 according to Robbins)
Extended neck The above resection procedures extended to include other groups of lymph nodes
dissection (deep mediastinal) or other structures (muscles or nerves)

Surgical Procedure
Central Lymph Node Dissection
– General anesthesia
– Resection Boundaries:
– Lateral = medial aspect of the carotid artery
– medial = trachea
– Central LAD always with thyroidectomy (ideally “en bloc”)
– Protection of the upper PG (parathyroid glands) extremely important
(possibly autotransplantation)

Surgical Procedure
Lateral Lymph Node Dissection
– General anaesthesia; following thyroid resection or as an independent
procedure
– sternocleidomastoid muscle, strap muscles pulled laterally dorsally
– transection of the omohyoid muscle
– Visualization of carotid artery, internal jugular vein, vagus nerve (vessel
loop)
– Exposure of hypoglossal nerve (= cranial dissection landmark and border)
– Exposure of the accessorius nerve in its course (important for its protection)
at the upper edge of the sternocleidomastoid muscle
– En bloc resection of the entire compartmental fibro-fatty tissue with LN
– Dorsal landmark and border = dorsal cervical fascia
 – Dorsal to note and spare: C3 to C7 fibers of the brachial plexus…
– Dorsal to note and spare: cervical sympathetic trunc (mediodorsal; avoid
Horner’s syndrome).
– Visualization of the subclavian vein dorsal to the clavicle (= caudal
dissection landmark and border)
– Caudomedial left: Protection of the thoracic duct…

Postoperative Complications
Bleeding Needing Revision
– Incidence = 0.3–5%
– Bleeding within a few hours to 24 h postoperatively
– Clinical signs: cervical swelling of the throat; dyspnoea; dysphagia
– Therapy: Immediate revision
Recurrent Laryngeal Nerve Lesion
– Temporary vs. permanent (passing after 6 months = permanent)
– Causes: transection, contusion, strain, electrocoagulation, pressure damage
(edema, hematoma)
– High rate of spontaneous remission of the paresis
– Unilateral lesion: often late diagnosis/unnoticed
– Clinical signs: Absence of glottis closure on coughing; discrete hoarseness;
deeper voice; usually only evident on postoperative laryngoscopy.
– Therapy: Speech therapy
– Bilateral lesion: Usually early and markedly symptomatic
– Clinical presentation: Stridor, dyspnea
– Therapy: In mild clinical condition: conservative (calcium, glucocorticoids,
if necessary NSAIDs, O2 administration); in case of respiratory impairment
(reintubation, if necessary translaryngeal laterofixation of the vocal cord, if
necessary tracheotomy).
Parathyroid Hypofunction (= Hypoparathyroidism; ◘ Fig. 6.6)
– Temporary vs. permanent (passing after 6 months = permanent)
– Prophylaxis: Intraoperative exposure/imaging of the parathyroid glands,
autotransplantation in case of reduced blood flow
– Clinical presentation, diagnosis and therapy (► Sect. 6.5.1)
Thyrotoxic Crisis
 – Rare; on the ground of hyperthyroidism.
– Life-threatening due to decompensation of the organism
– Diagnosis: Purely clinical (Akamizu criteria ◘ Table 6.8)
– Clinical presentation: (Classification according to severity: staging according to
Hermann ◘ Table 6.9)
– Prognosis: Overall lethality >15%; in stage 3 up to 30%
– Prophylaxis:
– Consistent preoperative preparation in patients with hyperthyroidism
(thyrostatic drugs, α/β-blockade if necessary) until euthyroidism is achieved.
– Avoidance of Iodin exposure
– Resection of all autonomies
– Therapy:
– Interdisciplinary intensive medical treatment
– Sedation + thyrostatic drugs + β-blockade + corticosteroids
– Symptomatic measures: Reduction of temperature, fluid and electrolyte
balance, high-calorie diet, possibly plasmapheresis
– In case of uncontrollable crisis: emergency thyroidectomy
Table 6.8 Akamizu criteria for the diagnosis of thyrotoxic crisis

Main Main criterion 2 Secondary criterion 1 Secondary criterion

criterion 1 2
Elevated fT4 CNS manifestations Body temperature ≥ 38 °C, heart rate Thyroid disease in
or fT3 levels (agitation, delirium, ≥ 130, cardiac decompensation, history, goiter,
psychosis, seizure, impaired gastrointestinal and hepatic exophthalmos
consciousness) manifestations
Definite Both main criteria + one of the Secondary criteria or main criterion 1 + at least three of the
thyrotoxic secondary criteria
crisis
Suspicion of Main criterion 1 and exactly two of the secondary criteria, or main criterion 2 + one of the
thyrotoxic secondary criteria 1 + all of the secondary criteria 2, or at least three of the secondary
crisis criteria 1 + all of the secondary criteria 2

Table 6.9 Staging of thyrotoxic crisis according to Hermann

Stage Clinical criteria

1 Tachycardia, arrhythmias, hyperthermia, adynamia, diarrhea, dehydration, tremor, agitation,
hyperkinesia, possibly increased thyroid hormone levels.
2 Stage 1 + disorientation, somnolence, stupor or psychosis
3 Stage 1 + coma
1–3a Age < 50 years
1–3b Age > 50 years
Tracheomalacia
– Rare
– Postoperative collapse of the trachea during inhalation
– Therapy:
– Intraoperative: Atraumatic lateral submucosal stabilization sutures (pillar
sutures) to sternocleidomastoid muscle
– Postoperative: Postoperative mechanical ventilation
– Endoluminal stenting
Postoperative Care
– Monitoring in the recovery room: HF (heart rate), SpO2 (pulse oximetric
oxygen saturation), neck circumference, hypocalcemia signs.
– Postoperative pain therapy (analogic pain scale)
– Workflow:
– On the first postoperative day: calcemia and PTH
– Calcium substitution if symptomatic hypoparathyroidism or very low level
– Before discharge: Postoperative laryngoscopy
– 1 week postoperatively: clinical follow-up (with discussion of the definitive
histopathology); possible initiation of substitution therapy; planning of
possible radioiodine therapy
– 3–4 weeks postoperatively: TSH control and eventual adaptation of
substitution
– Cervical ultrasound: after 6 months

6.2.4 Benign Thyroid Diseases

Hypothyroidism
Definition
– Insufficient central thyroid hormone production
– Limited peripheral activity of thyroid hormones
Symptoms
– During growth
– Delayed growth to dwarfism
– Mental retardation
– Rare: Rectal prolapse, umbilical hernia…
– In adults (incidious and slowly progressive; ◘ Table 6.2)
Diagnosis
– Laboratory diagnosis: Elevated TSH, low fT3 and fT4
Therapy
– Purely substitution therapy (levothyroxine)

Hyperthyroidism
Key Points
– Increased thyroid hormone secretion: hypermetabolism
– Extrathyroidal vs. thyroidal (toxic nodules, Graves’ disease, thyroiditis)
causes
– Definitive therapy possible only under euthyroidism
– Therapy: Medical and/or radioiodine and/or surgical therapy
Definition
– Increased thyroid hormone secretion
– As a consequence = hypermetabolism
Etiology
– Diseases of the thyroid (toxic nodules, autoimmune thyreopathy)
– central nervous system disease (increased TSH secretion or thyroid hormone
resistance)
– Exogenous thyroid hormone supply
– Molar pregnancy
– Thyroid cancer (► Sect. 6.2.5)

Symptomatology (◘ Table 6.2)

Toxic Nodules (= Autonomies)
Definition
– Thyroid cell clusters with autonomous (= TSH-independent) hormone secretion
– Forms: Unifocal vs. multifocal vs. multifocal disseminated
Epidemiology
– In regions with iodine deficiency: prevalence up to 65%
– In regions without iodine deficiency: prevalence <2–5%
– Prevalence increases with age
Symptoms
– Symptoms of hyperthyroidism (◘ Table 6.2) in up to 20% of patients (in
Germany, 4.1%/year hyperthyroidism in patients with unifocal autonomy)
Diagnosis
– Laboratory tests:
– TSH (low)
– Exclusion of an immunothyreopathy (Graves’ disease)
– Thyroid ultrasound: nodules; however, cannot prove/exclude an autonomic area
– Thyroid suppression scintigraphy with quantification of uptake: method of
choice to prove/exclude functional autonomy (toxic nodules)
Normal TSH (in 70% of patients with relevant autonomy) does not exclude the
diagnosis of hyperthyroidism.
Therapy
– Principle: Definitive therapy only in case of euthyroidism

Pretherapeutic/Preoperative Hyperthyroidism Treatment

– Thyrostatic agents: Thiamazole (initially 10–40 mg/day, then 2.5–10
mg/day), or carbimazole (initially 15–60 mg/day, then 5–15 mg/day), or
thiouracil (initially 25–150 mg/day, then 10–50 mg/day)
– Inhibitors of iodine uptake: (e.g. perchlorate) Inhibition of iodine uptake
prior to planned iodine contamination/contrast medium (CM) administration:
Irenate drops (1 mL = 300 mg sodium perchlorate), 3 × 25 drops/day
– Adjunctive therapy: β-blockers (attenuation of sympathetic activity and
reduction of peripheral conversion of T4; e.g. propranolol 3 × 20–40 mg/day
for 3–6 days before surgery), possibly benzodiazepines (to alleviate
symptoms)
– Lugol’s iodine solution (Plummer and Boothby 1923): If thyrostatic not
suitable because of side effects

– Radioiodine Therapy
– Indications/contraindications (limit = goiter >80–90 mL; ◘ Table 6.10)
– Low side effects + high success rate (85–95%)
– Principle:
– In-patient (2–5 days), with radiation protection measures (= isolation in
special departments)
 – Per os intake from131 I
– Risks/Side Effects:
– Sialadenitis
– Posttherapeutic hyperthyroidism (due to disintegration of the follicles)
– Radiation-induced thyroiditis
– Hypothyroidism: When excessive destruction of normal functional tissue
– Surgical Therapy
– Medical pretreatment obligatory (see above; exception: uncontrollable
thyrotoxic crisis)
– Indication for surgical therapy:
– Large goiter (volume > 80 mL); small strumen with poor iodine uptake.
– Goiter with persistent symptoms under medication
– Goiter with additional cold nodules
– For children, women of childbearing potential, pregnant women (when
medication is no longer possible or desirable)
– Autonomous adenomas (toxic nodules) with diameter > 3 cm
– Refusal of radioiodine therapy
– Iodine-induced thyrotoxicosis; thyrotoxic crisis that cannot be controlled
despite drug therapy
– Principle: High risk of recurrence: Therefore rather aggressive therapy
– Complications of surgery (Section “Cervical Lymphadenectomy (LAD)
for thyroid Cancer”)
– Posttherapeutic/postoperative therapy: objectives: Prophylaxis of recurrence +
treatment of postoperative hypothyroidism

Table 6.10 Indications and contraindications for 131I therapy

Indications Contraindications
Small to medium sized autonomous goiter Very large goiter with existing mechanical
complications
Focal toxic nodules (autonomous volume treatable with one Very large volume of autonomous tissue
radioiodine session)
Toxic nodules in recurrent goiter Low iodine uptake of the thyroid in
scintigraphy
Toxic nodules in patients with increased surgical risk Children and young people with toxic
nodules
Gravidity and lactation
Large cold nodules with suspected
malignancy
Immunothyreopathy: Graves’ Disease
Definition
– Merseburg Triassic
– Goiter
– Tachycardia (sinus tachycardia)
– Exophthalmos
– Hyperthyroidism
Epidemiology
– Prevalence = 0.5–2%; second most common cause of thyroid hyperfunction (in
Europe)
– Mostly between 20 and 50 years of age
– Most common reason for hyperthyroidism in children and adolescents
– Annual incidence = 40–60/100,000 population per year
– Women:Men = 5:1
– Association with other autoimmune diseases (5–10% of patients):
– Vitiligo
– Pernicious anaemia
– myasthenia gravis
– Diabetes mellitus type 1
– Addison’s disease
– Rheumatoid arthritis
Pathophysiology
– Pathogenesis not fully understood
– Autoantibodies: anti-TSH receptor = increased hormone production =
hyperplasia, hypertrophy
– Risk factors
– Genetic predisposition (familial clustering)
– Nicotine consumption
– Triggering factors: stress; iodine exposure to jodes; viral infection; influence of
sex hormones
Symptoms
– Caution: Oligosymptomatic courses
– Clinical presentation: (◘ Table 6.2 Symptoms of hyperthyroidism), in addition:
– Overheated moist skin
 – Systolic hypertension
– Auscultatory murmur over the thyroid (increased blood flow)
– Enlarged thyroid (80% of patients)
– Endocrine ophthalmopathy (not always synchronous), pathognomonic
– Pretibial myxedema, rare (4% of patients)
– Hypertrophic osteoarthropathy, rare
– Acropachy, rare
Diagnosis
– Laboratory Diagnosis:
– Low TSH: Hyperthyroidism
– Increased fT4 and/or fT3 (if TSH low and T4/T3 normal = subclinical
hyperthyroidism)
– Antibody determination: TSH receptor antibodies (= TRAb) elevated (grey
range = 1–1.5 IU/L)
– Blood count and liver values: Required if drug therapy is used
– Ultrasound:
– Hypoechogenicity with/without goiter
– Doppler: Diffusely increased perfusion
– Exclusion of focal findings
– Complementary Diagnosis:
– Scintigraphy: Not absolutely necessary
– Ophthalmological examination of an ophthalmopathy: measurement of the
protrusio
– In case of symptoms: cardiological examination, osteodensitometry
Therapy
– Primary (initial manifestation): Thyrostatic long-term therapy
– After completion: regular follow-up to exclude recurrence
– In case of recurrence/unsuccessful therapy: definitive therapy (surgery vs.
radioiodine therapy)
– Thyrostatic long-term therapy:
– 30–60% remission after long-term thyrostatic therapy
– Therapy duration at least 12 months (= better remission rate)
– Contraindications:
– Mechanical impairment due to thyroid volume
– Suspicion of malignancy
 – Severe side effects
– Multimorbid patient (if stable euthyroidism cannot be achieved by drug
therapy)
– Lack of compliance
– Desire to have children (= relative contraindication)
– Recurrence after long-term thyrostatic therapy
– Practical implementation (◘ Table 6.11)
– Radioiodine Therapy
– Indications:
– Graves’ disease with small or moderate goiter
– Increased risk of surgery, recurrent laryngeal nerve palsy, postoperative
hypoparathyroidism
– refusal of an operation
– Special occupations (using the voice): Singer, teacher, speaker
– Contraindications:
– Pregnancy (should be avoided 4 months after therapy)
– Breastfeeding women (wean at least 6–8 weeks before radioiodine)
– Suspected malignancy
– Practical implementation:
– Ablative concept: Complete thyroid ablation (about 250 Gy)
– Function-optimized concept: function-preserving therapy (about 150 Gy)
– Surgical therapy:
– Indications:
– Compression symptoms (tracheomalacia, tracheastenosis, stridor)
– Malignancy suspected
– Need for immediate therapeutic effect (e.g. severe side effects to
thyrostatic or radioiodine therapy)
– Therapy refractory hyperthyroidism
– Highly-active endocrine orbitopathy
– Lack of patient compliance
– Desire to have children
– For children/adolescents (relative indication)
– Preoperative preparation: (see above: Overview: Pretherapeutic/preoperative
hyperthyroidism treatment); Glucocorticoids: In Graves’ disease
hyperthyroidism with suspicion of endocrine ophthalmopathy
– Total/almost total thyroidectomy: therapy of choice
 – Intraoperative specific features:
– Increased blood flow = bleeding tendency
– Difficult identification of recurrent laryngeal nerve/parathyroid glands
– Follow-up: Specific features:
– Frequently postoperative hypocalcemia (disturbed bone metabolism)
– Discontinue thyrostatic medication immediately postoperatively
– Hormone replacement: levothyroxine (1.5 μg/kg BW/day)
Table 6.11 Thyrostatic long-term therapy of Graves’ disease (practical implementation)

Substance Initial therapy (mg/day) Maintenance therapy (mg/day)

Monotherapya,b
Thiamazole 10–40 2.5–10
Carbimazole 20–60 5–15
Propylthiouracil 150–300 50–200

Combination therapy a,b

a
Combination therapy with thyroid hormones (levothyroxine 100 μg/day)
possible: lower thyrostatic doses
b
Pregnancy: Absolutely monotherapy, as only thyreostatics pass the blood-
placental barrier. Multidisciplinary monitoring

Thyroiditis
Key Points
– Inflammatory diseases of the thyroid = histological evidence of inflammatory
cells in the thyroid
– Diagnosis based on clinical presentation + laboratory diagnosis (+++) +
ultrasound
– Therapy depending on entity (acute/subacute/Riedel’s/autoimmune
thyroiditis)

Acute Thyroiditis
Pathogenesis
– Rare
– Acute infection due to fungi/bacteria
– Especially in case of immunodepression (HIV, tuberculosis)
– Pathogen: Frequently Streptococcus pyogenes, Staphylococcus aureus
Symptoms
– Pain:
– Mostly one-sided
– Radiating pain (ears, lower jaw, retrosternal)
– Acute onset
– Fever
– Local redness, swelling, possible fluctuation
– Difficulty swallowing
– possibly hoarseness
Diagnosis
– Medical history: chronic diseases (HIV, tuberculosis)
– Palpation: pain/fluctuation
– Laboratory: leukocytosis, CRP elevation, temporary hyperthyroidism
– Ultrasound: Inhomogeneous image with hypoechogenic areas (= pus)
– CT: exclusion of a process involving more than one organ
– Contrast medium swallow: exclusion of a fistula with pyriform sinus/esophagus
– FNA: Confirmation of diagnosis + microbiological examination
Therapy
– No pus collection
– i.v. antibiotics, pathogen-oriented, at least 14 days
– Analgesia, cooling measures
– In case of pus collection
– Additional surgical drainage

Subacute Thyroiditis (De Quervain)

– Granulomatous disease
– Inclusions of multinucleated giant cells
Pathogenesis
– Often a few weeks to several months after viral infection (especially respiratory
tract)
– Predominantly women in the fourth and fifth decade
– Seasonal accumulation in early autumn
– Genetic predisposition (HLA B35)
Symptoms
– Acute onset with pain (radiating into the ears)
– subfebrile temperature
– Dysphagia
– Mild hoarseness
– Viral prodromes: muscle pain, general feeling of illness
– First mild hyperthyroidism then euthyroidism and possibly discrete
hypothyroidism
Diagnosis
– Palpation: Firm consistency, pressure pain
– Laboratory: mild leukocytosis, CRP elevated, extremely accelerated blood
sedimentation (>100 mm/h, almost pathognomonic), inflammation-related
anemia
– Ultrasound: Typical: Map-like (hypo/hyperechogenic areas)
– Scintigraphy: Decreased Tc uptake (in the affected thyroid)
– FNA: Granulomatous change with multinucleated giant cells
Therapy
– Mild course: Aspirin 2–3 × 500 mg/day; alternatively, diclofenac 50–150
mg/day.
– Pronounced symptoms: glucocorticoid therapy (over 6–12 weeks)
– If hyperthyroidism: symptomatic (β-blocker, e.g. propranolol 3 × 40 mg).
– Surgical therapy: in case of therapy resistance (rarely necessary)

Autoimmune Thyroiditis
– Lymphocytic organ infiltration
– Women:Men =7–10:1
– Genetic predisposition (HLA DR3, DR4, DR5, B8), environmental influences,
nutrition, infections, age, sex
– Autoimmune thyroiditis:
– Hypertrophic autoimmune thyroiditis (Hashimoto’s thyroiditis)
– Atrophic autoimmune thyroiditis (primary myxedema)
– Post-partum thyroiditis: In 3–11% of women after childbirth
– Asymptomatic “silent thyroiditis”: almost always an incidental finding
– Riedel’s goiter: extracapsular and infiltrating thyroiditis
Symptoms
– Often incidental finding when organ enlargement is detected
– Feeling of pressure or slight pain
– Hypothyroidism symptoms (Hashimoto’s and atrophic thryoiditis = most
frequent cause of hypothyroidism in adults; ◘ Table 6.2)
Diagnosis
– Positive family history
– Presence of other autoimmune diseases
– Mild cervical pressure sensation
– Palpation: Firm consistency of the thyroid
– Laboratory tests:
– Antibody elevation: anti-TPO (anti-thyroid peroxidase; frequent), anti-Tg
(anti-thyroglobulin)
– hyperthyroidism (at the beginning of the disease, short-term) then
hypothyroidism
– Ultrasound: Diffuse hypoechogenicity
– Scintigraphy: Diffuse low uptake
Therapy
– If hypothyroidism: L-thyroxine substitution therapy
– If hyperthyroidism: symptomatic therapy
– If Riedel’s thyroiditis: glucocorticoid therapy + surgery (to exclude cancer).

Special Forms of Thyroiditis

Traumatic Thyroiditis
– Thyroiditis induced by exogenous lesion (including radiation thyroiditis,
radioiodin-induced thyroiditis)
– Therapy: analgesia + anti-inflammatory drugs, local cooling, rarely
glucocorticoids
Drug-Induced Thyroiditis
– Drugs: e.g. cytokines such as interferons, Il-2, GM-CSF (“granulocyte
macrophage colony-stimulating factor”), amiodarone
– In case of hyperthyroidism, surgical therapy may be necessary

Goiter and Nodular Goiter

Key Points
– Organ enlargement of the thyroid with/without nodule
 – Etiopathogenesis: iodine deficiency + genetic predisposition
– Surgical therapy depending on: thyroid morphology, clinical presentation,
nodule behavior, FNA
Definition
Goiter
– organ enlargement over 18 mL in women, 25 mL in men
Nodular Goiter
– Enlargement of the thyroid due to multifocal thyrocyte proliferation
– Thyroid nodules: Clearly delineated clonal/polyclonal heterogeneous thyroid
formation
– Thyroid adenoma: histologically homogeneous monoclonal nodule with own
structure + capsule
Pathogenesis
– Genetic predisposition + acquired alterations: Direct influence on the progenitor
cells
– Iodine deficiency = hyperplasia-inducing
Diagnosis
Clinical Examination
– Inspection: Classification of goiter (grading, symmetry)
– Grading of goiter according to WHO
– 0a: No goiter
– 0b: Palpable, but not visible
– 1: Palpable and visible with head reclination
– 2: Visible goiter without reclination
– 3: Large visible goiter
– Palpation: estimation of thyroid volume; relationship to landmarks (e.g.,
retrosternal goiter); number and consistency of nodules; swallowing
displacement; presence of cervical lymph nodes.
Ultrasound
– Determination of the thyroid volume
– Localization, dimension and character (echogenicity, margins, vascularization,
calcifications) of the nodules (◘ Table 6.12 Ultrasound signs of malignancy)
Table 6.12 Ultrasound signs of malignancy
Malignancy sign Signs of benign node
Hypoechogenicity Purely cystic mass
Increased intranodal vascularization Spongiform mass
Irregular border
Microcalcifications
Absence of a halo
Nodule larger than wide in transverse image
Abnormal cervical lymph nodes

Scintigraphy (► Sect. 6.2.2)

– Only if there’s a nodule >1 cm on ultrasound…
Endemic euthyroid goiter: scintigraphy not required, neither for indication nor
for planning surgery.
FNA
– For the differentiation of benign/malignant thyroid nodules
– Indications: ► Sect. 6.2.2
Laboratory (Standard Levels, ► Sect. 6.2.2, Laboratory Thyroid Function
Tests)
– Basal TSH (sufficient if normal)
– In case of suppressed or elevated TSH: fT3 and fT4 for the detection of
hyperthyroidism/hypothyroidism
– In case of disseminated functional autonomy (scintigraphy): TPO + TSH
receptor antibodies to exclude Graves’ disease
– If malignancy is suspected: calcitonin to exclude medullary thyroid cancer
(caution: proton pump inhibitor (PPI) therapy and renal insufficiency)
Therapy
Medical Therapy and Prophylaxis
– Levothyroxine or iodide
– Limited therapeutic influence on the progression of nodular goiter
– Large nodules, large goiter = less sensitive to medical therapy
– In iodine deficiency endemic areas: Early prophylaxis of nodular goiter
indicated
– Radioiodine therapy: ultima ratio, only if surgical treatment is not possible
Surgical Therapy
– Procedure oriented to thyroid morphology and nodules
– Possible surgical procedures: Hemithyroidectomy, thyroidectomy…
– Indications:
– Large thyroid volume (grade III, volume > 60 mL)
– Suspicion of nodules (◘ Table 6.12)
– Patient’s request for pathology with smaller volume goiter
– Waiver of the patient for long-term control examinations
– Section “Preoperative Measures”
– Procedural choice:
– Hemithyroidectomy: When nodules localized on one side
– Hartley-Dunhill procedure: hemithyroidectomy + contralateral subtotal
resection
– Thyroidectomy: standard procedure for euthyroid multinodular goiter

Highly Recommended For All Thyroid Operations

– Use of magnifying loupes
– Visual identification of recurrent laryngeal nerve (gold standard)
– Intraoperative neuromonitoring (recurrent laryngeal nerve + vagus nerve)
– Visual identification of the parathyroid glands

Recurrent Goiter
– Prophylaxis in case of incomplete resections = drug-based recurrence
prophylaxis (administration of iodide/levothyroxine)
– Radical resection in primary surgery = thyroidectomy = most important
factor for recurrence prophylaxis
– Surgery on recurrent goiter: only by very experienced surgeons;
intraoperative neuromonitoring + readiness for frozen section obligatory

6.2.5 Malignant Thyroid Diseases

Key Points
– 1% of human malignancies
– 99% = epithelial tumours (carcinomas)
– Increase in incidence of thyroid cancers in the last 25 years (better diagnosis +
absolute increase)
 – Mortality declining (differentiated cancers = excellent prognosis)
– Evidence (RCT, meta-analyses): in the past 3 years in favour of limited
resection or surveillance without surgery for “low-risk” differentiated thyroid
cancers (not yet integrated in the current guideline)
German S3 guideline “Thyroid carcinoma”: currently in development with
planned completion by 30.04.2021.

Cancers of the Thyroid Gland

Definition (◘ Table 6.13)
– Differentiation: cancers with follicular epithelial cells vs. C-cell differentiation
– Distinction based on the different biological course:
– Differentiated cancers (papillary = PTC, follicular = FTC)
– Poorly differentiated cancers (medullary cancer = MTC, anaplastic
carcinoma)
– Specific features of thyroid cancers:
– Congenital (familial) or acquired in the course of life (sporadic)
– From childhood to old age
– Strong predominance in the female gender (especially PTC and FTC)
– Geographical differences = genetic, environmental factors
– Differentiated cancers = very good prognosis; anaplastic carcinomas = very
poor prognosis
– Incidence of thyroid microcarcinoma (carcinoma <1 cm) up to 35%.
Table 6.13 Classification of thyroid cancers

Carcinomas with follicular Differentiation according to Cell differentiation Subdifferentiation

epithelial cell differentiation biological course
Differentiated carcinoma Papillary carcinoma Conventional type
Variants
Follicular Minimally invasive
carcinoma
Broadly invasive
Other

Anaplastic carcinomaa
Carcinoma with C-cell Medullary carcinomaa Sporadic
differentiation
Autosomal dominant inheritance (MEN 2,
fam. MTC)
Mixed type (C-cell follicle differentiation)
Rare primary thyroid carcinomas Squamous cell carcinoma
 Mucinous carcinoma
Mucoepidermoid carcinoma
Mucoepidermoid carcinoma with eosinophilia
Spindle epithelial tumor with thymus-like differentiation (SETTLE)
Carcinoma with thymus-like differentiation (CASTLE)

MEN 2 multiple endocrine neoplasia type 2, MTC medullary thyroid carcinoma

a
Low-differentiated carcinoma = medullary + anaplastic carcinoma
Papillary Thyroid Cancer (PTC)
Epidemiology
– Most frequent malignant thyroid cancer (60–85%)
– Age peak = 40 years of age
– Women:Men = 3:1
Molecular Pathology BRAF Mutation: Approx. 50% of PTC
– RET/PTC rearrangements: 10–20% of the PTC
– NTRK1 rearrangements: 10% of the PTC
Prognosis
– Regional LN metastasis: Already with tumor sizes of a few mm
(microcarcinoma)
– distant metastases significantly later (= mainly lung)
– Normal life expectancy even with metastases
– Prognostic factors:
– Large PTC = poor prognosis
– High age = poor prognosis
– Radioiodine storage = good prognosis
Therapy Principles
– ► Section 6.2.3 “Principles of surgical therapy” and “Cervical
lymphadenectomy for thyroid cancer”:
– Total thyroidectomy + central lymphadenectomy
– Subsequent radioiodine therapy
– Lateral lymph node dissection:
– if positive lateral LN
– In case of special histology (poorly differentiated cancer)
– In case of recurrent PTC: primary therapy = surgery; in case of mediastinal LN
metastases or distant metastases: Primary radioiodine therapy
Hemithyroidectomy = sufficient for papillary/follicular thyroid
microcarcinoma (< 1 cm nodule) without extrathyroidal tumor manifestation.
Follicular Thyroid Cancer (FTC)
Epidemiology
– 10–30% of thyroid cancers
– High incidence in iodine deficient areas
– Women:Men = up to 5:1
– Age peak: 50 years of age
Molecular Pathology
– Adenoma-carcinoma sequence: FTC develops from an adenoma (genetic
alterations)
– Vascular invasion/metastasis: From tumor size of 1–2 cm
– No specific genetic change (= no marker)
Prognosis
– Hematogenous metastasis mainly (lung, bone): 10% in encapsulated tumors,
50% of broadly invasive FTC
– Lymph node metastases together with organ metastases
– Minimally invasive FTC = excellent prognosis; broadly invasive FTC = 40–
60% 10-year survival; oncocytic FTC = poorer prognosis.
Therapy Principles
– ► Section 6.2.3 “Principles of surgical therapy” and “Cervical
lymphadenectomy for thyroid cancer”
– Total thyroidectomy + central lymphadenectomy
– Subsequent radioiodine therapy
– Lateral lymph node dissection:
– if positive lateral LN
– In case of special histology (poorly differentiated cancer)
– In case of FTC recurrence: primary therapy = surgery; in case of mediastinal
LN metastases or distant metastases: Primary radioiodine therapy
Poorly Differentiated Thyroid Cancer
Epidemiology
– 4–7% of thyroid cancers (geographical differences)
– Women:Men = 2:0
Molecular Pathology
– Largest part = development de novo
– No specific genetic characteristic features
Prognosis
– 5-year survival rate = 50
– 10-year survival rate = 25–35%.
Therapy Principles
– ► Section 6.2.3 “Principles of surgical therapy” and “Cervical
lymphadenectomy for thyroid cancer”
– Total thyroidectomy + central lymphadenectomy
– Lateral lymph node dissection
– Subsequent radioiodine therapy
Anaplastic Thyroid Cancer
Epidemiology
– <5% of thyroid malignancies; 90% of deaths from thyroid cancers
– Predominantly older people
– Mostly in nodular goiters
– Women:Men = 1.5:1
Molecular Pathology
– Often de novo
Prognosis
– Mortality >90%
– Mean survival <6 months (= one of the most aggressive human malignancies)
– Short history, rapid metastasis (hematogenous + lymphogenous)
– At diagnosis: already extensive extrathyroidal extension (trachea, esophagus,
cervical vessels)
Therapy Principles
– Perioperative radiochemotherapy
– Operative therapy: If operable, ideally R0 resection
– Molecular genetic testing always indicated
– New targeted therapies/checkpoint inhibitor therapy: in evaluation within
studies
Medullary Thyroid Cancer (MTC)
Epidemiology
– 1–3% of all thyroid cancers
– Sporadic MTC (75%): Mostly in patients >45 years
– Familial MTC (FMTC; 25%): In patients from childhood to old age
Molecular Pathology
– Tumor markers: calcitonin, chromogranin A, CEA
– Familial MTC: Autosomal dominant in the context of the MEN-2 syndrome (2a
or 2b; ► Sect. 6.2.3)
– Familial MTC: detection/exclusion of the rearranged-during-transfection
(RET)-protooncogene mutation obligatory
Prognosis
– MTC without metastasis: Excellent prognosis
– MTC with metastasis: generally poor prognosis
Therapy Principles
– Sporadic MTC: thyroidectomy + central LN dissection + ipsilateral lateral LN
dissection ± contralateral lateral LN dissection (if calcitonin level > 200 pg/mL)
– Familial MTC: timing of therapy depends on mutation type: thyroidectomy +
LN dissection; in MEN 2B: surgery immediately after diagnosis
Familial Non-Medullary Thyroid Cancer (► Sect. 6.3)
– 5% of the differentiated PTC, FTC
– In the context of defined autosomal dominant syndromes (e.g. Gardner
syndrome, Cowden syndrome, Carney complex) or non-syndromic
Rare Thyroid Cancers
– Squamous cell carcinoma
– Mucinous carcinoma
– mucoepidermoid carcinoma
– Mucoepidermoid carcinoma with eosinophilia
– Spindle cell tumor with thymus-like differentiation (SETTLE)
– Carcinoma with thymus-like differentiation (CASTLE)
Primary Malignant Lymphoma of the Thyroid Gland
– Patients with Hashimoto’s thyroiditis = 70 times higher risk
– Predominant part = extranodal mucosa-associated lymphoid tissue (MALT)
marginal zone B-cell lymphoma
– Other lymphomas (non-Hodgkin or Hodgkin) very rare

Primary Sarcomas of the Thyroid Gland

– Rare
– Differential diagnosis with anaplastic thyroid cancer difficult
– Special type: angiosarcoma (poor prognosis)

Metastases in the Thyroid Gland

– Incidence of thyroid metastases in autopsies = 25%
– Origin tumors:
– Lung Cancer
– Breast Cancer
– Renal cancer
– Cancers of the GI tract
– Malignant melanoma

6.2.6 Workup of a Solitary or Dominant Thyroid Nodule

Key Points
– High prevalence of thyroid nodes in Germany (25–50%)
– Necessity of a rational workup for differential diagnosis (workup diagram)
– Central = anamnesis + clinical examination + laboratory thyroid function tests
+ ultrasound
– Increasing importance: FNA

Epidemiology
– Prevalence (25–50%) depending on: Geography (endemic area), age, sex
– In Germany approx. 16 million people with nodules; 2/3 = cold nodules
– Diagnostic workup = evidence-based rational approach (◘ Fig. 6.2: ATA
guideline 2015)
– Prevalence-oriented differential diagnosis of the solitary/dominant thyroid
nodule (with decreasing prevalence)
– Colloid Nodules
 – Thyroid adenoma, hormonally inactive (mostly follicular adenoma)
– Thyroid adenoma, hormonally active, toxic adenoma (autonomous adenoma)
– Thyroid Cyst
– Thyroiditis (Hashimoto)
– Thyroid cancer (papillary, follicular)
– Non-epithelial thyroid tumors and metastases
– Abscess

Fig. 6.2 ATA guidelines

Symptoms
– Mostly asymptomatic
– Mild symptoms/symptomatic: Only if large nodule
– Subjective globus sensation
– Swallowing disorders, to dysphagia
 – Stridor with tracheal compression

Diagnosis
– Rational approach
Medical History and Clinical Examination
– Age, sex, cervical radiation history, family history, familial thyroid disease
patterns (► Sect. 6.3, e.g., MEN, poliposis coli, Gardner and Cowden
syndromes).
– Time course of occurrence, growth
– Clinical examination:
– Solitary nodule: Isolated nodular finding
– Dominant nodule: Sudden change in a nodule in a nodular goiter.
– Local symptoms
– Metabolic activity
– Exclusion of malignant signs

Clinical Signs of Malignancy in Thyroid Nodules

– Rough consistency
– Palpable cervical lymphadenopathy
– Non-displaceable Thyroid
– Hoarseness (expression of a recurrent laryngeal nerve impairment)
– Horner’s syndrome = late sign

Laboratory Diagnosis (► Sect. 6.2.2 Laboratory Thyroid Functuon Tests)

Ultrasound
– Most sensitive examination method for solitary/dominant thyroid nodes
– Presentation of clinically inapparent nodules
– Best method for monitoring the progress of a nodule
– Guidance of the FNA
– Sonographic signs of malignancy (◘ Table 6.12)
Scintigraphy
– If low TSH
– In nodule endemic areas (even if TSH normal)
– To distinguish hot nodule (autonomous area) vs. cold nodule
– Diagnostic consequence: autonomous area (= no further diagnosis) vs. cold
nodule (= indication for FNA); check possibility of radioiodine therapy

FNA (► Sect. 6.2.2 Fine Needle Aspiration Cytology) (◘ Fig. 6.3)

Therapy
– Oriented to:
– Knowledge of the underlying disease/its prognosis (diagnosis)
– Risk factors of the patient
– Function of the thyroid
– Expectations of the patient
– Health condition of the patient

Fig. 6.3 Ultrasound and cytological FNA categories (classes) and recommended treatment

Indications for Surgical Therapy

– Higher-grade suspicion in FNA (Bethesda groups IV, V, and VI, TIR 3B/thy 3f
to TIR 5/Thy 5 ◘ Table 6.4)
– Benign nodule with compression symptoms
– Newly appeared growing nodule with cervical radiation history
– Solitary autonomous adenoma >3 cm
– Suspicious nodule in the absence of follow-up
Indications for Conservative Therapy
– Operation criteria not fulfilled
– Patient not fit for anaesthesia/surgery
With conservative therapy:
– Always plan regular follow-up (interval = 6–18 months)
– In case of nodule growth: reschedule follow-uo sooner and possibly indicate
surgery
Operative Therapy Principles
– Basically hemithyroidectomy + isthmus resection
– Enucleation: no longer justifiable; subtotal resection: actual controversial
discussion
– In case of suspected malignancy: intraoperative frozen section
– In case of malignancy: thyroidectomy (exception = microcarcinoma)
– Prophylactic central lymphadenectomy: controversy

6.2.7 Guidelines
Gharib H, Papini E, Garber JR et al. (2016) American Association of Clinical
Endocrinologists, American College of Endocrinology, and Associazione Medici
Endocrinologi medical guidelines for clinical practice for the diagnosis and
management of thyroid nodules—2016 update. Endocr Pract 22:S1–S60.
Haddad RI, Nasr C, Bischoff L et al. (2018) NCCN guidelines insights:
thyroid carcinoma, version 2.2018. J Natl Compr Canc Netw 16:1429–1440.
Haugen BR, Alexander EK, Bible KC et al. (2016) 2015 American Thyroid
Association Management Guidelines for adult patients with thyroid nodules and
differentiated thyroid cancer. The American Thyroid Association Guidelines Task
Force on Thyroid Nodules and Differentiated Thyroid Cancer. Thyroid 26:1–133.
Haugen BR, Sawka AM, Alexander EK et al. (2017) American Thyroid
Association Guidelines on the management of thyroid nodules and differentiated
thyroid cancer task force review and recommendation on the proposed renaming
of encapsulated follicular variant papillary thyroid carcinoma without invasion to
noninvasive follicular thyroid neoplasm with papillary-like nuclear features.
Thyroid 27:481–483.
Musholt TJ, Bockisch A, Clerici T et al. (2018) Update of the S2k guideline
Operative therapy of benign thyroid disease. Surgeon 89:699–709.
Patel KN, Yip L, Lubitz CC et al. (2020) The American Association of
Endocrine Surgeons Guidelines for the definitive surgical management of thyroid
disease in adults. Ann Surg 271:e21–e93.
 Perros P, Boelaert K, Colley S, British Thyroid Association et al. (2014)
Guidelines for the management of thyroid cancer. Clin Endocrinol 81:S1–S122.
S2k AWMF guideline (Register No. 088/007) Surgical therapy of benign
thyroid disease. Version as of 03.10.2015. ► https://​www.​awmf.​org/​uploads/​tx_​
s2leitlinien/​088-007l_​s2k_​operative_​Therapie_​benigner_​Thyroid_​Diseases_​2015-
10-verlaegert.​pdf
Wells SA, Asa SL, Dralle H et al. (2015) Revised American Thyroid
Association Guidelines for the managements of medullary thyroid carcinoma. The
American Thyroid Association Guidelines Task Force on medullary thyroid
carcinoma. Thyroid 25:567–610.

6.3 Familial Malignant Syndromes of the Thyroid

Gland
C. E. Gibson and R. Udelsman

6.3.1 Introduction (◘ Table 6.14)

Table 6.14 Hereditary predisposition syndromes for thyroid cancer

Familial PTEN Carney RET-associated DICER1

adenomatous hamartoma complex
polyposis tumor type 1
(Cowden)
Gene APC PTEN PRKAR1A RET DICER1
Pathognomonic >100 Mucocutaneous Multiple Medullary thyroid Pleuropulmonary
criteria colorectal lesions, pigmented carcinoma) blastoma)
adenomatous cerebellar skin lesions
polyps tumors (e.g. nevi,
(Lhermitte- blue nevi,
Duclos) lentiginous
N)
Other main – Breast, Blue nevi, Primary Germline stromal
manifestations endometrial, pigmented hyperparathyroidism, tumors, cystic
thyroid nodular pheochromocyt om, nephromas,
carcinoma, adrenal mucous neuromas multinodular goiter
macrocephaly gland,
cardiac
myxoma…
Ancillary Extracolic Fibrocystic Thyroid Hirschsprung’s Wilms tumor,
manifestations polyps, mastopathy, nodules, disease, cutaneous rhabdomyosarcoma,
congenital gastrointestinal melanotic lichen, ciliary body
hypertrophy hamartomas, schwanomas, amyloidosis… medulloepithelioma,
of the retinal lipomas, adrenal or pituitary gland
pigment fibromas, renal pituitary blastoma, nasal
 Familial PTEN Carney RET-associated DICER1
adenomatous hamartoma complex
polyposis tumor type 1
(Cowden)
Gene APC PTEN PRKAR1A RET DICER1
epithelium, cell adenomas, chondromesenchymal
thyroid carcinomas, HCC, hamartoma
nodules, SD uterine pancreatic
carcinoma, fibromas carcinoma
soft tissue
tumors,
desmoid
tumors,
osteomas
Prevalence of 40% 75% Up to 75% Up to 30%
thyroid
diseases
Benign 0.4–12% 35% <5% – Up to 30%
Malignant CMV-PTC PTC 50% FV- PTC FTC 100% MTC FTC FV-PTC
subtypes 63% FV-PTC PTC 28% FTC
25% PTC 14%
12%

CMV cribriform-morulare variant, FV follicular variant

Genetics
– Thyroid cancer three times more frequent in women than men
– Earlier age at diagnosis in women (40–50 s) versus men (60 s–70 s)
– Certain inherited mutations associated with different types of thyroid cancer:
– RET gene mutations: associated with medullary thyroid cancers/syndromes
such as MEN 2A, MEN 2B and FMTC
– APC gene mutations: associated with FAP and Gardner syndrome
– PTEN gene mutations: associated with Cowden disease
– PRKAR1A gene mutations: associated with Carney Complex, type I

6.3.2 Hereditary Medullary Thyroid Carcinoma

Medullary Thyroid Carcinoma (MTC)
Key Points
– Tumor of the parafollicular C cells
– accounts for ~5% of all thyroid cancers
– 25% = hereditary, 75% = sporadic
– responsible for disproportionate number of thyroid cancer deaths
 – all patients with MTC should be screened for germline mutations:
– linked to germ line activation of the Rearranged during Transfection (RET)
proto-oncogene
– several RET mutations identified; aggressiveness of MTC differs by
mutation (i.e. genotype-phenotype correlation)
Clinical Presentation
– Neck mass; cervical lymphadenopathy
– Asymptomatic/incidental finding (e.g. during work-up for unrelated medical
issues)
– Evidence of distant metastases: Liver, lung, bone and mediastinum
Diagnosis
– Neck ultrasound (US)
– Fine needle aspiration biopsy (FNA)
– Tumour markers: Elevated serum calcitonin + carcinoembryonic antigen (CEA)
levels
– Cytology: plasmacytoid cell pattern, spindle cells, stromal amyloid deposits,
positive calcitonin antibody
Treatment
– Surgery (total thyroidectomy; central and/or lateral neck lymph node dissection)
– External beam radiation (rarely used)
– Chemotherapy (rarely used)
– Tyrosine kinase inhibitor therapy

Multiple Endocrine Neoplasia 2A (MEN 2A)

Epidemiology
– Identified in <1000 kindreds to date
– High penetrance for the MTC
– accounts for 90% of MEN-2 cases
Genetics
– Germline mutation of the RET proto-oncogene
– Mutation in codon 609, 611, 618, 620, 630, 631 and 634 (most common;
mutation at extracellular cysteine residues)
– Autosomal dominant (AD); localized on chromosome 10q11.2
– Neoplastic cell proliferation (C-cell hyperplasia) = precursor lesion
– Characteristic features:
– MTC (often multifocal and bilateral)
– Pheochromocytoma
– Parathyroid neoplasia (typically benign asymmetric multiglandul
hyperplasia)
– Hirschsprung’s disease and cutaneous lichen amyloidosis (uncommon
associations).
Clinical Presentation
– Often asymptomatic
– If symptomatic, linked to hyperparathyroidism or pheochromocytoma.
– No correlation between extent of hypercalcemia and extent of symptoms
Diagnosis
– thorough family history
– Genetic testing = gold standard for diagnosis
– can detect >30 variants of RET mutations
– FNA biopsy:
– 85–99% specific for MTC
– Cytologic features: Plasmacytoid cells, spindle cells, stromal amyloid
deposits, eccentrically located nuclei, positive calcitonin staining.
– Tumor markers:
– Calcitonin levels >100 highly suggestive of MTC
– Elevated CEA levels may denote advanced disease
Treatment (◘ Table 6.15)
– Mostly level 2 risk (codon 634):
– Highest rate of pheochromocytoma and hyperparathyroidism
– Must rule out pheochromocytoma prior to neck surgery
– MTC development at earlier age than other codon mutations
– Prophylactic total thyroidectomy (± central LN dissection) prior to age 5
recommended
– Modified radical (= functional) neck dissection --> if clinical or cytologic
evidence of cervical metastases
Table 6.15 Management of patients with RET germline mutation detected on genetic screening. (From
Revised American Thyroid Association Guidelines for the Management of Medullary Thyroid Carcinoma
2015)
MEN RET mutation Age at presentation Recommended procedure
Type (years)
MEN 2A C609/F/G/R/S/Y Variable Thyroidectomy when serum calcitonin ↑, or based on
patient/parent preference
MEN 2A C611/F/G/S/Y/W Variable
MEN 2A C618/F/R/S Variable
MEN 2A C620F/R/S Variable
MEN 2A C634F/G/R/S/W/Y ≤ age 5 Thyroidectomy by age 5 years
MEN 2B A883F ≤ age 5
MEN 2B V804M + Y806C 20–30 years Thyroidectomy when serum calcitonin ↑, or based on
patient/parent preference
MEN 2B V804M + S904C 20–30 years
MEN 2B V804M + E805K 20–30 years
MEN 2B V804M + Q781R 20–30 years
MEN 2B M918T Infancy Thyroidectomy ± Level VI LN dissection within first
year of life

Multiple Endocrine Neoplasia 2B (MEN 2B)

Epidemiology
– Less common cause of inherited MTC (<5% of cases)
– MTC presents at earlier age than in MEN 2A
– Worst prognosis
Genetics
– Germline mutation in the RET proto-oncogene
– mutation in codon 804, 806, 883 and 918 (most common: methionine →
threonine)
– Autosomal dominant (AD); located on chromosome 10q11.2
– Neoplastic cell proliferation (C-cell hyperplasia); precursor lesion
– Characteristic features:
– MTC (early onset; infancy—early childhood)
– Pheochromocytoma (40% penetrance)
– Mucosal neuromas, gangliomas, megacolon, Marfanoid-like body habitus.
Clinical Presentation
– Often asymptomatic
– If symptomatic, linked to tumor compressive symptoms, high calcitonin levels,
or pheochromocytoma
Diagnosis
– Thorough family history
– Genetic testing = gold standard for diagnosis
– Can detect >30 variants of RET mutations
– FNA biopsy:
– 85–99% specific for MTC
– Cytologic features: Plasmacytoid cells, spindle cells, stromal amyloid
deposits, eccentrically located nuclei, positive calcitonin staining.
– Tumor markers:
– Calcitonin levels >100 highly suggestive of MTC
– Elevated CEA levels may denote advanced disease
Therapy (◘ Table 6.15)
– Mostly level 3 risk (codon 918):
– Highest rate of advanced disease (locally advanced or widely metastatic)
– C-cell hyperplasia or MTC development during infancy
– must rule out pheochromocytoma prior to neck surgery
– Prophylactic total thyroidectomy (± central LN dissection) by age 1
recommended
– Total thyroidectomy + central neck dissection + modified radical (functional)
neck dissection → if clinical or cytologic evidence of cervical metastases
– consider prophylactic functional neck dissection if clinically negative cervical
LN but high calcitonin and/or CEA levels

Familial Medullary Thyroid Carcinoma (FMTC)

Epidemiology
– Clinical variant of MEN 2A
– Affected patients suffer exclusively from MTC
Genetics
– Germline mutation in RET proto-oncogene
– Similar codon mutations as those found in MEN 2A; also codons 768 and 790
– Autosomal dominant (AD); located on chromosome 10q11.2
– Neoplastic cell proliferation (C-cell hyperplasia); precursor lesion
– Least aggressive form of MTC
Clinical Presentation
– Often asymptomatic
– Incidentally found
– Presents in second or third decade of life
Diagnosis
– RET mutation
– Thorough family history
– Must demonstrate the absence of pheochromocytoma or
hyperparathyroidism in ≥2 generations within a family
Treatment
– Mostly level 1 risk (codon 918):
– Total thyroidectomy ± central neck dissection
– Add functional neck dissection → if clinical or cytologic evidence of cervical
metastases

6.3.3 Familial Papillary Thyroid Carcinoma (FPTC)

Key Points
– First reported in monozygotic twins in 1955
– Now recognized as a distinct clinical entity
– Prevalence = 5–10% of well-differentiated thyroid cancer
– To date, no identifiable responsible genes, however heritability of FNMTC
(familial non medullary thyroid cancer) considered to be one of the highest of
all carcers

Clinical Presentation
– Neck mass; cervical lymphadenopathy
– May occur as a minor component of other familial cancer syndromes:
– Familial adenomatous polyposis (FAP)
– Gardner Syndrome
– Cowden’s disease
– Carney complex type I

Diagnosis
– Neck ultrasound (U/S)
– Fine needle aspiration (FNA) biopsy
– Genes: to date, none identified
– Cytology: trabecular struma with oxyphilia (some cases)
Screening Recommendations
– all first degree relatives of affected families to be screened; consider screening
of second degree relatives (nearly 50% of second degree relatives also affected)
– fine needle aspiration (FNA) biopsy
– Tumor markers: None
– Cytology: trabecular struma with oxyphilia (some cases)
Treatment
– Total thyroidectomy + prophylactic central neck lymph node dissection
FPTC felt to be more aggressive than sporadic cases:
– Earlier age of onset
– Higher incidence of:
– Multifocality
– Bilaterality
– Nodal involvement
– Intraglandular dissemination
– Extrathyroidal invasion
– Recurrence

6.3.4 Rare Genetic Syndromes Associated with Thyroid Cancer

Key Points
– 5% of well-differentiated thyroid cancers (WDTC) have familial disease, most
of which are NMFTC
– Some rare syndromes associated with WDTC include familial adenomatous
polyposis (FAP), Gardner syndrome, Cowden syndrome, and Carney complex
I
– Inheritance is likely autosomal dominant with reduced penetrance
– Most common type of thyroid cancer associated with these syndromes is PTC

Familial Adenomatous Polyposis (FAP)

– ► Section 3.​3.​3
Epidemiology
– Occurs in approximately 1:10,000 to 1:30,000 live births
– Men:Women = 1:1
– Accounts for <1% of all colorectal cancer cases in the U.S.
– 0.5–2% of cases associated with thyroid cancer; cribriform variant classically
associated with FAP
Genetics
– Germline mutation in the adenomatous polyposis coli (APC) tumor suppressor
gene
– Autosomal dominant (AD); located on chromosome 5q21–q22
– Near complete penetrance of colonic manifestations; variable penetrance of
extracolonic manifestations (◘ Table 6.16).
Table 6.16 Colonic and extracolonic manifestations of familial adenomatous polyposis (FAP)

Colonic manifestation Extracolonic manifestation

Adenomatous colorectal polyps (>100) by second to Gastric polyps: Rarely progress to gastric cancer
third decade of life Duodenal polyps: Up to 12% risk of duodenal
Colorectal carcinoma: 100% of cases (if not treated) cancer
Desmoid tumours
Cysts
Other benign tumors: lipomas, osteomas, fibromas,
adrenal adenomas

Clinical Presentation
– Often asymptomatic
– If symptomatic, linked to iron-deficiency anemia due to occult bleeding.
Diagnosis
– Suspect any patient with ≥10 colorectal polyps on colonoscopy
– Genetic testing for germline mutation in the APC gene = required for
DEFINITIVE DIAGNOSIS
Treatment
– total colectomy
– Excision of desmoid tumors:
– Destruction of adjacent vital intra-abdominal organs
– Leading cause of death in patients with FAP
– Thyroidectomy (if thyroid cancer present)

Gardner Syndrome
Definition and Epidemiology
– Considered a variant of FAP
– Combination of:
– Inherited colonic adenomatosis
– Along with extracolonic lesion (◘ Table 6.17)
Table 6.17 Extracolonic and extraintestinal manifestations of Gardner syndrome

Benign extra-intestinal lesions Extra-colonic malignancies

Osteomas, dental abnormalities Duodenum/periampullary (5%)
Desmoid tumours Thyroid (2%)
Cutaneous lesions Pancreastic (2%)
Adrenal adenomas Gastric (<1%)
Nasal angiofibromas CNS (<1%)
Congenital hypertrophy of the retinal pigment epithelium Hepatoblastoma (2%)
Adrenal (rare)

Genetics
– Germline mutation in adenomatous polyposis coli (APC) tumor suppressor gene
– Autosomal dominant (AD); located on chromosome 5q21–q22 (same as in
FAP)
Clinical Presentation
– Based on which extra-intestinal manifestation(s) present
Diagnosis
– Suspicion in any patient with known FAP and additional extra-colonic lesion:
– Genetic examination for germline mutation of the APC gene = required for
DEFINITIVE DIAGNOSIS
Treatment
– Total colectomy (as in FAP)
– Additional therapy based on presence of other extra-intestinal lesions

Cowden Syndrome
Epidemiology
– First reported in 1963
– Estimated prevalence = 1/200,000–250,000
Genetics
– Germline mutation in phosphatase tensin homolog (PTEN) tumor suppressor
gene
– Autosomal dominant (AD); located on chromosome 10q23
– Other mutations:
– Hypermethylation of the promoter of the Killin (KLLN) gene, also located
on chromosome 10q23
– Mutations in succinate dehydrogenase (SDH) gene, subunits B and D
– Germline PIK3CA and AKT1 mutations
– Characteristic features:
– Enlarged cranium
– Benign tumors on face, hands and feet
– Breast, renal and thyroid malignancies
Clinical Manifestations
– Mucocutaneous
– Distinctive and common manifestation of Cowden syndrome
– Trichilemmomas, acral keratoses, facial papules
– Breast
– Breast cancer = most common malignancy in Cowden syndrome
– Early onset (third and fourth decades of life)
– Lifetime risk = 25–50%
– Thyroid
– Thyroid disease: incidence in >50% of patients
– Risk of thyroid malignancy = 3–35%
– incidence of non-medullary thyroid cancer (NMTC) = 70-fold increased
– Genitourinary
– Endometrial Cancer
– Renal Cell Cancer
– Gastrointestinal
– Gastric, duodenal, colon polyps
– Colorectal cancer
– Other manifestations
– Macrocephaly
– Mental retardation
 – Immune dysfunction
– Vascular tumors
Diagnosis
– Thorough family history
– Genetic testing for PTEN, KLLN or other associated gene mutations
Management
– Genetic counselling
– Cancer surveillance:
– Annual physical exam (PE): with particular attention to skin, breast and
thyroid
– Thyroid ultrasound: beginning at age 18
– Colonoscopy: at age 35, then every 5 years
– Consider renal ultrasound by age 40
Treatment
– Therapy based on organ system(s) involved

Carney Complex I
Epidemiology
– Approximately 600 cases reported to date
– Incidence: Men:Women = 1:1
– Mean age at diagnosis = 20 years
Genetics
– Germline mutation in PRKAR1A gene on chromosome 17q22–q24; codes for
the type 1α regulatory subunit of protein kinase A
– Other associated mutations: PDE11A gene mutation; involved in cAMP
signaling
– Autosomal dominant (AD) inheritance; can also occur sporadically
– Characteristic features:
– Multiple endocrine disorders: Cushing syndrome, adrenocortical hyperplasia,
acromegaly, thyroid gland tumors (often WDTC= “well differentiated
thyroid cancers”)
– Other features: Atrial myxomas, schwannomas, osteochondromyxomas,
pigmented skin/mucosa lesions
Presentation
– Variable:
– Cushing’s
– Acromegaly
– Thyroid gland tumors
– Other lesions (see characteristic features)
– Numerous pigmented lesions (lentigenes, blue nevi), noted during adolescence
Diagnosis
– Detailed patient history with identification of 2 or more of the following: (◘
Table 6.18)
– Genetic testing = gold standard for diagnosis:
– PRKAR1A or PDE11A mutations
– Echocardiogram:
– To detect cardiac myxoma = life-threatening condition!
– Biochemical analysis:
– Hormones: cortisol, insulin-like growth factor, prolactin.
Table 6.18 Diagnostic features of Carney complex I

Endocrine abnormalities Non-endocrine abnormalities

Primary pigmented nodular adrenocortical disease (PPNAD) Cardiac myxomas
Thyroid tumors Lentiginosis
Acromegaly Multiple Blue Nevi
Testicular tumors
Schwannoma
Osteochondromyxoma
Skin myxoma

Treatment
– Treatement directed toward specific symptoms:
– Cardiac myxoma requires open heart surgical removal
– Pituitary adenoma → transphenoidal resection
– thyoid cancer (typically well-differentiated) → Thyroidectomy ± lymph node
dissection

6.3.5 Guidelines
Provenzale D, Gupta S, Ahnen DJ et al. (2018) NCCN Guidelines Insights:
colorectal cancer screening, version 1.2018. J Natl Compr Canc Netw 16:939–
949.
Kloos RT, Eng C, Evans DB et al. (2009) Medullary thyroid cancer:
management guidelines of the American Thyroid Association. Thyroid 19:565–
612.
Wells SA, Pacini F, Robinson BG et al. (2013) Multiple endocrine neoplasia
type 2 and familial medullary thyroid carcinoma: an update. J Clin Endocrinol
Metab 98:3149–3164.
Wells SA, Asa SL, Dralle H et al. (2015) Revised American Thyroid
Association guidelines for the management of medullary thyroid carcinoma.
Thyroid 25:567–610.

6.4 Anatomy and Physiology of the Parathyroid

Gland
F. Billmann

6.4.1 Anatomy
– 4 Parathyroid glands (PG)

Localization
– Variable (embryological):
– Lower PG: Posterior to the inferior pole of the thyroid gland, medial and
anterior to the recurrent laryngeal nerve; wide variability.
– Upper PG: posterior to the superior pole of the thyroid gland, lateral and
superior to the recurrent laryngeal nerve (1 cm around the junction of the
inferior thyroid artery and recurrent laryngeal nerve); relatively constant
– approx. 90% of the PGs in “normal position

Blood Supply
– Inferior thyroid artery (A. thyroidea inferior)
– Variations = A. thyroidea superior/A. thyroidea ima

6.4.2 Physiology (◘ Fig. 6.4)

Parathormone (PTH)
– Production + distribution by PG
– Function: Regulation of calcium-phosphate balance together with vitamin
D3/calcitonin (thyroid gland)
– PTH receptors:
– Type 1: Bones, kidneys, intestines
– Type 2: brain, intestine

Fig. 6.4 Regulation of the calcium balance

Calcium (Ca++)
– Functionally active calcium (Ca++) = non-albumin-bound Ca++ = ionized Ca++
– “CaSR” = Calcium Sensing Receptor (PG cells): Binding site for Ca++
– Regulation of calcium = central to cell signaling pathways, cranial nerve
function, muscle function, bone metabolism
Control Loop (Negative Feedback)
– Accurate control of calcium levels:
– Hypocalcemia → lower Ca++ receptor binding → increase in PTH secretion.
– Vitamin-D3-receptor (PG cells): Vitamin D3 deficiency → secretion of PTH
– PTH → PTH receptor → bone resorption + absorption from small intestine +
reabsorption in kidney → increase Ca++ level
– Increase in Ca++ level → higher Ca++ receptor binding → reduction in PTH
secretion

Normal Levels of Calcium Metabolism

– Serum PTH: 1.5–6.0 pmol/L (12–72 ng/L)
– Serum calcitonin: <2.8 pmol/L (<10 ng/dL)
– Serum calcium (total): 2.15–2.75 mmol/L
– Serum calcium (ionized): 1.0–1.5 mmol/L
– Urine calcium: 4.02–4.99 mmol/L in 24-h urine
– Serum phosphate: 0.84–1.45 mmol/L

6.5 Diseases of the Parathyroid Gland

F. Billmann

6.5.1 Benign Parathyroid Diseases

Primary Hyperparathyroidism (pHPT)
Key Points
– Most common cause of hypercalcemia; prevalence = 1–5/1000 (increasing
with age)
– Often asymptomatic
– Elevated/unexpected normal parathyroid hormone + elevated ionized serum
calcium
– Imaging diagnosis for localization before planned parathyroidectomy
– Indication for parathyroidectomy:
– Symptomatic pHPT patients
– Asymptomatic pHPT patients with compliance with the guidelines (◘ Table
6.20)
– Monitoring impossible
 – Monitoring without therapy = in asymptomatic patients (guidelines) =
obligation of regular controls (progression of the disease)
Definition
– One or more hyperactive PG
– Continuous hypersecretion of PTH
Forms
Sporadic pHPT
– Solitary adenoma (65–85%)
– Multiglandular hyperplasia (10–30%)
– Double adenoma (5–10%)
– Carcinoma (approx. 0.1%)
Hereditary pHPT (► Sect. 6.3)
– Multiple endocrine neoplasia type 1 (MEN 1)
– Multiple endocrine neoplasia type 2A (MEN 2A)
– Neonatal severe hyperparathyroidism (NSHPT; mutation CaSR, chromosome
3q13.3–q21)
– Hyperparathyroidism jaw tumor syndrome (HPT-JT, mutation parafibromin,
chromosome 1q25)
Epidemiology
– Prevalence: 1 per 1000 (USA), 3 per 1000 (Norway), 4.3 per 1000 (Sweden), 2–
4 per 1000 (Germany)
– Incidence: 27–30 new cases/100,000 population years (increases with age)
– Third most common endocrinological disease (after thyroid disease, diabetes
mellitus)
– Women:Men = 3:1
– Average age = 55 years

Caution
Radioiodine therapy = no pHPT incidence increase with currently used
radioiodine doses.

Symptoms
– Associated with hypercalcemia (not with elevated PTH)
– Mostly few symptoms/asymptomatic (truly asymptomatic patients <5%)
– Affected systems ◘ Table 6.19
Table 6.19 Symptoms of pHPT

Bones and muscles Muscle weakness

Myalgia
Bone pain
Osteoporosis/penia
Osteitis fibrosa cystica
Brown tumor
Kidneys Kidney stones/renal colic
Nephrocalcinosis
Dehydration/thirst
Polyuria/oliguria/anuria
Renal insufficiency
Neuropsychiatry Concentration problems
Poor memory
Restlessness
Depression
Confusion
Dementia/Paranoia
Ataxia
Hyporeflexia
Coma
Gastrointestinal tract Nausea/vomiting
Abdominal pain
Anorexia
Ulcer disease
Pancreatitis
Constipation
Weight loss
Cardiovascular system Hypertension
Vascular calcifications
QT interval reduction
Bradycardia
Myocardial block
Lethal arrhythmias
Other Visual changes
Linear keratopathy (corneal calcification)
Conjunctivitis
Pruritus
Caution
Hypercalcemic crisis:
– Metabolic emergency (calcium >3.5 mmol/L)
– Symptoms: Dehydration, metabolic encephalopathy (stupor to coma),
gastrointestinal symptoms (distended abdomen, bloating, constipation,
vomiting, ileus), renal (acute renal failure), cardiovascular symptoms
(tachyarrhythmia)
– Intensive medical treatment (rarely haemodialysis necessary)
Diagnosis
– 2 stages (laboratory diagnosis + imaging diagnosis)
Laboratory Diagnosis (◘ Fig. 6.5)
– Goal = Confirmation of the pHPT
– In serum: Ionized Ca++ + PTH + Vitamin D
– In urine: Ca++ + phosphate + creatinine
– In 24-h urine: Ca++ + phosphate + creatinine (ratio creatinine clearance/Ca++
clearance)
– 2 independent determinations (eventually after vitamin D deficiency
treatment)
– Strategy:
– PTH high/unusually normal = pHPT until proven otherwise.
– Phosphate, electrolytes, creatinine, 24-h urine: calcium and phosphate: to
exclude Differential diagnoses
– s. Normal levels (► Sect. 6.4.2)
Fig. 6.5 Positive diagnosis of primary (pHPT) and secondary hyperparathyroidism (sHPT). DD differential
diagnosis

Diagnostic Imaging
– Goal = Localization: Essential for minimally invasive surgery (= no exploration
of all 4 parathyroid glands)
– Standard = Ultrasound (US) + Scintigraphy
– Ultrasound (linear transducer 7.5–15 MHz)
– Adenoma: Hypoechogenic, peripheral blood circulation (Doppler)
– Possibility of US-guided fine needle aspiration (FNA)
– Scintigraphy:99 m Tc-Sestamibi-Scintigraphy
– SPECT (Single Photon Emission Computed Tomography): Tomographic
examination
– Dual99 m Tc- and123 I-scintigraphy: contrast enhancement by subtraction
imaging (subtraction of thyroid uptake)
– 4D-CT/MRI (not first-line method)
– Fusion possible: SPECT + CT, SPECT + MRT, PET + CT
– Invasive investigations (not first-line methods): Selective vein sampling;
Selective angiography; Fine needle aspiration (FNA)
Genetic Workup
– In young patient <40 years with pHPT + multiglandular disease + family
history or syndromic signs

Ectopic Parathyroid Glands

– Ectopic location = 4–16% of cases
– Necessity of a standardized neck exploration (localization OP technique)
– If adenoma not identifiable by exploration: abort surgery + need for further
imaging (MRI, CT, SPECT, selective vein sampling)

Differential Diagnosis (◘ Fig. 6.6)

Secondary (sHPT), Tertiary HPT (tHPT) (See Below)
Familial Hypocalciuric Hypercalcemia (FHH)
– Mutation of CaSR = loss of function of the receptor (kidney and parathyroid
gland) leads to a reduction in calciuria
– Findings: hypercalcemia, PTH normal or high, 24-h calciuria decreased,
Ca++/Crea clearance <0.01

Fig. 6.6 Laboratory test results in hyper- and hypoparathyroidism

Milk-Alkali Syndrome
– Caused by an excess of easily absorbable alkalis (e.g. bicarbonates) + calcium
(e.g. milk)
– Findings: hypercalcemia, metabolic alkalosis, decreased PTH, impaired renal
function
Lithium Therapy
– Especially psychiatry: depression, mania, schizophrenia, cluster headaches
– Elevated PTH, increased bone turnover
Malignancy-Associated Hypercalcemia
– Calcium release by osteodestructive metastases of a malignant tumor or by
tumor production and release of a parathyroid hormone-related peptide
(paraneoplastic; PTHrP)
– Findings: Elevated tumor markers, elevated PTHrP and calcitriol
Granulomatous Disease
– e.g. sarcoidosis, berylliosis, tuberculosis, histoplasmosis
– Renal involvement = increased hydroxylation of vitamin D = hypercalcemia
(increased absorption and reabsorption of calcium)
– Findings: hypercalcemia, PTH decreased, 24-h calciuria decreased
Endocrinopathies
– e.g. hyperthyroidism, adrenal insufficiency, pheochromocytoma, VIPoma
– Increased bone turnover
– Findings: Elevated calcium, low PTH
Drugs
– e.g. thiazides, vitamin D, calcium, vitamin A intoxication
– Elevated PTH, increased bone turnover.
Immobilization, Bed Rest
– Lack of stress on the musculoskeletal system = increased osteoclastic activity =
hypercalcemia

Therapy
Indications for Medical Therapy
– OP criteria not met
– Patient not fit for anaesthesia/surgery
– Monitoring absolutely necessary: Ca++ + creatinine + PTH control every 6–12
months (once PTH target level is reached).
– Therapeutic options:
– Hormonal therapy (especially postmenopausal women): e.g. raloxifene 60
mg daily
 – Bisphosphonates (e.g. alendronate): 70 mg once a week
– Calcimimetic (e.g. Cinacalcet): Starting at 30 mg/day (increase until PTH
goal is reached).
– Dietary recommendations: Sufficient water intake (at least 2–3 L/day),
calcium intake <1000 mg/day.
Indications for Surgical Therapy
– Symptomatic pHPT = always OP indication
– If monitoring is not desired/not possible
– For neurocognitive/neuropsychiatric symptoms
– Asymptomatic pHPT (75% of patients): Early surgery if OP criteria are met (◘
Table 6.20), for prophylaxis of morbidity/mortality
– Multiglandular hyperplasia: persistence and recurrence rate high; sporadic or in
the context of MEN; therapy = subtotal parathyroidectomy (± cryopreservation)
– Ectopic PG (up to 4–16% of patients): Treatment success depending on
treatment algorithm (◘ Fig. 6.7)
Table 6.20 Criteria for determining the indication for surgery in asymptomatic pHPT patients. (According to
Wang and Udelsman 2007 and 2016 AAES guideline)

Measured level Criteria for surgery Monitoring without surgery

Serum calcium (above the >1.0 mg/dL (0.25 mmol/L) above the Annual
upper normal level) upper normal level
24 h urine calcium >400 mg/day (>10 mmol/day) or Annual
nephrocalcinosis on imaging
Creatinine clearance Reduced <60 mL/min Annual
Bone density T-score < 2.5 (lumbar, femoral, or wrist), Every 1–2 years (lumbar,
and/or fracture risk femoral and wrist)
Age <50 years –
Fig. 6.7 Treatment algorithm for intraoperative identification of missing parathyroid glands (failure to find a
PG). (After Wang and Udelsman 2007)

Guideline Criteria for Determining the Indication for Surgery (◘ Table

6.20)
– Progressive disease (= only 25% of asymptomatic patients)
– Weighing morbidity/mortality of surgery vs. expected benefit
– If monitoring is not desired/not possible
– For neuropsychological/neuropsychiatric symptoms
– Caution: in Germany only S1 guideline

Operative Therapy Principles

– Bilateral neck exploration
– Indication: No definite localization, or suspicion of 4-gland hyperplasia
– Principle = exploration/visualization of all 4 parathyroid glands
– Resection of enlarged, abnormal TG (based on morphology)
– Intraoperative aids: Intraoperative PTH measurement, intraoperative frozen
section, localization by gamma probe
– Minimally invasive parathyroidectomy:
– Current method of choice
– Only if positive localization (sonography, sestamibi scan)
– Intraoperative aids: Intraoperative PTH measurement, intraoperative frozen
section, localization by gamma probe

Surgical Therapy
– Need for careful hemostasis: “Only a dry situs guarantees the identification
of the parathyroid glands”
– Advantage operative vs. medical: improvement of bone density and mass:
back to baseline (lasts up to 10 years postoperatively)
– Early: prevention of cardiovascular morbi/mortality

– If 3 or more PGs are enlarged or PTH does not decrease adequately after
removal of the first PG = 3½-gland resection or total parathyroidectomy +
autotransplantation of part of the most inconspicuous PG into
sternocleidomastoid/brachioradialis muscle
– Postoperative aftercare
– Postoperative calcium controls (1 time daily)
– Search for clinical signs of hypocalcaemia (see “Hypoparathyroidism”
below)
– Eventual substitution of temporary hypocalcemia:
– Calcium per os 1.5–3 g/day +1,25-dihydroxy vitamin D3 0.5–2 μg/day
– i.v. calcium administration in case of persisting symptoms
– Before discharge: PTH determination + videolaryngoscopy

Surgical Procedure
Bilateral Neck Exploration
– General anaesthesia (rarely locoregional anaesthesia possible)
– Extension of the cervical spine, roll or vacuum mattress under the shoulders
– Access: 4–5 cm Kocher collar incision, in skin fold approx. 1 finger width
above the jugular notch (preoperative marking)
– transection of the platysma muscle, formation of a subplatysmal flap (cranial
retraction by suture)
– Opening of the linea alba and retraction of the strap muscles laterally
– Start surgery on the side of the localization (if negative localization, start on
the right side)
– Visualization of the thyroid gland, ligation and transection of the middle
thyroid veins, mobilization of the thyroid gland anteromedially
– Identification and sparing of the recurrent laryngeal nerve and inferior
thyroid artery
– Lower PG = usually in the cervical thymus; upper PG = usually in a 1-cm
radius around the point where the recurrent laryngeal nerve inserts into the
cricothyroid membrane.
– Removal of the conspicuous PG (+ intraoperative PTH determination +
frozen section examination of the specimen)

Surgical Procedure
Minimally Invasive Open Parathyroidectomy
– Local/regional anaesthesia/general anaesthesia
– Moderate extension of the cervical spine
– Access: 2–4 cm Kocher collar incision, in skin fold approximately 1 finger
width above jugular notch (preoperative marking)
– transection of the platysma muscle + formation of a subplatysmal flap
(cranial retraction by suturing)
– Opening of the linea alba + retraction of the strap muscles to the lateral side
– Targeted preparation in the area of localization
– Mobilization of the thyroid gland (transection of the middle thyroid veins not
necessary)
– Identification + protection of the recurrent laryngeal nerve and the inferior
thyroid artery
– Visualization of the conspicuous PG, mobilization, ligation of the blood
supply and excision of the adenoma
– Intraoperative PTH measurement

Surgical Procedure
Minimally Invasive Video-Assisted Parathyroidectomy (MIVAP)
 – General anesthesia
– Thyroid positioning: Moderate extension of the cervical spine
– Access: 15 mm transverse neck incision, approx. 2 cm above the jugular
notch (preoperative marking); lateral access possible in case of reoperation
– transection of the platysma muscle
– Opening of the linea alba over 3 cm + retraction of the strap muscles to the
side
– Targeted dissection in the area of localization, mobilization of the thyroid
gland + transection of the middle thyroid veins
– Insertion of a 5 mm 30° endoscope through the access and purely endoscopic
continuation of the operation
– 2–3 surgeons required for surgery: Surgeon, first assistant (laparoscope
guidance + suction), second assistant (holding the retractor).
– Identification + protection of the recurrent laryngeal nerve and the inferior
thyroid artery (easier due to laparoscopic magnification effect)
– Visualization of the conspicuous PG, mobilization, ligation of the blood
supply and exheresis of the adenoma
– Intraoperative PTH measurement

Complications (Section “Postoperative Complications”)

Reoperation
– Indications
– pHPT persistence: persisting hypercalcemia after neck
exploration/recurrence of hypercalcemia within 6 months of initial surgery.
– pHPT recurrence: recurrence of hypercalcemia later than 6 months after
initial surgery
– Causes of persistence/recurrence ◘ Table 6.21
– Preoperative strategy ◘ Table 6.22
– Surgical strategy ◘ Table 6.23
Table 6.21 Causes of pHPT persistence or recurrence

pHPT Persistence Failure to identify or resect the PG adenoma

Failure to identify or resect all adenomas or 4-gland hyperplasia
Inadequate subtotal resection of a 4-gland hyperplasia
Subtotal resection of a parathyroid adenoma
Residual or metastatic parathyroid carcinoma
Parathyromatosis
Inadequate percutaneous ablation
pHPT Recurrence Recurrent growth of hyperplastic PG tissue (especially familial pHPT)
Recurrent growth of autotransplanted PG tissue
Recurrence of PG carcinoma or metastatic PG carcinoma
Parathyromatosis

Table 6.22 Preoperative strategy in case of reoperation

1. Reconfirmation of the diagnosis Complete medical history and clinical examination again
Repetition of laboratory diagnosis
Exclusion of a differential diagnosis (see Differential
diagnoses, ◘ Fig. 6.6)
2. Re-evaluation of the old localization (Essential information for planning the surgical strategy and
diagnosis, operation report, pathology the possible location of the adenoma)
findings
3. Planning of additional localization (Only if 1. and 2. did not provide further information)
examinations Ultrasound + Sestamibi
4D CT/MRI
Selective vein sampling
Selective arteriography
4. Discussion of the operative risk with the Injury to recurrent laryngeal nerve during reoperation 4–20%
patient (preoperative laryngoscopy); if bilateral, need for
tracheostomy
Transient/permanent postoperative hypocalcemia (transient:
10–20%, permanent: 5–10% after reoperation)

Table 6.23 Surgical strategy for reoperation

1. Selection of an adequate time Ideal time window: within the first week or 3 months after initial
window and adequate access for the surgery
reoperation Optimal strategy = targeted minimally invasive re-exploration
(reducing the risk of laryngeal recurrent nerve injury and
postoperative hypocalcaemia)
Lateral access (“back door”): due to adhesions in the central neck
Planning of the surgery on the basis of the localization diagnosis,
the operation and pathology report
2. Exploration of ectopic and unusual Most unidentified PG (40%) are found in normal anatomical
PG localizations localization
◘ Fig. 6.7 in the other cases
3. Consider intraoperative localization Intraoperative PTH measurement
methods Intraoperative ultrasound examination
Gamma probe examination (with radioactively labelled sestamibi)

Secondary Hyperparathyroidism (sHPT)

 Key Points
– Renal etiology = 90% of patients with sHPT
– Mostly asymptomatic
– sHPT: elevated parathyroid hormone + low/normal ionized serum calcium;
caution: differential diagnoses
– Primary therapy = conservative, medical
– Indications for surgical therapy:
– Failure of conservative therapy
– Hypercalcemia
– Normocalcemia, if PTH > tenfold elevated, refractory hyperphosphatemia,
advanced renal osteopathy, spontaneous fractures, calciphylaxis
Definition
– Compensatory hypersecretion of PTH in response to hypocalcemia
Etiology (◘ Table 6.24)
– Always outside the PG (= malfunction of one/several components of the
calcium control loop)
Table 6.24 Differential diagnosis of sHPT

Gastrointestinal causes Inadequate diet Food intolerance

Dietary restriction
Phytic acid (legumes, cereals, oilseeds)
Malabsorption Celiac disease
Pancreatic diseases (exocrine pancreatic
insufficiency)
Inflammatory bowel disease
Cystic fibrosis (mucoviscidosis)
Gastric bypass surgery
Cortisone therapy
Age
Vitamin D-associated Lack of sunlight Dark skin in northern latitudes
causes Cultural habits, clothing
Inadequate diet Vegan or lactovegan diet
liver or biliary disease Malabsorption, 25-hydroxylase deficit.
Liver cirrhosis
Cholestasis
Antiepileptic therapy Modified vitamin D metabolism
Vitamin D-dependent Hypophosphatemia
osteomalacia
Nephrological causes Chronic kidney disease Hyperphosphataemia
1α-hydroxylase deficiency: Decreased 1,25-
dihydrovitamin-D
Reduction of PTH clearance: (C-terminal)
PTH resistance
Cellular/tissue-associated Bones Growth
causes
Genetic causes Pseudohypoparathyreoisism Abnormal PTH receptor G protein/PTH
resistance(s)
“Hungry Bone
Syndrome”
Bisphosphonate therapy
Lactation, postlactation
period
Metastatic prostate Kidneys Diuretics
carcinoma Increased natriuresis
Idiopathic hypercalciuria
Soft Tissue Rhabdomyolysis: calcium deposition,
hyperphosphatemia, acute renal failure.
Acute pancreatitis
Sepsis

Renal sHPT
– 90% of patients with sHPT
– Renal insufficiency → increased phosphate level → inhibition of renal calcitriol
production → decreased serum Ca++ (hypocalcaemia) → stimulation of PTH
production and release
– Renal insufficiency → hyperphosphatemia, acidosis, hyperuricemia →
inhibition 1α-hydroxylase → hypocalcemia → compensatory hypersecretion of
PTH + hyperplasia of PG

Extrarenal sHPT (= Differential Diagnosis) (◘ Table 6.23)

Symptoms
– Initially no symptoms
– Symptoms of pHPT (see above “Primary hyperparathyroidism”)
– Extraosseous calcifications (perarticular with gout-like symptoms, vessels,
kidneys, myocardium)
– Calciphylaxis: extreme form of extraosseous calcifications (vessels, subcutis)
→ vasculitis + paniculitis → necrosis
Diagnosis (◘ Fig. 6.5)
– Same as in pHPT (see above “Primary hyperparathyroidism”)
Therapy
Conservative/Medical = Primary Therapy of sHPT
– Compensation of hyperphosphatemia: diet (reduction of intake) + phosphate
binders + withdrawal by dialysis
– Calcium administration as required
– Vitamin D3 substitution
– Calcimimetics (Cinacalcet): Receptor blockade of parathyroid cells (=
prevention of PTH release).
Surgical Therapy
– Indication for surgery: Only 5% of patients with sHPT
– Indications:
– Failure of conservative/medical therapy
– Hypercalcemia = absolute indication for surgery
– Normocalcemia and:
– PTH > tenfold above the norm
– Hyperphosphatemia resistant to therapy with extraosseous calcifications
or
– Advanced renal osteopathy or
– Bone pain refractory to treatment or
– Spontaneous fractures or
– Calciphylaxis
– Standard operations
– Subtotal parathyroidectomy: Smallest PG is left in situ with intact circulation
(recurrence risk = 16%)
– Parathyroidectomy + simultaneous orthotopic/heterotopic autotransplantation
Postoperative Follow-Up: Like pHPT (See Above “pHPT”)

Surgical Procedure
Parathyroidectomy with Simultaneous Orthotopic/Heterotopic
Autotransplantation
 – General anaesthesia (rarely locoregional anaesthesia possible)
– Extension of the cervical spine, roll or vacuum mattress under the shoulders
– Bilateral neck exploration (see above)
– A portion of the least modified PG is divided into small, 1-mm pieces =
implantation into the brachioradialis muscle of the non-shunt-bearing
forearm; clip marking
– Thymectomy + central LK dissection (due to possibility of supernumerary
PGs).

Tertiary Hyperparathyroidism (tHPT)

Definition
– PG function escaping the negative feedback within the framework of an sHPT
– Consequence of chronic sHPT (= PG hyperplasia):
– Under-expression of CaSR
– Reduction of vitamin D receptors
Etiology
– Chronic kidney disease (= most frequent cause)
– X-linked dominant hypophosphatemic rickets
– Autosomal dominant hypophosphatemic rickets
Therapy
– Always surgical therapy = parathyroidectomy + simultaneous
orthotopic/heterotopic autotransplantation (see above “Surgical procedure”)
– Postoperative follow-up like pHPT (see above “pHPT”)
After kidney transplantation (= etiological treatment of sHPT)
– PG hyperplasia remains (= increased PTH secretion)
– Development of PTH-induced hypercalcemia in 1/3 of patients after kidney
transplantation = risk for kidney transplant function!

Hypoparathyroidism
Epidemiology
– Incidence: Very controversial (especially for postoperative hypoparathyroidism)
Symptoms
– Symptoms of hypocalcemia:
– Paresthesias
 – Muscle spasms (up to tetany) (Chvostek/Trousseau sign)
– seizures (especially in acute hypoparathyroidism)
– Chronic hypocalcemia: Mostly and long asymptomatic
Chvostek/Trousseau sign:
– Chvostek: contraction of facial muscles on tapping the facial nerve trunk (1 cm
ventral to the ear lobe).
– Trousseau: carpopedal spasm (paw position) after inflation of a blood pressure
cuff above systolic pressure for 3 min
Diagnosis
– Serum Ca++ + PTH levels
Etiology
– Intraoperative iatrogenic damage to the PG
– Damage to PG blood flow
– Unintentional parathyroidectomy
– Other: Developmental defect of parathyroid gland: autoimmune, genetic (e.g.,
mutation PTH gene).
Therapy
– Calcium + vitamin D (or vitamin D analogues)
– Thiazides: stimulation of renal calcium reabsorption
– In acute hypoparathyroidism (e.g. postoperative hypoparathyroidism): i.v.
calcium and vitamin D administration
– PTH substitution in case of failure of other treatments

6.5.2 Parathyroid Cancer

Epidemiology
– Women:Men = 3:1
– Frequency peak: 40–60 years of age
– approx. 0.1–0.5% of HPT patients

Symptoms
– Symptoms of pHPT

Diagnosis
– Very high PTH levels
– Rarely preoperative diagnosis, mostly intraoperative (= infiltration of the tumor
into the surrounding area)/postoperative diagnosis (= histology)

Therapy
– Always surgical therapy
– Parathyroidectomy + ipsilateral hemithyroidectomy
– En bloc resection of the infiltrated soft tissues
– Systematic ipsilateral lymphadenectomy
– Postoperative follow-up like pHPT (see above “pHPT”)
– Palliative approach: chemotherapy ± radiotherapy

6.5.3 Guidelines
Bilezikian JP, Brandi ML, Eastell R et al. (2014) Guidelines for the management
of asymptomatic primary hyperparathyroidism: summary statement from the
Fourth International Workshop. J Clin Endocrinol Metab 99:3561–3569.
Wilhelm SM, Wang TS, Ruan DT et al. (2016) The American Association of
Endocrine Surgeons Guidelines for definitive management of primary
hyperparathyroidism. JAMA Surg 151:959–968.
German AWMF S1 guideline, registry no. 174–006. primary
hyperparathyroidism. ►https://​www.​awmf.​org/​uploads/​tx_​szleitlinien/​174-006l_​
S1_​primaerer-Hyperparathyreoi​dismus_​2016-05.​pdf

6.6 Anatomy and Physiology of the Adrenal Gland

F. Billmann

6.6.1 Embryology
– Each adrenal gland = 2 different glands in one capsule
– Adrenal cortex (cortex)
– Adrenal medulla
Aberrant adrenal tissue is possible along the migratory route of the urogenital
crest.
Extraadrenal chromaffin cells: Persistence of part of these cells = Zuckerkandl
organ (usually left of aortic bifurcation) + possibly paraganglion.

6.6.2 Anatomy
Topographic Anatomy
– Pairs gland (endocrine cells)

Location
– Retroperitoneal position
– Superomedially from the upper pole of the respective kidney
– Right adrenal gland:
– Often contact with right crus of the diaphragm
– Close to inferior vena cava
– Left adrenal gland:
– Between kidney and aorta
– Contact/relationship with pancreas tail + splenic artery
Adrenal glands = usually not directly visible in the retroperitoneum. Therefore
dissection + mobilization of the adjacent organs necessary.

Blood Supply
– Arterial blood flow:
– Branches of the inferior phrenic artery + renal artery + aorta
– Right adrenal gland: superior + inferior adrenal arteries
– Left adrenal gland: middle + inferior adrenal arteries
– Venous drainage:
– Right adrenal vein: drainage into inferior vena cava (short vein)
– Left adrenal vein: drainage into the left renal vein (variant: directly into the
inferior vena cava)
– Lymphatic drainage:
– Lymphatic plexus of the adrenal gland
– Drainage: Paraaortic + renal lymph node groups

Innervation
– Cortex: No direct innervation
– Medulla: Through preganglionic sympathetic nerves
– No parasympathetic component

Histological Anatomy
Adrenal Cortex
– 3 zones:
– Zona glomerulosa (outer zone)
– Zona fasciculata (intermediate zone)
– Zona reticulata (inner zone)
Adrenal Medulla
– 10% of the weight of the adrenal gland
– Cells: Content = catecholamines (fix chromium salts = chromaffin cells).

Positional Relationships
– Right adrenal gland:
– Posterior: right diaphragmatic crus, diaphragm
– Anterior: right lobe of the liver
– Medial: Inferior vena cava (medial and anterior)
– Cranial: diaphragm (cranial and posterior)
– Caudal: Right renal upper pole
– Left adrenal gland:
– Posterior: Left diaphragm, kidney
– Anterior: peritoneum from the bursa omentalis (further: stomach, spleen)
– Medial: left inferior phrenic artery, left gastric artery
– Lateral: Left kidney
– Cranial: diaphragm (cranial and posterior)
– Caudal: pancreatic tail and splenic artery

6.6.3 Physiology
– Adrenal glands: Synthesis + secretion of 2 groups of hormones:
– Steroid hormones (adrenal cortex)
– Catecholamines (adrenal medulla)

Steroid Hormones
Glucocorticoids
Effects of Glucocorticoids
Fig. 6.8 Feedback regulation of glucocorticoid production and secretion in humans

Regulation (◘ Fig. 6.8)

– On almost all organ systems of the human body
– Alteration of the metabolism of proteins, lipids, carbohydrates: increase of
blood sugar
– Preservation of intravascular volume, blood pressure
– Sensitization of β-adrenergic stimulation.
– Anti-inflammatory and immunosuppressive effect
– To summarize: Glucorticoids = response to stress
– Short-term exposure = anabolism
– Long-term exposure = catabolism
10–15% of cortisol in plasma unbound = active.
Mineralocorticoids
Effects of Mineralocorticoids

Fig. 6.9 Feedback regulation of mineralocorticoid production and secretion in humans

Regulation (◘ Fig. 6.9)

– Regulation of water + electrolyte balance: expansion of intravascular volume
40% of aldosterone in plasma unbound = active.
Adrenal Sexual Steroids
Effects of Adrenal Sexual Steroids
– Regulation ACTH-dependent
– Other mechanisms (not yet fully understood)
Regulation
– In adults: Mild peripheral effects:
– Development of secondary sexual characteristic features in men
– female virilization
– During development:
– development of the external male genitalia (vas deferens, epididymis,
seminal vesicles, prostate)
– During puberty:
– Development phallus, muscle mass, body hair
– Effects after peripheral transformation to testosterone
– Absence of androgens = development of female genitalia + vagina
– Adrenal gland = secondary source of secretion of sexual steroids (main source =
gonads)
Catecholamines
Regulation
– Basal state: Low catecholamine secretion
– Autonomic sympathetic nervous system: stimulation = catecholamine secretion
– Activation of the autonomic sympathetic nervous system = response to stress
– Physiological stress
– Psychological stress
Effects of Catecholamines
– β-adrenergic effects:
– Heart (β1-receptors): Increase heart rate, increase contractility
– Smooth muscle (uterus, bronchi, muscle vessels; β2-receptors): Smooth
muscle relaxation (= bronchodilation, increase muscle blood flow, etc.).
– α-adrenergic effects:
– Vessels (skin, GI tract, etc.; α1-receptors): Vasoconstriction
– Presynaptic receptors (CNS; α2-receptors): Reduction of sympathetic outflow
Action of the catecholamines
– Increase of blood supply and oxygen supply to brain, heart, muscle (“fight-and-
flight response”)
– Reduction of the supply to the other organs

6.7 Diseases of the Adrenal gland

F. Billmann
– In this chapter only adrenal diseases treated by surgery will be presented.

6.7.1 Primary Hyperaldosteronism (Conn Syndrome)

Definition
– Hypersecretion of aldosterone (► Sect. 6.6.3)
– Etiologies within or outside the adrenal gland

Etiopathogenesis
Etiologies
– Two main etiologies:
– Unilateral aldosterone-producing adrenal adenoma (= Conn’s adenoma) =
60%
– Bilateral adrenal hyperplasia or idiopathic hyperaldosteronism = 40%
– Rare etiologies:
– Unilateral primary adrenal hyperplasia
– Adrenocortical carcinoma (aldosterone-producing)
– Familial hyperaldosteronism
– aldosterone-producing ovarian tumor

Pathogenesis (► Sect. 6.6.3)

Clinical Presentation
– Mostly mild/unspecific
– Most common symptoms:
– Headache
– Muscle weakness
– Fatigue
– Polydipsia, polyuria and nocturia
– Arterial hypertension: Almost always, but mild (diastolic blood pressure
usually <120 mmHg)

Diagnosis (See Algorithm ◘ Fig. 6.10)

Laboratory Adrenal Function Tests
– To confirm primary hyperaldosteronism
Fig. 6.10 Diagnostic algorithm in patients with suspected primary hyperaldosteronism

Caution
Discontinue all diuretics 2 weeks prior to blood sampling in cases of suspected
hyperaldosteronism.

– aldosterone in plasma
– Plasma aldosterone/renin ratio
Aldosteronemia >20 ng/dL + plasma aldosterone/renin ratio > 30
(ng/dL:ng/mL/h); sensitive + specific in screening and diagnosis.
– Salt suppression test or 3-day sodium test: If aldosterone/renin not conclusive
Etiological Diagnosis
– To distinguish unilateral adenoma vs bilateral hyperplasia vs rare etiologies.
– Central to the decision-making process of therapeutic strategy:
– Unilateral Conn’s adenoma: surgery
– Bilateral hyperplasia: non-surgical (medical) therapy
Diagnostic Imaging
– CT abdomen or MRI abdomen
– Visualization of a tumour/mass of the adrenal gland (suspicion of adenoma)

Caution
Adrenal tumour may also be nonfunctional (2–8%).

Selective Venous Sampling

– Invasive examination (only in centres with expertise)
– Localization (right/left) of aldosterone hypersecretion
– Indication:
– None/Small adrenal tumor on imaging
– Bilateral tumour of the adrenal gland
– Complications:
– Adrenal Vein Thrombosis
– Adrenal Infarction
Therapy
– Depending on etiology
Medical (Drug) Therapy
– In bilateral hyperplasia/idopatic hyperaldosteronism
– Spironolactone (aldosterone antagonist) = therapy of arterial hypertension
– Possible combination with other antihypertensive drugs
Surgical Therapy
– Indication = aldosterone-producing adenoma
– Preoperative Preparation:
– Spironolactone: normalization hypertension
– Potassium substitution: normalization of electrolyte/fluid balance
– About 3–4 weeks
– Technique: (► Sect. 6.7.7) Minimal-invasive surgery
 Aldosterone-producing adenomas = mostly small + benign: minimally
invasive surgery = ideal modality.
– Low morbidity (fewer postoperative complications)
– Analogue success rate (vs. open)
Results
– Cure of hypokalemia: Almost all patients
– Cure of hypertension: 70% of patients (30% need further antihypertensives)

6.7.2 Cortisol-Producing Adrenal Adenoma

Definition
Cushing’s Syndrome
– Hypercortisolism (overproduction of cortisol)
– Different etiologies (see below)
Exogenous steroid use = most common cause of Cushing’s syndrome.
Cushing’s Disease
– Hypercortisolism (overproduction of cortisol)
– Small pituitary adenoma: stimulation of the normal adrenal gland
ACTH Syndrome
– Ectopic ACTH secretion (outside the pituitary gland; 15% of Cushing’s cases)
– Mostly malignant tumors (lung, pancreas, carcinoid tumor, thymoma)

Epidemiology and Etiology

– Exogenous steroid use (most common cause!)
– Endogenous causes:
– Cushing’s disease (pituitary adenoma, 70% of endogenous causes)
– ACTH syndrome due to ectopic ACTH secretion (malignant tumors; 15% of
endogenous causes)
– Cortisol-producing adrenal disease (10–20% of endogenous causes).
– Adrenal adenoma (50–60% of cases)
– Bilateral adrenal hyperplasia (20–30% of cases)
– Adrenocortical carcinoma (20–25% of cases)
– Primary adrenal hyperplasia
– Ectopic CRH (corticotropin releasing hormone) syndrome
Clinical Signs
– Weight gain = most common sign: Mostly on the trunk = centripetal obesity.
– muscular atrophy of the extremities
– Fat deposits on head (“moon face”) + neck
– Dorsal kyphosis (“bull neck” = “buffalo hump”)
– Abdominal striae (dark red, broad)
– Hypertonus
– Hyperglycemia
Subclinical Cushing’s syndrome: Absent or poorly developed clinic, usually in
patients with adrenocortical tumors.

Diagnosis
Laboratory Adrenal Function Tests
– To confirm Cushing’s syndrome
Diurnal variation in cortisol secretion: cortisol high early morning, low
evening: important for test interpretation.
Overnight Cortisol Suppression Test
– Most sensitive diagnostic test
– Principle: p.o. administration of 1 mg dexamethasone at 22 h or 23 h + cortisol
determination in the blood the next morning at 8 h
– False negative = 3%; false positive = 30%.
– Interpretation:
– Suppression (cortisol ≤5 μg/dL): Reliable exclusion of hypercortisolimsus
– Absence of suppression: suspicion of hypercortisolism
Free Cortisol 24 h Urine Test
– In patients with suspected hypercortisolism in the suppression test
– Less sensitive, higher specificity
– Normal: Urinary cortisol <80 μg/day
48-h Low-Dose Dexamethasone Test
– For patients with ambiguous results
– Administration of 0.5 mg dexamethasone every 6 h for 2 days
– Determination of pre- and post-dexamethasone 24-h-cortisol in urine
– Interpretation:
– No suppression: Autonomous cortisol secretion
 Patients with adrenal incidentaloma: Always perform tests to exclude
Cushing’s syndrome.

Etiological Diagnosis
– To find the etiology
ACTH in Plasma
– ACTH secretion: diurnal variations parallel to cortisol (approx. 1–2 h earlier)
– Suppressed ACTH in patients with:
– Adrenal Adenoma
– Adrenocortical carcinoma
– Cortisol-producing bilateral adrenal hyperplasia
– ACTH elevated/upper normal range: Cushing’s disease
– ACTH markedly/very elevated: Ectopic ACTH secretion (tumor)

Therapy
– Depending on etiology

Surgical Therapy
Cushing’s Disease
– Transsphenoidal pituitary adenoma resection (if resectable)
– Bilateral adrenalectomy:
– In patients with no improvement after drug therapy + transsphenoidal
pituitary adenoma resection.
– In patients with end-organ insufficiency in relation to hypercortisolism.

Caution
In the case of bilateral adrenalectomy: perioperative steroid therapy (◘ Table
6.25) + lifelong substitution.
Table 6.25 Recommendations for perioperative steroid therapy

Surgical Example Hydrocortisone Equivalency Duration

stress (mg). (days)
Small Hernia surgery 25 1
Medium Open cholecystectomy 50–75 1–2
Revascularization of the lower
extremity
Segmental colon resection
Surgical Example Hydrocortisone Equivalency Duration
stress (mg). (days)
Total joint replacement
Abdominal hysterectomy
Large Pancreaticoduodenectomy 100–150 2–3
Esophagogastrectomy
Total proctocolectomy
Cardiac surgery + cardiopulmonary
bypass

Ectopic ACTH Secretion Syndrome

– Identification of the secreting tumor + treatment
– Bilateral adrenalectomy: Only if tumor irresectable or consequences of
hypercortisolism not treatable with medication
Cushing’s Syndrome in Adrenal Tumor (Adenoma or Carcinoma)
– Unilateral adrenalectomy (of the affected side)
– Adenoma: Almost all adenomas = resectable
– Adrenocortical carcinoma: resectable in only 25–35% of cases
Medical (Drug) Therapy
– Chemotherapy: Poor results
– In case of metastases/non-resectable tumor: drugs with direct effect on adrenal
gland or on steroid synthesis (mitotane, aminoglutethimide, metyrapone,
ketoconazole)

6.7.3 Pheochromocytoma
Definition
– Neuroectodermal tumor; from chromaffin cells of the adrenal medulla
– Treatable form of endocrine hypertension
– Secretion of catecholamines: symptoms
– High morbidity/mortality if not treated

Epidemiology
– Incidence = 0.005–0.1% of the general population
– Incidence = 0.1–0.2% of hypertensive adults
– Bilateral tumor = 10% of cases; possibility of multiple tumors
– Extraadrenal localization: 10% of cases (= paragangliomas)
– Non-functional pheochromocytomas = rare (mostly extraadrenal)
– Malignant pheochromocytoma: 10% of cases (metastases: bone, liver, lung, less
frequently lymph nodes)
– 5-year survival rate:
– Benign phaeochromocytoma = 97%
– Malignant pheochromocytoma = 43%
– Familial pheochromocytoma: 10% of cases (currently more like 25%):
– MEN 2A or 2B (► Sect. 6.3): Often bilateral pheochromocytoma
– Neurofibromatosis type I or MEN 1 (► Sect. 6.3): Pheochromocytoma risk
<1%
– Hereditary paraganglioma syndrome (mutations in SDHD, SDHB and SDHC
genes)
In familial pheochromocytoma: Mandatory follow-up + regular screening.

Clinical Signs
– Variable clinical presentation: Over time and from one patient to another (up to
dramatic situations)
Arterial Hypertension
– Constant hypertension + paroxysmal peaks (variable frequency and severity)
– Paroxysmal symptoms due to e.g. physical stress, food containing tyramine
(chocolate, cheese, red wine)
Other Symptoms
– Excessive sweating
– Tachycardia
– Trembling
– Inner restlessness
– Thoracic pain
– Impaired glucose tolerance: with diabetes mellitus signs (polydypsia, polyuria)
Impaired glucose tolerance = consequence of catecholamine secretion.

Diagnosis
Laboratory Function Tests
– Confirmation of excessive catecholamine secretion
– In the blood:
– Free metanephrines in plasma: more sensitive than punctual metanephrines
in urine
– In the urine:
– Free catecholamines + metabolites in 24-h urine: to confirm elevation in
plasma
– Free catecholamines: Dopamine, epinephrine, norepinephrine…
– Metabolites: normetanephrines, metanephrines, vanillinmandelic acid
– Elevated levels in more than 90% of patients with pheochromocytoma
Phenylethanolamine-N-transferase = enzyme only in adrenal gland:
conversion of norepinephrine to epinephrine; as consequence: extraadrenal
pheochromocytoma = no epinephrine production.
Etiological Diagnosis = Localisation Diagnosis
– Goal = localization of the pheochromocytoma
– Only if laboratory diagnosis is confirmed (see above)
CT Scan
– Imaging of first choice for suspected pheochromocytoma
– Detects 95% of tumors >6–8 mm
MRI
– In selected cases
– T2 weighting: visualization of chromaffin cells (T2 adrenal/liver ratio > 3 in
pheochromocytoma)
MIBG Scintigraphy
– Localization of extraadrenal pheochromocytomas + metastases + bilateral
pheochromocytomas
– Method of choice in case of:
– positive Laboratory function tests and negative CT + MRI examinations
– Follow-up of patients with recurrent/metastatic disease
In (suspected) malignant pheochromocytoma: staging by standard imaging +
MIBG scintigraphy.

Therapy
Preoperative Preparation
Background
– Preoperative preparation = central to prophylaxis of intraoperative
cardiovascular crisis
– Intraoperative cardiovascular crisis: due to the release of catecholamines.
Principle
– α-adrenergic blockade
– β-adrenergic blockade for the prophylaxis of arrhythmias/tachycardia

Caution
β-adrenergic blockade: inhibition of α-blocker-induced vasodilation; β-
blocker alone: increase in hypertension + left ventricular congestion.

– Restoration of a normal electrolyte-fluid balance

Dosages for Pheochromocytoma

– Phenoxybenzamine (non-selective α-blocker): 10 mg 3 times/day
– Prazosin (selective α1 blocker): 0.5–1 mg titrated to 3–20 mg/day
– Metyrosine (tyrosine hydroxylase inhibitor): 250 mg 3 times/day titrated to a
maximum of 1.5–4 g/day
– Propranolol (non-selective β-blocker): 10–40 mg 3 times/day.

Surgical Therapy
Strategy
– Laparoscopic/retroperitoneoscopic adrenalectomy:
– In the case of unilateral small, benign appearing tumour with normal
opposite side
– Patients with MEN 2 or von Hippel-Lindau syndrome with small unilateral
findings (<6 cm)
– Bilateral minimally invasive adrenalectomy: For MEN 2 or von Hippel-
Lindau syndrome with small bilateral findings
Adrenal cortex-sparing adrenalectomy (subtotal) = method of choice for
bilateral benign disease (e.g. MEN 2 or von Hippel-Lindau syndrome):
prophylaxis of adrenal insufficiency.
– Open adrenalectomy:
– For findings >6 cm (high risk of malignancy = approx. 25%)
– In case of primary suspicion of malignancy
– Malignant pheochromocytoma + limited metastasis: resection possible in well-
selected patients
Principles of Surgical Therapy (► Sect. 6.7.7)
– Avoid intraoperative manipulation of the tumor
– Early ligation of the adrenal vein (interruption of the venous outflow of the
tumor)
Postoperative Monitoring/Follow-Up
– 24-h monitoring: blood pressure (compensatory hypotension due to
vasodilation), arrhythmias
– Annual: Plasmatic free metanephrines or urinary catecholamines
Chemotherapy
– Therapy regime
– High dose streptozocin
– Alternative: Cyclophosphamide + Vincristine + Darcarbazine
– Response rate = 50%
Radiotherapy
– For bone metastases
Palliative Therapy
– α-Methyltyrosine
– α-blockade + β-blockade

6.7.4 Adrenocortical Carcinoma

Definition
– Rare malignant endocrine tumor
– Surgical resection = only curative therapy

Epidemiology and Prognosis

Epidemiology
– Incidence = 0.5–2/1 million inhabitants per year in USA
– Bimodal age distribution:
– Peak in young children <5 years
– Peak in adults at 40–50 years
Prognosis
– Bad because of late diagnosis
– Most important prognostic factor = complete resection
– With complete resection: 5-year survival = 40%, median = 43 months
– In case of incomplete resection: Median survival = 12 months

Clinical Signs
Unclear Abdominal Complaints
– Secondary
– Due to progressive retroperitoneal mass.
Symptoms of Overproduction of Adrenocortical Hormones
– Majority of these tumors = functional
– Cushing’s syndrome: due to cortisol secretion (50% of tumors)
– Virilization/Feminization/Hypertension: due to androgen/estrogen or
aldosterone secretion (10–20% of tumors)

Diagnosis
Biochemical Screening
– Cortisol, aldosterone, androgens, estrogens
– Biochemistry = indicator for perioperative substitution therapy
– Exclusion of a pheochromocytoma: catecholamines + metabolites in plasma +
urine
Imaging
– High-resolution CT/MRI:
– MRI especially for the evaluation of a vena cava inferior infiltration
– With thorax: For detection of pulmonary metastases
– PET-CT: For detection of metastases + recurrence
Therapy
– 50% of tumors = localized at the time of diagnosis

Surgical Therapy
Strategy
– Complete resection = only curative option for local adrenocortical carcinoma
– Open resection:
– Adequate exposure
– Reduction of malignant cell spillage
 – Better control of the vessels (inferior vena cava, aorta, renal vessels)
– Radical en bloc resection (possibly multivisceral resection, if necessary)
– Laparoscopic resection technically possible, but high recurrence rate. Caution:
tumor fracture + peritoneal contamination.
– Recurrence/Metastases: Complete resection of recurrence + metastases
– Prolonged survival
– Reduction of hormone-associated symptoms
Postoperative Follow-Up
– Regular control of hormone levels
– Abdominal CT examinations (thorax + abdomen)
Chemotherapy
– For unresectable cancer(s)/metastases
– No chemotherapy active in terms of improving survival
– Reduction of symptoms due to anti-hormonal effect
– Mitotan:
– steroid inhibitor
– Inducer of atrophy of adrenocortical cells
– Side effects: Gastrointestinal + neuromuscular

Mitotan
– Need for close monitoring of hormone levels!
– Adjuvant mitotane therapy: currently being evaluated (in the context of
studies)
– Neoadjuvant mitotane therapy: currently being evaluated (in the context of
studies)
– Combinations of etoposide + doxorubicin + cisplatin + mitotane (EDP-M):
Currently being evaluated (in the context of studies), possible advantages in
recurrence-free survival/overall survival.
– Other active agents:
– Suramin, Ketoconazole
– Cisplatin, doxorubicin, vincristine

Radiotherapy
– Palliative therapy for bone metastases
6.7.5 Adrenal Incidentaloma
Definition
– Asymptomatic adrenal lesion as an incidental finding during imaging for
another reason
– Increasing frequency with use of abdominal CT

Epidemiology
– Frequency = 4% of routine abdomen imaging
– Frequency = 9% in autopsy series
– Most lesions = benign; hormone active vs. hormone inactive.

Clinical Evidence
Functional Incidentalomas
– All incidentalomas >1 cm: need for Hormonal workup
– All hormone-active lesions: Resection
– In case of radiological suspicion of malignancy: resection
– In case of size progression during follow-up: resection
Non-functional Incidentalomas
– Malignancy risk dependent on:
– Size of the mass: Best clinical indicator
– <4 cm: risk of malignancy = 2%
– 4.1–6 cm: risk of malignancy = 6%
– >6 cm: risk of malignancy = 35%
– Nonfunctional incidentalomas <3 cm: surveillance; nonfunctional
incidentalomas 3–5 cm: controversial.
– Radiological malignancy criteria/etiology (◘ Table 6.26)
Table 6.26 Characteristic features of adrenal incidentalomas in imaging (“imaging phenotype”)

Adrenocortical Adrenocortical Pheochromocytoma Metastases

adenoma carcinoma
Size Small, mostly ≤3 cm Large, mostly >4 Large, mostly >3 cm Variable, often <3
cm cm
Form Round or oval with Irregular with Round or oval with clear oval, irregular with
smooth margin unclear margin margin unclear margin
Texture Homogeneous Heterogeneous, Heterogeneous, with Heterogeneous,
with different cystic areas with different
densities densities
 Adrenocortical Adrenocortical Pheochromocytoma Metastases
adenoma carcinoma
Laterality Mostly solitary, one- Mostly solitary, Mostly solitary, one- Often bilateral
sided one-sided sided
Density (CT ≤10 HU >10 HU (mostly >10 HU (mostly >25) >10 HU (mostly
without CM) >25) >25)
Vessels in CM- Not highly vascular Mostly vascular Mostly vascular Mostly vascular
CT
Washout ≥50% after 10 min <50% after 10 min <50% after 10 min <50% after 10 min
MRI image Isointens in T2 Hyperintensity in Significantly Hyperintensity in
weighting T2 weighting hyperintensive in T2 T2 weighting
weighting
Necrosis, Rarely Frequently Bleeding + cystic areas Regular bleeding +
hemorrhage, frequent cystic areas
calcification
Size increase Mostly stable in Mostly fast (>2 Mostly slow (0.5–1.0 Different, slow–
progression or very cm/year) cm/year) fast
slow (<1 cm/year)

HU Hounsfield units, CM contrast medium

Evaluation Algorithm
– In patients with adrenal incidentaloma (◘ Fig. 6.11)
– In patients with adrenal incidentaloma and extraadrenal carcinoma (◘ Fig. 6.12)
Fig. 6.11 Algorithm for the evaluation of a patient with an isolated adrenal incidentaloma
Fig. 6.12 Algorithm for the evaluation and therapy of a patient with an adrenal incidentaloma in the context
of an extraadrenal carcinoma

Therapy
– Therapy = surgical therapy vs. monitoring
Indications for Surgical Therapy
– Hormone-active incidentalomas
– Incidentalomas with radiological signs of malignancy (regardless of size)
– Incidentalomas >6 cm
Incidentalomas 3–6 cm: Individual decision for surgery based on age + general
condition.

Strategy
– Laparoscopic adrenalectomy for:
– Incidentalomas without malignancy criteria on imaging
– Incidentalomas <4 cm
 – Open adrenalectomy: All other incidentalomas with indication for surgery
Background = Risk of capsular rupture and cell spillage during laparoscopic
adrenalectomy: Not adapted in the presence of malignancy.

6.7.6 Adrenal Metastases

Epidemiology
– Adrenal metastases = frequent
– In autopsies, adrenal metastases present in:
– 42% of lung cancers
– 16% of gastric cancers
– 58% of breast cancers
– 50% of malignant melanomas
– High percentage of prostate and kidney cancers

Clinical Signs
– Mostly asymptomatic
– If symptomatic:
– Unclear complaints
– Adrenal insufficiency: Very rare
Adrenal insufficiency: At least 90% of the adrenal cortex affected (massive
enlargement of the adrenal gland on CT).

Diagnosis
– Workup of patients with adrenal tumour in the context of extraadrenal
malignant disease (◘ Fig. 6.12)

Therapy
Surgical Therapy
– Resection of adrenal metastasis: in selected patients
– Selection Criteria:
– Prolonged disease-free interval
– Adequate tumor biology: good response to systemic therapy, history of
isolated metachronous metastasis, long disease-free interval
– Primary tumour localisation: better results for metastases from lung, colon,
kidney cancers and melanomas (worse for oesphagus, liver tumours or
sarcomas)
6.7.7 Principles of Adrenal Surgery
General (◘ Fig. 6.13)
Decisive Factors for the Choice of a Procedure
– Size and localization of the tumor
– Malignant potential of the lesion
– Unilateral vs. bilateral lesion
– Presence of extraadrenal manifestations
– Surgical procedure in the anamnesis
– Habitus of the patient
– Surgeon’s experience

Fig. 6.13 Access routes in adrenal surgery. (After Walz 2012)

General Rule
– Open access for large tumors and, if necessary, for tumors with malignant
potential

Open Adrenalectomy
Indications
– Known or suspected primary adrenocortical carcinoma
– Large tumors
– Tumor Recurrence
– Extension to adjacent organs
Technique
– 4 possible accesses:
– Anterior: Preferred for adrenocortical carcinoma
– Lateral: Ideal for obese patients
– Posterior: Rarely used; for small tumors
– Thoracoabdominal: Ideal for tumors requiring en bloc resection of adjacent
organs + lymphadenectomy

Surgical Procedure
Open Anterior Left Adrenalectomy
– Mostly longitudinal laparotomy
– Mobilization of the left colonic flexure + descending colon
– Entering retroperitoneum through incision along the lower edge of the
pancreas
– Medial visceral rotation of the spleen + pancreas tail (dissection on Gerota’s
fascia)
– Visualization of renal hilus and following renal vein to confluence with left
adrenal vein
– Left adrenal gland = left to the aorta, above the left renal vein
– Early ligation of the adrenal vein
– Supply of thin-caliber collaterals of the aorta, inferior diaphragmatic vessels
and renal vessels
– adrenalectomy
– No drainage

Surgical Procedure
Open Anterior Right Adrenalectomy
– Mostly longitudinal laparotomy
– Mobilization of the right ligamentum triangulare of the liver and
anteromedial rotation of the liver = access to the right adrenal gland
– Kocher maneuver: mobilization of the duodenum if necessary; thereby better
access to the right kidney + inferior vena cava
– Right adrenal vein: Mostly direct drainage into inferior vena cava; ligation of
the adrenalvein
– Control of the arterial inflow of the adrenal gland
 – adrenalectomy
– No drainage

Surgical Procedure
Open Posterior Adrenalectomy
– Patient in prone position; table bent 35°
– Oblique incision over the 12th rib; retraction of the sacrospinalis muscle
medially
– Resection of the 12th rib; reflection on pleura cranially
– Left: Cranial resection border = diaphragm; Right: Cranial resection border
= liver
– Adrenalectomy is performed like anterior technique

Surgical Procedure
Thoracoabdominal Approach
– Allows the best exposure: ideal for tumors requiring en bloc resection of
adjacent organs + lymphadenectomy
– Incision over tenth rib on the right and 11th rib on the left, with rib resection
– If infiltration V. cava or hepatic veins: Need for additional sternotomy

Surgical Procedure
Lateral Access
– Patient in lateral decubitus: using gravity for organ retraction
– Extraperitoneal approach to the adrenal gland
– sparing of extensive adhesiolysis in patients with postop. Adhesions
– Vascular control usually more difficult

Laparoscopic Adrenalectomy
– Standard access for small benign adrenal tumors
Advantages (Compared to Open Access)
– Less pain and less postoperative restrictions
– Shorter hospital stay
– Faster recovery
– Better cosmetic result
Good Candidates for Laparoscopic Adrenalectomy
– Patients with Conn adenoma
– Small functional adrenal tumors (<4 cm)
– Unilateral sporadic benign tumors
– MEN 2 or von Hippel-Lindau syndrome, patients with unilateral
pheochromocytoma
– Selected patients with adrenal metastasis

Laparoscopic Adrenalectomy
– Tissue-sparing subtotal adrenalectomy: Possible using laparoscopy
– In case of suspected malignancy: No laparoscopy
– In case of unclear incidentaloma: open approach recommended (potential
malignancy)

Surgical Procedure
Laparoscopic Left Adrenalectomy
– Patient in right lateral decubitus
– Operating table adequately padded; abdomen and thorax washed down from
areola to below the spina iliaca, and from right of umbilicus to spine
– Infracostal trocar 10–15 cm anterior to the anterior axillary line (open
technique)
– 3 × 10 mm trocars under direct vision: anterior axillary line, posterior
axillary line and one 5-cm posterior to posterior axillary line port, medial to
left kidney
– Dissection (e.g., ligasure, ultracision): Mobilization left colonic flexure,
using gravity; inferior and medial dissection
– Mobilization of the spleen by incision of the peritoneum lateral to the spleen
– Rotation of the spleen medially with pancreatic tail
– Dissection of the left adrenal gland from retroperitoneal fat
– Transection of the adrenal vein after mobilization of the gland and shortly
before complete resection (difference to the open technique): Vascular
stapler, energy sealing device (e.g.Ligasur) or clip (currently some authors
advise against clips: slippage of clips)
– Extraction of the adrenal gland in sterile plastic bag via umbilical caltrocar
 Surgical Procedure
Laparoscopic Right Adrenalectomy
– Patient in left lateral decubitus
– Trocar positions analogous to left
– Medial port for liver retraction
– Dissection of the adrenal gland caudally along the renal vein and medially
along the inferior vena cava
– Right adrenal vein usually short and thick-lumened
– Transection of the adrenal vein obligatory, by means of vessel stapler, energy
sealing (Ligasur), or vessel clips (currently some authors advise against
clips: slipping of the clips)
– Remaining dissection analogous to left

Retroperitoneoscopic Adrenalectomy
Indications
– Small benign adrenal tumors
– Isolated adrenal metastases

Caution
Positioning in knee-elbow position.

Advantages
– Minimally invasive method
– Reduction of hemodynamic or respiratory instability (vs. capnoperitoneum)
– No need for adhesiolysis or transperitoneal access
– Possibility of hemostasis by increasing the pressure (insufflator pressure up to
20–25 mmHg)
– Possibility of bilateral adrenalectomy without repositioning the patient
Contraindications
– Suspicion of adrenocortical carcinoma/malignant pheochromocytoma
– Adjacent Organ Infiltration
– Lesion >6 cm
– Morbid obesity
– Limited distance between costal arch and iliac crest

Surgical Procedure
 Retroperitoneoscopic Adrenalectomy
– Patient in knee-elbow position
– Palpation of the 12th rib, 1.5-cm incision just below the tip of the 12th rib.
– Opening of the retroperitoneum with scissors and widening of the access
with the finger
– Palpation with index finger and insertion of the 10 mm and 5 mm trocar
(possibly also possible under visual control): Medial trocar 5 cm medial to
the 12-mm trocar, lateral to the paraspinal muscles; the lateral trocar 5 cm
lateral to the 12-mm trocar under the tip of 11th rib.
– Insertion of a 12 mm trocar with balloon and CO2 insufflation with pressure
20–24 mmHg
– 30° 10 mm videoscope into the 12 mm trocar and start of blunt dissection:
retroperitoneal space
– Opening of the Gerota fascia + visualization of the renal upper pole.
Visualization of landmarks: paraspinal muscles, diaphragm, liver,
peritoneum parietale, inferior vena cava
– videoscope into paravertebral trocar
– Dissection along the renal upper pole
– Identification of inferior vena cava on right side
– Exposure of adrenal vein and supply by means of sealing (e.g. Ligasur)
– Complete mobilization of the adrenal gland and salvage using a plastic
salvage bag via 12-mm access
– haemostasis after reduction of pressure
– Removal of the trocar and closure of the accesses

6.7.8 Guidelines
AWMF S2k guideline, registry no. 088–008—Surgical therapy of adrenal tumors.
► https://​www.​awmf.​org/​uploads/​tx_​szleitlinien/​088-008_​S2k_​operative-
Therapie_​Nebennierentumor​en_​2019-07.​pdf
Fassnacht M, Arlt W, Bancos I et al.(2016) Management of adrenal
incidentalomas: European Society of Endocrinology clinical practice guideline in
collaboration with the European Network for the Study of Adrenal Tumors. Europ
J Endocrinol 175:G1–G34.
Funder JW, Carey RM, Mantero F (2016) The management of primary
aldosteronism: case detection, diagnosis, and treatment: an Endocrine Society
clinical practice guideline. J Clin Endocrinol Metab 101:1889–1916.
Gaujoux S, Mihai R, Joint working group of ESES and ENSAT (2017)
European Society of Endocrine Surgeons (ESES) and European Network for the
Study of Adrenal Tumors (ENSAT) recommendations for the surgical management
of adrenocortical carcinoma. Br J Surg 104:358–376.
Lenders JWM, Duh QY, Eisenhofer G et al. (2014) Pheochromocytoma and
paraganglioma: an Endocrine Society clinical practice guideline. J Clin Endocrinol
Metab 99:1915–1942.
Nieman LK, Biller BM, Findling JW et al. (2015) Treatment of Cushing’s
syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol
Metab 100:2807–2831.
Zeiger MA, Thompson GB, Duh QY et al. (2009) The American Association
of Clinical Endocrinologists and American Association of Endocrine Surgeons
medical guidelines for the management of adrenal incidentalomas. Endocr Pract
15:S1–S20.

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OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_7

7. Bariatric and Metabolic Surgery

Michel Gagner1 and Franck Billmann2
(1) Sacré Coeur Hospital, QC, Canada
(2) Department of General, Visceral and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Germany

Franck Billmann
Email: [email protected]

7.1 Epidemiology
7.1.1 Incidence
– Obesity = epidemic worldwide
– Obesity definition:
– Overweight (preadiposity): BMI (Body Mass Index) = 25–29.9
– Obesity grade 1: BMI = 30–34.9
– Obesity grade 2: BMI = 35–39.9 (“severe obesity”)
– Obesity grade 3: BMI ≥ 40 (“morbid obesity”)
– “Superobesity”: BMI ≥ 50
– Obesity in USA:
– Prevalence increase 15.3% of population (1995), 23.9% (2005), 27.2%
(2010), 39.8% (2016)
– 4.8% with BMI > 35, 3.7% with BMI > 40
– Prevalence development (◘ Figs. 7.1 and 7.2)
– Obesity worldwide (WHO data 2016):
– Delayed increase
– approx. 13% of the population obese; 39% overweight
Fig. 7.1 Age-standardized overall prevalence of overweight (upper curve) and obesity (lower curve)
in men and women (age > 20 years) (during 1980–2015). (From Chooi et al. 2019; courtesy of Chooi
et al.)

Fig. 7.2 Age-standardized regional prevalence of overweight (upper curve) and obesity (lower
curve) in men and women (age > 20 years) (over the period 1980–2015). (From Chooi et al. 2019;
courtesy of Chooi et al.)

7.1.2 Health Economic Consequences

– Obesity-associated morbidity (especially abdominal obesity): Increased
risk of
– Arterial hypertension
– Type-2 diabetes mellitus
– Hyperlipidemia
– Sleep Apnea Syndrome
– Coronary heart disease, steatosis hepatis and myocardial infarction
– Costs associated with obesity:
– Compared to normal weight: 30% higher costs in obese patients, 81%
higher costs in morbidly obese patients
– 147 billion $ annually in the USA (2008)
– 420,000 deaths in 2016 in U.S.

7.2 Pathophysiology and Principle of Action of

Bariatric Surgery
Key Points
– Pathophysiology of obesity = complex + not completely understood
– Operating principle of bariatric surgery:
– Gastric Restriction
– Intestinal malabsorption
– Learned behavioral changes
– Neural and endocrine signal change (postoperative)

7.2.1 Pathophysiology
– Complex and not completely understood
– Contributing factors:
– Genetic factors (approx. 67% of BMI variability)
– Behavioural factors (decrease in physical activity, increase in intake of
high calorie food)
 – Psychological factors
– Other, e.g. economic, socio-educational factors

7.2.2 Working Principle of Bariatric Surgery

– Bariatric surgery = altering the anatomy of the GI tract: reducing caloric
intake
– Simplified: 3 operating methods/principles:
– Restrictive methods
– Hormonal (hypoabsorptive) methods
– Combined methods (restrictive + hormonal)
– In reality: active principle of bariatric surgery = complex and interaction
of:
– Gastric Restriction
– Intestinal hypoabsorption
– Learned behavioural changes (e.g. after excessive food intake
following gastric restriction: nausea and vomiting lead to adaptation
of eating habits)
– Neural and endocrine signal change (postoperative)
– For example, gastric bypass: suppression of grhelin secretion leads
to a reduction in appetite
– Other hormones with implications after bariatric surgery:
cholecystokinin, peptide YY, GLP (glucagon-like peptide)-1

Restrictive Methods
– Principle of action of the gastric pouch: By reaching the feeling of satiety
early + delayed gastric emptying to limit food intake (volume limitation)
– Basic principle of the operation: Formation of a small gastric reservoir
with reduced outflow
– surgical procedures (◘ Fig. 7.3):
– Gastroplasty (e.g. sleeve gastrectomy)
– Gastric Balloon Insertion
– Laparoscopic gastric plication (new method under study)
– Endoscopic Sleeve Gastroplasty
– Historical method: adjustable gastric banding (“vertical banded
gastroplasty”)
Fig. 7.3 a–d Currently most frequent specific bariatric interventions
Hypoabsorptive Methods
– Active principle: Limitation of nutrient uptake
– Basic principle of the operation: forming a bypass of different portions of
the small intestine
– surgical procedures (◘ Fig. 7.3): Currently no purely hypoabsorptive
method
– Historical methods: Purely hypoabsorptive methods
– For example, jejunoileal bypass.

Combined Methods
– Principle of action: Limitation of food intake (volume limitation) +
Limitation of nutrient intake
– Basic principle of the operation: formation of a small gastric reservoir
with reduced outflow + formation of a bypass of different portions of the
small intestine
– Surgical procedures (◘ Fig. 7.3):
– Proximal Roux-Y-Gastric Bypass
– “Banded Gastric Bypass”
– Biliopancreatic diversion with/without duodenal switch
– Mini gastric bypass (new method under study)
– “single anastomosis duodeno-ileal bypass with sleeve gastrectomy”
(SADI-S); (new method under study)

7.3 Clinical Evidence

7.3.1 Bariatric Surgery: Laparoscopic Versus Open
– Reduced postoperative pain
– Reduction of morbidity:
– In particular, fewer pulmonary complications
– Fewer incisional hernias
– Fewer wound complications (infections, wound healing disorders)
– Reduction of hospital stay
– Faster recovery time
7.3.2 Bariatric Surgery: Prospective Controlled Studies
– No large randomized trial comparing bariatric surgery vs. medical
therapy
– Only 2 small randomized trials +3 cohort studies (all at high risk for bias)
+ meta-analyses:
– Bariatric surgery: weight loss on average = 20–50 kg
– Non-surgical therapy: Minimal weight loss
– A large prospective controlled case-control study (Swedish SOS study;
Sjöström 2013):
– Significantly better weight loss in surgery vs. non-surgery group after
2 years, and after 10 years
– Combined procedures better than purely restrictive procedures
– Median BMI decrease at 2 years after bariatric surgery: 50.0
(combined) to 32.6 (restrictive)
– Significantly lower morbidity after surgery (HR = 0.56; P = 0.01)
– Significantly fewer cardiovascular events after surgery (HR = 0.83; P
= 0.05)

7.3.3 Metabolic Consequences of Bariatric Surgery

– Randomized controlled trial and meta-analysis results
– Diabetes mellitus:
– Diabetes remission: 0% after no surgery vs. 22.4% after surgical
therapy (after 5 years)
– Diabetes-associated medication use: no treatment after 5 years: 0%
after no surgical vs. 45% after surgical therapy
– Effects independent of BMI at baseline
– Hyperlipidemia: treatment of 83% of patients
– NAFLD/NASH: improvement steatosis index/fibrosis index (tubular
stomach better than gastric bypass) (Billmann et al. 2021)
– Arterial hypertension: treatment of 66% of patients
– Sleep apnea: treatment of 88% of patients
– Improvement of end organ damage (especially those linked to Type-2
diabetes; e.g. diabetic kidney disease)
7.3.4 Mortality: Bariatric Surgery Versus Drug Therapies
– Matched case control studies only
– Last-published large study (Aminian et al. 2019): significantly lower all-
cause mortality in the surgery Group (10.0%) vs. no surgery Group
(17.8%)
– SOS study: Significant reduction in all-cause mortality of 31.6% in
surgery group vs. non-surgery group

7.4 Specific Current Bariatric Interventions

7.4.1 Roux-Y Gastric Bypass “Gastric Bypass”
– Combined method
– Procedure basis: division of the stomach − small gastric pouch (20–30
mL); Roux-Y gastrojejunostomy + jejunojejunostomy with formation of
a:
– Biliopancreatic limb: Jejunal limb from Treitz ligament to
jejunojejunostomy (confluence of biliary, pancreatic secretions and
alimentary flow); length = 30–100 cm
– Alimentary limb: Roux limb from gastrojejunostomy to
jejunojejunostomy; the limb passes food; length = 75–150 cm
– Common channel: rest of the small intestine after jejunojejunostomy
to ileocecal valve

Surgical Procedure
Roux Y Gastric Bypass
– Incision of the omentum minus 6 cm distal to the gastroesophageal
junction
– Dissection dorsolaterally along the posterior wall of the stomach and
finding the omental bursa
– Transsection of the stomach using the Endo-GIA device and formation
of the stomach pouch (volume target of the pouch = 20 mL)
– Performing a CEEA (“circular end-to-end anastomosis”) pressure
plate 25 mm by means of a gastric tube through the gastric pouch;
alternative = gastrojejunostomy by means of an Endo-GIA device, or
completely hand-sewn
 – Division of the omentum majus
– Measurement of the small intestine from Treitz ligament
– Antecolic end-to-side gastrojejunostomy by means of CEEA (device
transabdominal through lumen of distal jejunum)
– Test for leakage
– Measurement of the Roux thigh and side-to-side jejunojejunostomy
– Standard lengths: Roux length = 75–100 cm; distance between Treitz
ligament and jejunojejunostomy = 30–100 cm.
– Extended lengths: Roux and Treitz jejunostomy lengths 150 cm and
100 cm respectively
– Closure of mesenteric defects
– Leak-testing (not mandatory) and closure of the accesses

7.4.2 Banded Gastric Bypass

– Restrictive method
– Procedure basis: In addition to gastric bypass, a gastric band to prevent
regain of weight.
– Complications: Complications of vertical banded gastroplasty (s. ► Sect.
7.6.1)

7.4.3 Laparoscopic Adjustable Gastric Banding (LAGB)

– Restrictive method
– Procedure basis: Placement of an adjustable band (connected to a
subcutaneous port) approximately 1–2 cm aborally of the
gastroesophageal junction and formation of a 30 mL gastric pouch

Surgical Procedure
Laparoscopic Adjustable Gastric Banding
– Placement of the tape 1 cm below the esophagogastric junction
– Formation of a tunnel for placing the ligament through the pars
flaccida in the area of the small gastric curvature above the bursa
omentalis
– Using an intragastric calibration probe
– Tape is left blank at the beginning
 – Anterior extensive fixation of the ligament, especially at the large
curvature (fundus)
– Gastrogastric sutures for fixation of the ligament below the virtual
pouch, directly below the esophagogastric junction
– Port chamber placed on the rectus abdominis or epigastric muscle
– Adaptation of the band volume possible in the consultation
(depending on weight loss and symptoms)

7.4.4 Biliopancreatic Diversion (BPD)

– Combined method
– Basic principle of the operation: Distal subtotal gastrectomy (50–80%)
with reconstruction like gastric bypass; difference: enteroenterostomy
clearly more distal with formation of a common channel of approx. 50–
100 cm length

Surgical Procedure
Biliopancreatic Diversion (BPD)
– Devascularization of the large gastric curvature (mostly preservation
of the gastricae-breves vessels) + first part of the duodenum
– Transection of the duodenum with stapler, after isolation and
transection of the right gastric veins
– transection of the omentum minus along the small curvature up to
approx. 2 cm below the left gastric veins
– Horizontal gastrectomy (residual stomach = approx. 300 mL; approx.
5 cm from cardia along the small curvature), by means of endo-GIA
stapler
– Cholecystectomy
– Measure the common limb, mark 50 cm from the ileocecal valve.
– Cutting of the small intestine approx. 250 cm orally of the ileocecal
valve (formation of the alimentary limb)
– Anastomosis between biliopancreatic and alimentary limb by means
of side-to-side anastomosis at the level of the 50 cm marker
– Formation of a window in the mesocolon transversum and passage of
the gastric stump to the submesocolic region
– Enterotomy of the distal intestinal stump and posterior gastric wall
and gastroenterostomy using endo-GIA stapler
 – Closure of the common defect by means of hand suture (closure of the
mesenteric defects with non-absorbable suture)
– Exclusion of leakage/bleeding
– Irrigation, possibly drainage (not absolutely necessary), closure of the
accesses

7.4.5 Biliopancreatic Diversion with Duodenal Switch (BPD/

DS)
– Combined method
– Basic principle of surgery: like BPD with sleeve gastrectomy and
preservation of the pylorus, ileoduodenostomy behind the pylorus;
alimentary limb approx. 150 cm and biliopancreatic limb (different
lengths)

Surgical Procedure
Biliopancreatic Diversion with Duodenal Switch
– Devascularization of the large gastric curvature + first part of the
duodenum
– Transection of the duodenum with stapler
– Vertical gastrectomy (70% of the stomach), starting 6 cm proximal to
the pylorus parallel to the small curvature, using a 60 Fr.(French)-
nasogastric tube (placeholder)
– Exclusion of leakage of the stapler line (after duodenoileal
anastomosis)
– Measure the common limb, mark 100 cm from the ileocecal valve
– Cutting of the small intestine approx. 250 cm orally of the ileocecal
valve (formation of the alimentary limb)
– Antecolic anastomosis between biliopancreatic and alimentary limb
by means of side-to-side anastomosis at the level of the 100 cm
marker
– Duodenoileal anastomosis end-to-side
– Closure of the common defect by means of hand suture (closure of the
mesenteric defects with non-absorbable suture)
 – Irrigation, possibly drainage (not absolutely necessary), closure of the
accesses

7.4.6 Gastric Sleeve Resection

– Restrictive and hormonal method (especially in patients with high
perioperative risk)
– Basic principle of the operation: By means of splinting (32 to 40 Fr
probe) of the small curvature resection of the large gastric curvature:
– As definitive bariatric surgery
– In preparation for the BPD/DS
– Currently the most performed bariatric surgery worldwide

Surgical Procedure
Gastric Sleeve Resection “Sleeve Gastrectomy”
– Retraction of the liver (especially left lobe) and visualization of the
pylorus and the large curvature of the stomach
– Dissection of the greater omentum to open the lesser sac
– Dissection of the large curvature (starting 2–3 cm proximal to the
pylorus) and division of the short gastric vessels up to the
gastroesophageal junction
– Splinting of the small curvature of the stomach by means of a thick
gastric tube (approx. 36 Fr.) and vertical sleeve gastrectomy (by
means of an endo-GIA stapler) starting 4 cm orally of the pylorus up
to the gastroesophageal junction.
– Extraction of the resected part of the stomach
– Exclusion of leakage or bleeding along the stapler line
– Leak testing and closure of the accesses

Results Weight Loss

– 80% of gastric bypass patients achieve a weight loss of 60–80% of the
excessive weight in the first year; in the longer term stabilization at
50–60% of the excessive weight
 – Average weight loss: 30.19 kg for adjustable gastric band; up to 51.93
kg for BPD; after 10 years, stabilization of weight loss at 20–30 kg.
– 10–40% of patients do not achieve long-term weight loss

7.5 Complications
7.5.1 Mortality
– Between 0.1 and 2.0% in large studies
– No significant difference compared to non-op. General population (long-
term study)
– In Meta-analyses:
– After gastric bypass: 0.5%
– After gastric banding: 0.1
– After hypoabsorptive surgery: 1.1%

Causes of Mortality
– Pulmonary Embolism
– Anastomotic leakage and sepsis
– Myocardial Infarction
– Malignant/non-malignant neoplasms
– Ileus/gangrene due to hernias

Risk Factors
– Experience of the surgeon/department
– Advanced patient age
– Male gender
– Super obesity (BMI > 50)
– Comorbidities

7.5.2 Gastrointestinal Complications

– Relatively often

Nausea and Vomiting

– In more than 50% of patients with restrictive method
– Mostly because of dietary errors (too much, too fast)
– Anastomotic stenosis = other cause

Dumping Syndrome
– Neurohormonal syndrome
– Triggered by the ingestion of sugar
– Clinical presentation:
– Flush phenomenon of the face and upper half of the torso
– Drowsiness/dizziness
– Tachycardia
– Fatigue
– Diarrhea
– Incidence = 70% of patients after Roux-Y gastric bypass

Deficiency Symptoms
– After hypoabsorptive methods (e.g. gastric bypass): Iron, calcium, folic
acid, vitamin B12, possibly other nutrients
– After BPD: proteins, fat-soluble vitamins (A, D, E and K)
– Therefore, the need for regular laboratory control + substitution

Other Gastrointestinal Complications

– Dehydration
– Intestinal obstruction, ileus
– Anastomosis leaks and fistulas
– Strictures/stenoses
– Incisional hernias or internal hernias
– Cholecystolithiasis and choledocholithiasis

7.5.3 Other Complications

– Venous thromboembolism
– Wound infections
– Bleeding
– Splenectomy after injury (rare)
– Incisional hernias or internal hernias
– Early postoperative ileus
– Gallstones

Complications in the SOS Study (Sjöström 2013)

– Postoperative complications = 13% of patients, of which
– Bleeding = 0.5
– Embolism/thrombosis = 0.8%
– Wound complications = 1.8
– Pulmonary complications = 6.1

7.6 Historical Interventions and Interventions in

the Context of Studies
7.6.1 Historical Interventions
Jaw Wiring
– Historical intervention, no current application

Adjustable Gastric Banding (Vertical Banded Gastroplasty)

– Restrictive method
– Basic principle of the operation: Vertical partitioning of the stomach with
attachment of a gastric band (“mesh”) to control the diameter of the
gastric outlet.
– Complications:
– No long-term weight control
– Intolerance of gastric constriction: vomiting, gastroesophageal reflux
– Inflammatory reaction to the Band/Tube/Mesh: gastric stenosis
– Free perforation due to erosion of the band

Jejunoileal Bypass (Intestinal Bypass)

– Hypoabsorptive method
– Procedure basis: Purely intestinal bypass; transection of the proximal
jejunum, which is anastomosed distally to the ileum = bypass of up to
90% of the small intestine
– Complications:
– perioperative complications, hypoproteinemia
– Electrolyte dysregulation (loss via stool)
– hepatic insufficiency, nephrolithiasis, autoimmune complications
– bacterial overgrowth (SIBO)
– Due to high complication rate = abandoned technique

Stomach Partitioning
– Restrictive method
– Basic principle of the operation: exclusion of a part of the stomach by
double-row stapling to reduce the passage (no transection)
– Failure of this technique due to reopening of the stapler row or dilatation
of the oral part of the stomach

7.6.2 Interventions in the Context of Studies

Laparoscopic Gastric Plication “Gastric Plication”
– Restrictive method
– Basic principle of the operation: reduction of the size of the stomach by
inversion of the large curvature of the stomach internally
– Pros:
– Preservation of full stomach structure and function
– Comparable restriction as with sleeve gastric resection (see below)
– Disadvantages:
– No long-term results currently known
– Risk of gastric adaptation (due to distension) with renewed weight
gain
– Complications:
– Gastric perforation (<1%)
– Excessive gastric constriction (<1%)
– Slippage
– Portal or mesenteric thrombosis

Endoscopic Sleeve Gastroplasty “Gastric Plication”

– Restrictive method
– same as laparoscopic gastric plication, but using endoscopy
– lesser results and complications

Mini Gastric Bypass

– Combined method
– Basic principle of surgery: narrow long gastric pouch (close to the
gastroesophageal junction); anastomosis of the small intestine (150–200
cm distal from the Treitz ligament; without transection of the small
intestine) with the pouch
– Pros:
– Good weight loss (due to more hypoabsorption)
– Effective procedure for the therapy of diabetes mellitus type 2
– Shorter surgery time and anesthesia
– Disadvantages:
– Dumping Syndrome
– Hypoglycemia
– Intestinal obstruction and internal hernia, “afferent-loop syndrome”…
– Lifelong nutrient substitution (vitamins and minerals)
– Increased rate of biliary reflux (bile gastritis, bile esophagitis)
– marginal + gastric ulcers
– Complications:
– Postoperative ileus (2–4%) due to intestinal obstruction
– Anastomotic insufficiency (<1%)
– Bleeding (<1%)
– Need for conversion to Roux-Y-gastric bypass (5–10% of cases).

Ileal Transposition with/Without Sleeve Gastrectomy

– New method
– Procedural Basis:
– Transposition of a 100 cm distal segment of the ileum (completely
innervated and perfused) to the proximal jejunum
– Objective = early stimulation of the ileum by nutritional components

Laparoscopic “Jejunal Sleeve” (On Gastric Bypass/Sleeve)

– Combined method
– As a revision procedure after gastric bypass for weight gain or as a
primary procedure (in the context of studies)
– Procedural Basis:
– 40 Fr bougie for stomach/jejunum calibration
– Lateral resection of the gastric pouch (gastric sleeve)
– Resection of the blind end of the jejunum
– Sleeve resection of the jejunum over 15–25 cm
– Pros:
– Simple rescue method after gastric bypass
– BMI reduction of 5–10 extra points
– Disadvantages:
– Currently still within the scope of studies
– Medium- and long-term results missing
– Complications:
– Stapler line/anastomosis insufficiency
– Stenoses

Laparoscopic Single Anastomosis Duodenal Switch (SADI-S)

– Combined method
– Basic principle of the operation: formation of a tubular stomach;
transection of the duodenum approx. 3 cm distal from the pylorus;
duodenoileostomy approx. 2.5 m from the IC(ileocecal) valve (without
transection of the ileum) leads to the absorption of proteins and fats only
in the last 2.5 m of the ileum
– Pros:
– Only one anastomosis (compared to the conventional duodenal
switch), lesser operative time
– Good results in terms of weight loss
– Effective for reducing cholesterol and triglycerides
– Effective in the treatment of diabetes mellitus type 2
– Lesser risk of internal hernias
– Disadvantages:
– Less weight loss than classic duodenal switch (not studied in detail)
– No long-term results currently known
– Possible bile gastritis
– Complications:
– Intestinal obstruction (2–4%)
– Anastomotic insufficiency (<1%)
– Bleeding (<1%)
– Need for reversion due to excessive hypoabsorption (2–5%) or
insufficient weight loss

Myoelectric Gastric Stimulation

– Neurophysiological method
– Principle of action: Influencing the parasympathetic stimulation of the
stomach and the intrinsic myoelectric activity of the stomach by means
of a pacemaker
– Basic principle of the operation: Stomach stimulation by means of a
pacemaker and electrodes in the gastric curvature
– Within the framework of studies

7.7 Metabolic Surgery

– Rapidly increasing importance of metabolic surgery

7.7.1 Definition
– Metabolic surgery (◘ Fig. 7.4 and ◘ Table 7.1) = not clearly defined
– In most cases, concept to denote currently experimental procedures
– Proper definition: shift of the primary focus of surgery to treat weight
toward surgery to control metabolic disease (especially diabetes mellitus
in those patients without severe obesity)
Fig. 7.4 Definition of metabolic surgery based on the goal of surgical therapy. RYGB Roux-Y gastric
bypass, SG sleeve gastrectomy, GB gastric banding, BPD biliopancreatic diversion, DJB
duodenojejunal bypass, IT ileal transposition. (Mod. according to Rubino et al. 2014)

Table 7.1 Bariatric vs. metabolic surgery. (Rubino et al. 2014)

Comparison Bariatric surgery Metabolic surgery

parameters
Diseases Severe obesity “Metabolic” obesity, diabetes mellitus type 2, metabolic
syndrome
Primary Weight reduction Blood glucose and metabolic control, reduction of
objective cardiometabolic risk
Criteria for the Weight-oriented Abdominal circumference, BMI, disease-specific
surgical (BMI) parameters (HbA1c, C-peptide, insulin and glucose
indication levels), response to alternative therapies, associated
conditions that increase CVD risk and can be ameliorated
by surgery (hypertension, dyslipidemia, sleep apnea
syndrome, etc.)
Procedures RYGB, sleeve RYGB, sleeve gastrectomy, gastric banding, BPD,
gastrectomy, gastric BPD/DS, procedures within trials (duodenojejunal bypass,
banding, BPD, ileal interposition), device-based interventionsa
BPD/DS
Measurement of Overweight Glycemic control, dyslipidemia control, weight loss, CVD
the treatment reduction >50 risk reduction
success
Composition of Surgeon, Surgeon, endocrinologist, cardiologist, obesity specialist,
the treatment nutritionist, diabetes consultant, etc.
Comparison Bariatric surgery Metabolic surgery
parameters
team psychologist
Possible Simple, primarily Complex, neuroendocrine and/or metabolicc
mechanisms of mechanicalb
action

BMI body mass index, CVD cardiovascular disease, RYGB Roux-Y gastric
bypass, BPD/DS biliopancreatic diversion with duodenal switch
a
Endoluminal liners, electrophysiological devices, etc.
b
Restriction and/or malabsorption of energy intake
c
Changes in gastrointestinal hormones, changes in appetite and hunger
regulation, changes in nutrient perception, microbiotics, bile acid, etc.

7.7.2 Scientific Basis

– Bariatric surgery = reduction in relative risk (%) for comorbidities in
obese patients:
– Cancer risk (76%)
– Cardiovascular risk (82%)
– Endocrinological disease risk (65%; after 10 years: 82.9% treatment of
diabetes after bariatric surgery)
– Infectious disease risk (77%)
– Musculoskeletal disease risk (59%)
– Respiratory disease risk (76%)
– Psychiatric disease risk (47%)
– Bariatric surgery = reduction in direct treatment costs (over 5 years:
$8813 in operated patients vs. $11,854 in non-operated patients; SEER
registry, USA)

7.8 S3 Guidelines (February 2018)

7.8.1 Quality Assurance
– Bariatric/Metabolic Interventions only in clinics with certification or
aiming for certification
– The following procedures only in centers: age < 18 or age > 65, BMI ≥
60, non-standard procedures (center with special expertise)
– Necessity of suitable equipment; imaging diagnosis + endoscopy
available 24 h a day
– Entry of patients in national register; presentation of SOPs (Standard
Operating Procedures)

7.8.2 Diagnosis and Evaluation

– Necessity of interdisciplinary opinion before a surgical measure for
weight reduction
– Presentation to a physician experienced in conservative obesity
therapy is obligatory (e.g. nutritionist)
– Further presentations in other disciplines depending on the
comorbidities of obese patients (clinical psychology, psychosomatics,
psychiatry, endocrinology, nutritional counseling)
– Comprehensive preparation of major abdominal procedures (medical
history, documentation of concomitant diseases, current medication,
complaints, symptoms, ECG, chest X-ray, routine laboratory, sonography
of the abdomen, pulmonary function examination), exercise and
behavioural therapy
– Need for an esophagogastroduodenoscopy (EGD) before any bariatric
surgery
– Absolute exclusion of secondary causes of obesity (e.g. hypothyroidism)

7.8.3 Indication
– Primary indications for bariatric surgery:
– BMI > 50 kg/m2 + conservative weight loss attempt futile
(classification of a multidisciplinary team) or in case of severe
concomitant secondary disease without possible postponement
– BMI ≥ 40 kg/m2 without surgical contraindication after exhaustion of
conservative therapy and after comprehensive clarification
– 35 ≤ BMI < 40 + one or more obesity-associated sequelae/companion
diseases (e.g. diabetes mellitus type 2, coronary heart disease) and
after exhaustion of conservative therapy
 – Diabetes mellitus type 2 + 30 ≤ BMI < 35, if target levels cannot be
met
– Diabetes mellitus type 2 + BMI < 30, surgery can be offered as part of
a study
– In obese adolescents with significant comorbidities after failure of
multimodal conservative therapy
– Age alone (> 65 years) = no contraindication; indication must be
particularly justified (aim of the operation = prevention of immobility
and need for care)
– Desire to have children = no contraindication to bariatric surgery
– After treatment of contraindication: Reevaluation

7.8.4 Choice of Procedure

– Currently no flat rate procedure for all patients
– Currently effective surgical procedures as first-line therapy:
– Sleeve Gastrectomy (SM)
– Roux Y Gastric Bypass (RYGB)
– Omega Loop Bypass
– Biliopancreatic diversion with duodenal switch (BPD/DS)
– Further procedures:
– Biliopancreatic Diversion (BPD)
– One-anastomosis bypass (“mini-bypass”)
– Vertical Banded Gastroplasty (VBP)
– Choice of procedure depends on:
– BMI
– Age
– Gender
– Comorbidity
– Adherence
– Occupation
– Need for detailed consultation with the patient about:
– Common procedures
– Staged concepts (gastric balloon or sleeve gastric resection as first
step)
 – Possible treatment alternatives
– Possible complications (morbidity, mortality)
– Aftercare (possible lifelong supplementation, plastic follow-up
surgery)
– Need to consider patient preference in the absence of contraindication
– Indication + surgery by surgeons with expertise in hospitals with
institutional experience

7.8.5 Technical Aspects and Complications

– Gastric balloon:
– Necessity of the methylene blue sample for early diagnosis of balloon
dysfunction
– Previous gastric operations = contraindication with increased risk of
perforation
– Gastric band:
– Unconditional positioning of the band through pars flaccida of the
omentum minus (minimization of the ligament dislocation rate)
– Always laparoscopic
– Lowest mortality, but results inferior to other techniques
– Roux-Y gastric bypass:
– Laparoscopic surgery indicated
– Target = small stomach pouch
– Results: approx.—13–14 BMI points up to 5 years postoperatively
– Length of alimentary limb = approximately 150–200 cm (for adequate
weight loss + minor metabolic complications); biliopancreatic limb =
50–80 cm.
– Position of the alimentary limb = antecolic-antegastric
– for symptomatic reflux RYGB preferred
– BPD/DS:
– Laparoscopic surgery indicated
– Poutch: 200–500 mL
– Length of the common leg = approx. 100 cm
– DS with BMI > 50
– Monitoring/prevention of deficiency symptoms necessary
– Sleeve Gastrectomy:
– Laparoscopic surgery indicated
– Calibration for gastric tube formation obligatory
– For BMI > 60: Sleeve = procedure of choice (first stage of a
multistage strategy)
– Simultaneous cholecystectomy
– Indication in patients with preoperative symptomatic cholelithiasis
– In asymptomatic cholelithiasis: consider prophylactic cholecystectomy
– Incisional hernia: postponement of surgical treatment until stable weight
is reached
– Postbariatric plastic surgery

7.8.6 Aftercare
– Regular aftercare obligatory after bariatric surgery (experienced doctor +
nutritionist); if necessary with outpatient cooperation partner
– Need for close monitoring in the first year postoperatively; within the
first 3–6 months postop. by an bariatric surgeon
– Laboratory tests recommended for the detection of deficiency symptoms
– Supplementation with vitamins and minerals: obligatory for combination
procedures and hypoabsorptive methods, recommended for purely
restrictive methods with significant weight loss
– Psychological/psychosomatic/psychiatric care recommended in case of
postoperative occurrence of psychological disorders
– Possible recommendation of participation in self-help groups
– In patients of childbearing age: during rapid weight loss
Recommendation for contraception
– Consider possible dosage adjustment of medications

7.8.7 Guidelines
AWMF S3 guideline: Surgery for obesity and metabolic diseases. Register
No. 088–001. February 2018. ► https://​www.​awmf.​org/​uploads/​tx_​
szleitlinien/​088-001l_​S3_​Chirurgie-Adipositas-metabolische-
Erkrankungen_​2018-02.​pdf
References
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SE (2019) Association of metabolic surgery with major adverse cardiovascular outcomes in patients
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prospective controlled intervention study of bariatric surgery. J Intern Med 273:219–234
[Crossref][PubMed]

Suggested Reading
Carus T (2014) Operationsatlas Laparoskopische Chirurgie. Springer, Berlin/Heidelberg
[Crossref]

Gentileschi P, Kini S, Catarci M, Gagner M (2002) Evidence-based medicine: open and laparoscopic
bariatric surgery. Surg Endosc 16:736–744
[Crossref][PubMed]

Inabnet WB, DeMaria EJ, Ikramuddin S (2005) Laparoskopic bariatric surgery. Lippincott Williams
& Wilkins, Philadelphia/Baltimore/New York/London

Karcz K, Thomusch O (eds) (2012) Principles of metabolic surgery. Springer, Berlin/Heidelberg/New

York

Lucchese M, Scoparino N (eds) (2015) Minimally invasive bariatic and metabolic surgery. Principles
and technical aspects. Springer, Berlin/Heidelberg/New York

Migrone G, Panunzi S, De Gaetano A, Guidone C, Iaconelli A, Nanni G, Castagneto M, Bornstein S,

Rubino F (2015) Bariatric-metabolic surgery versus conventional medical treatment in obese patients
with type 2 diabetes: 5 year follow-up of an open-label, single center, randomised controlled trial.
Lancet 386:964–973
[Crossref]

Rubino F, Kaplan LM, Schauer PR, Cummings DE (2010b) The diabetes surgery Summit consensus
conference: recommendations for the evaluation and use of gastrointestinal surgery to treat type 2
diabetes mellitus. Ann Surg 251:399–405
[Crossref][PubMed]

Rubino F, Nathan DM, Eckel RH, Schauer PR, Alberti KG, Zimmet PZ, Del Prato S, Ji L, Sadikot
SM, Herman WH, Amiel SA, Kaplan LM, Taroncher-Oldenburg G, Cummings DE, Delegates of the
2nd Diabetes Surgery Summit (2017) Metabolic surgery in the treatment algorithm of type 2
diabetes: a joint statement by International Diabetes Organizations. Obes Surg 27:2–21
[Crossref][PubMed]

Schauer PR, Bhatt DL, Kirwan JP, Wolski K, Aminian A, Brethauer SA, Navaneethan SD, Singh RP,
Pothier CE, Nissen SE, Kashyap SR, Investigators STAMPEDE (2017) Bariatric surgery versus
intensive medical therapy for diabetes—5 years outcomes. N Engl J Med 376:641–651
[Crossref][PubMed][PubMedCentral]

Sundbom M, Näslund E, Vidarsson B, Thorell A, Ottoson J (2020) Low overall mortality during 10
years of bariatric surgery: nationwide study on 63,469 procedures from the Scandinavian Obesity
Registry. Surg Obes Relat Dis 16:65–70
[Crossref][PubMed]

White GE, Courcoulas AP, King WC, Flum DR, Yanovski SZ, Pomp A, Wolfe BM, Spaniolas K,
Pories W, Belle SH (2019) Mortality after bariatric surgery: findings from a 7-year multicenter cohort
study. Surg Obes Relat Dis 15:1755–1765
[Crossref][PubMed][PubMedCentral]

WHO (2020) Obesity and overweight. https://​www.​who.​int/​newsroom/​fact-sheets/​detail/​obesity-and-

overweight; Accessed 15 May 2021

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_8

8. Liver, Gallbladder and Bile Ducts

Katrin Hoffmann1 and Peter Schemmer2
(1) Department of General-, Visceral- and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Germany
(2) Department of General, Visceral and Transplant Surgery, University
Hospital Graz, Graz, Austria

Katrin Hoffmann
Email: [email protected]

Peter Schemmer (Corresponding author)

Email: [email protected]

8.1 Anatomy and Physiology of the Liver

8.1.1 Definitions
– Liver, gallbladder and bile duct anatomy = variable
– Anatomy of the portal vein branches, the hepatic veins and the hepatic
artery = important in liver surgery
– Liver function and liver regeneration = central concepts in understanding
the techniques of liver surgery

8.1.2 Macroscopic and Microscopic Anatomy

Macroscopic Anatomy
Ligaments and Ligamentous Attachments
– Lig. coronarium hepatis (Lig. triangulare dextrum + Lig. triangulare
sinistrum)/coronary ligament (left and right triangle ligament)
– Lig. falciforme hepatis /falciform ligament (separates segments 4–8 from
segments 2 + 3)
– Lig. teres hepatis (obliterated V. umbilicalis)/teres hepatic ligament
– Lig. venosum hepatis (obliterated Ductus venosus)/veneous hepatic
ligament
– Lig. hepatogastricum and Lig. hepatoduodenale (part of the omentum
minus)/gastrocolic and hepatoduodenal ligament
Functional Subdivision
– In segments (according to Couinaud)
– Oriented to vascular supply
– Subdivision:
– Right liver lobe (segments 5–8)
– Left liver lobe (segments 1–4)
– Caudate lobe (segment 1) + Lobus quadratus
– Cava-gallbladder line = dividing line between right/left liver lobe

Microscopic Anatomy
– Radial blood flow from portal vein and arterial blood through hepatic
sinusoids to central vein
– Intercellular transport of bile into bile ducts
– Glisson’s triad = interlobular branches of each portal vein, artery and bile
duct

8.1.3 Tasks of the Liver and Functional Liver Volume

Tasks of the Liver
– Liver = central metabolic organ with detoxification and synthesis
functions

Blood Formation Site (Embryonic Period)

Protein Biosynthesis and Degradation
– Under hormonal control
– In particular, formation coagulation factors (under the influence of
vitamin K)
– Protein degradation with formation of urea (excretion via kidney)
Cleavage of Carbohydrates and Glycogen Storage
– Glycogen storage
– Under the influence of: adrenaline, glucagon
Central Organ of Lipometabolism
– Formation and elimination of lipoproteins
Special Metabolic Services
– Bilirubin transport
– Biosynthesis of bile acids
Detoxification Function (Through Biotransformation)
– Protection of the organism from foreign substances and drugs
– In particular by means of cytochrome P450
Central Organ of the Trace Element Metabolism
– Trace elements: iron, copper, zinc, etc.
– Numerous vitamins: especially vitamin A
Immunological Function
– Phagocytosis of cellular elements (outdated erythrocytes), bacteria
– Elimination of immune complexes and endotoxins

Functional Liver Volume After Resection

Functional Liver Volume After Resection (FLR)
– Definition: Proportion (in %) of liver volume that must exist after
resection in order to maintain liver function
– Crucial for predicting liver function after liver resection
– Liver disease (Nonalcoholic steatohepatitis-NASH) or pre-damage of the
liver (chemotherapy associated steatohepatitis-CASH) = influence on
functional liver volume after resection
FLR Targets
– With normal liver function: FLR ≥20%
– After chemotherapy: FLR ≥30%
– In cirrhosis: FLR ≥40%
Measurement of FLR
– Mostly by 3D-CT volumetry: direct measurement of total liver volume
(TLV)
– Calculation of the standardized FLR (sFLR): taking into account the
portion to be resected

8.1.4 Location
Position Projection
– Hepatic superior border: medioclavicular = fourth intercostal space
(ICS); medioaxillary = sixth ICR; paravertebral = eighth ICS
– Liver inferior border: medioclavicular = eighth rib; medioaxillary = tenth
rib; paravertebral = tenth ICS
– Topographical relationship: to the inferior pleural space + base of the
lung
– Variable with inspiration/expiration

8.1.5 Measured Values

Weight
– General liver = 2.5% of body weight
– Adult man = approx. 1600 g
– Adult woman = approx. 1400 g
– Volume and weight increased during digestion (blood inflow approx. 500
g)

Linear Readings
– Cross diameter (right-left) = 28 cm (20–40 cm)
– Sagittal diameter (ventral-dorsal) = 8 cm (5–12 cm)
– Height (cranial-caudal) = 10 cm (up to 27 cm)

8.1.6 Blood Supply and Drainage

Blood Supply and Drainage of the Liver
Arterial Inflow = Hepatic Artery
– Common hepatic artery: direct outlet from truncus coeliacus
– Hepatic artery proper: after delivery A. gastroduodenalis and A. gastrica
dextra
– Division into right abd kifte hepatic artery
– Cystic artery: usually from right hepatic artery
– Numerous position variations
Numerous variants of arterial inflow
– Aberrant outflow from superior mesenteric artery possible (complete or
right hepatic artery)
– Left hepatic artery partly from left gastric artery or from coeliac trunc
directly
– Variants with accessory vessels e.g. separate branch to supply segment 4
from the hepatica propria artery
Portal Venous Inflow = portal vein
– Liver side of the confluence of splenic vein + superior mesenteric vein, if
necessary inferior mesenteric vein (position variants)
– Collaterals: V. coronarian vein + cystic vein, as well as branches to the
pancreatic head
– Division pattern in liver hilum:
– Right portal venous branch (segments 5–8); subdivision into:
– Anterior pedicle (segments 5 + 8 + parts of 4 if necessary) +
– Posterior pedicle (segments 6 + 7)
– Left portal venous branch (segments 2–4)
– Rare trifurcation
Venous Outflow
– Hepatic veins:
– Right hepatic vein (segments 6 + 7)
– Middle hepatic vein (segments 4, 5 + 8)
– Left hepatic vein (segments 2 + 3)
– Norm variant accessory right inferior hepatic vein (segments 6 + 7)
– The hepatic veins open directly subdiaphragmally into the inferior vena
cava
 Different variants
– Common orifice of middle + left hepatic vein
– Direct orifice of segmental veins into inferior vena cava (segment 1)
– Sonographically distinguishable from portal venous branches by the
absence of a connective tissue sheath
– Blood supply to the liver: 25% hepatic artery, 75% portal vein
– Proportion of oxygenation of liver blood: 40–50% hepatic artery, 60%
portal vein
– Autoregulation: low portal venous flow leads to increased arterial flow -
hepatic arterial buffer response

8.1.7 Terminology of Liver Resections (◘ Fig. 8.1)

– Consensus Terminology: International Conference (Belghiti et al. 2000)
Fig. 8.1 Functional subdivision of the liver and terminology of liver resections. (Mod. according to
Scott-Conner 2002)

Anatomical (= Typical) Liver Resections

– Right hemihepatectomy = right hepatectomy: resection of segments V–
VIII
– Left hemihepatectomy = left hepatectomy: resection of segments II–IV
– Left-lateral sectorectomy = Bisegmentectomy II + III: Resection of
segments II–III
– Extended right hepatectomy = right trisectorectomy: resection of
segments IV–VIII
– Extended left hepatectomy = left trisectorectomy: resection of segments
II–IV + V + VIII

Atypical Liver Resections

– Resections outside the anatomical landmarks (regardless of the resected
liver volume)

8.2 Diseases of the Liver

8.2.1 Benign Diseases
Key Points
– Adenomas are precancerous and need to be resected depending on both
size and histology
– Hemangiomas and FNH (focal nodular hyperplasia) are resected if
symptomatic

Hepatocellular Adenomas
Definition and Subtypes
Definition
– Clonal non-encapsulated neoplasms
– Mostly from highly differentiated hepatocellular cells
Subtypes
– HNF-1alpha-inactivated adenomas = 40% of all adenomas; association
with MODY type 3
– Inflammatory adenomas = 50% of all adenomas, increased risk of
bleeding due to ectasia of sinusoidal structures
– β-Catenin-mutated adenomas, high risk of malignant transformation
(approx. 40%)
– Unclassified adenomas = approx. 10% of all adenomas
– Adenomatosis ≥10 adenomas
Epidemiology and Risk Factors
Epidemiology
– Incidence = 3–4/100.000 in Europe
Risk Factors
– Taking oral contraceptives
– Anabolic Abuse
– Glycogen storage disorders, galactosemia
– perfusion disturbances of the liver
Symptoms and Complications
– Often diagnosed incidentially in asymptomatic patients
– Possible complications:
– Sponaneous rupture and hemorrhage
– Malignant transformation into HCC (hepatocellular carcinoma), risk
8–13% in β-Catenin-mutated adenomas
Diagnosis
– Sonography
– MRI
– Biopsy: indicated for all adenomas
Surgery Indication
– β-catenin mutated adenomas
– Adenomas >5 cm
– Adenomas in men—differentiation from HCC often difficult
– Adenomas with risk of rupture (in case of acute bleeding first
embolization, surgery two-sided)
– Size progression after discontinuation of oral contraceptives

Focal Nodular Hyperplasia (FNH)

Definition
– Tumor with hepatocellular origin, polyclonal
– Absence of central and portal veins (= absence of classical hepatic
architecture)
– Macroscopic: typical picture with central scar and ochre parenchyma
– Risk factor: taking estrogen-containing preparations
Epidemiology
– Second most common benign liver tumor
Diagnosis
– Sonography
– MRI
– Biopsy: not indicated with clear imaging
Complications
– Progressive growth
– Rupture
– Portal hypertension
– Bleeding: rare
– Necrosis: rare
– Malignant degeneration: not described

Therapy
Conservative Approach: Generally
Surgical Therapy
– Indications:
– Symptomatic patients
– Large displacing-growing FNH
– Cholestasis
– Vascular compression
Hemangiomas
Definition
– haemangioma of mesenchymal origin
Epidemiology
– Most frequent benign lesions = 0.5–7% of all patients
– Women three times more often affected than men
Clinical Presentation and Complications
Clinical Presentation
– Mostly incidental findings and often asymptomatic
Complications
– No malignant transformation risk
– Kasabach-Merritt syndrome:
– Hemangioma bleeding, thrombocytopenia and consumption
coagulopathy
– Rare complication of generalized giant hemangiomas
– Mortality risk in case of hemorrhage 30–40%
Diagnosis
– Sonography (also contrast medium supported ultrasound)
– CT
– Biopsy: not indicated

Therapy
Conservative Therapy
– Whenever there is no indication for surgery
Surgical Therapy
– Surgery indication:
– Symptomatic patients
– Lesion >5 cm
– Significant hemorrhage, location near the capsule with risk of rupture
– Acute bleeding without the possibility of control by interventional
radiology

Echinococcosis
Definition
– Tapeworm zoonosis
– Human = intermediate host
– Tapeworm species:
– Echinococcus granulosus = Echinococcus cysticus = dog tapeworm
– Echinococcus multilocularis = Echinococcus alveolaris = fox
tapeworm

Clinical Presentation
E. granulosus (Cysticus, Unilocularis), Dog Tapeworm
– Displacing growth
– Infestation of lung and pericardium also possible
– Pericyst partly calcified as membrane around cystic hydatid
– Often asymptomatic
– Due to size non-specific upper abdominal complaints or infection
symptoms
E. multilocularis (alveolaris), Fox Tapeworm
– Infiltrative (tumor-like) growth with formation of small cysts
– Recognised occupational disease in hunters and foresters
– Often asymptomatic
– Due to size non-specific upper abdominal complaints or infection
symptoms
Diagnosis
– Incidental finding on imaging
– Serological: Detection of antibodies
– Puncture: Contraindicated!

Therapy
Surgical Therapy
– Whenever possible
– Only under perioperative medication with mebendazole or albendazole
– Treatment only in cooperation with infectiologists

Surgical Procedure
Surgery for Echinococcosis Cyst
 – Rupture of the cyst and/or extravasation of cyst fluid must be avoided
at all costs, otherwise contamination of the situs with the development
of disseminated intra-abdominal disease
– Endocystectomy (procedure of choice for E. granulosus (Kniepeiss et
al. 2020)):
– Repositioning of the liver and the cyst with cloths soaked in 20%
saline solution
– Puncture of the cyst with a disposable trocar + aspiration of the cyst
fluid while filling with physiological saline solution
– Uncapping of the cyst
– Exclusion of connection to the bile duct system = e.g. White test
(white fat emulsion approved for i.v. infusion, e.g. lipofundin®,
intralipid® via ductus cysticus)
– Installation of compresses soaked in 20% saline solution for 20 min
= Caution: If bile ducts are opened, bile duct necrosis!—rule out
beforehand and over sew bile leaks if necessary
– Suturing of the caspel edge or bipolar coagulation
– Pericystectomy: Resection of the entire cyst plus surrounding liver
tissue—technically more difficult, risk of rupture!
– Typical/anatomical liver resection according to oncological criteria in
E. multilocularis

8.2.2 Malignant Diseases of the Liver

Key Points
– Distinguish primary—such as CCC (cholangiocarcinoma), HCC
(hepatocellular carcinoma)—and secondary malignant findings
(metastases)

Primary Tumors
Hepatocellular Carcinoma (HCC)
Epidemiology and Risk Factors
– Incidence
– Incidence = 10/100.000 in Germany
– Risk factors
– Cirrhosis of the liver of any etiology (alcohol, hepatitis,
hemochromatosis, etc.)
– Chronic hepatitis B/C virus infection
– Cumulative 5-year risk of developing HCC in patients with
HCV(hepatitis C virus)-associated liver cirrhosis in Europe = about 17%
– Non-alcoholic fatty liver hepatitis (NASH) as a consequence of
diabetes mellitus and the metabolic syndrome—number 1 rising risk
factor world wide
– Aflatoxin exposure
Early Detection
– Screening program for all patients with:
– Liver cirrhosis
– Chronic hepatitis B/C
– Fatty liver disease,
– Steatohepatitis
– Sonography every 6 months
Special Forms
– Fibrolamellar HCC—young patients often better prognosis
– Mixed differentiated tumors (combined HCC/intrahepatic
cholangiocarcinoma)
– Early HCC—transition from regenerated node to HCC
Clinical presentation and Classification
– Clinical presentation
– Mostly asymptomatic
– Conspicuous in routine examinations of cirrhotic patients
– TNM classification (HCC)
– T (tumor)
– T1 Solitary tumor without vascular invasion
 – T2 Solitary tumor with vascular invasion or multiple tumors all <5
cm
– T3a Multiple tumors >5 cm
– T3b Multiple tumors involving a major branch of the V. portae or
Vv. hepaticae
– T4 Tumor with invasion of adjacent organs or perforation of the
visceral peritoneum
– N (lymph nodes)
– N0 No locoregional lymph nodes
– N1 Locoregional lymph nodes
– M (metastases)
– M0 No distant metastases
– M1 Distant metastases
– UICC stages according to the TNM classification (eighth edition,
January 2018)

I T1 N0 M0 II T2 N0 M0
IIIA T3 N0 M0 IIIB T4 N0 M0
IVA Any T N1 M0 IVB Each T Each N M1

Diagnosis
– Diagnostic imaging
– CM Sonography
– Primovist MRI
– CT
– Characteristic signs in imaging
– Arterial hypervascularization with rapid washout of the contrast
medium and relative contrast reversal to the surrounding liver
parenchyma
– Biopsy
– Only if unclear imaging or therapeutic consequence
– Tumor marker
– AFP only suitable for assessment of progression, not for diagnosis
Surgical Therapy
– Liver resection (Lin et al. 2012; de Santibañes et al. 2017)
– Indications:
– Patients with potentially resectable HCC without cirrhosis
– Patients with potentially resectable HCC and Child A/B cirrhosis
– Presence of portal hypertension (ascites, platelets <100.000,
splenomegaly) = not a sole exclusion criterion for resection, but
significantly increases the surgical risk
– Atypical resections = leaving as much functional liver tissue as
possible
– 5-year survival = 30–50%, but high recurrence rates due to de novo
tumors in cirrhosis or micrometastases
– Liver transplantation (Lin et al. 2012)
– Indications depending on local/national legal regulations and/or
guidelines
– Treatment of HCC + underlying liver cirrhosis
– Prioritization and organ distribution according to local/national legal
regulations and/or guidelines (Eurotransplant region—MELD (Model
for End-Stage Liver Disease) score based:
– Patients receive extra points with increasing waiting time)
– Transarterial chemoembolisation (TACE), local thermal ablation
(up to 3 cm diameter), liver resection: allows bridging during
waiting period = recommended to avoid progression
– Other local ablation procedures (RFA (radiofrequency ablation),
PEI (percutaneous ethanol injection), SIRT (selective internal
radiotherapy), IRE (irreversible electroporation), cryotherapy)
– 5-year survival rates = up to 70%; local recurrence rate < 15%

Intrahepatic Cholangiocarcinoma: CCC

Definition
– Originating in the liver from the bile ducts
Epidemiology
– Incidence
– Incidence = 1–2/100.000 in Germany
– Risk factors
– Cholelithiasis
– Cirrhosis on the basis of chronic hepatitis C infection, alcoholic and
non-alcoholic hepatitis
– Uptake of carcinogens (nitrosamines, aflatoxins, anabolic steroids,
etc.)
– Congenital anomalies of the bile ducts
– Concomitant diseases (e.g. primary sclerosing cholangitis, ulcerative
colitis, α-antitrypsin deficiency)
Symptoms and Classification
– Symptoms
– Abdominal pain
– B-symptomatics
– Icterus
– TNM classification (CCC)
– T (tumor)
– Tis carcinoma in situ intraductal tumor
– T1 Solitary tumor without vascular infiltration
– T2a Solitary tumor with vascular infiltration
– T2b Multiple tumors with or without vascular infiltration
– T3 tumors with infiltration of the peritoneum or direct invasion of
extrahepatic structures
– T4 tumors with periductal invasion
– N (lymph nodes)
– N0 No lymph node metastases
– N1 Lymph node metastases
– M (metastases)
– M0 No distant metastases
– M1 Distant metastases
 – UICC stages according to the TNM classification

0 Tis N0 M0
I T1 N0 M0
II T2 N0 M0
III T3 N0 M0
IVa T4 N0 M0
Each T N1 M0
IVb Each T Each N M1

Diagnosis
– Imaging
– Sonography
– CT
– MRI with MRCP (with Primovist)
– ERCP (endoscopic retrograde cholangiopancreaticography)
– Tumor marker
– CA 19-9 = progress assessment
– Caution: Also elevated in cholangitis and jaundice
Therapy
– Surgical therapy
– Resection = only curative option
– Anatomical resection + lymphadenectomy in the hepatoduodenal
ligamentrecommended
– Contraindications:
– Satellite nodules and or bilobar manifestation
– Remote metastases
– Peritoneal carcinomatosis
– 5-year survival rates = 23–42% after R0 resection without lymph
node metastases
– Adjuvant therapy:
– according to local guide lines, preferably within the context of clinical
trials

CCC of the Common Hepatic Duct Bifurcation: Klatskin Tumors

Definition
– First described by Gerald Klatskin in 1965 (hence the synonym Klatskin
tumor).
– Perihilar cholangiocellular carcinoma in the region of the common
hepatic duct bifurcation
Epidemiology
– Incidence 1/100.000 in Germany
Symptoms and Classification
– Symptoms
– Icterus
– Pruritus
– Abdominal pain
– B-symptomatics
– Differential diagnosis
– HCC
– Liver metastases
– Pancreatic cancer
– Cholangitis
– Cholecystitis, choledocholithiasis
– Biliary strictures, bile duct cysts
– Classification according to Bismuth-Corlette in type I-IV
– I Distal common hepatic duct to the confluence of the right and left
bile duct
– II Common hepatic duct bifurcation
– IIIa Common hepatic duct bifurcation and right hepatic duct
– IIIb Common hepatic duct bifurcation and left hepatic duct
 – IV Common hepatic duct bifurcation and both hepatic ducts or
multifocal
– TNM classification (Klatskin tumours)
– T (tumor)
– Tis carcinoma in situ, intraductal tumor
– T1 Tumor limited to bile duct with spread to muscularis
– T2a Tumor infiltrates periductal fat tissue
– T2b Tumor infiltrates surrounding liver tissue
– T3 Tumor with infiltration of the equilateral portal vein or hepatic
artery
– T4 Tumor infiltrates main trunk of portal vein or bilateral portal
vein branches or common hepatic artery or secondary bile ducts
bilaterally or secondary bile ducts unilaterally with invasion of
contralateral portal vein or hepatic artery
– N (lymph nodes)
– N0 No lymph node metastases
– N1 Regional lymph node metastases (incl. metastases along the
cystic duct, choledochal duct, portal vein and hepatic artery)
– N2 Lymph node metastases periaortic, pericaval, along the superior
mesenteric artery and or truncus coeliacus
– M (metastases)
– M0 No distant metastases
– M1 Distant metastases
– UICC stages according to the TNM classification

0 Tis N0 M0
I T1 N0 M0
II T2 N0 M0
III T3 N0 M0
IVa T4 N0 M0
Each T N1 M0
IVb Each T Each N M1

Prognosis
– Prognostic factors:
– Lymphnode metastases
– Tumor differentiation
– Perineural invasion
– R1 resection status
Therapy
– Surgical therapy
– Resection = only curative option
– Indications:
– Type 1 Extrahepatic bile duct resection possibly with
duodenopancreatectomy
– Type 2–4 Extrahepatic bile duct resection + liver resection

Surgical Procedure
Klatskin Tumors
– Depending on the type (extended) hemihepatectomy right or left +
creation of biliodigestive anastomosis + lymphadenectomy in the
hepatoduodenal ligament
– if necessary in combination with resection of the equilateral portal
vein and hepatic artery in case of infiltration
– General portal vein resection without oncological advantage
– Intraoperative frozen sections from the proximal and distal resectional
margins; caution: discontinuous growth
– If bilirubin is highly elevated = preoperative PTCD
(cholangiodrainage) or ERCP + stent = relief of cholestasis = better
liver function and less morbidity postoperatively
– In preparation for augmentation procedures to increase functional
liver reserve prior to resection (portal vein embolization)

– Contraindication:
– Secondary bile ducts infiltrated on both sides
– Invasion of the portal vein or hepatic artery of the opposite side
– Bilateral vascular infiltration
 – Remote metastases
– Liver cirrhosis or pronounced fibrosis (functional liver reserve)
– Chemotherapy/radiochemotherapy:
– according to local guide lines, preferably within the context of clinical
trials

Liver Metastases
Indications
– Resection of metastases of colorectal cancers = standard therapy
– Resection of metastases from non-colorectal cancers:
– Increasing frequency
– Individual tumor biology and the possibility of R0 resection are
decisive here
– Recurrent resections as well as two-stage resections possible
Therapy
– All resection techniques and circumferences possible
– Decisive = possibility of extrahepatic tumor freedom
– Prognosis-limiting factor = R0 resection
– If necessary, combination with multimodal concepts—neoadjuvant
chemotherapy and/or interventional radiological procedures such as RFA
– Caution: Chemotherapy-pretreated liver = reduced regenerative
capacity, at least 45% residual volume required

8.2.3 Technique of Liver Resection

Planning of the Resection
Technical Conditions
– Review of vascular anatomy—inflow (hepatic artery and portal vein),
outflow (hepatic veins) and bile ducts; normative variants (aberrant or
accessory vessels)
– Exclusion infiltration of structures
Parenchyma Conditions
– Normal tissue, steatosis, steatohepatitis (after chemotherapy), fibrosis,
cirrhosis
– Liver function
– Planned extent of resection vs. volume of future liver remnant =
functional liver reserve
Caution
– Normal tissue 25% residual volume sufficient
– After chemotherapy at least 45% residual volume
– In cirrhosis only if hyper-Child-Pugh A or A and bilirubin <2—otherwise
high mortality after conventional (open) resection; laparoscopic
parenchymal-sparing minor resection also acceptable in Child C patients,
the minimal invasive robotic approach has similar complication rates as
open or laparoscopic procedures (Murtha-Lemekhova et al. 2022)
Strategy of Resection
– Limited resections only
– Anatomical resections along the segment boundaries
– Always choose the most parenchyma-sparing procedure if possible
– if necessary, two-stage resections or multimodal combination with
ablation

Resection Type
Minor Resection (<3 Segments)
– Monosegmentectomy
– Bisegmentectomy
– Anterior (segments V + VIII) or posterior (segments VI + VII)
sectorectomy
– Wedge resection = for superficial findings
– Atypical resection = incision after marking and sonography of vascular
structures which must be spared
Major Resection
– Right hemihepatectomy (segments V, VI, VII, VIII)
– Left hemihepatectomy (segments II, III, IVa, IVb)
– Extended right hemihepatectomy (segments IVa, IVb, V, VI, VII, VIII)
– Extended left hemihepatectomy (segments II, III, IVa, IVb, V, VIII)
– Mesohepatectomy (segment IVa, IVb, V, VIII)

Surgical Procedure
Liver Resection (Conventional/Open)
– Supine position
– Reversed L shape incision for right/left or extended right/left
resections; median laparotomy for left lateral resections
– Inspection and palpation of the liver, the hepatoduodenal ligament and
the entire abdomen
– Complete mobilization of the liver: use of the falciform ligament to
pull on the liver, transection of the triangular ligaments and the teres
hepatis ligament to obtain enough mobility for sonography and
resection
– Dissection along the vena cava:
– Caution: Makuuchi ligament between segment 8 dorsal to v. cava
often includes veins draining into v. caval vein (to be divided for
right hemihepatectomy)
– Caution: Mobilize the whole liver and not only the liver lobe that
will be resected!
– Intraoperative ultrasound to check vascular anatomy, extent of
findings and positional relationship of lesions to vascular structures
obligatory
– Complete lymphadenectomy in the hepatoduodenal ligament only
indicated for cholangiocarcinoma and gallbladder cancer; for liver
metastases of CRC in studies
– Cholecystectomy with long-left ductus cysticus—temporary closure
with Bulldog clamp for later white test (Lipofundin®, Intralipid®, or
similar) for visualization of the bile ducts at the resection area
– For all major resections:
– Intravascular control: exposure and tightening of the artery and
portal vein of the half of the liver to be resected (right pedicle in
the area of the Gans fissure)
– Outflow control: visualization and tightening of the hepatic vein to
be resected
 – Before parenchymal transection a CVP <5 is obligatory = less
bleeding! (Anti-Trendelenburg positioning, low volume supply, if
necessary vasodilators i.v.)
– Control of the arterial inflow by dividing the feeding artery by
means of clips or hemostatic suturing
– Dividing the portal vein and hepatic vein by means of a vascular
stapler or hemostatic suturing after clamping
– Parenchymal transection:
– Ultrasonic dissection (CUSA = Cavitron Ultrasonic Surgical
Aspirator) in combination with bipolar coagulation, clipping and
ligation = advantage: precise transection; disadvantage: time-
consuming
– Endovascular staplers (= stapler hepatectomy (Schemmer et al.
2006)) = crush clamp of the liver parenchyma with a straight clamp
with subsequent stapling = advantage: time-saving; disadvantage:
not applicable for all anatomical resections (e.g. isolated segment
VIII)
– Water jet dissection = similar to ultrasonic method
– Ultrasonic scissors = especially for atypical resections, less precise
than ultrasonic dissector
– Advanced coagulation technology = e.g. LigaSure®,
Ultracision®/Sonicision™, bipolar scissors, Habib-Sealer
(thermoablative)
– Finger fracture = crushing of liver tissue between fingers, supply of
vessels by means of clips or stitches

Technology
– It is essential to ensure that the vascular and bile duct structures of the
remaining liver areas are protected, irrespective of the resection
procedure used
– Pringle maneuver:
– Targeted short-term clamping of the portal vein + hepatic artery during
the transection phase using silicone reins in order to reduce the
tendency to bleed
 – Rarely used with modern open resection techniques as ischemia-
reperfusion damage in the remaining liver tissue as well as a higher
tumor recurrence rate can be detected
– No differences between intermittent and continuous Pringle
manoeuvre
– If necessary, maximum 20 min recommended
– Post-resection phase:
– Achiving haemostasis with argon beamer, bipolar electrocoagulation,
etc.; if necessary over sawing of vessels
– Retrograde control for bile leakage with White-Test (alternatively
diluted blue staining = lower detection rate) via cystic duct under
manual compression of the Ductus choledochus (Caution: Dislodging
of the Ductus choledochus with clamp or bulldog may lead to
necrosis) = if necessary re-positioning of bile ducts at the resection
area
– Optional application of haemostyptics or sealants
– If necessary, reconstruction of the bile duct by means of biliodigestive
anastomosis according to Y-Roux
Possible Complications
– Postoperative (postheoatectomy) liver failure PHLF—insufficient
residual volume—definition according ISGLS (Rahbari et al. 2011)
– Protein deficiency—edema, ascites
– Hepatorenal syndrome
– Postoperative bleeding
– Biliary leakage and bilioma formation—definition according ISGLS
(Koch et al. 2011):
– Low “sweating out” of bilirubin = usually suspended
– High volume leakage: indicates bile leakage = revise early + oversaw
– Superinfected fluid collection/abscess = interventional CT-guided
drainage
– Biliopleural fistula = rare, especially after simultaneous diaphragmatic
resection
– Atelectasis + pleural effusion on the right = respiratory training and
drainage if necessary
8.3 Liver Transplantation
8.3.1 General and Legal Basis
(National) Legal Basis
Definition
– Regulated by:
– Local official regulations and guidelines

Indication and Listing

Indication for Liver Transplantation
– Based on the local official regulations and guidelines
– Set in the interdisciplinary transplant conference in consensus between
the departments involved and legal guidelines
Listing
– Potentially all patients with rapidly progressing or already far advanced
irreversible chronic liver diseases, for which no conservative-internistic
or surgical treatment alternative with equal chances of success exists
– Listing for transplantation (waiting list), if probability of survival appears
to be greater with a liver transplant
– Listing based on assessment of liver function and tumor stage (HCC and
others (Talakic et al. 2021) as well as disease-specific complications
(dominant stenosis PSC)

Organ Allocation
Eurotransplant
– Organization for organ allocation based on national guidelines
– Coordinates the allocation of organs subject to transfer such as heart,
kidney, liver, lung, pancreas and intestine (§ 8 TPG)
– In Austria, Belgium, Croatia, Hungary, Luxembourg, the Netherlands,
Slovenia and Germany
Principles of Organ Allocation
– Allocation of organs to individual patients or centres by Eurotransplant
on the basis of national regulations
– Patients’ listing on the Eurotransplant wait list for transplantation as soon
as the treating transplant centre has made the indication and all necessary
examinations are available

8.3.2 Evaluation and Follow-Up of Liver Function

Clinical Follow-Up
– Basic parameters:
– Progressive physical weakness, fatigue, muscular deficits
– Hepatic encephalopathy
– Therapy refractory ascites
– Spontaneous bacterial peritonitis
– Hepatorenal syndrome
– Gastrointestinal bleeding/variceal bleeding
– Hepatopulmonary syndrome
– Progressive osteopathy
– Recurrent biliary sepsis
– Occurrence of hepatocellular carcinoma

Laboratory Parameters of Liver Synthesis and Excretion

– Only limited suitability for risk assessment

Hepatocellular Integrity
– GOT
– GPT
– LDH

Biliary Integrity
– alkaline phosphatase
– γ-GT

Synthesis Performance of the Liver

– Albumin
– Cholinesterase (CHE)
– PT
– INR
– Fibrinogen

Excretory Capacity of the Liver

– Bilirubin (direct, indirect)
– Bile acids

Scoring Systems for Liver Function and Prognosis

– For the assessment of patient survival and mortality risk (limited
possible)

Child-Pugh Score (◘ Table 8.1)

– Disadvantages:
– Subjective assessment of the therapeutic response of ascites and
encephalopathy
– Continuous deterioration of the patient’s condition is often not
reflected in a change in child classification

Table 8.1 Child-Pugh scorea

1 point 2 points 3 points

Encephalopathy None
Ascites, therapy No ascites Moderate, controlled by therapy Pronounced despite therapy
Bilirubin <35 μmol/L −35 to 50 μmol/L >50 μmol/L
Albumin >3.5 g/dL −2.8 to 3.5 g/dL <2.8 g/dL
INR <1.7 −1.7 to 2.3 >2.3

INR International Normalized Ratio

a
Child A = 5–6 points; Child B = 7–9 points; Child C = >10 points

MELD Score
– MELD Criteria:
– Bilirubin
– Creatinine
– Coagulation: INR (International Normalized Ratio; Prothrombin
Ratio)
– Formula:
– MELD score = 10 × (0.957 × log(creatinine) + 0.378 log(bilirubin) +
1.12 log(INR) + 0.643)

8.3.3 Indications for Liver Transplantation: Relevant

Underlying Diseases in Adults
Chronic Liver Disease
Underlying Disease for Liver Cirrhosis
– Hepatitis B, C, D
– Autoimmune hepatitis
– Alcololic liver disease
– Cryptogenic
Cholestatic Liver Disease
– Primary sclerosing cholangitis (PSC)
– Primary biliary cirrhosis (PBC)
– Secondary sclerosing cholangitis
– Familial cholestasis syndromes
– Biliary atresia

Chronic Drug Toxicity

Metabolic Diseases/Genetic Diseases
– α1-antitrypsin deficiency
– Wilson’s disease
– Hemochromatosis
– Glycogen storage diseases
– Galactosemia
– Tyrosinemia
– β-Thalassemia
– TTR (transthyretin) amyloidosis
– Cystic Fibrosis
– Hypercholesterolemia (LDL receptor deficiency, Crigler-Najjar
syndrome type 1)
– Erythropoietic protoporphyria
– Primary amyloidosis
– Urea Cycle Defects
Other Diseases
– Congenital cystic liver
– Echinococcosis of the liver
– Chronic Budd-Chiari syndrome
Acute Liver Disease
– Fulminant liver failure
– Etiologies:
– Poisoning
– Hepatitis
– Budd-Chiari Syndrome
– Drug toxicity, etc.
– Definition: Acute liver failure based on Kings College score, Clichy
criteria, or BiLE score
– Pregnancy-associated liver diseases
– Extensive liver trauma
– Postoperative liver failure after liver resection or transplantation

Malignant Diseases of the Liver

Hepatocellular Carcinoma (HCC)
– Based on national german guidelines indication only if within MILAN
criteria for the entire period prior to liver transplantation = singular HCC
<5 cm or up to 3 foci <3 cm (international scientific based guidelines i.e.
MILAN citeria (with possible downstaging), UCSF criteria, Up-to-5
criteria, Kyoto criteria, AASLD, EASL) (Bento de Sousa et al. 2021;
Cusi et al. 2022; European Association for the Study of the Liver.
 Electronic address: [email protected]; European Association for
the Study of the Liver 2018)
– 5 year survival up to 85%
Cholangiocarcinoma (CCC)
– Should be performed within prospective randomized clinical trials only

Epithelioid Hemangioendothelioma
Caution
– Indication for liver transplantation in the case of malignant primary
disease
– Transplantation only in patients who have a significantly better
chance for recovery and long-term survival with transplantation
than without transplantation or with the use of alternative therapies
– Liver metastases in other primary tumors (e.g. colorectal tumors) =
currently (at least outside of studies) no indication for liver
transplantation

8.3.4 Contraindications for Liver Transplantation

Lack of Patient Adherence/Psychosocial Problems
– Adherence = beyond consent to transplantation, willingness and ability to
cooperate in the treatments and examinations required before and after
transplantation
– Reliable intake of immunosuppressants + regular outpatient follow-up
examinations = absolute prerequisites for successful transplantation
– Psychological consultation before transplantation = obligatory
– Continued alcohol or drug abuse = clear contraindication until
completion of consistent withdrawal and addiction treatment

High Age
– Probability of presence of concomitant diseases that speak against
transplantation (such as cardiovascular problems) increases with age
– Above 65 years of age Clarify indication individually
– The decisive factor is the biological age of the patient
Cardiovascular and Pulmonary Concomitant Diseases
– To be excluded:
– Severe valvular heart disease
– Severe pulmonary hypertension
– (Alcohol Toxic) Cardiomyopathy
– Coronary vascular disease and myocardial infarction

Caution
Cardiovascular and pulmonary concomitant diseases = risk during
transplantation or longer-term transplant success.

– General fitness for anaesthesia must always be checked

Infections
– Chronic suppurative infections (e.g. osteomyelitis, sinusitis, abscesses):
– Need to be treated before transplantation
– So is spontaneous bacterial peritonitis
– In active tuberculosis:
– No liver transplant
– Tuberculostatic therapy required for at least 3 months (if possible for 1
year)
– HIV infection = no contraindication
– AIDS = contraindication

Extrahepatic Metastases
– Extrahepatic tumor manifestation = absolute contraindication

8.3.5 Surgical Principles

Patient Positioning
– Supine position
– Both arms alongside the body

Laparotomy
– “Reversed L-shape incision” in the right upper quadrant of the abdominal
wall (reversed angle incision to the right)
– Alternatively, transverse upper abdominal laparotomy (costal margin
incision) without or with median extension to the xyphoid (bilateral
rooftop incision with vertical extension)

Recipient Hepatectomy
– Severing of the falciform ligament between ligatures
– Mobilization of the diseased liver from the hepatoligamentous structures
– Ductus hepatocholedochus, hepatic artery and portal vein separated after
close-to-life preparation—corresponds to the beginning of the anhepatic
phase of the recipient
– Completely mobilized liver is dissected from the recipient vena cava
while supplying (clipping/LigaSure) numerous small hepatic veins
(Houben et al. 2014; Kniepeiss et al. 2020)
– 3 large hepatic veins are transected with the help of two endo-vascular
stackers
– Stop bleeding
– Inferior vena cava is partially clamped tangentially with a Satinski clamp

Machine Perfusion of the liver

– Novel method for organ preservation + reconditioning (van Rijn et al.
2021; Karangwa et al. 2020; Martins et al. 2020; Ceresa et al. 2022;
Sousa Da Silva et al. 2022)
– Considering shortage of donor livers suitable for transplantation, this
technique may help avoid needless wastage of organs
– Goals: (1) improvement of quality of marginal livers, (2) extension of
time for which liver can be preserved, (3) enabling an objective
assessment of liver quality/viability
– Hypothermic vs. normothermic machine perfusion

Implantation
– Donor organ placed in the right upper abdomen of the recipient

Vena Cava Anastomosis

– In modified piggy-back technique side-to-side cavocaval anastomosis
between donor and recipient
– Front and back wall each separately continuous with Prolene threads of
strength 4-0

Portal Vein Anastomosis

– if necessary, shortening of the portal vein to avoid kinking
– End-to-end portal vein anastomosis
– Separately for the front and back wall in continuous technique with
Prolene threads of strength 5-0

Reperfusion
– With the reperfusion of the liver the anhepatic phase is finished

Portal vein
– Portal venous reperfusion is widely considered as standard in adults
– Subsequently, completion of the portal venous anastomosis and closure
of the caudal opening of the donor’s vena cava; the cranial portion of the
donor’s vena cava was already closed during backtable surgery

Hepatic artery
– Artery is usually anastomosed with Prolene sutures of different
thicknesses (5-0 to 7-0) in continuous or single button suture technique.
– Here, the back wall can be sewn continuously; front wall in single button
technique
– To avoid anastomotic stenosis, the branch-patch technique is used so that
the anastomosis is located between the bifurcation of the gastroduodenal
artery (which is usually blocked) and the hepatic artery on the recipient
side and the bifurcation between the gastroduodenal artery and the
hepatic propriocele artery on the donor side.
After reperfusion, arterial and portal venous blood flow is determined in
mL/min.

Bile Duct
– Bile duct anastomosis:
– Usually as end-to-end anastomosis with 5-0 PDS (polydioxanon)
– Either single knots or running suture
– In selected cases (e.g. PSC of the recipient), a biliodigestive
anastomosis with Roux-Y reconstruction is indicated
– 2 percutaneously inserted easy-flow drains, one subphrenic, the other
subhepatic

Postoperative Phase
– Interdisciplinary intensive care unit
– Interdisciplinary treatment (e.g. gastroenterologists/hepatologists,
nephrologists, infectiologists, etc.)

8.4 Anatomy and Physiology of the Gallbladder

and Bile Ducts
8.4.1 Gallbladder (Vesica Biliaris)
– Lateral under lobus quadratus of liver segments IVb/V
– Positional relationship to right colonic flexure, duodenum and V. portae
– Consisting of:
– Fundus
– Corpus
– Infundibulum
– Collum (= transition to the cystic duct)

8.4.2 Bile Ducts

– Outflow into left (segments I–IV) and right hepatic duct (segments V–
VIII)
– Ductus hepaticus communis = union of right + left ductus hepaticus
– Ductus choledochus = Ductus hepaticus after inflow of the Ductus
cysticus
– Common orifice of the ductus choledochus with ductus pancreaticus
Orifice in papilla Vateri of the duodenum, pars inferior
– Calot’s triangle = bounded by cystic duct, hepatic duct and hepatic
subsurface
Variants:
– Course of the right posterior bile duct
– Crossing over/under the common bile duct, accessory bile duct of seg. IV
– Ostium of choledochal duct: common excretory duct with confluence,
common ostium without confluence, separate ostia of both ducts

8.4.3 Blood Supply and Drainage of the Gallbladder and Bile

Ducts
Blood Supply of the Extrahepatic Bile Ducts + Gallbladder
– Right hepatic artery, gastroduodenal and retroduodenal arteries
– Cystic artery from right hepatic artery (numerous variants, rarely cystic
artery directly from main hepatic artery)

Blood Supply of the Intrahepatic Bile Ducts

– Common hepatic artery from Coeliac trunc
– After delivery of the gastroduodenal and right gastric arteries as the
proper hepatic artery
– Branching into the right and left hepatic artery (variable anatomy)
– Venous outflow via the hepatic veins

8.5 Diseases of the Gallbladder and Bile Ducts

8.5.1 Benign Diseases of the Gallbladder
Key Points
– Gallstones in 10–15% of population (m:f = 1:1.8)
– 75% are asymptomatic at diagnosis
– 20%Of those turn symptomatic within 15–20 years
– Indication for surgery in symptomatic cholecystolithiasis with and
without complications, porcelain gallbladder and stones >3 cm
– Laparoscopic cholecystectomy is the standard procedure
– Acute cholecystitis = most frequent complication of cholecystolithiasis;
if detected = immediate indication for surgery
 – Gallbladder polyps rare overall, but if gallbladder polyps ≥1 cm or
symptomatic = indication for surgery

Cholecystolithiasis
Definition
– Inability to ensure the solubility of the bile component (cholesterol,
calcium, pigments)
– Precipitation and formation of concrements (gallstones)
Epidemiology
– Gallstone carriers = 15–20% of the population
– Risk factors:
– Overweight
– Rapid weight loss
– Pregnancy
– Multiparity
– Female gender
– First degree family history
– Medications: Ceftriaxone, postmenopausal estrogens, parenteral
nutrition.
– Geographical origin (Scandinavia, American Indians)
– Ileal diseases, secondary to resection or bypass of the small intestine
– Age (risk increased from 40 years)
– Symptomatic cholecystolithiasis: approx. 30% of carriers
– Hereditary component in the development of gallbladder stones about
25%
– More than 190,000 cholecystectomies/year in Germany
Classification
– Asymptomatic cholecystolithiasis
– Symptomatic uncomplicated cholecystolithiasis
– Symptomatic complicated cholecystolithiasis with complications
– Due to trapped concrement
– Acute cholecystitis = most frequent complication
Symptoms
– Colicky attacks of pain
– Of more than 15 min duration in the epigastrium/right upper abdomen
– Radiation into the back and right shoulder
– Nausea, occasionally bilious vomiting
– Possibly intolerance for fat, alcohol
– Dyspepsia and flatulence
– Additionally in case of complicated form:
– Fever
– Chills
– Painful jaundice (due to stone entrapment in the choledochal duct)
– Defensive tension in the upper abdomen (in acute cholecystitis,
gallbladder empyema or perforation)
– Possibly signs of purulent cholangitis or biliary pancreatitis, liver
abscesses
Complications
– Stone impaction in the choledochal duct
– Acute cholecystitis
– Gall bladder empyema/perforation
– Purulent cholangitis
– Liver abscesses
– Biliary pancreatitis
– Mirizzi’s syndrome: larger trapped stone in the cystic duct compresses
the common hepatic duct or the choledochal duct:
– Penetration into the duodenum—gallstone ileus
– Development of biliodigestive fistulas possible
– Shrinking gallbladder: after recurrent inflammation and scarring
– Porcelain Bubble:
– Chronic calcifying cholecystitis
– Increased risk of carcinoma
Annual complication rate
– After first colic = 1–3%
– In asymptomatic stone carriers = 0.1–0.3%

Diagnosis
Clinical Presentation
– Pressure pain right upper abdomen
– possibly palpable tumor
– Murphy’s sign (focal pain under direct pressure) on inspiration
– possibly sclerenicterus
Lab Chemistry
– Bilirubin, AP, γ-GT, if necessary GOT, GPT, lipase, amylase, blood
count, CRP
Imaging Non-Invasive Procedures
– Sonography:
– Sensitivity >95
– Standardized transcutaneous B-mode sonography
– Complete visualization of the gallbladder in variable sectional planes
(in at least 2 patient positioning variants, offset by 90° to each other)
– Assessment of: Gallbladder stones, sludge, wall composition, caliber
ductus hepaticocholedochus (up to 7 mm normal), free fluid,
cholestasis intrahepatic, liver abscesses, pancreatic head, multilayered
wall (cholecystitis)
– CT:
– For poor sound conditions
– In the case of complicated courses in individual cases
– In case of suspected tumor for differential diagnosis
– MRI/MRCP:
– In case of suspected tumor for differential diagnosis
– Oral/i.v. cholangiography:
– Almost no longer used for the diagnosis of gallstones
Imaging Invasive Procedures
– ERCP (endoscopic retrograde cholangiopancreaticography):
– For choledocholithiasis
– If necessary in combination with papillotomy
– In biliary pancreatitis with cholestasis/icterus and/or signs of
cholangitis: as soon as possible
– For cholangitis within 2 h after admission
– Therapeutic splitting: Bile duct repair by ERCP before cholecystectomy
(CCE)
– PCT (percutaneous transhepatic cholangiography):
– Only rarely, if ERCP is not possible
Further Etiological Clarification
– In case of unusual clinical constellation (e.g. family history, occurrence
in childhood and adolescence, intrahepatic microstones, association with
diarrhoea)
– Possible etiologies: e.g., hemolytic anemias, bile acidosis syndrome, drug
history, infections

Therapy
Conservative Therapy
– Serious complications: only 2% of gallstone carriers
– If asymptomatic cholecystolithiasis = monitoring, no indication for
therapy
Surgical Therapy
– Surgery indications
– Symptomatic uncomplicated or complicated cholecystolithiasis
– Asymptomatic patients with porcelain gallbladder: due to increased
risk of carcinoma
– Asymptomatic patients with gallbladder stones >3 cm in diameter:
Because of increased risk of carcinoma (in men nine- to ten-fold)
– In major abdominal surgery: simultaneous cholecystectomy even for
asymptomatic stones
 – Surgery may be considered in
– patients with chronic hemolytic diseases due to increased risk of
biliary symptoms
– undergoing solid organ Tx due to increase risk of developing
symptoms post-Tx
– bariatric surgery patients

Caution
– No indication in asymptomatic cholecystolithiasis with gallbladder
stones <3 cm in diameter
– In the first and second trimester of pregnancy only in case of urgent
indication (laparoscopic, intra-abdominal pressure below 12 mmHg,
intraoperative fetal monitoring), otherwise post partum
– OP procedure

– Laparoscopic/robotic cholecystectomy:
– Worldwide standard procedure (more than 93% of all
cholecystectomies started laparoscopically)
– Conversion rate to open cholecystectomy = 4–7%
– Identical complication rates with shorter hospital stay and shorter
convalescence (fewer wound infections)
– Contraindication laparoscopic: Manifest portal hypertension (relative),
liver cirrhosis MELD score >8, gallbladder carcinoma, severe
pulmonary obstruction, gravidity third trimester
– Open cholecystectomy:
– Well suited for unclear conditions
– In case of suspected tumor or strong bleeding tendency
– Mini-laparotomy cholecystectomy:
– Laparotomy <8 cm
– No differences to laparoscopic cholecystectomy with regard to
complication rates, length of hospital stay and convalescence times
– NOTES cholecystectomy (“natural orifice transluminal endoscopic
surgery”):
– Transvaginal or transgastric
– Elective surgery only
– Complication rate = 3.1%, conversion rate = 4.9% (German NOTES
registry)

Surgical Procedure
Laparoscopic Cholecystectomy
– Supine position with legs apart or flat supine position
– Surgeon between legs or left
– Access pneumoperitoneum at 12 mmHg, (Veress needle) or mini-
laparotomy via subumbilical skin incision approx. 1.5 cm
– Insertion camera trocar + 10 mm working trocar + 1–2 × 5 mm
working trocars
– Elevating the gallbladder and pulling the infundibulum to the right
– Representing the Calot Triangle
– Dissection of the cystic duct and cystic artery, including visualization
of the opening into the choledochal duct.
– Clip supply—2 each to central, 1 to peripheral
– Subserosal release of the gall bladder and transfer to salvage bag
– Hemostasis
– Moving the camera in 10 mm trocar
– Removal of the gallbladder via a subumbilical skin incision, if
necessary with a spreading instrument (using a salvage bag)
Inspection of the surgical area, removal of the trocars under visual
control, fascial closure, skin suture.

Surgical Procedure
Open Cholecystectomy
– Rib-arch margin incision or transrectal incision
– Antegrade subserosal extirpation of the gallbladder
– Settling of the cystic duct + cystic artery in Calot’s triangle near the
gallbladder
 – Clip supply of these structures—2 each to central, 1 to peripheral

– Complications
– Recurrent cholelithiasis = 2% of cases
– Bile leak (mostly liver bed) = 0.4–1.5%
– Wound infection = 1.3–1.8%
– Pancreatitis = 0.3
– Bleeding = 0.2–1.4
– Bile duct injury rate = 0.2–0.4%
– Subhepatic abscess
– Infected bilioma/hematoma
– Occlusive icterus due to remaining concrements
– Post surgical care
– Clinical control first post-op day
– Blood count, CRP and bilirubin on post-op day 2
– Discharge when symptom-free + laboratory inconspicuous
– In case of bilirubin elevation: sonography to exclude cholestasis

Gallbladder Polyps
Definition
– Benign tumors of the gallbladder wall
– Subdivision into 2 groups:
– Benign pseudotumors (e.g. cholesterol polyps or adenomatosis)
– Adenomas
Epidemiology
– Prevalence of gallbladder polyps = between 1 and 7%
– Polyps ≥1 cm in diameter = significantly increased probability of
neoplastic genesis (adenomas) = risk of carcinoma in up to 50%
– Risk factors for adenoma development:
– Age > 50 years
– Solitary polyps
– Gallstones
– Presence of more than one polyp = speaks against an adenoma and for
the presence of cholesterol polyps
– Risk factors for malignancy:
– Age > 60 years
– Coexistence gallstones
– Size increase
– Size >10 mm
Clinical Presentation
– Mostly asymptomatic
– Otherwise see Cholecystolithiasis
Diagnosis
– Sonography
– No change in position when repositioning the patient
– If necessary endosonography and CT

Therapy
Surgical Therapy
– Surgery indication
– Gallbladder polyps ≥1 cm: Independent of symptoms
– For polyps >18–20 mm: Because of the significant risk of malignancy,
primarily consider open cholecystectomy
– Laparoscopic vs. open cholecystectomy
Conservative Therapy
– For polyps <1 cm
– Sonographic control:
– Initially every 6 months
– Later annually, if no increase in size

Acute Cholecystitis
Definition
– The most frequent complication of cholecystolithiasis
– Acute inflammation of the gallbladder wall

Pathophysiology
Course
– Stone entrapment with passive or permanent occlusion of the cystic duct
– Gallbladder hydrops with abacterial infection
– Secondary colonization by ascension from duodenum or
hematogenous/lymphogenous dissemination
– possibly gallbladder empyema and ulcerophlegmonous course
Other Risk Factors
– Diabetes mellitus
– Atrial fibrillation
– Terminal renal failure
– Severe liver dysfunction

Symptoms
Symptoms
– Colicky upper abdominal pain
– Fever + possibly chills
– Nausea + vomiting
Complications of Acute Cholecystitis
– Gallbladder gangrene
– Gall bladder empyema or perforation
– Rare formation of biliodigestive fistula (60% to the duodenum)
– Gallstone ileus

Diagnosis
Clinical Presentation
– Positive Murphy sign
– Defensive tension right upper quadrant in peritonitis
Lab
– AP, γ-GT, transaminases, bilirubin, lipase, coagulation parameters (INR,
PTT), CRP and blood count
– Clear signs of infection: leukocytes plus CRP elevated
– Cholangitis: AP, γ-GT elevated
Sonography
– Sensitivity = 94%; Specificity = 78%
– Wall thickening (>4 mm) with possibly triple stratification of the
gallbladder
– Pericholecystitis with free fluid
– Dense internal pattern: with empyema
– Evidence of covered or open perforation, if applicable
Therapy
– Always operative
Indication
– If acute cholecystitis is detected, surgery is indicated immediately
– In patients on anticoagulants:
– If necessary, adjustment of coagulation or start of fluid substitution
– Antibiotic administration and electrolyte balance
– Subsequent early selective surgery within 1–3 days
– If patient cannot be operated early (diagnosis too late, other medical
reasons (= too high risk of surgery): Cholecystectomy in interval only
after 6 weeks
– ACDC (“acute cholecystitis: early versus delayed cholecystectomy”)
study (Gutt et al. 2013): early selective laparoscopic cholecystectomy vs.
interval cholecystectomy after primary conservative antibiotic therapy:
– Reduction of morbidity and mortality
– Total in hospital time significantly lower
– Significantly reduced hospital costs
– Comparable numbers of bile duct injuries and bile leakages
OP Procedure
– Laparoscopic cholecystectomy:
– Standard procedure
– Conversion rate 2–7% (some series up to 20%)
– Primary open surgery:
– In case of expected complications
– In case of multiple previous operations
– For “intensive gallbladder”
Acute cholecystitis in patients requiring intensive care (acute acalculous
cholecystitis):
– Incidence = 0.2–0.4% in patients who were in an intensive care unit for
more than 2 days
– Often associated with high morbidity and mortality
– Surgical rehabilitation obligatory as long as no clinical contraindications
are present
– Open procedure justified with similar peri- and postoperative
complication rates
– No operability given:
– Interventional percutaneous cholecystostomy or endoscopic
transpapillary bile duct drainage
– Secondary cholecystectomy after re-evaluation and stabilization of the
patient (early or late elective = no clear recommendations)

8.5.2 Benign Diseases of the Bile Ducts

Key Points
– Choledocholithiasis in up to 15% of patients with cholecystolithiasis
– Hyperbilirubinemia + sonographically dilated bile duct suspicious
– Therapeutic splitting ERC (endoscopic retrograde cholangiography)
and CCE (cholecystectomy) recommended
– Choledochal cysts very rare overall

Choledocholithiasis
Definition
– Concrement in the common bile duct (Ductus choledochus)
– Most frequently: formation of the calculus in the gallbladder and
migration into the choledochal duct; rarely formation directly in the
choledochus
Epidemiology
– Prevalence of gallstones in patients with cholecystolithiasis = age-
dependent: 5–15%
– High probability of simultaneous choledocholithiasis in:
– Sonographically dilated bile duct (>7–10 mm) + hyperbilirubinemia +
elevated γ-GT/GPT
– Bile duct >10 mm + gallbladder stones + colic
– Direct sonographic detection of stones in the bile duct
Clinical Presentation
– Strong evidence of choledocholithiasis
– Cholangitis
– Stone visible in ultrasound
– Icterus
– Hyperbilirubinemia + sonographically dilated bile duct

Therapy
Indication
– Patients with gallbladder + bile duct stones = therapeutic splitting
recommended
– Preoperative ERC = primary procedure in combination with papillotomy
– In case of cholangitis or severe biliary pancreatitis within 24 h
– Cholecystectomy only after pancreatitis has subsided
– In case of cholecystolithiasis under risk assessment = cholecystectomy
within 6 weeks if possible
– Symptomatic bile duct stones in gravidity: primary endoscopic
papillotomy + stone extraction
– If ERCP is not possible:
– Laparoscopic cholecystectomy + simultaneous surgical bile duct
revision (transcystic bile duct exploration or laparoscopic
 choledochotomy, cholangiography and extraction via grasping
forceps, basket, Fogarty catheter if necessary with bougienage of the
papilla), if expertise available
– Insertion of a T-drainage possible

Choledochal Cysts
Definition
– Cystic dilatation of the choledochus
– Affects extra- and/or intrahepatic bile ducts
– Mostly indication for surgical therapy
Epidemiology
– Rare clinical picture: incidence = 1/100.000 to 1/150.000 in western
countries
– More common in Japan
– Women: Men = 7–8: 1
– Genetic predisposition
Pathogenesis
– Abnormal connection between the choledochus and the pancreatic duct
– Reflux of pancreatic juice into distal choledochus = chronic
inflammation = slackening of the choledochal wall
– Classification according to Todani/Alonso-Lej
Clinical Presentation
– Classic triad (only 10% of patients):
– Pain in the right upper abdomen
– Icterus
– Abdominal mass
– Complications (if left long term without surgical treatment)
– Portal hypertension
– Cirrhosis of the liver
– Biliary obstruction
– Malignant degeneration = 2.5–26%
Diagnosis
– Lab
– Liver dysfunction (60% of cases)
– Sonography
– CT/MRI abdomen
– ERCP/percutaneous transhepatic cholangiography (PTC)
Therapy
– Targets
– Symptom relief
– Preventing complications
– Technique = cholecystectomy + resection of the extrahepatic cyst-bearing
bile ducts if necessary biliodigestive anastomosis

8.5.3 Gallbladder Carcinoma

Epidemiology
– Incidental finding in 0.2–0.4% of cholecystectomies
– Proportion of potentially resectable gallbladder carcinomas at the time of
diagnosis = 10–30%
– Risk factors:
– Disposition due to cholecystolithiasis (1–3%)
– Porcelain gallbladder (−20%) = indication for surgery even without
tumor evidence in imaging
– Gallbladder polyps = metaplasia-dysplasia pathway and adenoma-
carcinoma sequence identified

Symptoms
– Often asymptomatic
– History of cholecystolithiasis
– Courvoisier sign = painless palpable enlargement of the gallbladder, if
applicable
– Later: Icterus, B-symptomatics
Diagnosis
Sonography
– Mural tumor
– Expansion in the liver bed
– Metastases intrahepatic
CT Abdomen and Thorax
– Environment diagnosis
– Exclusion of metastases intrahepatic
– OP planning
Alternative MRI with Magnetic Resonance Cholangiopancreatography
(MRCP)
– Exclusion of intrahepatic metastases
– OP planning
– Assessment of the intra- and extrahepatic bile ducts
– If necessary ERCP for the evaluation of the intra- and extrahepatic bile
ducts

TNM Classification and Staging (UICC 2010)

TNM Classification
– T (tumor)
– T1 Tumor infiltrates lamina propria or musculature
– T1a Tumor infiltrates mucosa
– T1b Tumor infiltrates bile duct muscles
– T2 Tumour infiltrates perimuscular connective tissue, but no spread
via serosa or liver
– T3 Infiltration of serosa or infiltration of liver and/or other organ such
as stomach, colon, pancreas, extrahepatic bile ducts or other organs
– T4 infiltration of portal vein or hepatic artery or multiple extrahepatic
organs
– N (lymph nodes)
– N0 No regional lymph nodes affected
– N1 Regional lymph nodes affected
– M (metastases)
– M0 No distant metastases
– M1 distant metastases
UICC Stages According to the TNM Classification (2010)

Stage Ia T1 N0 M0
Stage Ib T2 N0 M0
Stage IIa T3 N0 M0
Stage IIb T1, T2, T3 N1 M0
Stage III T4 Each N M0
Stage IV Each T Each N M1

Therapy
Surgical Therapy
– Complete resection = only curative approach
OP Indication/Strategy
– Extent of surgery depends on TNM stage (see above):
– T1a cholecystectomy
– T1b Radical cholecystectomy—resection in the liver bed 3 cm hem
apical + lymphadenectomy
– T2 en bloc resection Couinaud segments IVb and V +
lymphadenectomy; 5-year survival 40% vs. 90%
– T3 Extended right hemihepatectomy + lymphadenectomy
– T3 with infiltration of an extrahepatic organ or T4 individual decision
only; 5-year survival <10%
– If incidental finding after cholecystectomy:
– Early resection within 2 to max. 4 weeks
– Goal = Avoid lymphogenic and peritoneal metastasis according to
recommendations
– If incidental finding during cholecystectomy:
– Switch to open procedure
– Resection according to recommendations
 – If no expertise for liver resection available = early presentation to liver
center within 2 to max. 4 weeks
– If gallbladder carcinoma suspected in diagnosis:
– Either diagnostic laparoscopy in case of frequent early peritoneal
metastasis and open resection according to recommendations
– If no expertise for liver resection available = immediate presentation
to liver center
– Early lymphogenic metastasis = extensive lymphadenectomy,
ligamentum hepatoduodenale to the truncus coeliacus
– Always excise trocar injection channels to avoid cutaneous metastases
Adjuvant Therapy
– According to current guidelines
– Preferably inclusion of patients in randomized controlled trials
Malignant diseases of the bile ducts are discussed in the chapter on
malignant diseases of the liver with Klatskin tumours (► Sect. 8.2.2).

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OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_9

9. Pancreas
Kim C. Honselmann1 and Tobias Keck1
(1) Department of Surgery, University Hospital Schleswig-Holstein,
Luebeck, Germany

Kim C. Honselmann
Email: [email protected]

Tobias Keck (Corresponding author)

Email: [email protected]

9.1 Anatomy and Physiology

9.1.1 Definition, Location and Structure
– Endocrine (increment) and exocrine (excretion) gland
– Retroperitoneal position at the level of lumbar vertebrae I–II
– Structure in four parts:
– Pancreatic head (= Caput pancreatis)
– Pancreatic isthmus (= Collum pancreatis)
– Pancreatic body (= Corpus pancreatis)
– Pancreatic tail (= Cauda pancreatis)

9.1.2 Anatomy and Embryology

Embryology (◘ Fig. 9.1)
– Development from endoderm (= into 2 opposite epithelial buds at caudal
end of foregut; at end of fourth week of development):
– Ventral pancreas: In the angle of the intestine and bile duct, with
connection to the choledochal duct
– Dorsal pancreas: larger, in connection with the foregut (= later
duodenum)
– Formation of the pancreatic head (caput pancreatis; ◘ Fig. 9.1):
– Due to right rotation of the intestine + growth of the duodenum
– Ventral pancreas origin is located caudal to the dorsal pancreas +
fusion of the bile ducts (Ductus Wirsungianus) + excrete together in
Papilla duodeni major (Papilla Vateri)-in case of fusion disorder
Pancreas divisum
– Formation of the pancreatic corpus + tail
– Dorsal pancreas alone forms the corpus and cauda pancreatis,
– Ductus Santorini: Original excretory duct of this origin, opens further
proximally in the duodenum at the papilla duodeni minor; frequent
obliteration (Cano et al. 2007)
Fig. 9.1 a–c Development of the pancreatic origin (embryology). (Mod. according to Schumpelick
2011)

Anatomy
– Localization and size
– Approx. 16 cm × 3 cm × 2 cm (L × W × D), 60–80 g weight
– Retroperitoneal at the level of lumbar vertebrae 1–3
– Pancreatic head in duodenal C; pancreatic tail to splenic hilum
– Pancreatic body = dorsal border of the bursa omentalis (ventral to the
abdominal aorta, inferior vena cava and left adrenal gland)
– Arterial blood supply (◘ Fig. 9.2):
– Celiac trunc: Superior posterior and anterior pancreaticoduodenal
artery and from superior mesenteric artery: anastomosis to
gastroduodenal artery (Arcade described by Rio-Branco) + dorsal
pancreatic artery from the splenic artery
– Superior mesenteric artery: inferior pancreaticduodenal artery (forms
arcade with superior pancreaticoduodenal artery and connection to
gastroduodenal artery, see above)
– Venous outflow via:
– Pancreaticoduodenal veins (via pancreatic head) into superior
mesenteric vein and portal vein
– Pancreatic veins (multiple veins) flow into the splenic vein (pancreatic
tail area)

Fig. 9.2 Anatomical location of the pancreas. 1 Head, 2 Uncinate process, 3 Neck, 4 Body, 5 Tail, 6
Duct of Wirsung, 7 Duct of Santorini, 8 Duodenum, 9 Spleen, 10 Proper hepatic artery, 11 Splenic
artery, 12 A. and Superior mesenteric vein, 13 Vena cava, 14 abdominal aorta. (From Schumpelick
2011)

9.1.3 Physiology
– Two functions: Exocrine and endocrine

Exocrine Function
– External secretion (i.e., in this case, into the intestinal lumen)
– 1.5–3 L secretion daily
– Secretion stimulants: secretin and cholecystokinin
– Pancreatic exocrine tissue (98% of pancreatic tissue) =
– Acinar cells + ductal epithelial cells
– Arrangement in acini (=cell groups) around excretory ducts
– Secretion composition:
– Ductal epithelial cells: Bicarbonate formation (creation of an alkaline
environment) + chloride resorption (production of an isotonic fluid)
– Acinar cells: Production of digestive enzymes (e.g. lipase, amylase,
proteinases)

Endocrine Function
– Internal secretion (= hormone; i.e. in this case into the plasmatic
compartment)
– Endocrine pancreatic tissue = about 2% of the cells (= islets of
Langerhans)
– A(α)-cells: 10% of the endocrine cells, hormone = glucagon (leads to
glucose production from glycogen in the liver as well as from
triglycerides from the adipocytes)
– B(β)-cells: 80% of endocrine cells, hormone = insulin (stimulates
glucose absorption in liver, fat cells and muscle cells)
– D(δ)-cells: 10%, hormone = somatostatin (inhibits the secretion of
pancreatic enzymes, gastrin and pepsin)

Control of the Functions

– Exocrine secretion:
– Cephalic phase (olfactory, gustatory, visual stimuli)
– Gastric phase (stretching stimuli of the stomach wall + release of
gastrointestinal hormones)
– Intestinal phase (via release of gastrointestinal hormones)
– Endocrine secretion: hormonal control loop

9.2 Benign Diseases

9.2.1 Acute Pancreatitis
Key Points
– Mild edematous and severe necrotizing types
– Potentially lethal clinical course in severe form of progression
– Incidence = 18/100,000 adults in Germany
– Mortality of severe form: 10–15%
– Most frequent etiology: alcohol (m > f) or gallstones/sludge (f > m)
– Laboratory diagnosis: threefold elevation of pancreatic serum amylase
above normal levels (definition), lipase, liver function tests, electrolyte
imbalance, coagulation imbalance
– Diagnostic imaging: ultrasound of the abdomen within 24 h to assess
the bile ducts, if CT, then wait until 72 h after admission
– Therapy:
– Conservative: endoscopic retrograde cholangiopancreatography
(ERCP), fluid intake + enteral nutrition (jejunal feeding tube if
necessary), antibiotics only therapeutically (if microbiology cultures
are positive), not prophylactically
– Operative/Interventional: As late as possible (>4 weeks), only in
case of complications such as necrosis or abscess, infection,
pseudocyst (step-up approach: drainage → minimally invasive
necrosectomy → open necrosectomy)

Definition
– Upper abdominal pain and serum amylase three times above normal
– Temporal inflammatory process
– Autodigestion of the pancreas gland (usually only partial)

Forms
Acute Edematous Pancreatitis
– Self-limiting
– Mild progressive form (80%)
Acute Necrotizing Pancreatitis
– Formation of necrosis (20%)
– Risk = secondary infection of necroses

Epidemiology
Incidence
– 40 new cases per 100,000 population (USA)
– 73 per 100,000 (Finland)
– 18 per 100,000 (Germany)
– Women:Men = 1:1 (different aetiology see above)
– Age = 38–70 years

Etiology
– Biliary (about 40%): Originating from stones in the common bile duct
and secondary obstruction of the duct of Wirsung
– Alcohol-induced (approx. 40%)
– Hypertriglyceridemia (approx. 10%)
– After abdominal trauma
– Side effects from medication: Azathioprine, sulfonamides, tetracyclines,
valproate, methyldopa, estrogens, 6-mercaptopurine, 5-aminosalicylic
acid (5-ASA), corticosteroids, octreotide, furosemide.
– Hereditary
– Viral (children: mumps)
– Hypercalcemia
– Mechanical obstruction (tumor, pancreas divisum, papillary stenosis)
– Tropical pancreatitis

Symptoms
– Severe epigastric pain with belt-like radiation into the back
– Abdomen is taut and elastic: “rubber belly”
– Meteorism
– Fever
– Paralytic (sub)ileus
– Vomiting
– Hypocalcemia
– Skin signs (rare) as a sign of coagulation disorder and as a result of fat
tissue necrosis (severe course):
– Cullen’s sign (periumbilical)
– Grey Turner sign (flank)
– Fox sign (inguinal)
– Sepsis
– Septic shock

Diagnosis
Laboratory Diagnosis
– In the serum:
– Amylase (threefold above normal)
– More specific (at more than 48 h after symptom onset) = lipase and
pancreatic amylase (as distinct from salivary amylase)
– Coagulation: Onset of systemic inflammatory response syndrome
(SIRS)
– Urea (elevation indicative of severe course)
– Cholestasis parameters: Bilirubin, γ-GT, alkaline phosphatase (AP):
Biliary etiology
– C-reactive protein (for differentiation between edematous and
necrotizing pancreatitis) (>120 mg/L); highly sensitive, correlation
with progression/development of necrosis, also procalcitonin (PCT)
– Blood sugar (low is indicative of a severe course)
– Hematocrit (increase indicative of a severe course)
– In urine: amylase (rare)

Caution
No correlation between level of pancreatic enzymes and severity of
pancreatitis, but correlation present for CRP, urea and hematocrit.
Diagnostic Imaging
– Chest and abdominal X-rays:
– To exclude free abdominal air
– Detection of pleural effusion, calcifications due to pancreatic
secretion, air-fluid level formation
– Contrast-enhanced CT
– In the presence of necrosis (necroses do not absorb contrast media)
– Significance for disease course only after 72 h
Risk Assessment (◘ Table 9.1)
– Ranson criteria:
– For mortality estimation
– For risk assessment of necrotizing pancreatitis: (1 point per item)
Table 9.1 Ranson criteria for acute pancreatitis

Time Criterion
On admission (1 point each) – Age >55 years
– WBC >16 G/L
– AST >250 U/L
– LDH >350 U/L
– Glucose >200 mg/dL
After 48 h (1 point each) – Volume deficit >6 L
– BUN increase by >5 mg/dL
– Base deficit >4 mmol/L
– Drop in PaO2 to 60 mmHg
– Drop in serum calcium <2 mmol/L
Point total after 48 h Mortality
0–2 points <1%
3–4 points 15%
5–6 points 40%
>6 points 100%
WBC White blood cell count, AST Aspartate Aminotransferase, LDH lactate
dehydrogenase, BUN Blood urea nitrogen, PaO2 arterial oxygen partial
pressure

Differential Diagnosis
– Acute cholecystitis/cholecystolithiasis
– Mesenteric ischemia/venous thrombosis
– Abdominal aortic aneurysm (AAA)
– Mechanical bowel obstruction
– Perforated gastric ulcer
– Colonic diverticulitis

Therapy
Etiology-Oriented Therapy
– Goal = Elimination of cause, if possible
– Biliary pancreatitis:
– ERCP + papillotomy (within 24 h)
– Laparoscopic cholecystectomy: after approx. 5–7 days (during the
same inpatient stay for mild pancreatitis); Rationale: Biliary
pancreatitis = high recurrence rate at 30%, early (<48 h) laparoscopic
cholecystectomy possible for mild pancreatitis (Ranson score <3)
– Alcohol-induced pancreatitis: secondary alcohol withdrawal therapy in
the interval
– Hypertriglyceridemia-induced pancreatitis: lower blood lipids
– Medication pancreatitis: discontinue medication
Conservative Therapy
– In edematous pancreatitis:
– Inpatient admission and monitoring of vital parameters
– Analgesia: paracetamol, metamizol, tramadol or buprenorphine (use
opiates with restraint due to papillary spasm, but not as strictly as in
the past)
– Fluid intake (target = urine output >0.5 mL/kg bw/h)
– Aim for early enteral feeding (but often gastric emptying disorder)
– Propulsive medication
 – Gastric tube to prevent vomiting
– Ulcer prophylaxis
– Compensation for electrolyte deficiency, calcium only from corr.
Calcium level of 0.9 mmol/L [corr. Ca2+ = measured Ca2+ (mmol/L) ×
(0.025 × albumin (g/L)) + 1]
– In acute necrotizing pancreatitis:
– Edema to edematous pancreatitis
– Volume-controlled therapy (PICCO, CVC, pulmonary catheter)
– Intensive care unit with invasive monitoring
– No prophylactic antibiotic administration
– Antibiotics for positive microbiology cultures after diagnostic
puncture of fluid accumulation or FNA
– Early enteral nutrition, if necessary via jejunal tube
Step-Up Approach
– In necrotizing pancreatitis with infected necrosis
– First CT-guided drain insertion percutaneously/endoscopically
– In the absence of improvement after 72 h (= improved function of at least
2 organ systems or at least 10% improvement of 2 out of 3 parameters,
white blood cell count/CRP and temperature):
– Retroperitoneoscopic necrosectomy or
– Transgastric necrosectomy or
– Open procedure
Operative Therapy Principles
– (Laparoscopic)/Open transabdominal retroperitoneal necrosectomy
(disadvantage = elimination of compartmentation)
– Indication: in the event of ineffective or unsuccessful drainage
– Wait until the findings are consolidated (if at all possible wait more
than 4 weeks until the operation)
– Imaging of the pancreas
– Relief from fluid retention
– Removal of the clay-like necrosis areas in digital preparation—
Beware of venous bleeding!
Surgical Procedure
Retroperitoneoscopic Necrosectomy
– CT-guided drainage of the retentive cavity with target drain
– General anesthesia
– Supine position with elevation of the punctured side
– Indwelling urinary catheter
– Access: Five circular incision around the inserted retroperitoneal
drainage
– Digital exploration: drainage of the fluid accumulation
– Digital opening of the fluid collection, then insertion of a
retroperitoneoscope, necrosectomy above (with grasping
forceps/laparoscopic suction/via retroperitoneoscope as optical
channel)
– Alternatively, insertion of a long 10-mm trocar + long 10-mm 0° optic
via incision into the retroperitoneum
– Inspection of the retroperitoneal cavity + removal of the remaining
loose areas with forceps (caution: venous bleeding from the splenic
vein, if necessary tamponade with tamponade strips and revision after
24 h)
– Final placement of 2 large luminal drains
– Extensive rinsing, if necessary continuous rinsing via drains
(disadvantage: rinsing lanes)

Surgical Procedure
Open Retroperitoneal Necrosectomy
– General anesthesia
– Supine position, indwelling urinary catheter
– Approach: Large bilateral subcostal incision
– Opening of the omental bursa
– Mobilization of both colonic flexures
– Removal of necrotic areas by blunt dissection with fingers: paracolic,
around the mesenteric root and in the lesser sac (omental bursa)
(caution: high risk of bleeding). Carefully remove necrotic tissue
– Extensive rinsing
– Insertion of several drains with relaparotomy on demand or
 – Insertion of e.g. an ABthera vacuum dressing (3M, St. Paul, MN
55144-1000, USA) (= continuous irrigation of the necrosis area and
permanent suction) (caution: intestinal fistulas) with repeated
relaparotomies (caution: irrigation lanes)
– Postoperative treatment
– Intensive monitoring
– SIRS/sepsis therapy

Prognosis
– Lethality (acute edematous pancreatitis) = 1%
– Lethality (acute necrotizing pancreatitis with infected necrosis) >20%

9.2.2 Chronic Pancreatitis

Key Points
– Recurrent episodes of pain
– Alcohol = most common cause
– Pain + complications = surgically treatable
– Alcohol abstinence + nicotine abstinence (progression effect) should be
recommended
– Imaging diagnosis: In case of chronic recurrent course = detection of
complications + localization before planned pseudocyst removal

Definition
– German Society for Gastroenterology, Digestive and Metabolic Diseases
(guideline):
– Pancreatic disease
– Recurrent inflammatory episodes and pain
– Gradual fibrotic remodeling of the gland
– Progressive loss of exocrine + endocrine pancreatic function

Forms
– Chronic pancreatitis with focal fibrosis
– Chronic pancreatitis with segmental/diffuse fibrosis
– Chronic pancreatitis with or without calcifications (inflammatory
pancreatic head pseudotumor)
– Special form:
– Obstructive chronic pancreatitis (duodenal diverticulum, pancreas
divisum, tumors, papillary stenosis)
– Hereditary chronic pancreatitis
– Idiopathic chronic pancreatitis: when no cause is found

Complications
– Pseudocysts: cyst-like pancreatic structure without epithelial lining
– Pancreatic duct stenosis: inflammation-induced narrowing of the
pancreatic duct (pearl cord-like duct)
– Duodenal stenosis: inflammation-related narrowing of the duodenum
– Vascular complications: Arterial hemorrhage, aneurysm rupture, portal
vein stenosis and thrombosis
– Compression or scarring stenosis of the bile ducts, with obstructive
jaundice
– Duct rupture with pancreatogenic ascites or pancreato-pleural fistula

Epidemiology
– Prevalence: 25–30 cases/1 million inhabitants
– Incidence: 23 new cases/100,000 inhabitants (increases with age)
–m>f
– Average age: 3rd–4th decade of life (social problems, disability)
– 10-year survival rate: 70%
– Overall lethality: 30–35%
– Risk increase for pancreatic cancer (tenfold)

Etiology
– Mostly unclear
Alcohol Abuse (75–90%)
– Most important factor
– For women, >40 g alcohol/day for 6–12 years is considered as threshold.
– For men, >80 g alcohol/day is considered as threshold
– Time between onset of alcohol abuse and onset of chronic pancreatitis:
on average 18 ± 11 years
Nicotine Abuse
– Leads to progression of the disease

Hyperparathyroidism (with Ca2+ Elevation)

Hereditary
– Prevalence 1/300,000
– Mutation in the cationic trypsinogen gene (PRSS1): approx. 67% of
patients with hereditary pancreatitis
– Other responsible genes: SPINK1 gene, CFTR gene
Autoimmunological (IgG-4 and Lymphoplasmocytic Infiltrates)
– Plasma cellular infiltrates in the pancreas
– IgG-4 elevation in serum
– CT morphologically bulky pancreas often without visible ductal changes

Symptoms
Abdominal Pain
– Main symptom
– Mechanism = infiltration of the parenchyma, nerve myelin sheaths +
pressure increase in the pancreatic duct (obstruction)
– Neuropathic pain
Symptoms Associated with Loss of Function
– Malnutrition
– Steatorrhea (lipase secretion reduced by more than 90%): Greasy stools
– Weight loss
– Vitamin deficiencies (fat-soluble vitamins A, D, E, K)
– Pancreatogenic (type III) diabetes mellitus
– Chronic pain syndrome

Diagnosis
Genetic Examination
– Indications for mutation analysis of the PRSS1 gene:
– Positive family history (one or two first-degree relatives with
idiopathic chronic pancreatitis)
– Two or more episodes of acute pancreatitis without identifiable cause
before the age of 25 years
– Idiopathic chronic pancreatitis with first symptoms before the age of
25
Laboratory Diagnosis (◘ Table 9.2)

Table 9.2 Non-invasive pancreatic function testsa

Test Mild exocrine Moderate exocrine Severe exocrine Specificity

insufficiency insufficiency insufficiency (%)
sensitivity (%) sensitivity (%) sensitivity (%)
Stool Elastase 54% 75% 95% 85%
Qualitative stool 0% 0% 78%b 70%b
fat determination
Chymotrypsin <50% Approx. 60% 80–90% 80–90%
activity
13C-breath test 62–100% 90–100% 80–90%
(mixed
triglycerides)

a
The direct invasive pancreatic function tests (secretin or secretin-
pancreazymin test) were used as reference procedures
b
Related to the quantitative stool fat determination

Diagnostic Imaging
– Only to be used in case of insufficient correlation of clinical,
morphological and functional parameters or for the assessment of
complications
Sonography
– Inhomogeneous organ with normal pancreatic duct, possibly
calcifications = uncertain sign
Endosonography
– Highest sensitivity
– Endosonographically assisted fine needle aspiration (not percutaneous!):
– To confirm the histological diagnosis (often false negative in cancer
and chronic pancreatitis)
– To confirm autoimmune pancreatitis (plasma cells, IgG-4)
CT/MRI/Magnetic Resonance Cholangiopancreaticography (MRCP)
– Supplementary for unclear pancreatic changes
– MRCP helpful for pancreatic duct assessment
ERCP
– Disadvantages of ERCP (vs MRCP):
– Increased morbidity (5–10% overall; 3.47% post-ERCP pancreatitis)
– Increased mortality (3.3‰)
– Therefore, diagnostic endoscopic retrograde
cholangiopancreaticography (ERCP) should be omitted in favor of
MRCP for purely diagnostic indications
– Evaluation criteria of ERCP (according to Cambridge classification)
– Cambridge classification (for different examination procedures; ◘ Table
9.3)
Table 9.3 Evaluation criteria according to the Cambridge Classification

Endoscopic retrograde cholangiopancreaticography (ERCP)

Cambridge No pathological changes on complete visualization of the pancreatic duct
0
Cambridge <3 pathological side branches, main duct regular
1
Cambridge >3 pathological side branches, main duct regular
2
Cambridge Pathological side branches plus pathological main duct
3
Cambridge Like 3 plus cyst, duct stones, strictures, involvement, adjacent orifices
4
Transabdominal ultrasound
Cambridge Normal organ, duct <2 mm, smooth contour
0
Cambridge Echo dense organ contour, organ enlarged, duct <3 mm, lobulated texture with echo
1 dense segments
Cambridge Irregular contour, irregular echo-enhanced main duct >3 mm, lobulated texture with
2 echo-dense septa
Cambridge Like 2 and cysts, focal calcifications
3
Cambridge Like 3 and ductal stones, ductal obstruction, tumorous distention of the organ >2-
4 fold, splenic vein thrombosis
Endosonography (EUS)
Cambridge None
0
Cambridge Honeycomb texture, honeycomb-like, aisle <3 mm
1
Cambridge Hyperechogenic duct, hyperechogenic foci, echo-dense contour, duct <3 mm
2
Cambridge Lobulated, septate, hyperechogenic foci, duct >3 mm, irregular duct, no duct stones
3
Cambridge Like 3 and calcifications, duct stones, cysts
4
Computed tomography (CT)/Magnetic resonance cholangiopancreaticography (MRCP)
Cambridge None
0
Cambridge Not delineable with current methods in CT/MRCP
1
Cambridge Two or more of the following changes:
2 – Pancreatic duct >2 and <4 mm in the corpus pancreatis
– Mild pancreatic duct enlargement
– Heterogeneous parenchyma structure
– Small cystic changes (<10 mm)
– Duct irregularities
– >3 pathological secondary ducts
Cambridge All changes mentioned in 2 plus pathological main duct (>4 mm)
3
Cambridge One of the changes listed at 2 and 3 plus one or more of the following:
4 – Cystic structures >10 mm
– Parenchymal calcifications
– Intraductal filling defects (limestones)
– Duct obstructions (strictures)
 – Severe duct irregularities

Cambridge 0: No chronic pancreatitis (CP), Cambridge 1: Possible CP,

Cambridge 2: Low CP, Cambridge 3: Moderate CP, Cambridge 4: Heavy
CP

Differential Diagnosis
– Cystic Fibrosis
– Cystic pancreatic neoplasms (especially main duct IPMN)
– Schwachmann-Diamond syndrome (autosomal recessive bone marrow
disease)
– Johansen-Blizzard-Syndrome (disturbed development pancreas, nose and
galea)
– Congenital enzyme defects (trypsinogen, α1-antitrypsin deficiency)
– Divisive pancreas

Therapy
Treatment Strategy
Indications for Conservative Therapy
– For nicotine abuse: nicotine abstinence
– Pain medication according to WHO guidelines (◘ Table 9.4)
– Enzyme substitution
– Indication: steatorrhea, pathological pancreatic function tests, weight
loss
– Start with 20,000–40,000 units per meal, 10,000–20,000 units for
small snacks, doubling or tripling of dose possible if insufficient effect
Table 9.4 WHO guidelines for pain medication in chronic pancreatitis

Active substance Dosage Analgesics effect

Paracetamol 2–3 times 500–1000 mg Peripheral
Metamizole 1–4 times 500–1000 mg Peripheral
Tramadol 4 times 100 mg, 2–3 times 200 mg retard Low potent central
Tilidine 3 times 50–200 mg Low potent central
Buprenorphine 3–4 times 0.2–0.4 mg Highly potent central
Morphine Individual according to effect Highly potent central
Active substance Dosage Analgesics effect
Levopromazine 3–5 times 10 mg tricyclic antidepressant
Clompramine 1 time 50–100 mg tricyclic antidepressant

Indications for Interventional or Surgical Therapy

– Sustained pain requiring analgesics (new guideline: over 3 months:
consider surgical therapy)
– Complications:
– Strictures of the common bile duct, cholestasis, jaundice, cholangitis
– Inflammatory or unclear malignant suspicious masses
– Pancreatic pseudocysts
– Pancreatic duct stones
30–60% of patients with chronic pancreatitis develop pain or a
complication.
– Strategy:
– Endoscopic procedures: Possibility of short-term pain reduction (66%
of cases/few years)
– Mid-term/long-term pain control: significant superiority of surgery vs.
endoscopic therapy
– Symptomatic pancreatic duct stones, stenoses in the pancreatic head
and pseudocysts: according to the guideline, endoscopic or surgical
therapy (► Sect. 9.2.3)
– In the case of suspected pancreatic cancer (unclear mass): Surgical
therapy (oncological pancreatic head resection)
– For symptomatic pseudocysts + complication (e.g. gastric outlet
stenosis, bleeding, cholestasis, vascular stenosis): Higher success rate
for surgical procedures (vs. endoscopic pseudocyst drainage into
duodenum or stomach), use according to expertise
– Asymptomatic pancreatic pseudocysts >5 cm in diameter without
regression after 6 weeks: Individual treatment possible (41% of cases
lead to complications)
Operative Therapy: Principles
Division: Resecting/Draining Procedures
– Resecting procedures:
– Surgery according to Kausch-Whipple
– Pylorus-preserving pancreatic head resection according to Longmire
and Traverso (PPPD)
– Surgery according to Beger, Frey or Bern Modification: Duodenum-
preserving pancreatic head resection (DPPHR)
– Rarely distal pancreatic resection (inflammatory pancreatic head
tumor remains)
– Draining procedures
– Partington-Rochelle or Puestow procedure
– V-shape excision (Hamburg modification of the Frey procedure for so-
called small duct disease)
– Total pancreatectomy and autologous islet cell transplantation (TPIAT)—
so far not relevantly used in Europe
Surgical Strategy
– Inflammatory pseudotumor of the pancreatic head:
– Standard = pancreatic head resection (classic Whipple
operation/pylorus-preserving pancreatoduodenectomy/duodenum-
preserving pancreatic head resection)
– Duodenum-preserving therapy: better perioperative quality of
life/results in some studies, long-term results equal to PPPD
– Indication for draining procedures:
– Congested pancreatic duct >7 mm without inflammatory pseudotumor
in the pancreatic head
– Obstruction of the pancreatic duct system in risk constellations (portal
hypertension with occlusion of the mesentericoportal axis)
– Long-term inferiority of draining procedures compared to resecting
procedures with regard to freedom from pain
Poor long-term results of draining procedures compared to resecting
procedures with regard to freedom from pain.
– Surgery according to Kausch-Whipple or PPPD (► Sect. 9.3.1)
– Surgery according to Beger
– Indications:
– Segmental chronic pancreatitis with pain syndrome
– Expression of an inflammatory pancreatic head tumor
– Summary: Duodenum-preserving pancreatic head resection with
subtotal resection of the pancreatic head (a very small margin of
pancreatic tissue remains for blood supply to the duodenum) followed
by anastomoses to the duodenal C, bile duct, and pancreatic remnant
(corpus).

Surgical Procedure
Operation According to Beger
– General anesthesia
– Approach: Transverse laparotomy of the upper abdomen or median
laparotomy
– Opening of the omental bursa
– Limited Cooker Maneuver
– Identification of the superior mesenteric vein at the lower edge of the
pancreas and careful undermining of the pancreas (difficult step)
– At the upper edge of the pancreas identification of the portal vein +
detachment from their adhesions
– Looping of the common bile duct and the common hepatic artery
– Transection of the pancreas after undermining, if necessary gradual
transection without undermining (risky step)
– Subtle hemostasis of the pancreas after transection with single sutures
(5-0 PDS)
– Free preparation at the upper edge of the pancreatic head up to the
common bile duct
– For visualization of the intrapancreatic bile duct, insertion of a probe
via the cystic duct
– Subtotal pancreatic head resection and uncinate process along the wall
of the intrapancreatic bile duct segment (◘ Fig. 9.3)
– Extensive hemostasis on remaining pancreatic head margin (5–8 mm)
at the duodenum with single sutures (preservation of gastroduodenal
artery branches)
– For reconstruction: pull up a retrocolic jejunum;
– Two anastomoses as an interposition with the distal pancreas (end-to-
side or end-to-end pancreato-pancreaticojejunostomy) and the head of
the pancreas (side-to-side) connected with a biliodigestive
anastomosis to the intrapancreatic bile duct (◘ Fig. 9.3)
– Connection of the inflow and outflow jejunum loop via a Y-Roux
reconstruction
– Drainage of the anastomoses
– Surgery according to Partington-Rochelle
(pancreaticojejunostomy)
– Indication: only in case of massive ductal dilatation (≥7 mm)
without pancreatic head pseudotumor
– Modification of the Puestow operation, in which only a distal
pancreaticojejunostomy is performed via the mesentericoportal axis
to the head of the pancreas
– Summary: Longitudinal opening of the pancreatic duct from the
tail of the pancreas to the prepapillary segment; the duct of the
Santorini towards the papilla should also be opened
longitudinally; drainage of the opened duct via a deactivated
jejunum loop side-to-side anastomosis according to Roux-Y.
– Results:
– Primary pain resolution 61–90%
– Long-term success = only in 50% of cases (with complete
freedom from pain)
– Surgery according to Frey (latero-lateral pancreaticojejunostomy with
duodenum-preserving pancreatic head resection (extensive excavation
of the pancreatic head))
– Indication:
– As in Partington-Rochelle, but with inflammatory pancreatic
head tumor.
– Proximal or long pancreatic duct stricture + inflammatory
pancreatic head tumor
 – Synopsis:
– Hybrid procedure = combination of a longitudinal
pancreaticojejunostomy (ductal drainage) according to
Partington-Rochelle + an extensive but careful local pancreatic
head excision (in the case of inflammatory pancreatic head
tumor, the extent of the removed pancreatic head tissue is
decisive)
– Hamburg modification (Izbicki procedure or V-shape excision): in
advanced disease with large inflammatory pancreatic head tumor and
small duct disease (without obstructed pancreatic duct) = V-shaped
excision in pancreatic corpus and tail

Fig. 9.3 a, b Operation according to Beger. (Mod. after Strobel et al. 2009)

Surgical Procedure
Operation According to Partington-Rochelle
– General anesthesia
– Supine
– Approach: Transverse laparotomy of the upper abdomen or median
laparotomy
– In case of uncertain localisation of the duct (not palpable) = small
incision with removal of an elliptical shaped tissue part in the
pancreatic body or tail (if necessary palpation and pre-puncture with a
thin needle or intraoperative ultrasound)
– Longitudinal opening of the pancreatic duct over an overholt (curved
clamp) in a small segment and then in full length
– Dissection of a V-shaped tissue block over the pancreatic duct
– If necessary, removal of pancreatic duct stones
– Pulling up an aboral jejunal loop retrocolically
– Creation of a side-to-side anastomosis
– Drainage of the pancreatic anastomosis

Surgical Procedure
OP According to Frey (◘ Fig. 9.4)
– General anesthesia
– Supine
– Transverse laparotomy of the upper abdomen or median laparotomy
– Access to the omental bursa through dissection of the gastrocolic
ligament
– Identification of the superior mesenteric vein at the lower edge of the
pancreatic neck
– Identification of the portal vein at the upper edge of the pancreatic
neck + release from their adhesions
– Undertunneling of the pancreatic body on the mesentericoportal axis
not necessary (advantage in portal hypertension or severe adhesions)
– Identification of the pancreatic duct with 20G needle + aspiration of
pancreatic juice
– Wide opening of the pancreatic duct from the pancreatic tail to the
neck and further to the pancreatic head
– Probing of the duct of Wirsung with Overholt (curved clamp)
– Placement of prophylactic haemostatic sutures at the pancreatic head
to the duodenum (4-0 or 3-0 Prolene)
– Generous sharp peeling of the pancreatic head up to the pancreatic
duct with scalpel (sufficient extent is important for the surgical result).
Some pancreatic tissue must remain caudally
– Hemostasis and manual compression
– If bile duct stricture due to compression (inflammatory, fibrotic tissue
in the pancreatic head) excision pancreatic head up to the common
bile duct with subsequent door-wing-like anastomosis of the common
bile duct in the pancreatic head (Bern modification)
 – Pulling up the aboral jejunum loop retrocolic and creation of a side-to-
side pancreaticojejunostomy, Roux-Y reconstruction
– Postoperative treatment
– Blood glucose daily profile
– Drainage analysis: amylase and bilirubin
– Bleeding control (caution bleeding into the anastomosed jejunal
small bowel)
– Enteral nutrition: from the first postoperative day (water + tea),
from the third postoperative day: liquid diet

Fig. 9.4 Operation according to Frey. (a) Situs after resection of pancreatic head and tail. (b)
Situs after reconstruction by latero-lateral pancreatico-jejunostomy. (Mod. after Strobel et al.
2009)

Monitoring and Follow-Up

– Rationale: mortality 20 years after initial diagnosis (ED) increased by
38% = need for follow-up, exocrine and endocrine dysfunction.
– Possible recurrence of pain after limited surgical procedures (drainage)
– Annual inspection:
– Clinical examination
– Transabdominal ultrasound
– Laboratory with HbA1C

9.2.3 Guidelines
German S3 guideline for chronic pancreatitis. Definition, etiology,
diagnosis, conservative, interventional, endoscopic and surgical therapy of
chronic pancreatitis. DGVS guideline 2012 Renewed version 2021.

9.3 Malignant Diseases

9.3.1 Pancreatic Carcinoma
Key Points
– Fourth leading cause of cancer death (incidence = mortality)
– Ductal adenocarcinoma: Histologically leading (>95%)
– 5-year survival = 10%
– Mostly asymptomatic for a long time
– So far the only curative treatment = radical removal of the tumour +
regional lymph node removal

Definition
– Pancreatic carcinoma = malignant tumour of the pancreas

Epidemiology
– Incidence: 14.4 new cases/100,000 in men; 10.9/100,000 in women
(Robert Koch Institute)
– Women:Men = 1:1.5
– Age peak: 65–75 years of age
– Localization: approx. 65% of all pancreatic tumors = in the pancreatic
head, uncinate process or pancreatic neck
– Risk factors:
– Age (>80-year-olds: 40-fold increase in risk compared to 40-year-
olds)
– Nicotine abuse (relative risk = 1.5-fold increased)
– Diabetes mellitus type 2
– Obesity
– Hereditary syndromes

Etiology
– Malignant degeneration of the exocrine part of the pancreas
– Precursor stages = pancreatic intraepithelial neoplasia (PanIn; ◘ Fig. 9.5)
or cystic neoplasia

Fig. 9.5 Adenoma-carcinoma sequence in pancreatic cancer

Underlying Genetic Defects

– KRAS mutation (most common mutation)
– Inactivation of the tumor suppressor genes p16, p53 and Smad/DPC4
Hereditary Syndromes
– Increased risk of pancreatic cancer: Oncology guideline programme
(German Cancer Society 2013, ► Sect. 9.3.2)
– Familial atypical multiple mole melanoma syndrome (FAMMM
syndrome)
– Hereditary pancreatitis
– HNPCC (“hereditary non-polyposis colorectal cancer”)
– Familial breast cancer (BRCA 1 and BRCA 2)
– Peutz-Jeghers Syndrome
– Familial adenomatous polyposis
– Li-Fraumeni Syndrome
– Fanconi anemia
– Von Hippel-Lindau Syndrome

Forms
– Ductal adenocarcinoma (very common)
– Serous cystadenocarcinoma (very rare)
– Mucinous cystadenocarcinoma
– Intraductal papillary mucinous carcinoma
– Pancreatoblastoma
– Solid-pseudopapillary carcinoma
– Acinar cell carcinoma
– Adenosquamous carcinoma

Symptoms
– Initially asymptomatic
– Often unspecific
– Symptoms
– Weight loss
– Painless jaundice in bile duct obstruction (leading symptom)
– New-onset diabetes mellitus over the age of 50 (caution!)
– Upper abdominal or back pain

Diagnosis (◘ Fig. 9.6)

Patient History + Clinical Examination
Imaging Techniques
– Preoperative assessment of resectability
– Preferred modalities:
– Multidetector computed tomography (thin-slice angio-CT) or MRCP
– Endosonography
– Criteria studied (of resectability):
– Resectable (R) and borderline resectable (BR) tumors:
– No distant metastases
– No infiltration of the superior mesenteric vein or portal vein (R)
– No complete encasement of the superior mesenteric vein or portal
vein (R)
– No long-distance venous occlusion not allowing reconstruction
(BR)
– No encasement of the gastroduodenal artery up to the hepatic artery
(BR)
 – No walled superior mesenteric artery or cealiac trunc >180° of
circumference (BR)

Fig. 9.6 Treatment tree according to German S3 guideline of exocrine pancreatic cancer 2013

Therapy
Curative Therapy
Preoperative Therapy
– Preoperative biliary drainage using a stent: indications:
– Cholangitis
 – If surgery cannot be performed promptly. Avoid if possible, as
significant increase in infectious complications
– Neoadjuvant chemotherapy: Already used in trials for borderline
resectable and locally advanced tumours, aim for inclusion in trials
Operation
– If surgery is possible: increase of 5-year survival rate to over 20% (with
adjuvant FOLFIRINOX up to 55%)
– Partial duodenopancreatectomy with or without pylorus preservation
– Depending on the location, distal pancreatic resection or pancreatectomy
(RAMPS, Strasberg)
– In case of infiltration of the neighbouring organs = corresponding
extension of the resection (adrenal gland, portal vein, mesocolon, colon)
– Removal of at least 10 locoregional lymph nodes

Surgical Procedure
Pylorus-Preserving/Classical Pancreatoduodenectomy
(PPPD/Kausch-Whipple Operation; ◘ Fig. 9.7)
– Supine position (left arm resting, right arm extended)
– Right transverse laparotomy/longitudinal laparotomy
– Exploration of the abdominal cavity, exclusion of metastases
– Opening of the omental sac while sparing the gastroepiploic vessels
with transection of the gastrocolic ligament
– Mobilization of the right colonic flexure
– Triggering of duodenal C from its retroperitoneal connections: Kocher
maneuver
– Mobilization of the pancreatic head up to the mesentericoportal axis
– Open cholecystectomy
– Opening of the lesser omentum and exposure of the common hepatic
artery at the upper edge of the pancreas
– Lymphadenectomy in the area of the hepatoduodenal ligament,
visualization of all structures
– Ligation of the gastric and gastroduodenal arteries
– Slinging of the common bile duct and dissection proximal to the
cystic duct
– Exposure of the portal vein at the upper edge of the pancreas
– Presentation of the superior mesenteric vein at the lower edge of the
pancreas and ligation of the outlet of the right gastroepiploic vein
– Tunelling of the pancreas and placement of hemostatic stay sutures at
the upper and lower parenchymal border
– Transsection of the duodenum post pylorus, with the GIA stapler or
distal 2/3 gastric resection (classic Whipple operation)
– Transection of the pancreas at the level of the mesentericoportal axis
– Transection of the jejunum approx. 30 cm from Treitz
– Raising the proximal jejunum retrocolically
– Detachment of the uncinate process along the superior mesenteric
artery
– Completion of the dissection of the mesopancreas along the superior
mesenteric artery and removal of the specimen
– Marking of the incision margins with ink or sutures, intraoperative
quick incision
– Start of the reconstruction phase
– Reconstruction by means of hepaticojejunostomy and either
pancreaticojejunostomy and end-to-side gastrojejunostomy or
pylorojejunostomy or pancreatogastrostomy with Y-Roux
reconstruction or omega loop with or without Braun anastomosis (◘
Fig. 9.7)
– Warren-Catell pancreaticojejunostomy: End-to-side anastomosis with
duct-to-mucosa anastomosis (◘ Fig. 9.8)
– Posterior wall suture: pancreatic parenchyma to jejunal serosa
made in single button technique (4-0 PDS). The sutures are
presented
– After tie knotting, the anterior wall of the pancreatic duct is
presented with double-armored sutures (5-0 or 6-0 PDS)
– Punctual opening of the jejunum opposite of the pancreatic duct
and single sutures of the posterior wall (duct-to-mucosa, 5-0 PDS);
if necessary splinting of the pancreatic duct and completion of the
anterior wall (duct-to-mucosa)
– The external anterior wall suture is performed using a single suture
technique (4-0 PDS) so that the jejunum covers the anastomosis
– Pancreatogastrostomy (◘ Fig. 9.9): between pancreatic parenchyma
and stomach
– A larger (7 cm) anterior gastrotomy and a small (depending on the
cross-sectional size of the organ) (2 cm) dorsal gastrotomy are
performed as the approach.
– The mobilized pancreatic tail is invaginated into the stomach via
the dorsal gastrotomy (posterior wall of the stomach) and fixed by
means of a circular tabac pouch suture in the stomach wall and
several parenchyma to serosa sutures
– Bilioenteric anastomosis: Hepaticojejunostomy
– Length of the small intestine loop approx. 60 cm (Y-Roux)
– Rear and front wall: single layer, interrupted, PDS 5-0/6-0
– In case of narrow duct, if necessary, extension plastic according to
Gütgemann
– In case of high hilar anastomosis hepaticojejunostomy according to
Hepp-Couinaud may be necessary
– Enterotomy in the size of the hepatic duct
– Roux-Y reconstruction (2 loops), Omega reconstruction (1 loop) or 3-
loop reconstruction (one isolated Roux-Y loop each to the pancreas
and the bile duct as well as to the postpyloric duodenal remnant
(PPPD)/stomach (Whipple))
– At the end of the reconstruction: rinsing and insertion of 4 easy-flow
drains ventrally and dorsally of the pancreatic and biliodigestive
anastomosis respectively (according to pancreatic fistula score
possibly omitting drains all together)
Fig. 9.7 Pylorus-preserving or classic pancreaticoduodenectomy according to Whipple, (a)
normal situs, (b) after Whipple operation. 1 pancreatic anastomosis, 2 bile duct anastomosis, 3
gastroenterostomy. (Mod. according to Künzli et al. 2004)
Fig. 9.8 a–e Pancreaticojejunostomy
 Fig. 9.9 Pancreatogastrostomy

Pathology
– R0-narrow, if circumferential resection margin (CRM) ≤1 mm
– R0-wide or CRM negative if CRM >1 mm
– With stringent pathological workup (Leeds protocol) high R1 resection
rate (up to 60%)
– N0 (0 pos. LK), N1 (1–3 pos. LK), N2 (>3 pos. LK) (8th version of
AJCC)
Postoperative Complications
– Post-pancreatectomy hemorrhage (gastroduodenal artery arterial
hemorrhage and pancreatic sedimentation marginal hemorrhage), late
post-pancreatectomy hemorrhage (late PPH)

Caution
Mortality of the arrosion hemorrhage up to 50%.

– Pancreatic fistula (type A-C) = 20%

– Gastric emptying disorders (higher with pancreatogastrostomy) = 20%.
– Bile leakage/bilioma
– Anastomosis insufficiency
– Residual pancreatitis (postoperative pancreatitis)
– Diabetes mellitus requiring insulin
– Endocrine and exocrine pancreatic insufficiency
– Wound infection = 10% (for open surgery)
Postoperative Treatment: Adjuvant Chemotherapy
– Adjuvant chemotherapy in UICC stages I-III
– Contraindications to adjuvant chemotherapy:
– Eastern Cooperative Oncology Group (ECOG): Performance Status
>2
– Uncontrolled infection
– Liver cirrhosis Child B and C
– Severe coronary artery disease; heart failure (NYHA III and IV)
– Preterminal and terminal renal failure
– Impaired bone marrow function
– Inability to attend regular check-ups
– Adjuvant therapy = improvement of 5-year survival after curative
resection from 10% to 20% (with mFOLFIRINOX to 55%)
– 5-Fluorouracil plus gemcitabine for 6 months
– mFOLFIRINOX for 6 months (PRODIGE-Group)
Palliative Therapy
Indications
– For locally advanced or metastatic pancreatic cancer
– ECOG 0–2 (◘ Table 9.5)
Table 9.5 Eastern Cooperative Oncology Group (ECOG)a (according to Oken et al. 1982)

Points ECOG performance status

0 Normal, unrestricted activity, as before the disease
1 Restricted during physical exertion, able to walk, light physical work possible
2 Able to walk, self-care possible but not able to work, can stand up more than 50% of
waking time
3 Limited self-care possible; confined to bed or chair for 50% or more of waking hours
4 Completely dependent, self-care not possible, completely confined to bed or chair
5 Death

a
Performance status describes the physical condition of cancer patients and
is used to quantify general well-being and limitations in activities of daily
living
Therapy Regime
– First-line therapy: Gemcitabine (1000 mg/m2) (to be discussed)
– 5-FU with or without folinic acid: not as sole first-line therapy
– Alternative to monotherapy with gemcitabine: combination with the EGF
(epidermal growth factor) receptor tyrosine kinase inhibitor erlotinib
depending on the development of skin exanthema
– In healthier patients (ECOG 0–1, age ≤75 years and a bilirubin level
below 1.5 times the normal level): Combination of 5-FU/folinic acid,
irinotecan and oxaliplatin (FOLFIRINOX protocol)
– Nab-paclitaxel plus gemcitabine

9.3.2 Guidelines
Oncology guideline program (German Cancer Society, German Cancer Aid,
AWMF): S3 guideline Exocrine pancreatic cancer, long version 1.0, 2013,
AWMF register number: 032-010OL, ► http://​leitlinienprogra​mm-
onkologie.​de/​Leitlinien.​7.​0.​html, Renewed 2022.

9.4 Cystic Neoplasms

Key Points
 – Increasing incidence and detection of cystic neoplasms in the last two
decades
– About 90% of pancreatic cystic neoplasms are classified into four
entities:
– Intraductal papillary mucinous neoplasia (IPMN)
– Serous cystic neoplasia (SCN)
– Mucinous cystic neoplasia (MCN)
– Solid pseudopapillary neoplasia (SPN)
– Frequently incidental findings
– Malignant progression of mucinous cystic lesions in 10–50% of cases

9.4.1 Intraductal Papillary Mucinous Neoplasia (IPMN)

Definition
– Macroscopically visible, mucin-producing epithelial tumors arising from
pancreatic duct epithelium (papillary)
– Precursor lesion of IPMN carcinoma
– WHO classification: inclusion of IPMN in this classification in 1996
– Breakdown:
– Main-duct-IPMN
– Branch-duct-IPMN
– Mixed-type IPMN
– IPMN with low, intermediate or high grade dysplasia or with invasive
cancer
– Histologically prognostically relevant subclassification:
– Gastric
– Intestinal
– Pancreatobiliary
– Oncocytic

Epidemiology (◘ Table 9.6)

– Estimated incidence = 1/280,000 patients
– Women:Men = 1:1
– Frequency peak: 60–70 years of age
– Often incidental findings
– 5-year survival from MD (“main-duct”)-IPMN = 31–54%
– Intestinal IPMN (20%): Roughly correspond to villous neoplasms of
the colon; if invasive, 5-year survival rate = 50%
– Pancreatobiliary IPMN (8–10%): “High-grade tumors”; in >50%
presence of an invasive component; a 5-year survival = like ductal
adenocarcinoma of the pancreas
– Oncocytic IPMN: Extremely rare; frequently “high-grade carcinomas”
Table 9.6 Clinical and imaging features of cystic neoplasms of the pancreas. (According to
Grützmann et al. 2011; Tanaka et al. 2012)

IPMN MCN SCN SPN

Age (average) 64 years 47 years 70 years 30 years
Male (%) 60% 5% 30% 13%
Symptoms Frequently 50% Rarely Rarely
Localization Mainly pancreatic Almost always Variable Mainly
head pancreas tail pancreatic head
Main course Dilated (“main duct Normal Normal Normal
type”)
Non-dilated (“branch
duct type”)
Calcifications No Rarely Central scar (30–
40%)
Main aisle Always Sometimes No No
connection
Muzin Yes Yes No No
Appearance “Grape-like” “Orange-like” “Honeycomb-like”
Malignancy Frequently (Sendai Very often >70% Very rarely <5% Up to 10%
Criteria)
Special features Main and side aisle Ovarian stroma Microcystic and Young women
Type oligocystic
Therapy MD: Operation Operation Watch Operation
always, BD: ◘ Fig.
9.10

IPMN intraductal papillary-mucinous neoplasia, MCN mucinous-cystic

neoplasia, SCN serous-cystic neoplasia, SPN solid pseudopapillary
neoplasia, MD main duct, BD branch duct
Etiology
– Unclear
– Association with extrapancreatic primary tumors (colorectal, breast, and
prostate cancer)

Symptoms
– Most frequently due to pancreatic duct obstruction
– Nausea
– Vomiting
– Abdominal discomfort (59%)
– Back pain
– Weight loss (29%)
– Jaundice (biliary obstruction) (16%)
– Previous episodes of pancreatitis (14%)
– Diabetes mellitus (IDDM)

Diagnosis
CT or MRI
– MRI (MRCP) = better method in centers with experience (duct
association and main duct connection)
Imaging Signs
– Endosonography (ductal association and worrying nodules)
– Dilated pancreatic duct
– BD-IPMN = “Grape-like configuration”

Therapy (◘ Fig. 9.10)

Surgical Therapy of MD-IPMN
Indication for Surgery
– All MD-IPMN with main duct diameter >1 cm
– Since 62% of all MD-IPMN = malignant and 43% of all MD-IPMN =
invasive
Fig. 9.10 Flowchart for the treatment of cystic neoplasms. (After Tanaka et al. 2017)

Aim of the Operation

– Removal of the lesion ideally before malignant transition
Principle
– Resection according to localization: R0 resection to be aimed at
(oncologic radical operation)
– Frequent PPPD vs. classical pancreatic head resection vs.
pancreatectomy for multifocal type
– If necessary, total pancreatectomy in multifocal IPMN, decision
according to histology of leading lesion
– Operate main finding, if frozen section shows high-grade dysplasia at the
sedimentation margin, resect further until total pancreatectomy. If low-
grade dysplasia, no further resection and organ-preserving procedure

Further Indications for Total Pancreatec tomy

 – Positive margins at the pancreatic incision margin in pancreatic head
carcinoma as isolated positive margin
– Multifocal metastases of renal cell cancer (urological consultation)
– Multifocal advanced neuroendocrine tumors
– Refractory pain syndrome in chronic pancreatitis (TPIAT (see above)
—very controversial!)
– Resection margin:
– In case of high-grade dysplasia = extension of the resection
– In moderate and low-grade dysplasia = no further additional
resection necessary
– If the main duct diameter is <1 cm = further evaluation (◘ Fig.
9.10)

Preoperative for planned splenectomy: vaccination against Pneumococcus,

Haemophilus influenzae group B and Meningococcus group C 2 weeks
before planned surgery.

Surgical Procedure
Total Pancreatectomy with Splenectomy
– Supine position (left arm supported, right arm extended)
– Transverse upper abdominal laparotomy, right and left extended
– exploration of the abdominal cavity
– Opening of the omental sac while sparing the gastroepiploic vessels
with transection of the gastrocolic ligament
– Mobilization of the right colonic flexure
– Release of duodenal C from its retroperitoneal connections (Kocher
maneuver)
– Lifting of the duodenum and pancreas from the inferior vena cava up
to the left renal vein
– Extension of the Kocher maneuver by mobilization of the pars
horizontalis duodeni up to the superior mesenteric vein, presentation
of the same from caudal right in the region of the mesenteric root
– Elevation of the pancreatic neck = view of the avascular plane dorsal
to the pancreas, here preparation up to the sinus confluens venosum,
 exposure of the superior mesenteric artery just to the left of the vein in
this area (mesenteric artery first approach)
– Open antegrade cholecystectomy, opening of the hepatoduodenal
ligament with exposure of the choledochal duct and the common
hepatic artery. Caution: Expose the right hepatic artery with
intersection of the bile duct (often variable course)
– Dissection and ligation of the gastroduodenal artery and the right
gastric artery
– Dissection and ligation of the splenic artery and confluent placement
and suturing of the splenic vein
– In spleen-preserving pancreatectomy, visualization of the pancreatic
tail from caudal and cranial and stepwise visualization of the
individual branches from the splenic artery and into the splenicvein
– Separation of the splenorenal ligament and medial elevation of the
spleen together with the pancreatic tail, so that the retroperitoneal
layer is exposed
– Mobilization of the distal stomach and the duodenojejunal flexure
– Approx. 10–15 cm aboral of the ligament of Treitz = deposition of the
jejunum
– Removal of the specimen en bloc after stepwise separation of the
pancreatic head from the mesentericoportal axis (pancreas, distal
stomach, duodenum, spleen)
– Reconstruction with end-to-side hepaticojejunostomy and end-to-side
duodenojejunostomy if pylorus-preserving, otherwise
gastrojejunostomy (◘ Fig. 9.10)

Postoperative Management After Pancreatectomy

– Screening/prophylaxis/therapy of weight loss (80% of patients loose
>10% of their weight)
– Enzyme substitution (median 8 capsules/day, taken regularly with each
meal)
– Insulin administration in pancreatogenic (type III) diabetes (median 25
IU/day)
– In total pancreatectomy, sugar control is more difficult with reduced
hypoglycemia sensitivity
Surgical Therapy of BD (“Branch-Duct”)-IPMN
– Indication:
– Consider surgical therapy, ideally before transition to carcinoma; in
selected series, up to 26% of all BD-IPMN are malignant and up to
18% are invasive carcinomas
– Patients <65 years and cyst size >2 cm = resection (due to cumulative
malignancy rate)
– Patients with “worrisome features” (nodules, wall thickening) or
symptoms (pain, new-onset diabetes mellitus, etc.)
Conservative therapy for BD-IPMN (see below)
– Only in Sendai (Fukuoka)-negative tumors: i.e. <2 cm without symptoms
or “worrisome features”
– Annual malignancy rate of only 2–3%
– Patients with BD-IPMN = significantly older
– Conservative therapy + check-ups
Postoperative Follow-Up
– Recurrence rate after 5 years = 0–20% (disease of the entire pancreas!)
– 5-year survival in resected non-invasive IPMN = 80–100%
– 5-year survival in resected invasive IPMN = 40–60%
– 5-year survival rate for IPMN carcinoma = 20% (like adenocarcinoma—
thus avoid transition to carcinoma by prophylactic surgery in high-risk
constellations)
– Control examinations after 2 and 5 years due to general risk of
development of IPMN at further sites in the pancreas (R0-situation)
Conservative Therapy of MD-IPMN (5–9 mm Main Duct) and BD-
IPMN
(Caution!) 5 mm might still be dangerous as far as development of IPMN
cancer
– ◘ Figure 9.10

9.4.2 Serous Cystic Neoplasms (SCN)

Definition
– Benign tumors consisting of numerous cysts
– 10–20% of cystic pancreatic lesions
– Honeycomb structure
– Star-shaped scar in 20% of patients
– Virtually never degenerate malignant
– Localization: Pancreatic corpus and tail (70%)

Epidemiology (◘ Table 9.6)

– Women > Men = 5:1
– Frequency peak: >60 years of age
– 18–39% of all cystic neoplasms

Symptoms
– Mostly asymptomatic
– Nausea
– Vomiting
– Abdominal discomfort
– Back pain
– Weight loss

Diagnosis
– Multi-slice CT
– MRI
– Endosonography

Therapy
Surgical Therapy
– From a size of >4 cm, due to increased growth and all with symptoms
Conservative Therapy + Monitoring
– In all other cases

9.4.3 Mucinous Cystic Neoplasia (MCN)

Definition
– Solitary, round tumors with uni- or multilocular cysts
– Cysts lined by mucin-forming cells
– Ovarian stroma (probably scattered ovarian cells)
– Approx. 10% of cystic tumors of the pancreas
– Mostly in the body-tail area
– Potential precursor for pancreatic cancer

Epidemiology (◘ Table 9.6)

– 95% women
– Frequency peak: 40–60 years of age
– Malignancy rate = 30–50%
– Prevalence of invasive cancer = up to 15%
– 5-year survival rate of invasive MCN = 57%
– 5-year survival rate of MCN adenocarcinoma = 20%

Symptoms
– 20% = asymptomatic
– Non-specific abdominal complaints

Diagnosis
– Multi-slice CT
– MRI

Therapy
– Always surgical therapy
– Principles:
– MCN <4 cm without mural nodules = parenchyma-sparing or
laparoscopic (central or distal) pancreatectomy
– Otherwise, classic pancreatic resection with lymphadenectomy (LAD)
and (often) splenectomy, if necessary
– ◘ Figure 9.10

9.4.4 Solid Pseudopapillary Neoplasia (SPN)

Definition
– Solid, only secondary pseudocystic-degenerative tumors
– <5% of cystic pancreatic tumors
– Typically solid tissue at the edge and hemorrhagically disintegrating
centrally

Epidemiology (◘ Table 9.6)

– Young women (20–30 years)
– Low malignancy potential, often very large tumors
– Metastases (liver and peritoneum): In 10–15% of cases with a long time
interval to resection of the primary site, then resection again
– 5-year survival = 97%

Symptoms
– Asymptomatic
– Mostly incidental finding

Diagnosis
– Multi-slice CT
– MRI
– Endosonography

Therapy
Always Operative
– Even in metastatic stage
Principles
– Distal pancreatic resection with/without splenectomy
– Pancreaticoduodenectomy (PPPD/Whipple)

Surgical Procedure
Laparoscopic Spleen-Preserving Pancreatic Left Resection
– Y-positioning (= suppine position with spread leg; = French position)
– Access by means of a total of 4 trocars in a semilunar line around the
main findings
– Pneumoperitoneum
– Exploration of the abdominal cavity for pathologies not previously
described (liver/peritoneum)
 – Intracorporeal sonography of the liver and the peripancreatic region as
well as the pancreas
– Positioning in anti-Trendelenburg position, beach-chair positioning
– Visualization of the pancreas by mobilization of the left colonic
flexure as well as the transverse colon up to the right flexure
– Opening of the omental sac
– Visualization of the gastroepiploic artery and the confluens venosum
of portal vein to avoid complications
– Dissection of adhesions between upper pancreatic margin and
stomach and lymphadenectomy
– Pancreas mobilization starting at the lower edge, from here
visualization of the splenic vein and the venous confluence
– Visualization of the celiac trunc and the splenic artery
– Completion of the oncological lymphadenectomy at the upper
pancreatic margin
– Dissection + transection of the small vessels of the pancreatic body
and tail in an alternating manner centrally (confluens venosum) and
peripherally (splenic hilus) = e.g. Ligasure device or PDS/metal clips
– Separation of the pancreas tail with a linear stapler (GIA with coating
if necessary) and salvage using a salvage bag
– Insertion of two drains dorsal and ventral to the pancreas
– Further operative possibility = method according to Warshaw:
– Spleen supply only via left gastroepiploic artery and short gastric
arteries
– Splenic artery and vein are severed (short gastric vessels)
– Caution: Higher rate of secondary splenectomies for ischemia.

9.4.5 Guidelines
Tanaka M, Chari S, Adsay V, Fernandez-del Castillo C, Falconi M, Shimizu
M, Yamaguchi K, Yamao K, Matsuno S, and International Association of
Pancreatology (2006) International consensus guidelines for management of
intraductal papillary mucinous neoplasms and mucinous cystic neoplasms
of the pancreas. Pancreatology 6:17–32.
Tanaka M, Fernandez-del Castillo C et al. (2012) International
consensus guidelines 2012 for the management of IPMN and MCN of the
pancreas. Pancreatology 12:183–197.
 Tanaka M, Fernández-del Castillo C, Kamisawa T, Jang JY, Levy P,
Ohtsuka T, … Wolfgang CL (2017) Revisions of international consensus
Fukuoka guidelines for the management of IPMN of the pancreas.
Pancreatology 17(5):738–753.

9.5 Endocrine Neoplasms

Key Points
– Rare, approx. 3% of all pancreatic neoplasms
– 5-year survival of malignant neuroendocrine tumors of the pancreas
approx. 30–40%
– Grouping into functional and non-functional neuroendocrine tumors

9.5.1 Definition
– Neuroendocrine tumors (NET) of the pancreas = rare tumors
– Initiation from endocrine cells
– Classification of NET of the pancreas:
– Functional (hormone-active) NET (gastrinoma, insulinoma, VIPoma,
somatostatinoma, PPoma): Production and release of hormones
– Non-functional (hormone-inactive) NET

9.5.2 Epidemiology
– Incidence: 0.4–1.5 new cases per year/100,000 population
– Increasing prevalence
– Insulinoma and gastrinoma (Zollinger-Ellison syndrome) = 1:500,000 per
year
– Glucagonoma (diabetes dermatitis syndrome) = very rare
– Vipoma = Verner-Morrison Syndrome
– Nonfunctional NET of the pancreas (exclude MEN-1 syndrome in case
of familial clustering)

9.5.3 Symptoms
Insulinoma
– Whipple triad:
– Hypoglycaemia (glucose <45 mg/dL) + associated neurological
symptoms (= feeling of weakness, confusion, dizziness, visual
disturbances, headache, loss of consciousness)
– Autonomic symptoms (palpitations, tachycardia, sweating and
sometimes aggressiveness)
– Rapid improvement in symptoms with glucose infusion
– Weight gain (20% of patients)
– Mechanical complications possible, but rarely due to the rather small
tumors
– Mostly very small tumors
– Malignant insulinomas (10%): Production of various hormones:
calcitonin, melanocyte-stimulating hormone (MSH), adrenocorticotropic
hormone (ACTH), etc. = variable symptomatic picture

Gastrinoma (Zollinger-Ellison Syndrome)

– Gastrin overproduction leads to:
– Excess stomach acid = multiple ulcerations
– Upper abdominal pain (multiple refractory gastric ulcers)
– Reflux Disease
– Complications of ulcers: Upper GI (gastrointestinal) bleeding + gastric
or duodenal perforation

VIPom
– Massive diarrhea
– Mechanism = release of vasoactive intestinal peptide
– Resulting in:
– Dehydration
– Hypochloridemia
– Hypokalemia
– Hypomagnesemia

Glucagonom
– Severe migratory necrotizing exanthema
– Moderately elevated blood glucose levels
– Weight loss
– Anemia
– Stomatitis

Somatostatinoma
– Often clinically inapparent
– Increased fat storage: due to partial inhibition of thyroid function
– gastric distention
– Inhibition of hormones in the gastrointestinal tract results in
– Malabsorption signs with fatty stools
– Gallstones due to gallbladder motility disorders

Pancreatic Carcinoid Syndrome

– Paroxysmal flush
– Intestinal complaints
– Diarrhea
– Signs of right heart failure

Non-Functional NET (95%)

– Generally late diagnosis, often incidental findings
– Abdominal discomfort
– Weight loss
– Prognosis and grading according to grading (G1-G3) and proliferation
rate (Ki-67 index <2%, 2–20%, >20%)

9.5.4 Diagnosis
Laboratory Diagnosis
– Determination in serum
– Detection of all hormones in serum with associated NET
Chromogranin A
– General marker for NET
– Also good follow-up parameter for diagnosis of recurrence
Caution
False-positive chromogranin A levels with proton pump inhibitor (PPI)
therapy (discontinue at least 1 week before testing).
Gastrin
– In Zollinger-Ellison syndrome:
– Fasting gastrin level >1000 pg/mL and gastric pH of <2
– Secretion test >200 pg/mL above basal level
– Also discontinue PPI inhibition (false positive levels of gastrin)
Fast Test
– For insulinoma: until hypoglycemia is reached
Insulin, Plasma Glucose
– Insulin (μU/mL)/plasma glucose (mg/dL) ratio >0.33
– C-peptide >0.7 mg/L (differential diagnosis hypoglycaemia facitata due
to insulin injection)
5-Hydroxyindoleacetic Acid
– Degradation product of serotonin
– In the acidified 24-h collected urine
– Increased in carcinoid syndrome and small bowel NET

Imaging Techniques
Contrast Enhanced Ultrasound
– Echo-negative structure, more often hypervascular perfusion
– Not sufficient to confirm the diagnosis
Endosonography
– Very good representation of the positional relationship to surrounding
organs
– Superior to other methods in localization diagnosis
– Good method for long-term follow-up of MEN-1 syndrome
Multidetector CT
– Hyperintense visualization of the NET in the early contrast phase
(hypervascularized)

9.5.5 Therapy
Benign Solitary NET with Local Resection Option (>2 cm)
– Enucleation

Caution
High pancreatic fistula rate after enucleation up to 80%!

NET Without Local Resection Option

– Operation by location:
– Pancreatic head resection
– Central pancreatic resection
– (Spleen-preserving) pancreatic left resection
– Systematic lymphadenectomy in case of Ki-67 index >2%, CT
suspicious LN metastases or tumor size >4 cm

Local Recurrences or Metastases of NET

– Surgical therapy

Diffuse Metastasized NET

– Treatment with somatostatin alone
– Treatment with somatostatin + α-interferon

Surgical Procedure
Central Pancreatectomy with Pancreatogastrostomy
– Supine positioning
– Transverse laparotomy of the upper abdomen or median laparotomy
– Exploration of the abdominal cavity for pathologies not previously
described
– Opening of the omental sac
– Mobilization of the lower edge of the pancreatic neck and body
 – Visualization of the superior mesenteric vein, at the inferior border of
the pancreas
– Mobilization of the upper edge of the pancreas (lymphadenectomy in
the area of the hepatica artery)
– Dissection of the pancreas from the portal vein
– Ventral luxation of the pancreas with loops around the pancreatic
body/neck
– Identification of the splenic vein and transsection of the pancreas
– The splenic artery is usually located separately from the neck and
proximal pancreatic body
– After complete exposure of the pancreatic body = transection with
stapler (endo-GIA) on the mesentericoportal axis
– Stapling of the proximal pancreatic remnant or two-row suturing
(PDS 4-0 MH, V-shape closure)
– Pancreatogastrostomy (or pancreato-pancreaticojejunostomy) between
the distal pancreatic remnant and the posterior wall of the stomach or
intestine (jejunum), after removal of the row of staples in the area of
the pancreatic duct (creation as described above)
– Insertion of 2 drains at the distal and proximal pancreatic remnant

9.5.6 Guidelines
Falconi M, Bartsch DK, Eriksson B, Kloppel G, Lopes JM, O’Connor JM,
Salazar R, Taal BG, Vullierme MP, O’Toole D, Barcelona Consensus
Conference (2012) ENETS Consensus Guidelines for the management of
patients with digestive neuroendocrine neoplasms of the digestive system:
well-differentiated pancreatic non-functioning tumors. Neuroendocrinology
95:120–134.

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© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_10

10. Kidney Transplantation

Bernd Jänigen1 , Franck Billmann2 and Przemyslaw Pisarski1
(1) Department of General, Visceral and Transplant Surgery, University
Hospital Freiburg, Freiburg, Germany
(2) Department of General, Visceral and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Germany

Bernd Jänigen (Corresponding author)

Email: [email protected]

Franck Billmann
Email: [email protected]

Przemyslaw Pisarski
Email: [email protected]

10.1 Introduction
– First successful kidney transplantation (KTx) in 1954 in identical twins
in Boston, USA
– Safe standard procedure with very good success:
– 2018 in Germany: 2191 NTx, thereof 638 living donations
– Problem: Serious organ shortage. Reasons = complex, additional
decrease since scandal with organ transplantation
– Alternative: Therapy of terminal renal failure by replacement procedures:
– Hemodialysis (HDi)
– Peritoneal dialysis (CAPD)
10.1.1 Legal Framework
– German Transplantation Act (GTA/TPG) implemented in 1997, last
amendment 2012
– Goal: Promote willingness to donate organs
– Content German Transplant Act (GTA):
– Public education
– Organ donation (post-mortem and living)
– Organ allocation
– Organ Transplantation
– Since the introduction of the GTA: Irreversible loss of brain function
(formerly: brain death) = recognition as criterion of death
– Transplantation according to urgency, likelihood of success and equality
of chances

10.1.2 Structure in Germany

– Organization of organ donation: German Foundation for Organ
Transplantation (DSO)
– Organ allocation: Eurotransplant (ET)
– Organ transplantation: Transplant Centres
Organ donation, allocation and transplantation by independent
institutions.

German Foundation for Organ Transplantation

– Structure—7 regions
– Coordination and implementation of organ donation

Eurotransplant
– Non-profit organisation based in Leiden (The Netherlands)
– Organ Allocation for:
– Austria, Belgium, Croatia, Germany, Hungary, Luxembourg,
Netherlands, Slovenia
– For Germany: Allocation according to allocation guidelines of the
German Medical Association (Bundesärztekammer)
Transplant Centers
– Preparation of the potential recipients
– Carrying out organ transplantation
– Follow-up of the recipients after transplantation

10.2 Indication for Transplantation and

Preparation of the Recipient
– Indication for kidney transplantation: Basically in all patients with end-
stage renal diseases (ESRD)
– Prior to registration in the waiting list: Obligatory medical evaluation of
the recipient concerning indication of KTx and operability

10.2.1 Indication for Transplantation

Indication = ESRD [End-Stage Renal Disease]
– Liberal indication due to high comorbidity and loss of quality of life due
to alternative procedures
– Waiting time for a post-mortem organ: currently approx. 8–10 years
– Alternative: Living donation: Also possible preemptively (before the
onset of dialysis requirement): sensible starting at approximate
glomerular filtration rate (GFR) <15 mL/min

Contraindications
– Severe acute or chronic infections
– Malignant disease (waiting period between 1 and 5 years)
– Severe cardiovascular diseases
– Severe pulmonary diseases
– Severe psychiatric illnesses
– Severe extrarenal diseases
– Alcohol or drug abuse
– Non-adherence: due to the need for immunosuppression

Causes of End-Stage Renal Disease

– Glomerulonephritis
– Interstitial nephritis
– Congenital malformations
– Bilateral nephrectomy for tumor or trauma
– Metabolic diseases (e.g. diabetes, oxaluria, etc.)
– Drug toxicity
– Hereditary diseases
– Obstructive diseases
– Reflux nephropathies
– Systemic diseases
– Haemolytic-Uraemic Syndrome (HUS)
– Irreversible acute renal failure
– Vasculopathies

Most Common Underlying Condition for New Waiting List

Enrollment in 2018 (n = 2348) (Eurotransplant Statistics)
– Cystic kidney disease: 421
– Chronic nephritic syndrome: 333
– Chronic kidney disease: 372
– Type 1 diabetes: 220
– Hypertensive kidney disease:206
– Nephrotic syndrome: 127
– Chronic tubulointerstitial nephritis: 40
– Rapid progressive nephritic syndrome: 40
– Other diagnoses: 589

10.2.2 Recipient Preparation

Detailed Information—Medical Aspects
– Before evaluation of transplantability and qualification for the waiting
list
– About all aspects of kidney transplantation:
– Registration in the waiting-list
– Waiting time
– Transplantation
– Need for immunosuppression
 – Risks and complications
– Results

Medical Evaluation
– For each organ recipient
– Objectives/content:
– Determination of general operability
– Exclusion of contraindications
– Compliance review

Caution
Due to the permanent immunosuppression required postoperatively,
one focus of preoperative diagnosis is the exclusion of possible sources
of infection.

Diagnostic Procedure
– Preparation of the recipient = ideally at an early stage before the onset of
the dialysis requirement (since a preemptive transplantation can take
place in the case of a living donation)
– Recipient diagnosis in close cooperation with the responsible dialysis
center

Detailed Anamnesis and Clinical Examination

– Underlying renal disease
– Dialysis initiation and procedures
– Tumor disease
– Cardiovascular risk factors and pre-existing conditions
– Pre-existing pulmonary disease
– Previous operations
– Infection status
– Addictive disorders
– Family medical history
– Current medication
– Clinical examination
– Vascular status
Laboratory Tests
– Blood count, kidney function, electrolytes, liver function, coagulation,
inflammatory signs, endocrine diagnosis (HbA1c, parathormone, thyroid
levels), PSA (prostate specific antigen, men >45 years)
– Virology/Bacteriology: Hepatitis B (HBV) and C (HCV); human
immunodeficiency virus (HIV), Epstein-Barr virus (EBV),
cytomegalovirus (CMV), herpes simplex virus (HSV), varicella-zoster
virus (VZV), syphilis (TPHA)
– Determination of the blood group
– HLA (“human leucocyte antigen”) typing
– Donor-specific antibodies
– Urinalysis
– Cross-match (living donation)

Apparative Examination
– ECG
– Chest X-ray
– Lung function
– Cardiac echocardiography
– Exercise ECG, myocardial scintigraphy, coronary angiography if
necessary
– Abdominal sonography
– Vascular status—if necessary pelvic CT native/angiography
– CCDS (color-coded Doppler sonography) carotid, if necessary,
– Urologic evaluation—including cystoscopy

Screening
– Urological screening (men >45 years)
– Gynaecological screening (women)
– Colonoscopy (>50 years)

Evaluation by Specialist
– ENT
– Dentist
– Dermatologist
– Urologist
– Psychological examination, if necessary

Vaccinations
– Influenza
– Tuberculosis
– COVID
– Hepatitis A and B
– Tetanus
– Diphtheria
– Polio
– Pneumococcus
– Meningococcus
– Live vaccines: varicella, measles/mumps/rubella (these are
contraindicated under immunosuppression)

Caution
If other diseases are diagnosed, therapy must have been started or
completed before registration in the waiting list (e.g. focal treatment for
ENT/dental infections, interventional and surgical therapy for CHD,
cholecystectomy for symptomatic cholecystolithiasis).

10.2.3 Registration in the Waiting List

Indication/Registration
– Indication is made individually by an interdisciplinary conference (6-
eyes-principle)
– Registration with Eurotransplant as soon as
– the treating transplant centre has determined indication and
– all necessary examinations are available
Waiting time = time from the first day of dialysis. The time of
registration in the waiting list and the registration status have no influence
on this.
– Currently more than 7500 patients in Germany are on the kidney waiting
list
Urgency Status
– Distribution (allocation) of organs:
– Through Eurotransplant
– According to the urgency status (◘ Table 10.1)
– Causes for immunisation:
– Previous transplants
– Blood transfusions
– Pregnancies
– High-urgency status = extremely rare. Prerequisites:
– Lack of dialysis access option (vascular surgery report required)
– Life-threatening situation that can only be resolved by a kidney
transplant
– Severe bladder problems (recurrent cystitis, haematuria) after
simultaneous pancreas-kidney transplantation with bladder drainage of
exocrine pancreatic secretions. These may occur with loss of graft
kidney function and functioning pancreas graft
Table 10.1 Urgency levels according to Eurotransplant (► http://​www.​eurotransplant.​org)

Notification status (MUC) Description transplantability Urgency Allosensitisation (PRA)

HU “High urgency” Urgent –
T “Transplantable” Normal None; PRA <6%
I “Immunized” Normal Present; 6 <PRA <85%
HI “Highly immunised” Normal Present; PRA >85%
NT “Not transplantable” None –

MUC Medical Urgency Codes, PRA panel reactive antibodies: Indicates the
percentage of the recipient’s antibodies against HLA versus the cross-
section of the population

Overview: HU (“high urgency”) status:

– Application to Eurotransplant
– Review by expert panel
10.3 Deceased Organ Donation
Key Points
– In case of consent to organ donation + presence of irreversible loss of
brain function: coordination of organ donation by the German
Foundation for Organ Transplantation (DSO)
– Procedure: Exclusion of contraindications, donor notification to
Eurotransplant (ET), allocation of the organ, followed by donor surgery

10.3.1 Organ Donation and Donor Selection

Organ Donation
– Notification of a potential organ donor by the organ retrieval hospital to
the DSO
– Consent to Organ Donation:
– Existence of written will (e.g. donor card)
– Oral will (relatives)
– Presumed will of the patient (relatives)
– Decision according to the relatives’ own values if the patient’s
presumed will is unknown
– Irreversible loss of brain function:
– Clinical diagnosis by two specialists experienced in the care of
severely neurologically ill patients (one of whom is a specialist in
neurology/neurosurgery)
– Apparative diagnosis: e.g. detection of cerebral perfusion arrest, EEG
with zero-line

Caution
– Notification to Eurotransplant + further diagnosis: Only permitted
after irreversible loss of brain function and if consent to organ
donation has been given.
– Donation in case of cardiocirculatory death: not allowed in Germany
– Eurotransplant: Deadline of 6 h for the allocation of organs
Donor Selection
– Exclusion of a contraindication to organ donation:
– Generalized, chronic infection [HIV, HBV, HCV]
– Malignant disease (waiting period usually = 5 years)
– Exceptions = non-metastatic brain tumours, skin tumours (excluding
malignant melanoma), early-stage prostate tumours
– Kidney disease
– Carrying out the donation operation in the donor hospital
– Exact timing depends on the schedule of transplant centers that accept
organs with short ischemia time (especially heart, lung)

Ischemia Times of Organs

– Heart: <6 h
– Lungs: 8 h
– Liver: 12 h
– Pancreas: 12 h
– Kidney: 30 h

10.3.2 Organ Allocation

– Organ allocation by Eurotransplant

Allocation Programs
– AM (Acceptable Mismatch) Program:
– All highly immunized patients (PRA >85%)
– Based on the available HLA typing, organs are selected that are most
likely to result in a negative cross-match
– Patients in the AM program have priority and are allocated before all
other patients
– ETKAS program (Eurotransplant Kidney Allocation System, donors <65
years)
– Allocation within the blood groups
– Organ allocation (◘ Table 10.2)
– ESP program (Eurotransplant Senior Program, donors ≥65 years)
– Recipient ≥65 years
– Regional allocation: short ischemia time
– Allocation only based on waiting time
– Waiting time shorter compared to ETKAS
– No HLA match: immunological risk difficult to assess and usually
higher
Table 10.2 ETKAS scoring system (Eurotransplant Kidney Allocation System: ► http://​www.​
eurotransplant.​org)

Scoring based on the following seven factors

1. HLA typing (HLA-A, -B and -DR Per fitting HLA match (max. 6) 66.6 points, max. 400 points
loci) HLA bonus for paediatric recipients (double points)
2. Mismatch probability (MMP) Calculation of the probability to get a 0- or 1-mismatch
kidney depending on the results of AB0- and PRA-screening
3. Waiting time 33.3 points per waiting year
4. Paediatric bonus: 100 extra points. Definition of paediatric recipient:
Dialysis started before the age of 18 years
Listing possible from GFR <20 mL/min
5. Distance between collection For Germany: Organ from D: +100 points, organ from
centre and recipient centre (max. 300 federal state of recipient centre: +200 points
points)
6. High urgency (HU) status HU recipient = 500 extra points
7. Kidney after other organ If kidney transplant required 90–360 days after other organ
transplantation (except pancreas) transplant and dialysis requirement existed prior to other
transplant = 500 extra points

HLA human leucocyte antigen, PRA panel reactive antibodies

10.3.3 Organ Retrieval

Principle
– All kidneys from donors <2 years of age must be retrieved en bloc
– Kidneys from donors between 2 and 5 years of age should be retrieved en
bloc, but may be divided depending on the recipient profile
– Kidneys from donors >5 years of age are retrieved as single kidneys as
described below
Surgical Procedure
Visceral Organ Perfusion (◘ Fig. 10.1)
– Insertion of a perfusion cannula in the aortic bifurcation or the
common iliac vessel
– Free preparation of the subdiaphragmatic aorta (above the truncus
coeliacus)
– Ligation of both iliac arteries distal to the catheter as well as the distal
vena cava
– Full heparinisation before the cross clamp
– Ligation/clamping of the aorta subdiaphragm—cross clamp
– Immediate start of perfusion, usually with cooled (4 °C) histidine
tryptophanetoglutarate (HTK) solution (approx. 8 L)
– Incision of the distal inferior vena cava as well as the thoracic inferior
vena cava, if necessary insertion of a drainage catheter
– Cooling of the situs with slush ice/Ringer solution
 Fig. 10.1 Situs during perfusion

Surgical Procedure
Kidney Retrieval
– Exposure of the orifice of the left renal vein and dissected at the level
of the orifice into the vena cava
– Transverse transection of the inferior vena cava directly at the upper
edge of the confluence of the right renal vein
– Longitudinal opening of the aorta starting at the aortic bifurcation and
extending to the renal arteries
– Separation of the aorta just above the renal arteries, cutting of the
aortic posterior wall, the aorta is left as a patch
– Transverse transection of the vena cava in the area of the incision
– After removal of the liver and, if necessary, the pancreas, the kidneys
are detached from the retroperitoneum laterocranial.
– The ureter should be removed as long as possible (clearly beyond the
pelvic axis) with surrounding fatty tissue to protect the accompanying
vessels
Kidney packing

Caution
Wide variation in renal arterial supply:
– Often additional superior and/or inferior pole vessels (sometimes
several centimeters distant from the main artery)
– Atypical course of the vessels (e.g. right inferior pole artery often
ventral to the vena cava)
– Ideally, polar arteries should be harvested together with the main
artery on a patch

10.4 Living Kidney Donation

Key Points
– Living donation = alternative to post-mortem kidney transplantation
– Precise regulation in the GTA (e.g. who is eligible as a donor)
 – Legal guidelines: Very strict in Germany compared to other countries
– For any living donation: Presentation to an Ethics Committee after
extensive medical evaluation; then scheduling of organ donation
– Organ donation (open or laparoscopic): immediately before
transplantation

10.4.1 Prerequisites
– Adults at least 18 years of age
– First and second degree relatives
– Spouse, partner
– Persons who have a special personal relationship with the donor

10.4.2 Legal Limits in Germany

– Altruistic donation
– Cross-over: Two pairs crossed over in case of AB0 incompatibility,
positive cross-match or immunisation
– Chain transplants: Many pairs crossed over in AB0 incompatibility,
positive cross-match or immunisation

10.4.3 Advantages of Living Kidney Donation

– Short waiting time or preemptive transplantation
– Better survival of the transplanted kidney
– Better overall survival of the recipient
– Mostly direct organ function due to short ischemia time
Absolute priority for living kidney donation = safety of the donor
(kidney donor = healthy person with no medical indication for surgery).

10.4.4 Donor Evaluation [Preparation and Diagnosis]

Initial Interview with the Potential Donor
– Verification of the conditions
– Information about risks for the donor and opportunities for the recipient
– Exclusion of obvious contraindications by anamnesis
– Blood group, HLA typing and cross-matching

Detailed Medical Examination (Often Inpatient)

– Anamnesis
– Medication
– Clinical examination
– Lab test
– Extended virologic examination including e.g. HBV, HCV, HIV, CMV,
EBV, etc.
– 24 h collection urine
– Urine status/sediment
– Lung function
– ECG
– Stress ECG
– Heart Echography
– If necessary, long-term blood pressure measurement
– OGTT (oral glucose tolerance test)
– Chest X-ray
– Kidney duplex sonography
– Abdominal Sonography
– Psychiatric evaluation if necessary
– Angio-CT/MRI
– Renal scintigraphy
– Screening depending on the age of the donor

Presentation to an Ethics Committee

– Directly in advance detailed explanation by doctor and informed consent
– Verification of voluntariness
– Exclusion of organ trafficking
– Verification of the conditions

10.4.5 Donor Operation

OP Procedure
– Open surgery
– Laparoscopic
– Retroperitoneoscopic

Open Surgery
– Advantages:
– Good overview
– Good control of the situs
– Disadvantages:
– Cosmetic (pararectal or lumbar scar)
– Risk of incisional hernia
– Risk of abdominal wall relaxation
– More postoperative pain
– Longer convalescence

Surgical Procedure
Open Nephrectomy (Minimal Incision; ◘ Fig. 10.2)
– Positioning: Supine
– Pararectal section starting from the costal arch (8–10 cm)
– Severing the lateral abdominal wall
– Medialisation of the peritoneal sac
– Exposing the lower pole of the kidney
– Mobilisation of the entire kidney from laterocaudal
– Exposure and preparation of the ureter to just below the iliac axis
– Transection of the ureter (ligation distally, proximally the ureter is not
closed)
– Preparation of the renal vessels from the renal hilus toward central
– Heparin administration before clamping optional
– Clamping of the renal vessels centrally and dissection of the vessels
– Transfer of the kidney for perfusion, cooling and preparation
– Supply of the vessel stumps with non-absorbable suture material
– Drainage, wound closure
 Fig. 10.2 Open living kidney donation on the left. The V. ovarica/testicularis and V. suprarenalis
are already detached and ligated. The renal vein and artery are centrally connected. Caudally the
ureter is visible

Laparoscopic/Retroperitoneoscopic
– Advantages:
– Good overview
– Cosmetics (Pfannenstiel incision)
– Less pain
– Shorter convalescence
– Lower risk of hernia
– No relaxation of the abdominal wall
– Disadvantages:
– Loss of 0.5–1 cm vessel length (can be optimized with narrower staple
suture devices)
– In case of conversion, loss of time due to emergency laparotomy
Surgical Procedure
Laparoscopic (Hand-)Assisted Nephrectomy (◘ Fig. 10.3)
– Positioning: back with slightly raised operating side, on vacuum
mattress with pelvic support on opposite side
– Five trocars (3 × 12 mm, 2 × 5 mm). Placement: 12-mm trocars in the
latter Pfannenstiels incision, subumbilical and in the upper abdomen
medioclavicular on the contralateral side. 5-mm trocars in the axillary
line on the explantation side and epigastric.
– Entering the retroperitoneum with mobilization of the colon
(+duodenum on the right, +pancreas tail and spleen on the left)
– Right transection of the ovarian vein (technically easier)
– Exposure and transection of the ureter just below the pelvic axis, the
stump is clipped distally
– Preparation of the ureter up to the hilus
– Mobilisation of the kidney from laterocaudal and cranial in
rendezvous
– Exposure of the vessels from the hilus to central
– On the left side, pay attention to the ovaric vein, suprarenal vein and
lumbar branches. These must be carefully dissected out and severed
between PDS (polydioxanone) clips.
– Pfannenstiel incision (6–8 cm) and insertion of the port
– Completing the preparation on the upper pole and vessels
– Central renal artery and vein disconnection with endo-GIA (vascular)
– Recovery of the kidney and transfer for perfusion, cooling and
dissection
– Drainage, wound closure
 Fig. 10.3 The renal vessels are placed as centrally as possible with an endo-stapler. This results
in a loss of approx. 0.5–1 cm of vessel length (width of the stapler)

Surgical Procedure
Perfusion Living Donation
– Start cooling in 4 °C cold solution (HTK solution, etc.)
– Irrigation of the kidney with 100 mL heparin solution (50 IU/mL) via
the renal artery (in the case of minimally invasive removal, removal
of the staple suture line)
– Flush the kidney with approx. 500 mL perfusion solution until clear
reflux via renal vein.
– Preparation analogous to back-table preparation for postmortem
organs
– Store at 4 °C until transplantation

10.4.6 Risks and Complications

Mortality
– Total mortality = 0.03%
After kidney removal, creatinine levels may be elevated.

Morbidity
– Morbidity: low
– Bleeding
– Nausea/vomiting
– Wound infections
– Chronic pain
– Thrombosis
– Embolism
– Pneumonia

Long-Term Risks
– Proteinuria
– Arterial hypertension: in about one-third of the cases
– Risk of needing dialysis: approx. 0.2% (donor) vs. 0.02% (comparable
non-donor)
– Surgical complications
The living kidney donor carries a small residual risk. Therefore, a
detailed explanation in the transplant centre (surgeon and nephrologist) is
essential before living kidney donation. Perioperative graft loss is
particularly stressful.

10.4.7 Donor Aftercare

– By family doctor or nephrologist
– 3–5 controls during the first year
– After the first year once a year
– Recommendation: kidney function, protein excretion and blood pressure
control

10.5 Kidney Transplantation

Key Points
– Before the start of the recipient operation: preparation of the organ
“back-table”.
– Subsequent retroperitoneal implantation of the kidney (standardized
technique)
 – The presence of vascular anomalies in the donor organ/recipient can
make kidney transplantation very difficult or, extremely rarely, even
impossible

10.5.1 Back-Table Preparation of the Kidney

– Living kidney donation: Back-table preparation immediately following
removal and perfusion
– Post-mortem kidney removal: back-table preparation is the responsibility
of the recipient centre

Surgical Procedure
Back-Table Preparation
– Tracing of the vessels to the hilus, excess fatty tissue is removed with
clamps and ligatures
– Lateral branches, such as branches to the adrenal gland, are ligated
– If necessary, vessel reconstruction in case of vessel variations, e.g.
polar arteries, accessory vessel supply
– Exposure of the ureter: The accompanying vessels must be spared at
all costs to minimize the risk of ureteral necrosis
– Caution: Ureter fissus as a norm variant
Checking the seal of the vessels

10.5.2 Surgical Technique of Transplantation

Implantation Site
– Iliac fossa (mostly right), simpler venous vascular access
– Extraperitoneal position
– Easy vascular and bladder access
– Short distance to the bladder
– Good accessibility for biopsy, ultrasound

Surgical Procedure
Kidney Transplantation (◘ Fig. 10.4)
– Positioning: Supine
– Preparation: Central venous catheter, sterile insertion of a bladder
irrigation catheter, filling of the bladder
– Hockey stick-shaped cut in the lower abdomen
– Opening of the lateral abdominal wall: M. obliquus externus, M.
obliquus internus and M. transversus
– Medialization of the peritoneal sac
– Sparing preparation of the iliac axis in order to ligate as few lymphatic
vessels as possible
– If necessary, shortening of the transplant kidney vein and end-to-side
anastomosis to the distal inferior vena cava, common iliac vein or
external vena cava, continuous suture with non-absorbable suture
material
– Implantation of the artery mostly on the A. iliaca communis (aorta or
A. iliaca externa also possible), continuous suture with non-
absorbable suture material
– The vessels should come to rest without kinking or torsion
– Reperfusion, if necessary haemostasis in the area of the anastomoses,
the hilus and the renal capsule
– Opening the bladder roof
– Ureterocystoneostomy and antirefluxplasty according to Lich-Gregoir
– Ureter must lie free of torsion and tension
– Implantation of a DJ (double J) catheter optional
– Drainage, wound closure
 Fig. 10.4 Situs after kidney transplantation. The vessels lie stretched. The ureter is then
anastomosed with the bladder

Immunosuppression
– Started shortly before reperfusion by steroid bolus
– 250 mg methylprednisolone i.v.
Application of 20% mannitol and Lasix before anastomotic opening =
optional and not evidence-based.
– pAVK in the recipient can significantly complicate implantation; central
= create anastomosis proximal to a stenosis; if possible:
thrombendarterectomy with vessel reconstruction before actual
transplantation
– In paediatric recipients: anastomosis mostly to aorta and v. cava =
compensation of a discrepancy of recipient and donor vessels +
 possibility of transplantation of kidneys from adult donors to small
recipients

Caution
A living donor transplant is technically more challenging due to the
lack of an arterial patch.

10.5.3 En Bloc Renal Transplantation

Principle
– Organs from donors <5 years and <10 kg weight
– Aorta and v. cava of the donor serve as vascular elongation for
connection to recipient vessels

Advantages
– Double nephron mass by transplantation of two kidneys
– Good long-term prognosis

Disadvantages
– Higher complication rate
– Transplantation must be critically reviewed in young women due to
possible pregnancy
– Difficult biopsy in case of rejection

10.5.4 Surgical Complications

Vascular System (Incidence <5%)
Postoperative Bleeding/Haematoma
– Localisation: Retroperitoneum, anastomoses, renal parenchyma
– Occurrence: Immediately postoperative
– Cause: coagulation disorder, medication (ASS) and infections
– Therapy: Depending on dynamics and clinical presentation, reoperation
if necessary
Arterial Thrombosis
– Symptom: Sudden stop of diuresis
– Occurrence: Early postoperative
– Diagnosis: Color-coded duplex sonography
– Therapy: Immediate reoperation and attempt at revascularization
Graft Vein Thrombosis
– Symptoms: Sudden stop of diuresis
– Occurrence: Early postoperative
– Diagnosis: Color-coded duplex sonography
– Therapy: Immediate reoperation and attempt at thrombectomy
Renal Artery Stenosis
– Symptoms: creatinine increase, arterial hypertension
– Occurrence: Medium/long term after transplantation
– Diagnosis: Color-coded duplex sonography
– Therapy: If possible, interventional therapy with stent, reoperation is
likely to cause complications

Urological Complications (Incidence 2–10%)

Urinary Leakage
– Localization: Laterocranial to the bladder
– Symptoms: pain, creatinine increase, signs of infection
– Diagnosis: Sonography, puncture, Mibi (microbiology)/creatinine in the
punctate, localization by retrograde pyelography
– Cause: Frequently high bladder pressure in contracted bladder, ureteral
necrosis, injury to urinary drainage system during transplantation
– Therapy: Depending on location, low-pressure drainage with indwelling
bladder catheter (BDK), Splint if necessary, ureteral reimplantation if
necessary
Ureteral Stenosis
– Localization: Mostly prevesical
– Symptoms: Urinary retention, pain, creatinine increase, late signs of
infection
– Diagnosis: Sonography, retrograde pyelography
– Cause: Often swelling in the anastomosis area, scarred stricture of the
anastomosis, ureteral necrosis
– Therapy: Splint, in case of persistence ureteral reimplantation
Ureteral Necrosis
– Localization: Prevesical
– Symptomatology: Clinical presentation of ureteral leakage or ureteral
stenosis
– Diagnosis: Depending on the symptoms
– Therapy: Reimplantation of the ureter with resection of the necrotic
portion

Lymphatic Complications (Incidence 2–18%)

Lymphocele
– Localization: Mostly mediocaudal to the kidney
– Symptoms: Urinary retention, pain, increase in retention levels
– Diagnosis: Sonography, puncture
– Therapy: Laparoscopic or open fenestration after intraperitoneal

10.6 Postoperative Treatment

Key Points
– After kidney transplantation: monitoring of the patient, if possible
intermediate care unit
– After approx. 5 days: start of intensive training of the patients (focus =
regular intake of medication)

10.6.1 Inpatient Stay

– Length of stay: approx. 14–21 days

Content
– Monitoring of urine production
– Balanced electrolyte and water balance
– Early mobilisation
– Adjusting immunosuppression
– Prophylaxis: Ulcer (pantoprazole), Candida infections (fluconazole),
Pneumocystis jirovecii (cotrimoxazole), CMV (valganciclovir; ◘ Table
10.3)
Table 10.3 Valganciclovir prophylaxis

Risk constellation Duration of therapy

High risk: Donor +/Recipient − 6 months
Medium risk: Donor +/Recipient + 3 months or biweekly CMV PCR
Moderate risk: Donor −/Recipient + 3 months or biweekly CMV PCR
Low risk: Donor −/Recipient − No prophylaxis
For induction therapy:
Antithymocyte globulin 6 months
AB0-incompatible transplantation 3 months
Basiliximab 3 months

Delayed Graft Function (DGF)/Acute Tubular Necrosis (ATN)

– Ischemia reperfusion injury
– Incidence: Up to 30% in postmortem renal transplantation, incidence
increases with increase of cold ischemia time
– No therapy possible, ensure sufficient fluid intake, adequate
immunosuppression
– Problem: Differential diagnosis of other causes of graft failure:
– Acute rejection: biopsy
– Circulatory disorder: Color-coded duplex examination

Caution
A clinical assessment is not possible in DGF/ATN due to lack of
excretion. Therefore, in this situation, a kidney biopsy is always
indicated after 7 days.

10.6.2 Immunosuppression
Key Points
– Highest immunological risk = at the time of transplantation
– Risk decreases exponentially over time

Immunosuppressive Therapy
– Induction phase (transplantation and early postoperative phase)
– Triple therapy (see below) in higher dosage
– If necessary, (in case of high immunological risk) additional induction
therapy with: Monoclonal (non-depleting) antibodies (e.g.
basiliximab) or polyclonal (depleting) antibodies (e.g. antithymocyte
globulin)
– Maintenance phase (starting approx. 6 months after transplantation)
– Triple therapy in low dosage

Standard Triple Therapy

– Calcineurin inhibitor (Tacrolimus)
– Antimetabolite (mycophenolate mofetil, azathioprine)
– Glucocorticoids (prednisolone)

Immunological Risk Factors

– Long cold ischemia time
– Low histocompatibility (≥3 HLA mismatches)
– Donor organ (donor >40 years)
– Recipients (<50 years)
– Retransplant
– Immunized recipient (PRA level elevated)

Immunosuppressive Agents
– Mechanism of action, side effects and application ◘ Table 10.4
– Dosing according to phase ◘ Table 10.5
Table 10.4 Functionality, side effects and use of immunosuppressants

Active Active Mechanism of Side effects Application

ingredient substance action
group
Glucocorticoids Prednisolone Inhibition of the Cushing’s habitus, Maintenance
entire immune hypertension, therapy,
response (non- hyperlipidemia, rejection
specific) osteoporosis, leukocytosis, therapy
cataract, psychosis,
pancreatitis, gastrointestinal
bleeding, gastric/duodenal
Active Active Mechanism of Side effects Application
ingredient substance action
group
ulcers, skin atrophy,
diabetes, impaired wound
healing
Calcineurin Ciclosporin A Inhibits Hypertension, Maintenance
inhibitors calcineurin by nephrotoxicity, hirsutism, therapy
binding to gingival hyperplasia, CNS
immunophilin toxicity
Tacrolimus Inhibits Nephrotoxicity, CNS
calcineurin by toxicity, diabetes,
binding FK- hypertension
binding protein
Antimetabolite Mycophenolic Blocks ionosine Gastrointestinal side effects, Maintenance
acid mono-phosphate leukopenia, anaemia, wound therapy
dehydrogenase healing disorders
Azathioprine Interferes with Pancytopenia, alopecia,
lymphocyte cholestatic hepatosis,
proliferation pancreatitis
m-TOR Sirolimus, Blocks T-cell Hyperlipidemia, Maintenance
inhibitors Everolimus activation thrombocytopenia, therapy
pneumonia, rash, wound
healing disorder
Monoclonal Basiliximab, IL-2 receptor Nausea, drowsiness Induction
antibodies Dacli blockade Therapy
Rituximab CD-20 receptor Nausea, edema, skin rash, AB0-
blockade leukopenia, incompatible
thrombocytopenia transplantation
Polyclonal Antithymocyte Lymphocyte Allergic reaction, Induction
antibodies globulin depletion leukopenia, anaemia, therapy,
(unspecified) opportunistic infections, rejection
increased risk of malignancy therapy

Table 10.5 Phase-appropriate dosage of immunosuppressants

Active ingredient Dose Target mirror

group
Glucocorticoids – Initially 3 mg/kg BW/intraoperatively then tapered to
0.1 mg/kg BW/day (approx. 3–6 months)
– Rejection therapy: 500 mg Boli for 3 days
Tacrolimus 0.1–0.2 mg/kg BW/day 8–12 ng/mL (6–8
weeks)
Active ingredient Dose Target mirror
group
6–8 ng/mL (after 6–
8 weeks)
4–6 ng/mL (after 3
months)
Ciclosporin A 3–6 mg/kg BW/day 150–250 ng/mL (6–
8 weeks)
100–150 ng/mL
(after 6–8 weeks)
50–100 ng/mL
(after 3 months)
Mycophenolate 2 × 1 g daily
mofetil 2 × 750 mg daily after 3 months
2 × 500 mg after 6 months
Azathioprine Initial 2–3 mg/kg BW/day
Long-term 1–2 mg/kg BW/day
Basiliximab 2 × 20 mg (preoperative and day 4)
Rituximab AB0i:
375 mg/m2 BSA 4 weeks before planned
transplantation
Antithymocyte 1.5 mg/kg BW/day
globulin Cumulative dose max. 6–10 mg/kg BW

BW body weight, BSA body surface

Caution
In all immunosuppressed patients:
– Significantly increased risk of infection
– Therefore, early anti-infective therapy is obligatory.
– Opportunistic infections must be included in the differential
diagnosis
Individual immunosuppression of each patient weighing the
immunological risk versus the risk of infection.

ABO-Incompatible Transplantation (Living Donation)

– Special preparation of the recipient
– In Europe: 375 mg/m2 BSA (body surface area) rituximab (Mabthera®) 4
weeks before planned transplantation
– 1 week before transplantation start immunoadsorption/plasmapheresis:
removal of circulating blood group antibodies against donor blood group
– IgG titre in target range (IgG <4), then transplantation
– Triple therapy: Start 1 week preoperative
– Induction with basiliximab
– 1 week postoperative daily: Titre control and immunadsorption in case
of rising titres (in our lab IgG >8)
– 2 weeks postoperative every 2 days: Titre control and
immunadsorption in case of rising titres (in our lab IgG >16)
– Accommodation (exact mechanism unclear): From >2 weeks
postoperative: No more titre controls
– Results regarding long-term function of the grafts identical to AB0-
compatible living donation
– Higher risk of developing lymphoceles: Preoperative mycophenolate
mofetil administration or immunoadsorption as a cause are discussed
AB0-incompatible living kidney donation for blood group
incompatibility = safe standard therapy.

10.6.3 Organ Rejection

Hyperacute Rejection
– Extremely rare since the introduction of the cross-match
– Aetiology: Circulating antibodies against the donor organ
– Minutes after reperfusion of the graft
– Cross-match: Compatibility testing of recipient serum with donor blood,
spleen or lymph node cells

Acute Rejection
– Rejection within days to months after transplantation: In approx. 10% of
all kidney transplants
– Clinical presentation: creatinine increase >20%, decrease in excretion,
painful, swollen graft
– Color-coded duplex sonography: increase in intrarenal resistance index
– Gold standard: kidney biopsy (Banff classification; ◘ Table 10.6)
 – Therapy:
– T-cell mediated rejection: steroid boli
– Vascular rejection: antithymocyte globulin
– Humoral rejection: antithymocyte globulin, plasmapheresis
– For all forms, increase the dose of maintenance immunosuppression
Table 10.6 Banff classification of acute and chronic renal rejection

Grade Definition
Acute kidney rejection
Borderline Focal mild tubulitis (1–2 mononuclear cells per cross-section) without intimal
damage arteritis
IA Significant interstitial infiltration (>25% of parenchyma affected) and focal,
moderate tubulitis (>4 mononuclear cells per tubular cross-section or 10 tubular
cells)
IB Significant interstitial infiltration (>25% of parenchyma affected) and focal, severe
tubulitis (>10 mononuclear cells per tubular cross-section)
IIA Significant interstitial infiltration with mild to moderate intimal arteritis
IIB Significant interstitial infiltration with moderate intimal arteritis (>25% of vessel
lumen)
III Transmural arteritis or fibrinoid changes and necrosis of the smooth muscle cells of
the media
Chronic kidney rejection
I Signs of minor chronic ischemia with mild graft glomerulopathy, minor interstitial
fibrosis, and tubular atrophy (<25% of cortical surface area)
II Signs of moderate chronic ischemia with moderate graft glomerulopathy, moderate
interstitial fibrosis and tubular atrophy (26–50% of cortical surface area)
III Signs of severe chronic ischemia with severe graft glomerulopathy, extensive
interstitial fibrosis, and tubular atrophy (>50% of cortical surface area)

Chronic Rejection
– Rejection within months to years after transplantation
– Pathomechanism:
– Formation of donor-specific antibodies (de novo DSA), connection
with poor adherence (immunosuppressants) is currently being
discussed
– Recurrent subclinical rejections
 – Presence of memory cells (B lymphocytes) for the formation of donor-
specific antibodies, not detected by cross-match and HLA typing (e.g.
in the case of living donation from child’s father to mother)
– Clinical presentation: Slow, continuous deterioration of renal function.
– Therapy:
– Increasing immunosuppression
– For donor-specific antibodies mostly frustrating

10.6.4 Infections
– Increased risk due to immunosuppression

Bacterial Infections
– Mostly urinary tract infections or pulmonary infections
– Atypical germs must be included in differential diagnosis
– Early resistance-appropriate antibiotic therapy

Opportunistic Infections
– Mostly in the first year after transplantation (higher immunosuppression)
– Mostly viral infections (CMV, BKV)
– Common pathogens:
– CMV
– Aspergillosis
– Candida
– Clostridium difficile

10.6.5 Aftercare
– After discharge from hospital:
– Initially 2–3 times weekly in cooperation with a nephrologic centre
– In the course increase of the interval
– One visit per year to the transplant centre
– Main focus: creatinine progression, immunosuppression

10.7 Results
– Survival rates ◘ Table 10.7
Table 10.7 1- and 5-year survival rates of the transplant and the recipient comparing post-mortem
organ donation vs. living donation

Graft Recipient
1 year 5 years 1 year 5 years
Post-mortem donation 91% 69% 96% 84%
Living donation 95% 80% 98% 91%

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_11

11. Spleen
Therezia Bokor-Billmann1 and Franck Billmann2
(1) Department of Dermatology, University Hospital Heidelberg,
Heidelberg, Germany
(2) Department of General, Visceral and Transplant Surgery, University
Hospital Heidelberg, Heidelberg, Germany

Therezia Bokor-Billmann (Corresponding author)

Email: [email protected]

Franck Billmann
Email: [email protected]

11.1 Spleen: Generalities

Key Points
– Coffee bean shaped organ; volume = 160 mL
– Intraperitoneal position, left hypochondriacal region
– Vascularization through splenic artery and vein (variations)
– Organ of filtering/degradation of erythrocytes and thrombocytes +
Organ of lymphatic defence

11.1.1 Embryology and Developmental Disorders

Embryology
– Development in the dorsal mesogastrium
– Colonization of the spleen by vascular plexus
– Colonization by reticulum cells + lymphocytes (formation of splenic
pulp)

Developmental Disabilities
– Aplasia (= agenesis = asplenia)
– Complete absence of the spleen
– Cause = Absence of the vasa splenica (developmental disorder 2nd–
5th embryonic week)
– Often associated with cardiac malformation/situs inversus
– Congenital hypoplasia
– Primary growth inhibition of the spleen
– Very rarely cardiac malformation/situs inversus
Splenic hypoplasia: Usually = secondary (atrophy)

11.1.2 Anatomy
Structure
Definition (◘ Fig. 11.1)
– Upper splenic pole = Extremitas superior
– Lower splenic pole = Extremitas inferior
– Posterior margin = Margo posterior
– Anterior margin = Margo anterior
– Surfaces:
– Diaphragmatic surface (Facies diaphragmatica): relationship to the
diaphragm
– Visceral surface (Facies visceralis): divided into two facets: Facies
gastrica (for the stomach) + Facies renalis (for the left kidney);
between the two: splenic hilus.
Fig. 11.1 View of the spleen from ventromedial. (From von Lanz and Wachsmuth 2004)

Form
– Similar to a coffee bean
– Variations: possible
– Parenchymal depression: at the anterior border of the spleen
Variations
– Double spleen: two separate, equally sized and adjacent partial spleens
(rare)
– Multiple spleens: up to 10 spleens of unequal size; supplied by splenic
artery
– Adjacent spleen: common (6–10% of population); very small roundish
spleen adjacent to a regular spleen: CT hypervascular; DD: primitive
neuroectodermal tumor

Measurements
– Depending on the blood supply of the organ/eventual pathology
– Expansion capacity of the spleen = 2–3 times the basal volume
– Decrease in height/weight from age 40 onwards
Measurements for Adults
– Length = 12.2 cm
– Width = 7.8 cm
– Thickness = 2.9 cm
– Weight (median):
– Men = 162 g
– Women = 155 g
– Volume (median) = 160 cm3

Location
– Intraperitoneal, left hypochondriacal region
– Spleen longitudinal axis = course of 10th rib
– Position dependent on movements of the diaphragm (breathing)
– Between lower margin 8th rib and upper margin 12th rib
– Held in position by peritoneal ligaments = phrenicosplenic ligament +
splenorenal ligament + gastrosplenic ligament

Vessels and Innervation

Vessels
– Splenic artery (A. splenica) = main vessel; one of the 3 branch vessels of
the truncus coeliacus
– Polar arteries = variations:
– In number: 1–5 splenic polar arteries
– In origin: mostly from splenic artery; from left gastroepiploic (A.
gastroomentalis sinistra), aorta, left gastric artery (A. gastrica sinistra),
inferior pancreatic artery (A. pancreatica inferior), superior mesenteric
artery (A. mesenterica superior)
– Splenic vein (V. splenica) to portal vein (V. portae hepatis)
– Blood circulation = 100–300 mL/min
Innervation
– Almost exclusively sympathetic postganglionic rr. splenici
– Few parasympathetic cholinergic fibers
– Spleen pain: Visceral pain, radiating from the front into the back

11.1.3 Physiology
Filter Function
Filter/Degradation Organ of the Altered Erythrocytes
– Filtering of pathologically altered red blood cells
– Storage of hemoglobin (destruction of the altered erythrocytes)
Storage/Degradation Organ of Platelets (and Clotting Products)
– Storage: up to 30% of circulating platelets
– Filtering of coagulation products in serum (parallel)

Lymphatic Defense Organ

– Formation of new B-lymphocytes
– Presentation of antigens to lymphocytes
– Spleen = most important organ of lymphocyte recirculation

11.2 Spleen Diseases

Key Points
– Benign diseases: Especially of hematological origin
– Malignancies: Lymphomas vs. leukemias vs. metastases
– Splenic trauma: Mostly blunt abdominal trauma; conservative vs.
surgical approach.
– Post-splenectomy morbidity: vaccination (possible pre- or
postoperatively)

11.2.1 Benign Haematological Diseases

Idiopathic Thrombocytopenic Purpura (ITP)
Definition
– Thrombocytopenia: platelet count <150,000/mm3
– Normal bone marrow function
– Exclusion of another cause of thrombocytopenia
Mechanism
– Increased platelet destruction
– By autoantibodies against platelet membrane antigens
– Phagocytosis of platelets in the reticuloendothelial system (spleen)
Epidemiology
– Young women > Men
– 72% patients >10 years old
– 70% young women >40 years old
Clinical Examination
– Purpura
– Epistaxis
– Bleeding gums
– Less common: GI (gastrointestinal) bleeding, hematuria, cerebral
hemorrhage.
Diagnosis
– Laboratory diagnosis: thrombocytopenia
– Exclusion of other causes of thrombocytopenia (◘ Table 11.1)
– Diagnosis follows this exclusion strategy
Table 11.1 Differential diagnoses of immune thrombocytopenic purpura

False-low platelets – EDTA-induced in vitro platelet clumping or cold agglutinins

– Giant platelets
Common causes of – Pregnancy (pregnancy thrombocytopenia, preeclampsia)
thrombocytopenia – Drug/drug-induced thrombocytopenia (heparin, quinidine,
quinine, sulfonamides)
– Viral infections (HIV, infectious mononucleosis, etc.)
– Hypersplenism (chronic liver failure)
Other causes of – Myelodysplasia
thrombocytopenia – Congenital thrombocytopenias
– Thrombotic thrombocytopenic—purpura/hemolytic uremic
syndrome
– Chronic disseminated intravascular coagulation
Secondary thrombocytopenias – Autoimmune diseases (e.g. lupus)
 – Lymphoproliferative diseases (CLL, non-Hodgkin lymphoma)

EDTA ethylenediaminetetraacetic acid, HIV human immunodeficiency

virus, CLL chronic lymphocytic leukemia
Therapy
– Depending on the severity of thrombocytopenia
Strategy
– Platelets >50,000/mm3 + asymptomatic: monitoring
– 30,000 > Platelets >50,000/mm3 + asymptomatic
– Monitoring without therapy
– Glucocorticoids: prednisone (1 mg/kg BW/day)
– Platelets >20,000/mm3 + few symptoms: Glucocorticoids (see above)
– Platelets <20,000/mm3 + bleeding: Inpatient treatment
– Platelet transfusion only if there is serious bleeding.
– I. v. immunoglobulins in case of bleeding and preoperatively (1 g/kg
BW/day for 2 days)
Splenectomy
– Indications:
– Refractory severe ITP
– Patients requiring very high or toxic doses of glucocorticoids
– Thrombocytopenia recurrence
– Incomplete response after drug therapy
– Pregnant women after drug therapy with a risk of bleeding
– Modality:
– Open splenectomy
– Laparoscopic splenectomy (see below)
– Result: Immediate response (within 10 days postoperatively) = 71–95%

Surgical Procedure
Laparoscopic Splenectomy
 – Preoperative skin marking, pressure-free right lateral positioning,
slight angulation of the operation table to increase the distance
between the costal arch and the spina iliaca
– Inferior dissection of the spleen, possible partial mobilization of the
left colonic flexure
– Transection of the splenogastric ligament and the gastricae-breves
vessels (ligasure)
– Visualization of the splenic hilus and vessels
– Minimal skeletonization of the splenic artery and clipping by means
of laparoscopic clips/vascular clips + transection
– Initial clipping of the artery: reduction of the spleen volume
– Dissection of the lateral and retroperitoneal splenic ligaments
– Identification of the pancreas tail and mobilization of the pancreas
without injury
– Visualization of the splenic vein, skeletonization, clipping by means
of laparoscopic clips/vascular clips and transection
– Transection the splenodiaphragmatic ligament
– Extraction of the spleen using of a laparoscopic extraction bag
– Irrigation and closure, drainage if necessary

Hereditary Spherocytosis
Definition
– Hereditary autosomal dominant disease
– Genetic defect: Alteration of the proteins of the ankyrin complex (=
erythrocyte cytoskeleton protein) (e.g. ankyrin, α- and β-spectrin protein)
Mechanism
– Ankyrin/spectrin alteration/deficit = erythrocyte cell membrane deficit =
erythrocytes smaller, rounder, non-deformable
– Consequence = increased osmotic fragility
– In the spleen: spherocytes increasingly trapped = erythrocytopenia (=
haemolysis)
Clinical Examination
– Variable: asymptomatic carrier to severe hemolysis
– Anemia
– Possibly jaundice
– Splenomegaly
Diagnosis
– Spherocytes
– Increased reticulocyte count
– Increased osmotic fragility of erythrocytes
– Negative Coombs test
Therapy
– Splenectomy
– Mechanism: Reduction of hemolysis by abolishing destruction in the
spleen; no influence on spherocytosis

Hemolytic Anemia Due to Erythrocyte Enzyme Defect

Definition
– Two enzymatic defects:
– Glucose-6-phosphate dehydrogenase (G6PD): X-linked inheritance
– Pyruvate kinase: autosomal inheritance
– Mechanism: defect = abnormal glucose metabolism = abnormal
erythrocyte deformability = hemolysis in the spleen
Clinical Examination
– Glucose-6-phosphate dehydrogenase deficit: anemia following drug,
medication, or chemical exposure
– Pyruvate kinase deficit: anemia + splenomegaly
Therapy
– Glucose-6-phosphate dehydrogenase deficit: splenectomy rarely
indicated
– Pyruvate kinase deficit: splenectomy

Haemoglobinopathies
Definition
– Two entities:
– Sickle cell anemia: Autosomal recessive disease
– Thalassemia: Autosomal dominant disease
– Mechanism:
– Sickle cell anaemia: deformation of erythrocytes in haemoglobin S
homozygous patients = destruction of erythrocytes in spleen =
anaemia; in heterozygous patients deformation in certain reduced
PaO2-situations
– Thalassemia: defect in hemoglobin synthesis = hemolytic anemia
Clinical Examination
– Sickle cell anaemia: haemolytic anaemia + thrombosis =
microinfarctions (especially in the spleen) + hypersplenism
– Thalassemia: Hemolytic anemia + splenomegaly + splenomegaly +
hypersplenism
Hypersplenism + splenic sequestration
– For sickle cell disease and thalassaemia
– Consequence: Acute splenomegaly = severe pain + need for transfusion
Therapy
– Medical/conservative therapy: transfusions, painkillers, etc.
– Splenectomy:
– Indication: recurrent acute sequestration crises; recurrent blood
transfusions; massive splenomegaly; splenic abscesses
– Open vs. laparoscopic splenectomy

11.2.2 Other Benign Diseases

Splenic Cysts
Classification
– True cysts (parasitic/non-parasitic)
– Pseudocysts: Mostly secondary after trauma
– Cystic-impressing tumors: cystic lymphangiomas, cavernous
hemangiomas
Clinical Examination
– Mostly asymptomatic
– Symptoms: vague pain in the left upper abdomen, left back or shoulder
pain, pleuritic chest pain, shortness of breath
– Acute symptoms due to hemorrhage of the cyst; usually due to increase
in size of the cyst
Epidemiology
– True cysts: mostly parasitic (Ecchinococcus species)
– Non-parasitic cysts: 70–80% = pseudocysts
Therapy
– Suspicion of parasitic cysts: Preoperative serological confirmation
– Parasitic cysts/pseudocysts: splenectomy
– Non-parasitic cysts: partial splenectomy
– Pseudocysts: If <4 cm: Monitoring

Splenic Abscess
– Rare, potentially life-threatening disease
– Incidence = 0.7%
– Mortality:
– 15–20% in previously healthy patients
– Up to 80% in immunocompromised patients
Risk Factors
– Malignant diseases
– polycythemia vera
– Endocarditis
– Haemoglobinopathies
– I. v. drug abuse
– AIDS (“acquired immunodeficiency syndrome”)
– Urinary tract infections
Clinical Examination
– Often non-specific:
– Abdominal pain in the left upper quadrant
– Fever
– Peritonitis signs
– Pleuritic chest pain
Diagnosis
– CT abdomen with contrast medium
Therapy
– Unilocular abscesses: CT-guided drainage (success rate = 75–90%) +
antibiotics.
– Multilocular abscesses: Splenectomy + peritoneal drainage + antibiotics

11.2.3 Malignant Diseases

Lymphomas
– Hodgkin lymphoma: surgical therapy = staging laparoscopy
– Non-Hodgkin lymphoma: splenectomy in patients with isolated splenic
involvement

Leukemia
– Hairy-cell leukemia: splenectomy in patients with splenomegaly.
– Chronic lymphocytic leukemia: splenectomy = palliative measure for
symptomatic splenomegaly
– Chronic myelocytic leukemia: splenectomy = palliative measure in
symptomatic splenomegaly or hypersplenism

Non-hematological Malignant Tumors

– Metastases:
– Mostly from cancers of the breast, lung or melanoma
– Mostly asymptomatic
– Symptoms if: Splenomegaly/spleen rupture
– Splenectomy = effective palliative treatment
– Vascular tumors:
– Benign: Hemangiomas
– Malignant: angiosarcomas, hemangiosarcomas; highly aggressive
tumors = splenectomy (diagnostic, therapeutic, palliative)

11.2.4 Spleen Trauma

– See polytrauma; blunt abdominal trauma

Epidemiology
– Spleen trauma = most frequent indication for laparotomy after blunt
abdominal trauma
– Mechanisms:
– Car/truck accident most frequent
– Other: Falls; pedestrian vs. car/motorcycle; bicycle accident; sports
accidents.

Pathophysiology
– Injury by:
– Rapid deceleration (with avulsion of the splenic ligaments) = capsular
avulsion
– Compression
– Energy transfer through the posterolateral thoracic wall
– Impaling through a (fractured) rib
– Spleen perfusion = 5% of cardiac output = minimal splenic trauma =
severe bleeding

Diagnosis
Anamnesis
– Mostly orienting/typical
– Important = accident mechanism
Clinical Examination
– Peritoneal irritation (pain, defense)
– External signs of direct force (deformity, haematoma, etc.)
– Hemodynamic status (hypotension, tachycardia)
Ultrasound Examination
– FAST (Focussed Assessment with Sonography in Trauma)
– Non-invasive, quickly available, cost-effective
– Quickly leads to the diagnosis and treatment strategy
Diagnostic peritoneal lavage
– Obsolete
– Has been replaced by FAST and CT
– CT abdomen:
– If patient is hemodynamically stable
– Excellent morphological representation of the injury
– Classification according to American Association for the Surgery of
Trauma Splenic Injury Scale (◘ Table 11.2)
Table 11.2 American Association for the Surgery of Trauma Spleen Injury Scale (2018 revision)

Gradea AISb Imaging criteria (CT) Surgical criteria Pathological criteria

severity
I 2 Subcapsular hematoma Subcapsular hematoma Subcapsular hematoma
<10% of the surface <10% of the surface <10% of surface
Parenchyma lazeration <1 Parenchyma lazeration <1 Parenchyma lazeration
cm depth cm depth <1 cm depth
Capsule injury Capsule injury Capsule injury
II 2 Subcapsular hematoma Subcapsular hematoma Subcapsular hematoma
10–50% of the surface; 10–50% of the surface; 10–50% of the surface;
intraparenchymal intraparenchymal intraparenchymal
hematoma <5 cm hematoma <5 cm hematoma <5 cm
Parenchymal laceration Parenchyma laceration 1– Parenchymal laceration
1–3 cm 3 cm 1–3 cm
III 3 Subcapsular hematoma Subcapsular hematoma Subcapsular hematoma
>50% of the surface; >50% of the surface or >50% of the surface;
ruptured subcapsular or progressive in size; ruptured subcapsular or
intraparenchymal ruptured subcapsular or intraparenchymal
hematoma ≥5 cm intraparenchymal hematoma ≥5 cm
hematoma ≥5 cm
Parenchymal laceration Parenchymal laceration Parenchymal laceration
>3 cm in depth >3 cm in depth >3 cm in depth
Gradea AISb Imaging criteria (CT) Surgical criteria Pathological criteria
severity
IV 4 Any injury with lesion of Parenchymal laceration Parenchymal laceration
the splenic vessels or with involvement of with involvement of
active intracapsular segmental or hilar splenic segmental or hilar
splenic hemorrhage vessels with >25% splenic vessels with
devascularization >25% devascularization
Parenchymal laceration
with involvement of
segmental or hilar splenic
vessels with >25%
devascularization
V 5 Any injury with lesion of Hilar vascular injury with Hilar vascular injury
the splenic vessels and splenic devascularization with splenic
active bleeding into the devascularization
peritoneal space; injury of
spleen-adjacent structures
Destroyed spleen Destroyed spleen Destroyed spleen

Vascular injury defined by pseudoaneurysm or arteriovenous fistula;

presents as focal collection of contrast from a vessel with decrease in
intensity on delayed imaging. Active bleeding from a vascular lesion
defined as contrast leakage from a vessel, focal or diffuse, with increase in
size or decrease in intensity on delayed imaging. Vascular thrombosis can
lead to infarction of the organ
a
Grade classification based on highest classification by imaging, surgery or
pathology. For multiple injury: Grade + 1 to Grade III
b
AIS abbreviated injury scale

Therapy
– Medical/conservative vs. surgical therapy
– Surgical therapy: spleen-preserving surgery vs. splenectomy
– Indication for surgical exploration:
– Hemodynamic instability
– Progressive intra-abdominal blood loss (in ultrasound, CT)
– Large hemoperitoneum
– Relative: Pseudoaneurysm in traumatized splenic area

11.2.5 Post-splenectomy Morbidity

Asplenia: Pathophysiology
– Thromboembolic consequences:
– Due to thrombocytosis (= increase in platelet concentration in the
blood)
– Increased risk of deep vein thrombosis + pulmonary embolism
– Lifetime pulmonary embolism risk = 35.6% vs. 9.7% (in control
group)
– Immunological consequences:
– Due to the absence of splenic function
– OPSI (Overwhelming Post-Splenectomy Infection)
– Rare, but high mortality = 50–70%
– Often prodromes (malaise, myalgias, vomiting)
– Pneumonia
– Rapid progression of the disease
– Multi-organ failure: hypotension → disseminated intravascular
coagulation → respiratory failure → coma → death within hours of
onset.
– Pathogen: Mostly S. pneumoniae; other pathogens: Haemophilus
influenzae, Neisseria meningitis, Salmonella species

Prophylactic Therapy in Asplenia Patients

– Vaccination (◘ Table 11.3):
– Three to four weeks preoperatively before elective splenectomy
– Three weeks postoperatively after non-elective splenectomy
– Pneumococcal, Haemophilus influenzae type b, meningococcal and
influenza vaccinations
– Connection to asplenia outpatient clinic
– Antibiotics: Currently no data on antibiotic prophylaxis in
splenectomized patients; however, recommended in children <5, 2 years
after splenectomy in children of all ages, and adults after
postsplenectomy sepsis.
– From 1 million platelets/mm3: prophylactic ASS (acetylsalicylic acid)
administration (thrombosis prophylaxis)
Table 11.3 Recommended vaccinations for asplenia patients

Vaccinationa Before elective splenectomyb After splenectomyc

Pneumococcal – Sequential vaccination with conjugate – If vaccination has not yet taken
vaccination vaccine (PCV13 Prevenar 13®) followed place, sequential PCV13 and
PPSV23 8 weeks later
by PPSV23 (Pneumovax 23®) 8 weeks
later (children <24 months should be – If vaccination with PCV13 has
vaccinated with PCV13 only) already taken place, then only
PPSV23 at the earliest 2 weeks
– Repeat vaccination (PPSV23) and after splenectomy (if follow-up
booster every 6 years uncertain PPSV23 before
discharge)
– Repeat vaccination (PPSV23)
and booster every 6 years
Haemophilus – Single vaccination with single vaccine Id. as before elective splenectomy
influenzae type b Act-Hib® or Hiberix®
vaccination
– No repetition/refresher
Meningococcal – Vaccination against meningococci of Two doses of MenACWY with an
vaccinations serogroups ACWY interval of 8–12 weeks between
– Children >2 months and adults: single doses
dose of a 4-valent conjugate vaccine
MenACWY (Menveo®, Nimenrix®)
– Currently no refresher recommended
– Vaccination against meningococci of Id. as before elective splenectomy
serogroup B
– Since 2015 STIKO recommendation for
vaccination using Bexsero® or
Trumenba® (risk assessment by the
treating physician)
– Currently no refresher recommended
Influenza – Annual influenza vaccination in autumn Id. as before elective splenectomy
vaccination – Children and adolescents up to 17 years
of age may be vaccinated with inactivated
vaccine or live nasal attenuated vaccine
(LAIV)

a
Recommendations of the Robert Koch Institute “Vaccinations in Asplenia”
b
If possible, the vaccinations should have taken place at least 2 weeks
before the operation
c
After splenectomy, vaccination can be given as soon as the patient is in a
stable general condition
References
von Lanz T, Wachsmuth W (2004) Praktische anatomie. Hals. Springer, Berlin
[Crossref]

Further Reading
Beauchamp RD, Holzman MD, Fabian TC, Weinberg JA (2008) The spleen. In: Townsend CM Jr,
Beauchamp RD, Evers BM, Mattox KL (eds) Sabiston textbook of surgery. The biological basis of
modern surgical practice, 18th edn. Saunders, Elsevier, Philadelphia

Bhangu A, Nepogodiev D, Lal N, Bowley DM (2012) Meta-analysis of predictive factors and

outcomes for failure of non-operative management of blunt splenic trauma. Injury 43:1337–1346
[Crossref][PubMed]

Cooper N, Ghanima W (2019) Immune thrombocytopenia. N Engl J Med 381:945–955

[Crossref][PubMed]

Feig BW, Ching CD (2019) The MD Anderson surgical oncology handbook, 6th edn. Wolters
Kluwer, Philadelphia

Germer CT, Keck T, Grundmann RT (2017) Evidenzbasierte Viszeralchirurgie benigner

Erkrankungen. Springer, Berlin
[Crossref]

Iolascon A, Andolfo I, Russo R (2019) Advances in understanding the pathogenesis of red cell
membrane disorders. Br J Haematol 187:13–24
[Crossref][PubMed]

Keck T, Germer CT (2017) Minimal invasive Viszeralchirurgie. Springer, Berlin

[Crossref]

Leonhardt H, Tillmann B, Töndury G, Zilles K (2003) Lehrbuch und Atlas der Anatomie des
Menschen, Innere Organe, vol 2, 3rd edn. Thieme, Stuttgart

Piel FB, Steinberg MH, Rees DC (2017) Sickle cell disease. N Engl J Med 376:1561–1573
[Crossref][PubMed]

Piel FB, Weatherall DJ (2014) The α-thalassemias. N Engl J Med 371:1908–1916

[Crossref][PubMed]

Rauber A, Kopsch F (1948) Lehrbuch und Atlas der Anatomie des Menschen, Eingeweide, vol 2,
17th edn. Thieme, Leipzig

Robert Koch Institut (2019) Impfungen bei Asplenie. https://​www.​rki.​de/​sharedDocs/​FAQ/​Impfen/​

AllgFr_​Grunderkrankunge​n/​FAQ01.​html. Accessed on 7.6.2021

Rubin LG, Schaffner W (2014) Clinical practice. Care of the apslenic patient. N Engl J Med
371:349–356
[Crossref][PubMed]
Standring S (2009) Gray’s anatomy. the anatomical basis of clinical practice, 40th edn. Churchill
Livingstone, Elsevier, London

The American Association for Surgery of Trauma (2018) Injury scoring scale. A resource for trauma
care professionals. http://​www.​aast.​org/​library/​traumatools/​injuryscoringsca​les.​aspx#spleen.
Accessed on 7.6.2021

Theilacker C, Ludewig K, Serr A, Schimpf J, Held J, Bögelein M, Bahr V, Rusch S, Pohl A,

Kogelmann K, Frieseke S, Bogdanski R, Brunkhorst FM, Kern WV (2016) Overwhelming
postsplenectomy infection: a prospective multicenter cohort study. Clin Infect Dis 62:871–878
[Crossref][PubMed]

von Lanz T, Wachsmuth W (2004) Praktische Anatomie. Bauch. Springer, Berlin

[Crossref]

Zilles K, Tillmann B (2010) Anatomie. Springer, Berlin

[Crossref]

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_12

12. Peritoneum
Jörg Pelz1
(1) Department of General, Visceral and Oncological Surgery, St.
Bernward Hospital, Hildesheim, Germany

Jörg Pelz
Email: [email protected]

12.1 Anatomy and Physiology of the Peritoneum

12.1.1 General Anatomy
Definition
– Peritoneum = serous membrane
– One of the largest organ in terms of area
– Mesothelial covering of the abdominal cavity and the organs therein
(peritoneum viscerale) (excluded: retroperitoneal and extraperitoneal
organs)
– Total area: 1.6 and 2.0 m2
– Blood flow: approx. 100–200 mL/min
– Lymphatic absorption rate
– Approx. 1–2 mL/min
– Drainage via the lymphatic channels to the thoracic duct

Structure (Histology)
– Mesothelium (Tunica serosa) = serous membrane
– Extracellular matrix (lamina propia)
– Connective tissue layer (Tela subserosa)
– Thickness = 90–130 μm

Division
– Visceral peritoneum (Peritoneum viscerale) (80%)
– Covering of the abdominal viscera and organs
– Innervated = pain (visceral pain)
– Parietal peritoneum (Peritoneum parietale) (20%)
– Covering of the inner side of the abdominal wall (somatic pain)
– Peritoneal fluid (physiologically production 50–70 mL/day)

12.1.2 Physiology (Tasks) of the Peritoneum

Organ Mobility
– Mobility (sliding) of the abdominal organs
– Through liquid film on the serosal epithelium

Resorption
– Within minutes; in cranial direction transdiaphragmatic
– Metabolically highly active membrane (semi-permeable)
– Used for peritoneal dialysis or intraperitoneal chemoperfusion/therapy

Immunological Tasks (= Defence)

– Especially for infections of the abdominal cavity
– Inflammatory process (vasodilation, phagocytic clearance)
– Involved in macrophage activation
– Humoral immunity by complement system
– Clearance limitation: Critical limit at >105 germs/mL
– High regeneration capacity

12.2 Benign Diseases of the Peritoneum

Key Points
 – Peritonitis (inflammation of the peritoneum)
– Manifestation up to a severe septic course
– Medical/conservative vs. surgical treatment
– Peritoneal adhesions = most frequent cause of small bowel obstruction
(ileus)
– Surgical therapy for ileus

12.2.1 Peritonitis
Definition
– Inflammation of the peritoneum
– Evolution: Up to a severe septic course

Etiology
Primary Peritonitis (Without Previous Abdominal Disease)
– About 1% of peritonitis
– By hematogenous seeding of bacteria
– Frequent association with liver cirrhosis
Secondary Peritonitis
– Inflammations (cholecystitis, appendicitis, etc.)
– Perforation of an abdominal organ (gastric ulcer, anastomotic
insufficiency, etc.)
– Perforation of the abdomen (e.g. stab wounds)
Spontaneous Bacterial Peritonitis (e.g. in Ascites, Liver Cirrhosis)
Special Form: Peritonitis After CAPD Catheter Insertion
– Frequently!
– CAPD: “continuous ambulatory peritoneal dialysis”.

Classification
– According to aetiology (see above)
– According to localization
– Local peritonitis
 – Diffuse (generalized) peritonitis
– According to clinical course
– Acute peritonitis
– Chronic peritonitis
– Localized peritonitis
– Generalized peritonitis

Symptoms
– Abdominal tension
– Fever
– Increase in inflammatory signs (leukocytosis, CRP, erythrocyte
sedimentation rate ESR, procalcitonin)
– Pain (localized/generalized)
– Fluid shift

Diagnosis
– Anamnesis
– Clinical examination
– Abdominal tenderness and guarding
– Abdominal rigidity
– Pain localization
– Laboratory (leukocytosis, CRP elevation)
– Ultrasound
– Computer tomography CT
– Contrast medium image of the parietal peritoneum
– Search of the source of peritonitis
– Peritoneocentesis as direct pathogen detection

Therapy
Surgical Therapy
– Eradication of the source of infectious (if present)
– Closed peritoneal lavage
– Irrigation of the peritoneal space (through drains placed during the
operation) after appropriate operations
 – For accelerated removal of inflammatory secretion/pus as a
consequence of various surgical diseases of the abdominal cavity
– Caution: Often irrigation limited to area directly adjacent to drains
(not entire peritoneal cavity)
– Staged lavage (= programmed lavage)
– For high-grade inflammatory diseases of the abdominal cavity
– Planned relaparotomy (with irrigation) performed at set intervals
evidence-based worse than on-demand lavage (according to patient’s
clinical condition)

Caution
In pancreatitis usually conservative approach.
Conservative Therapy
– Calculated antibiotic administration
– Adequate fluid management (volume and catecholamine controlled,
hemodynamic monitoring)
– Intensive care unit monitoring for severe peritonitis

12.2.2 Peritoneal Adhesions

Epidemiology
– Mostly postoperative
– Most frequent cause of small bowel obstruction (ileus = approx. 60% of
obstructions)
– More often in the lower abdomen:
– After gynecological surgery
– After appendectomy
– After colorectal resection

Clinical Presentation
– Asymptomatic to full image of ileus (► Sect. 16.​2)
– Usually nonspecific, occasionally crampy abdominal pain

Diagnostic Imaging
– CT: Indirect evidence of adhesions: dilated small bowel loops on the
anterior abdominal wall; caliber change of the small bowel.
– MRI: MRI-sellink if necessary

Therapy
– Asymptomatic adhesions/nonspecific pain:
– Symptomatic therapy preferred
– Adhesiolysis: Only in individual cases, due to high incidence of
recurrence.
– In case of manifest ileus: surgical therapy (► Sect. 16.​2)

12.3 Pseudomyxoma Peritonei

12.3.1 Definition
– Accumulation of mucus masses in the abdominal cavity due to a mucus-
forming tumour. Lymph node (<5%) or distant metastases may rarely
occur.

12.3.2 Classification
– Three groups (1995 classification)
– “Disseminated peritoneal adenomucinosis (DPAM): Rather benign
appearance.
– “peritoneal mucinous carcinomatosis” (PMCA): cause = disseminated
carcinoma cells, malignant appearance
– Intermediate category
Controversy: Benign disease vs malignant disease without infiltrative
growth.

12.3.3 Aetiology
– Mucinous cystadenoma (= mucocele) of the appendix vermiformis
– Second most common tumor of the appendix (after appendiceal
carcinoid)
 – In case of accidental detection: indication for metachronous or
synchronous right hemicolectomy (better outcome)
– In 50% of cases further intra-abdominal manifestation at diagnosis
– Mucinous tumor of the ovary (= mucocele)
– Rare
– Tumour cell spillage of malignant tumours of the abdominal cavity (e.g.
appendix, ovary, colon, uterus)

12.3.4 Clinical Presentation

– Local problems due to displacing growth, subileus

12.3.5 Therapy
– ► Section 12.5: Cytoreductive surgery (CRS) + hyperthermic
intraperitoneal chemotherapy (HIPEC)
– Poor/no effect of systemic chemotherapy
– Absolute indication for HIPEC therapy (highest evidence)
– Right hemicolectomy for R0-resected “low-grade tumours” = not
recommended

12.3.6 Prognosis
– With CRS + HIPEC: 10-year survival rate approx. 70%
– Low-grade tumor with significantly better outcome

12.4 Malignant Diseases of the Peritoneum

Key Points
– Peritoneal carcinomatosis = most frequent secondary, malignant disease
of the peritoneum
– Therapy: Paradigm shift with cytoreductive surgery (CRS) + HIPEC
– CRS + HIPEC:
– Significant survival benefit in selected tumor entities
– Optional treatment strategy in the current S3 guidelines for
colorectal cancer
 – High morbidity between 25% and 60%
– Mortality between 2 and 10
– The impact of chemoperfusion is unclear!

12.4.1 Mesothelioma
Definition
– Primary malignant disease of the peritoneum

Epidemiology
– Most common primary malignant disease of the peritoneum
– Mostly limited to the abdomen
– Median survival = 4–12 months (due to advanced stage at diagnosis)
– history of asbestosis: 50–70% of patients

Clinical Presentation
– Abdominal pain
– Ascites
– Weight loss

Prognosis
– Frequent infiltration of the other intra-abdominal organs (liver, intestine,
bladder, abdominal wall)
– Men with significantly worse outcome
– Median (overall) survival of 33 months
– Better survival in epithelioid subtype and after CC-0 resection

Therapy
– Difficult to treat
– Goal = complete surgical resection
– Concept of CRS + HIPEC

12.4.2 Peritoneal Carcinomatosis

Epidemiology
– Synchronous peritoneal carcinomatosis in 5–10% of all gastrointestinal
tumors
– Another 5–15% = metachronous
– Mean survival time of these patients is severely limited

Clinical Presentation
– History of malignancy
– Abdominal pain
– Ascites
– Weight loss

Diagnosis
– Pretherapeutic staging = essential
Imaging Techniques
– CT, MRI or PET-CT
– Frequent understaging:
– Sensitivity between 50 and 96
– Specificity between 62 and 100
– Radiologic diagnosis of carcinomatosis of the small intestine: in only
50% of cases
Staging Laparoscopy
– Small tumor nodules can be detected earlier
– Biopsy and pathological workup possible
– If necessary, limited tumor removal is possible laparoscopically
– Disadvantage: Second-look operation is usually necessary

Therapy
– Cytoreductive therapy alone not sufficient for cure
– Systemic chemotherapy = currently palliative standard treatment
procedure
– Interdisciplinary treatment concept = central (tumor board)
– Development of new therapeutic strategies (CRS + HIPEC; ► Sect.
12.5)
Systemic Chemotherapy
– Always as an interdisciplinary approach
– Important factor in multimodal treatment
– Results:
– Median survival = 9–12 months (palliative chemotherapy)
– Median survival = up to 20 months (modern combination
chemotherapeutics)
– Median survival = up to 30 months in selected patients
– Pros:
– Systemic effect (influence also on potential distant metastases)
– Fewer complications when compared with surgery
– Disadvantages:
– Still limited efficacy in peritoneal carcinomatosis

12.5 Cytoreductive Surgery (CRS) and HIPEC

– Definition:
– Combination of cytoreductive surgery (CRS) followed by
hyperthermic intraperitoneal chemoperfusion (HIPEC)

12.5.1 Curative CRS and HIPEC

Theoretical Approach
– Cytoreductive surgery: goal = removal of all visible tumor manifestations
– HIPEC: goal = destruction of remaining tumor cells after CRS
– Chemotherapeutic agents can be distributed throughout the abdomen
– Intraoperative application = dose-intensified + timely
adjuvant/additive chemotherapy with cytotoxic concentration (not
possible with systemic administration)
– Schematic representation of a HIPEC perfusion: ◘ Fig. 12.1
Fig. 12.1 Schematic representation of HIPEC according to Pelz

Surgical Procedure
Cytoreductive Surgery
– Median laparotomy from the xyphoid process to the symphysis pubica
– Incision of the abdominal wall without incision of the peritoneum,
detachment of the closed parietal peritoneum
– Opening the peritoneum
– Staging = documentation of the Peritoneal Cancer Index (PCI)
according to Sugarbaker (a total of 13 regions to be assessed) =
prognostic factor and relevant for indication:
– Abdominal cavity divided into 9 regions
– Small intestine into further 4 regions
– Point system depending on tumor size in each region: Absence of
tumor = 0 points (LS0); up to 0.5 cm = 1 point (LS1); up to 5 cm =
2 points (LS2); > 5 cm = 3 points (LS3)
– PCI between 0 and 39 points (◘ Fig. 12.2)
– Removal of all visible tumor manifestations (up to multivisceral
resection); if a stoma is necessary: Only after HIPEC
– Postoperative classification: Completeness of Cytoreduction (CC)
according to Sugarbaker (◘ Fig. 12.3) = prognostic factor
– Therapy goal = CC-0 or CC-1
– Placement of 4 26-CH silicone drains (= preparation HIPEC and
drainage):
– One inflow: At the site of greatest tumor burden
– Three drains: Subphrenic right, subphrenic left, Douglas…
– Two intra-abdominal temperature probes
– HIPEC:
– Following cytoreductive surgery (◘ Fig. 12.1)
– Inflow temperature 41–43 °C
– Perfusion time 30–120 min
– Open or closed perfusion
– Chemotherapeutics
– At present, there is no uniform recommendation on the type and
dose of cytostatic drug to be used

Fig. 12.2 Peritoneal carcinoid index according to Sugarbaker. (Mod. according to Jacquet and
Sugarbaker 1996)
 Fig. 12.3 Completeness of Cytoreduction (CC score) according to Sugarbaker. Assessment of
postoperative tumor burden after cytoreductive surgery. Surgical score CC-0 means that no tumor
was left; CC-1 tumor remnants up to 0.25 cm, CC-2 tumor remnants of 0.25–2.5 cm, CC-3 tumor
remnants larger than 2.5 cm. (Mod. according to Jacquet and Sugarbaker 1996)

Concept and Goals

– Resection of the primary tumor
– Macroscopic complete removal of all accessible tumor metastases
– HIPEC (= hyperthermic intraperitoneal chemoperfusion) = destruction of
remaining tumor cells after CRS
– The value of hyperthermic perfusion is unclear. Preliminary publications
of the PRODIGE-7 study do not show any survival benefits with the use
of oxaliplatin.

Indication
– Limited tumor extension
– Good general condition
– No or <3 resectable distant metastases
– Little evidence regarding the success of the therapy
– Few/old studies
– Difficult comparability
– Problems with patient selection
– Tumor entities: (see overview)

Indications of CRS and HIPEC According to Tumor Entities

– Pseudomyxoma peritonei:
– Absolute indication for HIPEC therapy = Highest level of evidence
 – Ten-year survival rate of 69% after CRS and HIPEC
– Low-grade tumor with significantly better outcome
– Right hemicolectomy not recommended for R0-resected low-grade
tumors
– Colorectal cancer:
– Only one randomized trial (Verwaal et al. 2003) = significant
survival benefit with respect to HIPEC therapy
– Five-year survival = 54%; median survival = 62 months in highly
selected patients (= benefit for patients likely linked with surgery)
– Careful patient selection = absolute prerequisite!
– Ovarian cancer:
– Systemic chemotherapy = currently therapy of choice
– CRS + HIPEC currently under investigation
– Controversial publications: tendency to lack of benefit for CRS +
HIPEC
– Therefore no recommendations in the current S3 guidelines
– Interdisciplinary approach needed (gynaecologist + surgeon)
– Mesothelioma:
– Difficult to treat tumor
– Men with significantly worse outcome
– Median (overall) survival = 33 months
– Better survival in epithelioid subtype and after CC-0 resection
– Stomach:
– Overall poor prognosis
– Systemic therapy = therapy of choice
– Median survival = 15 months
– Perioperative lethality rate of 4.9%
– Contraindication for PCI >10 and/or signet ring cell carcinoma
– Rather HIPEC in synchronous peritoneal carcinomatosis (Gastripec
randomized trial)
– Intra-abdominal sarcoma:
– Very poor prognosis despite multimodal therapy
– Median survival = 26 months
 – Systemic chemotherapy = therapy of choice
– Only in studies
– Tumors of the hepatobiliary system:
– Very poor prognosis
– Systemic chemotherapy = therapy of choice
– Only in studies

Risk-Benefit Analysis
– To be determined individually for each patient
– Interdisciplinary consideration in a tumor board
– No uniform standards for the indication of CRS + HIPEC
– Individual and interdisciplinary assessment of risks: potential benefit
must be higher than risk of complications
– Caution: Delay of palliative systemic chemotherapy

12.5.2 Prophylactic/Adjuvant CRS and HIPEC

– Theoretical approach
– Risk minimization of metachronous peritoneal carcinomatosis
– Use in high-risk patients
– The Prophylochip study failed to show any benefit in these patients

Indications
– High-risk patients
– T3/4 carcinomas with tumor rupture
– Mucinous or signet-ring cell carcinoma
– Isolated omental metastases/Krukenberg tumors

Evidence
– Currently no evidence
– Some evidence for a significant improvement in tumor-free survival
– No increase in complication rate due to chemoperfusion
– Randomized trial ongoing (prophylactic HIPEC)

Learning Curve
– Significant reduction in morbidity and lethality

12.5.3 Palliative Therapeutic Concepts for Ascites

– Therapy of choice for ascites = paracentesis
– Systemic chemotherapy = conditional control of malignant ascites
– HIPEC for ascites control (short-term effect only)

12.5.4 Complications
– With the use of CRS and HIPEC complication rate increases with the
extent of resection
– Complication rate depends on the type and dose of cytostatic drug used

Most Common Complications

– Anastomotic insufficiency (4.5–33%, depending on author and tumor)
– Fistula (3–6%)
– Intra-abdominal abscess (10–17%)
– Bleeding (1.5–12%)
– Ileus (2–17%)
– Wound healing disorders (2–15%)
– Toxicity (20–26%)
– Nephrotoxicity 16–19%
– Haematotoxicity 14–20%

Strategies to Minimize Complications

– Patient selection
– Learning curve
– As few gastrointestinal anastomoses as possible
– Protective stomas should be generously indicated (especially for
anastomoses in the rectal region)
– Monitoring in the intensive care unit
– Close monitoring of laboratory parameters = obligatory (focus on blood
count)
– Postoperative chest X-ray in extended peritonectomy of the diaphragm
– Chemoperfusion always with a certified perfusion device
– Establishment of Centers of expertise
 Learning Curve/Patient Selection
– Learning curve: 100 procedures required to:
– Reduction of complications
– Improvement of CC-0 resection rate from 35.6% to 65.1%
– Reduction in morbidity from 71.2% to 34.1%
– Reduction in the average length of stay in hospital from 21 to 17
days
– Reduction of the reoperation rate from 30% to 10%.
– Planning and training
– Patient selection by:
– Advanced imaging
– Interdisciplinary discussion (tumor board)
– Individualized decision—by means of e.g. Peritoneal Surface
Disease Severity Scores (PSDSS; Pelz et al. 2009)
– Lack of standardization of selection criteria for surgical therapy
(guidelines from the 2006 consensus conference in Milan)

12.5.5 Results
– Significant survival benefit in selected tumor entities
– Optional situation in the current german S3 guidelines for colorectal
cancer (◘ Fig. 12.4)
– High morbidity between 25% and 60%
– Mortality between 2% and 10%
– Quality of life:
– Postoperative: deterioration in the first days to weeks after surgery
depending on the size of the surgical intervention and the peri- and
postoperative complications
– Reaching the preoperative baseline level: After about 3–4 months
– In a proportion of patients: Long-term improvement
Fig. 12.4 Clinical pathway using the example of colon cancer

12.5.6 PIPAC (Pressurized Intraperitoneal Aerosol

Chemotherapy)
– The PIPAC is a palliative therapy
– Possibility of laparoscopic local therapy
– Pure chemotherapy (no cytoreduction)
– Repeatability of the therapy
– Improvement of overall survival in selected patient population
– Still little valid data

12.5.7 Guidelines
AWMF Guideline “Colorectal Carcinoma” ► https://​www.​awmf.​org/​
uploads/​tx_​szleitlinien/​021-007OLk_​S3_​Kolorektales-Karzinom-KRK_​
2019-01.​pdf
AWMF Guidelines “Ovarian Cancer” ► https://​www.​awmf.​org/​
uploads/​tx_​szleitlinien/​032-035OLl_​S3_​Ovarialkarzinom_​2020-04.​pdf

References
Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with
peritoneal carcinomatosis. Cancer Treat Res. 1996;82:359–74. https://​doi.​org/​10.​1007/​978-1-4613-
1247-5_​23. PMID: 8849962.

Pelz JO, Stojadinovic A, Nissan A et al (2009) Evaluation of a peritoneal surface disease severity
score in patients with colon cancer with peritoneal carcinomatosis. J Surg Oncol 99:9–15
[Crossref][PubMed]

Verwaal VJ, van Ruth S, de Bree E, van Sloothen GW, van Tinteren H, Boot H, Zoetmulder FA
(2003) Randomized trial of cytoreduction and hyperthermic intraperitoneal chemotherapy versus
systemic chemotherapy and palliative surgery in patients with peritoneal carcinomatosis of colorectal
cancer. J Clin Oncol 21:3737–3743. https://​doi.​org/​10.​1200/​JCO.​2003.​04.​187
[Crossref][PubMed]

Suggested Reading
Baratti D, Kusamura S, Azmi N, Guaglio M, Montenovo M, Deraco M (2020) Colorectal peritoneal
metastases treated by perioperative systemic chemotherapy and cytoreductive surgery with or
without mitomycin C-based HIPEC: a comparative study using the peritoneal surface disease severity
score (PSDSS). Ann Surg Oncol 27(1):98–106

Ceelen W (2019) HIPEC with oxaliplatin for colorectal peritoneal metastasis: the end of the road?
Eur J Surg Oncol 45(3):400–402. https://​doi.​org/​10.​1016/​j.​ejso.​2018.​10.​542. Epub 2018 Oct 31

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_13

13. Hernia
Jens Otto1 , Thorsten Lindenau1 and Karsten Junge1
(1) Department of General, Visceral and Minimally-invasive Surgery,
Rhein-Maas Hospital, Würselen, Germany

Jens Otto (Corresponding author)

Email: [email protected]

Thorsten Lindenau
Email: [email protected]

Karsten Junge
Email: [email protected]

13.1 Anatomy and Classification

13.1.1 Hernia Anatomy
– Hernia gate
– Gap in the layers of the abdominal wall, pelvic floor, diaphragm or
back muscles.
– Hernia sac
– Lining of the hernia; protrusion of the parietal peritoneum.
– Content of hernia sac
– All components of the abdominal cavity possible.

13.1.2 Epidemiology
Hernia Incidence
– In the normal population = 2–4%.
– In older age = up to 20.
– 10–15% of general surgery procedures.

Hernia Distribution
– Proportion of external hernias = 95.
– Inguinal hernias (75%).
– Scar hernias (10%).
– Umbilical hernias, thigh hernias and rare forms of hernia (5–7% each).
– Proportion of internal hernias = 5.

13.1.3 Pathogenesis and Classification

Pathogenesis
– Preformed or secondary fascia gap.
– Development of the hernia = protrusion of the parietal peritoneum
through the gap.

Hernia Classification
– According to position relative to the abdominal wall (◘ Table 13.1):
– External hernias: Protrusion of the peritoneum through the abdominal
wall “outwards” (e.g. inguinal hernias).
– Internal hernia: Hernia within the abdominal cavity, no external
appearance (e.g. ileocecal hernias).
– According to clinical aspects:
– Reponible/Irreponible: hernial contents to be forced back into
abdominal cavity (or not).
– Incarcerated: Non-reducible, acutely painful protrusion
(incarceration).
– By etiology:
– Congenital hernia (e.g. open processus vaginalis peritonei in indirect
inguinal hernia).
 – Acquired hernia (e.g. incisional hernia).
Table 13.1 Classification of hernias according to position in relation to the abdominal wall

External hernias Internal hernias

Inguinal hernia (inguinal hernia) Treitz hernia
Direct
Indirect
Femoral hernia (thigh hernia) Paracoecal hernia
Umbilical hernia (umbilical hernia) Hernia through foramen omentale (Winslowi)
Paraumbilical hernia Hernia at the mesosigma
Epigastric hernia Hernia at the mesentery (postoperative mesoslit), Petersen
hernia
Incisional hernia (also parastomal Pelvic floor peritoneal hernia
hernia)
Spieghel hernia (linea semilunaris) Paravesical hernia
Rare hernias
Bochdalek
Grynfelt
Trigonum lumbale (petit)

13.1.4 Hernia-Specific Complications

Intestinal Incarceration
– Most common complication.
– Emergency situation
– Clinical presentation:.
– Severe pain, palpable, bulging elastic tumor, local environmental
irritation, irreducibility, vomiting, ileus, intestinal perforation,
peritonitis.
– Caution: The sun must neither rise nor set over a pinched hernia (OP
within max. 12 h).

Hernia
– Danger of spontaneous perforation.
– Clinical presentation: swelling, redness, hyperthermia, painfulness.
Mains Clamping
– Clinical presentation: Pressure painful, non-responsive hernia with little
impairment of the patient.

13.2 General Diagnosis and Therapy Principles

13.2.1 Diagnosis
Physical Examination
– Palpation of the hernial gap.
– Palpate contents of hernia sac if necessary.
– Assessment of reproducibility.
– In the case of irreducible hernias, differentiation between chronic
irreducibility (e.g. in the case of adhesion or excessive extent of the
hernia) vs. acutely occurring irreducibility = incarceration.

Sonography
– Most important tool for assessing fracture aperture, fracture content and
size indication.

Further Investigations
– MRI or CT.
– Only in the case of corresponding symptoms, complex situation
(recurrent findings) or poor assessability (e.g. obesity).

13.2.2 Therapy Principles

– In the case of acute incarceration = manual reduction of the fracture
tumour only within the first few hours.

Caution
Bowel perforation/reposition of gangrenous bowel with delayed
attempt at reduction.

– Successful reduction = inpatient monitoring + surgical repair of the

hernial gap during the same stay.
– Unsuccessful reduction = operation as soon as possible + vitality check
of the content of hernia sac.

Procedure for Surgical Therapy

– Direct fascia suture/fascia doubling: Shouldice, Bassini.
– Sublay mesh implantation.
– Combination methods.
– Laparoscopic procedures: e.g. TAPP (transabdominal preperitoneal
plasty), TEP (total extraperitoneal plasty).

13.3 Incisional Hernia

13.3.1 Definition
– Post-operative fascia defects in the area of a fascia scar.
– Hernial gap/hernia sac (► Sect. 13.1).
Distinction: Incisional hernia – rectus diastasis – burst abdomen:
– Differentiation by hernial gap and hernia sac.
– Rectus diastasis: widening of the linea alba with divergence of the rectus
muscles.
Abdominal laceration: early postoperative abdominal wound
dehiscence after laparotomy.

13.3.2 Incidence
– Most frequent postoperative complication.
– Up to 18%, depending on the investigation period.
– In Germany: 100,000 incisional hernias per 700,000 laparotomies per
year; only 30% of hernias with surgical treatment.
– Longitudinal laparotomies more affected than transverse laparotomies.

13.3.3 Aetiology
– Impaired scar formation due to impaired collagen metabolism
(congenital/acquired).
– Multifactorial risk profile.
– Risk factors s. Overview:
 Risk Factors for Incisional Hernias
– Patient-related:
– Age (>45 years).
– Male gender.
– Obesity (BMI > 25).
– Ascites.
– COPD (chronic obstructive pulmonary disease).
– Consuming disease.
– Diabetes mellitus.
– Renal failure.
– Anemia (Hb < 10 g/dL).
– Nicotine abuse.
– Chemotherapy.
– Steroid therapy.
– Collagen metabolism disorder.
– Surgical:
– Recurrent surgery.
– Emergency surgery.
– Contaminated surgical field.
– Experience of the surgeon.
– Fascia suture technique Prevention through small bites – technique.
– Thread material.
– Postoperative condition:
– Wound infection.

– In the search for the risk profile for the development of incisional
hernias, the multifactorial genesis is always emphasized.
– Aim for etiological treatment (before surgical therapy or in parallel).

13.3.4 Clinical Presentation

– Mostly asymptomatic.
– Noticeable only by increase in size.
– Advancement of peritoneal organs; risk = “loss of domain”.
– Entrapment symptoms (e.g., irreducibility, pain, passenger disruption):
Up to 15.
– With intestinal strangulation: Up to 2%.

13.3.5 Diagnosis
Clinical Examination
– Examination always lying down + standing (+ pressing).
– Clinical signs ► Sect. 13.3.4.

Sonography
– Standard procedure.
– Confirmation of diagnosis + exclusion of a complication.
– Extent of the hernia gap (planning of the operation).

CT or MRI
– In case of unclear findings.
– For complex recurrence findings with implanted mesh materials.
– With pronounced obesity.

13.3.6 Surgical Therapy

Operation Indication:
– Every incisional hernia = indication for surgery.
– If incarceration is suspected = emergency.

Open Procedures
Suture Procedure
– Direct suture of the fascia gap
– Only indicated for small trocar hernias with unclear initial occlusion
Sublay Technique
– Conventional standard care.
– Incisional hernia repair in the sense of abdominal wall reinforcement
using a textile mesh implant in the retromuscular position (◘ Fig. 13.1).
– Pros:
– Extraperitoneal mesh position
– Restoration of fascia continuity = restoration of abdominal wall
function.

Fig. 13.1 Schematic drawing of retromuscular meshplasty (sublay position). (After Schumpelick
2011)

Abdominal Wall Replacement

– When tension-free fascial closure is impossible.
– Defect bridging by mesh.

Caution
In both the sublay technique and abdominal wall replacement, care
must be taken to ensure sufficient overlap between the mesh prosthesis
and the tissue of at least 5 cm in all directions.

Laparoscopic Procedures
– IPOM (intraperitoneal onlay mesh) = standard laparoscopic treatment.
– Advantage laparoscopic vs. open = reduced rate of wound complications.
– Basics:
– Visualization of the entire anterior abdominal wall (= adhesiolysis).
– Mobilisation of the content of the hernia sac, display of the complete
hernia gap.
– Cover the entire scar with an overlap of at least 5 cm on all sides (of
the hernia gap and the scar; ◘ Fig. 13.2).
– If necessary, cut through fatty tissue structures such as the ligaments
falciforme and teres hepatis or open up the prevesical space in the
lower abdomen.
Fig. 13.2 Examples of mesh coverage. Trolar and Mesh placement in the case of: (a) Midline
Incision Hernia; (b) Subcostal Incision Hernia; (c) Transversal Incision Hernia
IPOM mesh materials must achieve rapid and stable incorporation on
the parietal side and prevent adhesions on the visceral side.

Special Procedures
Component Separation According to Ramirez
– Mobilization technique of the fascia to close large gaps of the median
lines.
– Useful in combination with mesh reinforcement for large defects.
 Surgical Procedure
Component Separation According to Ramirez
– Lateral: Longitudinal splitting of the external aponeurosis (approx. 1–
2 cm lateral to the rectus sheath) = separation of the internal oblique
muscle and external oblique muscle.
– Medial: Longitudinal splitting of the rectus sheath on both sides from
the median line.
– Medialization of the rectus sheath blades = closure of the defect on the
median line.

13.3.7 Guidelines
Bittner R, Bingener-Casey J, Dietz U, Fabian M, Ferzli GS, Fortelny RH,
Köckerling F, Kukleta J, Leblanc K, Lomanto D, Misra MC, Bansal VK,
Morales-Conde S, Ramshaw B, Reinpold W, Rim S, Rohr M, Schrittwieser
R, Simon T, Smietanski M, Stechemesser B, Timoney M, Chowbey P, IEHS
(2014) Guidelines for laparoscopic treatment of ventral and incisional
abdominal wall hernias. International Endohernia Society (IEHS) – Part 1.
Surg Endosc 28: 2–29.
Bittner R, Bingener-Casey J, Dietz U, Fabian M, Ferzli GS, Fortelny
RH, Köckerling F, Kukleta J, Leblanc K, Lomanto D, Misra MC, Bansal
VK, Morales-Conde S, Ramshaw B, Reinpold W, Rim S, Rohr M,
Schrittwieser R, Simon T, Smietanski M, Stechemesser B, Timoney M,
Chowbey P, IEHS (2014) Guidelines for laparoscopic treatment of ventral
and incisional abdominal wall hernias. International Endohernia Society
(IEHS) – Part 2. Surg Endosc 28: 353–379.
Bittner R, Bingener-Casey J, Dietz U, Fabian M, Ferzli GS, Fortelny
RH, Köckerling F, Kukleta J, Leblanc K, Lomanto D, Misra MC, Bansal
VK, Morales-Conde S, Ramshaw B, Reinpold W, Rim S, Rohr M,
Schrittwieser R, Simon T, Smietanski M, Stechemesser B, Timoney M,
Chowbey P, IEHS (2014) Guidelines for laparoscopic treatment of ventral
and incisional abdominal wall hernias. International Endohernia Society
(IEHS) – Part 3. Surg Endosc 28: 380–404.
Dietz UA, Wiegering A, Germer CT (2014) European Hernia Society
guidelines on the treatment of inguinal hernia in adult patients; Hernia
(2009) 13: 343–403; ► https://​doi.​org/​10.​1007/​s10029-009-0529-7
Surgeon ► https://​doi.​org/​10.​1007/​s00104-014-2814-y

13.4 Inguinal and Femoral Hernia

13.4.1 Anatomy, Definition and Classification
Anatomy
– Inguinal canal (= inguinal canal):
– Course from the inner, lateral (= Anulus inguinalis profundus) to the
outer, medial (= Anulus inguinalis superficialis) inguinal ring.
– Contents: In the male spermatic cord + vessels supplying the testis; in
the female Lig. rotundum.
– Anatomical border: anterior wall = aponeurosis of the obliquus
externus muscle; posterior wall = fascia transversalis and peritoneum;
upper border = inferior border of the obliquus internus muscle and the
transversus muscle; inferior border = inguinal ligament.
– Femoral canal (= thigh canal):
– Anulus femoralis: entrance into the femoral canal; limited by V.
femoralis, Lig. inguinale, Lig. lacunare and Pecten os pubis.
– Canalis femoralis: 3–4 cm long canal in the medial section of the
lacuna vasorum (femoral vessels).

Definition
– Inguinal hernia: abdominal wall hernia in the area of the trigonum
inguinale (immediately above the lig. inguinale).
– Femoral hernia: abdominal wall hernia below the lig. Inguinale through
the femoral canal.

Division
– Classification of hernias in relation to the inguinal ligament:
– Inguinal hernia: hernial gap in regio inguinale, cranial of the lig.
Inguinale.
– Femoral hernia (= thigh hernia): Hernial gap = annulus femoralis
(caudal to the inguinal ligament), hernial sac medial to the femoral
 vein (always acquired).
– Topographical classification of inguinal hernia (in relation to the
epigastric vessels):
– Medial (= direct) inguinal hernia (30–40% of all inguinal hernias,
always acquired): Hernial gap directly in the area of the medial
inguinal fossa.
– Lateral (= indirect) inguinal hernia (mostly congenital, can also be
acquired): Hernial gap = inner inguinal ring, course through the
inguinal canal.

13.4.2 Epidemiology
Inguinal Hernia
– Incidence = 25% of all men and 2% of all women during their lifetime.
– Peak of manifestation: childhood and adolescence + older adulthood
(>40 years).
– Bilateral findings = 15–30%.
– 220,000 interventions in Germany.
– Men: Women = 8: 1.
– In children almost always indirect; in adults 70% indirect.

Femoral Hernia
– Incidence = 5–7% of all hernias (significantly less frequent than inguinal
hernia).
– Mostly women of advanced age (75%).
– Association with simultaneous inguinal hernia: Up to 9% in women; up
to 50% in men.

13.4.3 Pathophysiology
– Risk factors:
– Increased intra-abdominal pressure (e.g. obesity, chronic cough,
COPD, prostatic hyperplasia or constipation).
– Connective tissue disorders (e.g. Ehlers-Danlos syndrome, Marfan
syndrome or osteogenesis imperfecta).
– Change in collagen composition.
– Smoking.
13.4.4 Clinical Presentation
Asymptomatic Small Inguinal Hernia
– Incidental finding in the course of a clinical or sonographic examination.
– Initially no indication for surgery = “watchful waiting”.

Symptomatic Inguinal Hernia

– Visible/palpable protrusion (= size progression).
– Under stress (abdominal press, lifting weights, etc.) Pain in the groin
region.
– Possibly swelling in the groin.

Complications
– Intestinal incarveration: severe, persistent pain + a palpable, turgid
swelling of the groin region.
– Irreducibility: increase in symptoms → vomiting + ileus symptoms →
intestinal perforation + peritonitis.

Caution
Intestinal incarceration, ileus or perforation = indication for immediate
emergency surgery.

– Entrapment of parts of the omentum majus: often pressure-painful

swelling.

Femoral Hernia
– Typical hernial growth below the inguinal ligament.
– Mostly unspecific feeling of pressure.
– Signs of incarceration.
– Differential diagnoses: lymphadenopathy, subsidence abscess, lymph
node, lipoma.
– Exclusion by sonography.

13.4.5 Diagnostic Procedures

Palpation of the Inguinal Canal
– Lying down + standing patient under abdominal pressure (cough) =
assessment of potential hernia/hernial gap.
– Clarification of reproducibility.

Sonography
– In combination with palpation = reliable statement on hernia size,
position, content, reducibility and incarceration.

CT/MRI Examination
– Only in exceptional cases (e.g. previous operations/obesity).

13.4.6 Therapeutic Principles

Evidence-Based Strategy (◘ Fig. 13.3)
EHS (European Hernia Society) Recommendation
– All male adults aged 30 years and older with symptomatic inguinal
hernia → Surgical procedure with mesh implantation.
– Open Lichtenstein surgery/minimally invasive surgical procedures
(TAPP and TEP) = best evidence-based methods for the treatment of a
primary unilateral inguinal hernia.
– Suture procedure without mesh implantation (Shouldice procedure):
Only in individual cases (e.g. young patient with a small hernia gap and
sufficient fascial conditions or young patient with a desire to have
children).
Fig. 13.3 Flowchart for the treatment of inguinal hernia (IH) in men aged 30 years and older; TEP
total extraperitoneal plasty, TAPP transabdominal preperitoneal plasty. (European Hernia Society
Guidelines 2009)

Principles
– Surgery = therapy of choice.
– Aim of the surgical procedures = reinforcement of the posterior wall of
the inguinal canal by means of a mesh implant.
– Laparoscopic procedures (TAPP/TEP):
– Lower rate of wound infection/hematoma formation.
– Shorter convalescence time but longer OP time.
 In women (high rate of femoral hernias) = laparoscopic repair
procedures are more likely, since the possibility of clarifying the treatment
of the femoral hernia is better.
– Open procedures:
– Surgery under local anesthesia possible.
– Intraoperative decision option pro vs. contra mesh (in case of strong
fascia conditions and small hernia gap = suture procedure without
mesh application, e.g. Shouldice possible).

Emergency Surgery
– Incarceration + signs of mechanical ileus = emergency surgery.
– Acute hernia incarceration (<6 h) = one reduction attempt + inpatient
monitoring.
– Suspected bowel necrosis/acute abdomen = emergency surgery.

Complications
– Essential in preoperative education.
– Inguinal hernia recurrence (1–10%).
– Chronic groin pain:
– Definition: Groin pain that has been present for 3 months or more.
– Incidence: open procedure 18%; laparoscopic procedure 6%; however,
after approx. 2.5 years the incidence is the same for both surgical
procedures.
– Injury to the spermatic cord = potential infertility/testicular necrosis.
– Intraoperative conversion to open surgical technique, even median
laparotomy in case of emergency.
– Injuries to the bowel, urinary bladder or iliac vessels.
– Wound infection (1–2%).
– Seroma formation.
– Bleeding (0.5%).
– Injury or compression of the femoral artery, femoral vein and femoral
nerve (1%), thromboembolism (1%).
– Lethality <1%.
Caution
In case of incarceration with bowel resection: lethality up to 20%.
Intraoperative EHS Classification (2007)
– Assessment of:
– Frequency of hernia (primary and recurrent).
– Localization (medial, lateral, femoral, combined).
– Size of hernial gap (1 = 1.5 cm corresponding to ≤1 finger, 2 = 1.5–3
cm corresponding to 1–2 fingers, 3 ≥ 3 cm corresponding to 3 fingers
and more).
– Target:
– Standardization of the hernia description.
– Enabling large international comparative studies.

Surgical Technology
Inguinal Hernia
Operative Procedure
Shouldice Herniotomy
– Opening of the inguinal canal, attachment of the spermatic cord with
appendages, separation of the hernia sac.
– Important = protection of the ilioinguinal nerve, iliohypogastric nerve
and the rami genitales as well as femorales of the genitofemoral nerve.
– 2-row doubling of the fascia transversalis (continuous Prolene suture)
starting at the tuberculum pubicum.
– Narrowing of the inner inguinal ring.
– Continuous suture of the transversus abdominis and obliquus internus
muscles to the inguinal ligament (2-row).
– Continuous closure of the external aponeurosis, skin suture.

Surgical Procedure
Herniotomy According to Lichtenstein
– Opening of the inguinal canal, attachment of the spermatic cord with
appendages, separation of the hernia sac.
– Important = protection of the ilioinguinal nerve, iliohypogastric nerve
and the rami genitales as well as femorales of the genitofemoral
nerve.
– Treatment of the hernia, resection of the preperitoneal lipoma if
necessary; repair of the posterior wall with non-absorbable mesh (8 ×
12 cm); continuous mesh fixation at the inguinal ligament.
– In the male, slit in the lateral part and new formation of the inner
inguinal ring around the spermatic cord and testicular vessels and
subsequent closure of the slit.
– Externus aponeurosis closure.

Surgical Procedure
TAPP (= Transabdominal Preperitoneal Plasty)
– Only intubation anesthesia because of capnoperitoneum.
– Periumbilical camera trocar plus two working trocars, creation of the
capnoperitoneum.
– Incision of the peritoneum + dissection of the preperitoneal inguinal
region with visualization of the hernial gap, inguinal ligament and
inguinal canal structures.
– Dissection of the hernia back into the peritoneal space.
– Placement of a non-absorbable mesh implant (mesh size at least 10 ×
15 cm); fixation only if necessary by means of tissue adhesive,
absorbable stapler – more important is sufficient overlapping of the
mesh in relation to the fracture edge.
– Closure of the peritoneum (continuous suture).

Surgical Procedure
TEP (= Total Extraperitoneal Plasty)
– Preparation in the preperitoneal space by mechanical + CO2
insufflation.
– Intubation anesthesia only.
– Subumbilical access to the preperitoneal space; CO2 insufflation.
– Two additional working trocars in the midline and suprasymphysary:
preparation of the preperitoneal inguinal region with visualization of
 the hernial gap, inguinal ligament and inguinal canal structures.
– Placement of a non-absorbable mesh implant (mesh size 10 × 15 cm);
fixation with tissue adhesive/no fixation.
– Relief CO2, fascial closure of the subumbilical incision, skin suture.

Surgical Procedure
Recurrence of Inguinal Hernia
– Supply principle depends on the previous operation.
– After open hernia repair = laparoscopic procedure recommended.
– After laparoscopic hernia repair = open procedure recommended.

Surgical Procedure
Femoral Hernia
– Optimal treatment possible with TAPP – Open surgery: visualization
of the femoral portal via inguinal access/crural access.
– Closure of the hernial gap:
– Continuous suture of the pectineal ligament to the inguinal ligament
(according to Moschkowitz/Fabricius).
– Single button sutures between M. obliquus internus and M.
transversus abdominis and Lig. pectineale and Fascia transversalis
(after Lotheissen/McVay).
– Closure of the hernial gap by mesh (Gilbert prosthesis via inguinal;
plug from crural).

Aftercare
– Rule of thumb: do not lift more than 10–15 kg for 4–6 weeks.
– Immediately postoperative: pain-adapted stress possible.
– Refrain from heavy lifting/sporting activities for approx. 2–3 weeks,
followed by pain-adapted loading.

13.4.7 Guidelines
European Hernia Society guidelines on the treatment of inguinal hernia in
adult patients; Hernia (2009) 13: 343–403; ► https://​doi.​org/​10.​1007/​
s10029-009-0529-7
Simons MP, Aufenacker T, Bay-Nielsen M, Bouillot JL, Campanelli G,
Conze J, de Lange D, Fortelny R, Heikkinen T, Kingsnorth A, Kukleta J,
Morales-Conde S, Nordin P, Schumpelick V, Smedberg S, Smietanski M,
Weber G, Miserez M (2009) European Hernia Society guidelines on the
treatment of inguinal hernia in adult patients. Hernia 13: 343–403.
Bittner R, Arregui ME, Bisgaard T, Dudai M, Ferzli GS, Fitzgibbons
RJ, Fortelny RH, Klinge U, Kockerling F, Kuhry E, Kukleta J, Lomanto D,
Misra MC, Montgomery A, Morales-Conde S, Reinpold W, Rosenberg J,
Sauerland S, Schug-Pass C, Singh K, Timoney M, Weyhe D, Chombey P
(2011) Guidelines for laparoscopic (TAPP) and endoscopic (TEP) treatment
of inguinal hernia [International Endohernia Society (IEHS)]. Surg Endosc
25: 2773–2843.

13.5 Umbilical and Epigastric Hernia

13.5.1 Umbilical Hernia
Definition
– Hernia.
– Incidence = approx. 5.
– Women > Men.
– Risk factors: Ascites, obesity, pregnancy…

Division
– Infantile umbilical hernias:
– Hernia gap at the annulus umbilicalis.
– Spontaneous closure in the first years of life (98%) = no indication for
surgery before the age of 2 years.
– In adulthood:
– Mostly paraumbilical hernia next to the umbilical pillar.
– No spontaneous regression tendency at all.
– Risk factors: Obesity, liver cirrhosis or ascites; in combination with
rectus diastasis.
13.5.2 Epigastric Hernia
Definition
– Fascia defect in the linea alba between xyphoid and umbilicus.
– Incidence = approx. 5.
– Men > Women.
– Overview: Clinical presentation, diagnosis and indication for surgery is
similar for umbilical and epigastric hernia.

13.5.3 Clinical presentation

– Unspecific abdominal pain.
– Pain when stretching or tensing the abdominal wall muscles.
– protrusion of the abdominal wall.
– Depending on the findings, up to intestinal obstruction, with ileus or
strangulation.

13.5.4 Diagnosis
– Palpation.
– Sonography: fascial gap, hernia contents.

13.5.5 Therapy
– Small asymptomatic findings = “watchful waiting”.
– Symptomatic findings (e.g. increase in size, incarcerations or also non-
specific abdominal pain) = surgical therapy:
– Small fascial defects (<1 cm): continuous sutureplasty (non-
absorbable, transverse), joint to joint.
– Fascia defects >1 cm and/or a BMI of >30: Additional mesh implant
(alloplastic material).

13.5.6 Guideline
Guidelines for treatment of umbilical and epigastric hernias from the
European Hernia Society and Americas Hernia Society N. A. Henriksen, A.
Montgomery, R. Kaufmann, F. Berrevoet, B. East, J. Fischer,W. Hope, D.
Klassen, R. Lorenz, Y.Renard, M. A. Garcia Urena and M. P.
Simons5onbehalf of the European and Americas Hernia Societies (EHS and
AHS).
 Surgical Procedure
Umbilical Hernia
– Semicircular left lateral umbilical incision (extendable to cranial and
caudal).
– Sharp detachment of the navel (caution: skin injury and blood
circulation).
– Preparation of the hernia sac up to linea alba and hernia gap.
– Repositioning the hernia sac.
– Transverse hernia closure: Direct non-absorbable or slow-absorbable
suture for small gaps less than 1 cm; mesh implantation in
preperitoneal position (preperitoneal umbilical mesh plasty = PUMP)
with an overlap of 3 cm for gaps of 1–4 cm.
– Refixation of the umbilicus and skin closure.

Surgical Procedure
Epigastric Hernia
– Transverse skin incision.
– Then procedure analogous to the umbilical hernia.

13.5.7 Differential Diagnosis: Rectus Diastasis

Definition
– Divergence of the rectus musculature in the area of the linea alba.
– No fascial defect: bulge-like protrusion.

Etiology and Pathogenesis

– Congenital rectus diastasis.
– Acquired rectus diastasis.

Clinical Presentation
– Protrusion in the area of the diastasis when tightening the abdominal
muscles (or straightening).
– Mostly only an aesthetic problem.
Therapy
– In the absence of a fascial defect, no risk of incarceration = primary
conservative therapy.
– Only rarely (e.g. for cosmetic reasons) correction indicated = procedure
as for incisional hernia = mesh implant in retromuscular position
(sublay).
– High recurrence rate without mesh reinforcement.

13.6 Parastomal Hernia

13.6.1 Definition
– Protrusion of any kind near a stoma.

13.6.2 Epidemiology
– Incidence depending on stomatal type:
– Terminal colostomy: 4–48%.
– Terminal ileostomy: 2–28%.
– Loop colostomy: 0–31%.
– Loop ileostomy: 0–6%.
– High recurrence rate after treatment.

13.6.3 Risk Factors and Prevention

Risk Factors
– General risk factors (similar to the pathogenesis of incisional hernias, ►
Sect. 13.3.3): Obesity, wound infections, advanced age,
immunosuppression, COPD.
– Special risk factor = opening too large for stoma passage (fascial passage
should be as narrow as possible).

Prevention
– Expulsion of the stoma through rectus muscle.
– abdominal wall gap (fascia gap) as small as possible.
– Tunneling technique under peritoneum (valve mechanism) (before
abdominal wall passage).
– Prophylactic mesh implantation: Significant reduction in the rate of
parastomal hernias = recommended for open/laparoscopic creation of
permanent terminal small and large bowel stomas (Level 1
recommendation, Bittner et al. 2014).

13.6.4 Clinical Presentation

– Often symptomatic
– Especially supply problems with plate and bag.
– Voiding disorder, pain.
– Incarceration, ileus.

13.6.5 Relevant Complications

– Hernia incarceration.
– Ileus.
– Progressive voiding obstruction.
– Chronic pain and subileus conditions.

13.6.6 Therapy
– Mains supply = standard.

Open Procedures
– Local suture procedures with only fascia constriction (= recurrence rate
50 to 76%) = not recommended.
– Stomal Relocation:
– Usually by means of relaparotomy.
– Relocation alone without prophylactic mesh implantation =
disappointing recurrence rates (30–45% parastomal hernias at the
newly created stoma site) (analogous to initial placement).
– Treatment of the defect at the initial stoma site in the sense of a scar
hernia (in 33% here recurrent scar hernia).
– A further disadvantage of relocation is scar fractures in the
relaparotomy area in 10–20% of cases.
– Repair by means of mesh implantation (◘ Fig. 13.4):
– Reduction of the fracture.
 – Fascia constriction and reinforcement using textile mesh implant;
– Reticular tunneling and lateralization of the intestine (Sugarbaker
technique).
– Classification of the technique according to mesh positioning:
epifascial (onlay), retromuscular (sublay) intraperitoneal mesh
reinforcement (◘ Fig. 13.4).

Fig. 13.4 Mesh position in parastomal hernia repair. a Onlay, b Sublay, c Intraperitoneal mesh
position

Minimally Invasive Procedures

– Laparoscopic hernia reduction + intraperitoneal mesh reinforcement.
– Techniques:
– IPST mesh (intraperitoneal stoma mesh): Mesh with a central tunnel
(from 2–3.5 cm diameter) is placed around the terminal stoma and
fixed to the abdominal wall.
– Sugarbaker technique: reduction of the hernia + lateralization of the
executing intestinal loop with a covering mesh.
– Sandwich technique: reduction of the hernia + positioning of a
keyhole-shaped mesh around the terminal intestinal loop + additional
lateralization of this loop with another mesh in the sense of the
Sugarbaker technique.

13.6.7 Guidelines
Bittner R, Bingener-Casey J, Dietz U, Fabian M, Ferzli GS, Fortelny RH,
Köckerling F, Kukleta J, Leblanc K, Lomanto D, Misra MC, Bansal VK,
Morales-Conde S, Ramshaw B, Reinpold W, Rim S, Rohr M, Schrittwieser
R, Simon T, Smietanski M, Stechemesser B, Timoney M, Chowbey P, IEHS
(2014) Guidelines for laparoscopic treatment of ventral and incisional
abdominal wall hernias. International Endohernia Society (IEHS) – Part 3.
Surg Endosc 28: 380–404. ► https://​doi.​org/​10.​1007/​s00464-01m3-3172-4

13.7 Other Rare Hernias

13.7.1 Spieghel’s Hernia (Hernia Lineae Semilunaris)
– Hernia form in the lower middle abdomen.
– Hernial gap: between the aponeurosis of the internal oblique muscle and
the outer edge of the rectus sheath (linea semilunaris).

13.7.2 Hernia Obturatoria

– Hernia of the pelvic floor (◘ Fig. 13.5).
– Hernial gap in the obturator foramen, along the vasa obturatoria/N.
obturatorius.
– Especially for older women.
– Clinical presentation: Typically pain in the lower abdomen + radiation to
the inner thighs.
– Caution: Not visible externally.
Fig. 13.5 Rare hernias of the pelvic floor

13.7.3 Hernia Ischiadica

– Hernial gap = foramen ischiadicum in the region of the gluteus maximus
muscle (◘ Fig. 13.5).

13.7.4 Hernia Perinealis

– Hernia through ischiorectal fossa (◘ Fig. 13.5).
– Course: to the perineum or into the labia majora.

13.7.5 Lumbar Hernia

– Hernia in the region of the upper (Trigonum lumbale superius)/lower
lumbar triangle (Trigonum lumbale inferius).
– Trigonum lumbale = weak point of the lumbar abdominal wall.
– Trigonum lumbale superius: triangle between lateral border of the
Mm. erector spinae and medial border of the M. obliquus internus,
below the 12th rib.
 – Trigonum lumbale inferius (Petit): between edges of the M. obliquus
externus and latissimus dorsi, above the iliac crest.

References
European Hernia Society guidelines (2009) on the treatment of inguinal hernia in adult patients.
Hernia 13:343–403. https://​doi.​org/​10.​1007/​s10029-009-0529-7
[Crossref]

Schumpelick V (ed) (2011) Gastroenterologische Chirurgie, 3rd edn. Springer, Berlin/Heidelberg

Further Reading
Bakker WJ, Aufenacker TJ, Boschman JS, Burgmans JPJ (2020) Lightweight mesh is recommended
in open inguinal (Lichtenstein) hernia repair: a systematic review and meta-analysis. Surgery
167:581–589
[Crossref][PubMed]

Bernardi K, Olavarria OA, Holihan JL, Kao LS, Ko TC, Roth JS, Tsuda S, Vaziri K, Liang MK
(2019) Primary fascial closure during laparoscopic ventral hernia repair improves patient quality of
life: a multicenter, blinded randomized controlled trial. Ann Surg 271:434–439
[Crossref]

Blonk L, Civil YA, Kaufmann R, Ket JCF, van der Velde S (2019) A systematic review on surgical
treatment of primary epigastric hernias. Hernia 23:847–857
[Crossref][PubMed][PubMedCentral]

Bökkerink WJV, Koning GG, Malagic D, van Hout L, van Laarhoven CJHM, Vriens PWHE (2019)
Long-term results from a randomized comparison of open transinguinal preperitoneal hernia repair
and the Lichtenstein method (TULIP trial). Br J Surg 106:856–861
[Crossref][PubMed][PubMedCentral]

Bullen NL, Massey LH, Antoniou SA, Smart NJ, Fortelny RH (2019) Open versus laparoscopic
mesh repair of primary unilateral uncomplicated inguinal hernia: a systematic review with meta-
analysis and trial sequential analysis. Hernia 23:461–472
[Crossref][PubMed]

Caro-Tarrago A, Olona C, Millán M, Olona M, Espina B, Jorba R (2019) Long-term results of a

prospective randomized trial of midline laparotomy closure with onlay mesh. Hernia 23:335–340
[Crossref][PubMed]

Gavriilidis P, Davies RJ, Wheeler J, de’ Angelis N, Di Saverio S (2019) Total extraperitoneal
endoscopic hernioplasty (TEP) versus Lichtenstein hernioplasty: a systematic review by updated
traditional and cumulative meta-analysis of randomised-controlled trials. Hernia 23:1093–1103
[Crossref][PubMed][PubMedCentral]

Gutlic N, Gutlic A, Petersson U, Rogmark P, Montgomery A (2019) Randomized clinical trial

comparing total extraperitoneal with Lichtenstein inguinal hernia repair (TEPLICH trial). Br J Surg
106:845–855
[Crossref][PubMed]

van den Hil LCL, van Steensel S, Schreinemacher MHF, Bouvy ND (2019) Prophylactic mesh
placement to avoid incisional hernias after stoma reversal: a systematic review and meta-analysis.
Hernia 23:733–741
[Crossref][PubMed][PubMedCentral]

Kohler A, Lavanchy JL, Lenoir U, Kurmann A, Candinas D, Beldi G (2019) Effectiveness of

prophylactic intraperitoneal mesh implantation for prevention of incisional hernia in patients
undergoing open abdominal surgery: a randomized clinical trial. JAMA Surg 154:109–115
[Crossref][PubMed]

Lockhart K, Dunn D, Teo S, Ng JY, Dhillon M, Teo E, van Driel ML (2018) Mesh versus non-mesh
for inguinal and femoral hernia repair. Cochrane Database Syst Rev 9:CD011517
[PubMed]

Obermaier R, Pfeffer F, Hopt UT (eds) (2009) Hernienchirurgie. Elsevier Urban & Fischer, München

Patterson TJ, Beck J, Currie PJ, Spence RAJ, Spence G (2019) Meta-analysis of patient-reported
outcomes after laparoscopic versus open inguinal hernia repair. Br J Surg 106:824–836
[Crossref][PubMed]

Sheen AJ, Montgomery A, Simon T, Ilves I, Paajanen H (2019) Randomized clinical trial of open
suture repair versus totally extraperitoneal repair for treatment of sportsman’s hernia. Br J Surg
106:837–844
[Crossref][PubMed]

Shrestha D, Shrestha A, Shrestha B (2019) Open mesh versus suture repair of umbilical hernia: meta-
analysis of randomized controlled trials. Int J Surg 62:62–66
[Crossref][PubMed]

Berger D (2010) Laparoskopische reparation der parastomalen Hernie. Chirurg 81:988–992

[Crossref][PubMed]

Jones HG, Rees M, Aboumarzouk OM, Brown J, Cragg J, Billings P, Carter B, Chandran P (2018)
Prosthetic mesh placement for the prevention of parastomal herniation. Cochrane Database Syst Rev
7:CD008905
[PubMed]

Reinforcement of Closure of Stoma Site (ROCSS), Collaborative and West Midlands Research
Collaborative (2020) Prophylactic biological mesh reinforcement versus standard closure of stoma
site (ROCSS): a multicentre, randomised controlled trial. Lancet 395:417–426
[Crossref]

Rosch R, Conze J, Junge K, Neumann U (2010) Konventionelle Reparation der parastomalen Hernie.
Chirurg 81:982–987
[Crossref][PubMed]

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_14

14. Gastrointestinal Stromal Tumors and

Sarcomas
Daniel Oertli1 , Holger Bannasch2 , Athanasios Tampakis3,
Christoph Kettelhack3 and Tobias Keck4
(1) Department of General Surgery, University Hospital Basel, Basel,
Switzerland
(2) Department of Plastic, Hand and Aesthetic Surgery, Schwarzwald-
Baar-Hospital Villingen-Schwenningen, Villingen-Schwenningen,
Germany
(3) Clarunis—Universitäres Bauchzentrum Basel, University Hospital
Basel, Basel, Switzerland
(4) Department of Surgery, University Hospital Schleswig-Holstein,
Luebeck, Germany

Daniel Oertli
Email: [email protected]

Holger Bannasch
Email: [email protected]

Christoph Kettelhack
Email: [email protected]

Tobias Keck (Corresponding author)

Email: [email protected]

14.1 Gastrointestinal Stromal Tumours (GIST)

D. Oertli and T. Keck

Key Points
– GIST = Most common soft tissue tumors in the gastrointestinal tract.
– Overexpression of the KIT tyrosine kinase receptor (CD 117):
– Diagnostic marker.
– Simultaneous target for systemic therapy.
– Smaller tumors consistently benign; with larger diameter = malignant
potential (occasionally liver metastases).
– Radical surgical excision indicated (goal = R0 resection) = avoidance
of local recurrences.
– Adjuvant/neoadjuvant antibody therapy: with imatinib (“first
line”)/with sunitinib (“second line”).

14.1.1 Definition
– GIST (gastrointestinal stromal tumors) = soft tissue tumors of the GI
tract.
– Malignant potential dependent on:
– Differentiation (G = Grading).
– Tumor size.
– Metastasis.

14.1.2 Epidemiology and Tumour Localisation

Epidemiology
– Annual incidence = 1/100,000 in general population.
– Women: Men = 1: 1.
– Peak incidence = 5th–7th decade of life.

Localization
– Stomach: 60–70%.
– Small intestine: 20–30.
– Colorectum: <5%.
– Other localization: esophagus, omentum, mesentery <5%.
– Most GIST = sporadic.
– Solitary in 95% of cases.

Tumor Biology
– Locally displacing growth.
– Tumor originates from the tunica muscularis.
– Usually no lymphatic metastasis.
– Hematogenous or peritoneal metastasis possible, 20% of tumors are
metastatic at diagnosis.

Risk Factors
– Neurofibromatosis.
– In the context of the Carney triad: extraadrenal paraganglioma,
pulmonary chondroma, GIST.
– Familial accumulation possible.

14.1.3 Clinical Presentation

Manifestation
– 70% symptomatic.
– 20% Incidental finding during endoscopy or surgery.
– 10% autopsy finding.

Symptoms
– Often incidental finding.
– Gastrointestinal bleeding 52%; if localized in the stomach: bleeding in
70% of cases.
– Abdominal pain 32%.

Prognosis at Diagnosis
– 5-year survival of the total collective (SEER database) = 45%.
– Primary tumor = 53% (5-year survival = 64%).
– Locoregional recurrence = 19%.
– Stage IV (metastatic) = 23% (5-year survival = 13%).

14.1.4 Pathology
Conventional Histology
– Homogeneous proliferation of mesenchymal cells.
– gastrointestinal tract: cells with characteristic features of interstitial Cajal
cells (pacemaker cells for peristalsis located in muscle wall with
characteristic features of both smooth muscle and autonomic nerve cells).
– Histological subtypes:
– Spindle cell type (70%).
– Epithelioid cell type (20%).
– Mixed type (spindle + epithelioid cells = 10%).

Immune Phenotype
– KIT tyrosinase receptor (CD 117) overexpression: 95% of cases.
– CD 117 = product of the c-KIT gene (= protooncogene).
– Diagnostic marker in immunohistochemistry.
– Most other mesenchymal tumors (except Ewing’s sarcoma) = CD 117-
negative.
– Other positive markers:
– CD 34: 70%.
– “Smooth muscle actin” (SMA): 25%.
– S-100 <10%.
– Desmin <5%.

Mutation Detection
– Mutation in KIT receptor / in platelet-derived growth factor receptor α
(PDGFRA): Functionally corresponding to protooncogene.
– Mutated protooncogene permanently active: cell proliferation and tumor
formation.

14.1.5 Diagnosis
Diagnostic Imaging
– Primary diagnosis by esophagogastroduodenoscopy (EGD), in case of
tumor localization in the small intestine push/double balloon endoscopy
 (advancing the endoscope into the small intestine with the help of
balloons) or capsule endoscopy necessary.
– In the case of unclear tumour extension or to exclude metastases,
additional CT-scan.
– For tumor search in the small intestine: dynamic magnetic resonance
examination with oral contrast medium (MRI-Sellink) useful.
– Standard diagnosis CT, MRI and FDG PET if necessary for therapy
assessment.

Tissue Biopsy
– Indications:
– If endoscopically accessible (± endoluminal sonography).
– If tumor is unresectable or metastatic.
– If neoadjuvant pretreatment is planned.
– Contraindication/no indication: If tumour appears to be primarily
completely resectable.

Caution
Tumor seeding on biopsy and/or postinterventional bleeding from
tumor.

14.1.6 Therapy
Surgical Therapy
Indication
– Surgery = standard therapy for resectable GIST.
General Principles
– Minimum distance to the tumour: of 1–2 cm sufficent under certain
circumstances.
– Partial resection of the stomach possible (e.g. wedge resection):
Gastrectomy necessary in rare cases.
– Segment resections of the small and large intestine.
– Local R0 resection of the rectum permitted.
– No systematic lymphadenectomy (because predominantly hematogenous
metastasis).

Caution
A complete microscopic resection (R0 resection) should always be
aimed for!
GIST of the Stomach
– Tumour diameter 0.3–6.5 cm = mostly “low risk”.
– Small tumors: well localizable + resectable by laparoscopy
+/intraoperative gastroscopy.

Caution
If tumor rupture (spontaneous, traumatic, iatrogenic, intraoperative)
and tumor cell seeding:
– Adjuvant therapy (see below).
– Without imatinib high local recurrence risk.

Adjuvant Treatment
Antibodies (Ab) Against the Tyrosine Kinase Receptor
– Imatinib (e.g. Gleevec) = selective thyrosine kinase inhibitor on c-KIT
and PDGFRA.
– Central role of the c-KIT mutation status (see below “Molecular genetic
analysis”).
– Dosage = 400–800 mg daily.
– Median time to maximum response: 197 days.
– Results
– Complete remission = 5%.
– Partial remission = 47%.
– Disease stabilisation = 32%.

Molecular Genetic Analysis as a Guide for Ab Treatment

– c-KIT exon 11: mutation higher response rate than c-KIT exon 9
mutation.
– c-KIT mutation: Higher response rate than wild type.
 – As a consequence 2 strategies possible:
– Pro: High dosage (800 mg daily) in wild type and in exon 9
mutation.
– Contra: 400 mg in all patients, increase to 800 mg if progression.
– Side effects: Anemia, neutropenia, fatigue, edema, rash, nausea,
diarrhea.

Second-Line Therapy
– In case of resistance to imatinib (Gleevec):
– 5% primary resistance.
– 14% Secondary resistance.
– Sunitinib: Different clinical benefit depending on genotype: KIT exon 9:
63%; wild type 56%; KIT exon 11: 36%; PDGFRA 25%.

Neoadjuvant Treatment
Goals
– Achieving resectability in primary irrectable GIST.
– Tumor regression (size): Can function-preserving surgery be
facilitated? Can a multivisceral resection be avoided?
– Minimization of tumor fragility.
– Based on imatinib (Gleevec).
Indications/Target Organs
– Locally advanced tumor.
– Target Organs:
– Rectum.
– Esophagus.
– Pancreas.
Multimodal Therapy
– Indication depends on the risk of recurrence; this can be assessed, for
example, with the score according to Fletcher et al. (2002a, b) (◘ Table
14.1) or Miettinen and Lasota (2001).
Table 14.1 Risk stratification according to Fletcher in GIST

Risk Tumor size Microscopy: number of mitoses

Very low <2 cm <5/50 HPF
Low 2–5 cm <5/50 HPF
Intermediary <5 cm 6–10/50 HPF
5–10 cm <5/50 HPF
High >5 cm >5/50 HPF
>10 cm Each number
Any size >10/50 HPF

HPF high power field

Results of Neoadjuvant Therapy
– Maximum tumor response after 6–12 months: Surgery.
– Despite R0 resection: local recurrence (up to 76% of patients).
– In this case salvage surgery: 15 months median survival.
– Prognostic factors (poor prognosis):
– Tumor size.
– Tumor localization (duodenum, rectum).
– Incomplete resection.
– High proliferation rate (Ki-67 index).
– Mutation status (deletion in KIT exon 11).

Assessment of the Therapeutic Success in Imaging

Caution
Cross-sectional imaging needed to evaluate therapeutic success.

– Absence of increase in size of tumor and metastases = response.

– Especially if tissue density changes under Ab therapy.
Computer Tomography (CT scan)
– Therapy success = no increase in size (not necessarily shrinkage of the
lesion).
– Decrease in measured density (in Hounsfield units) >15% = remission.
Positron Emission Tomography (PET)
– Decrease in SUV (“standardized uptake value”) by more than 40% from
baseline or SUV <3.4 = response to neoadjuvant therapy.

14.1.7 Guidelines
ESMO (2018) Clinical Practice Guidelines for diagnosis, treatment and
follow-up. Ann Oncol 29 (Suppl 4): iv 68–78.

14.2 Soft Tissue Tumours of the Extremities

H. Bannasch

14.2.1 General: Classification

Key Points
– Benign soft tissue tumors 90% vs. Soft tissue sarcomas 10%.
– In 60% of cases = Extremities affected.
– Diagnosis: Ultrasound, MRI, PET.
– Prognosis: Depending on tumor size + grading.

Epidemiology
General Information
– Benign soft tissue tumors.
– Large majority of all soft tissue tumours (at least 100 times more
frequent than soft tissue sarcomas).
– Incidence = approx. 300/100,000.
– Soft tissue sarcomas (STS)
– Rare and heterogeneous group of malignant tumors.
– Mesenchymal origin.
– STS = only 1% of all adult malignancies.
– Incidence in adults = approx. 2–3/100,000.
– Incidence increase of STS in the last decades (possibly only apparent
increase due to better data collection).
– No differences in the geographical frequency distribution.
– In children: Proportion of STS in all malignancies significantly higher
(5–8%) despite lower incidence.
STS Body-Distribution Pattern
– 60% Extremities.
– Lower extremity more often than upper extremity.
– In each case, higher incidence for the proximal limb sections.
– 20–35% retro- and intraperitoneal sarcomas including GIST (► Sect.
14.1).
– 15–20% trunk, head and neck region.

Caution
Soft tissue sarcomas in adults vs. children: significant differences with
regard to distribution pattern in the body, histological frequency
distribution of the subtypes and tumor biology!

Rare Hereditary Risk Factors

– Li-Fraumeni Syndrome.
– Very rare autosomal dominant disease with germline mutation of the
tumor suppressor gene p53.
– Very high risk for numerous malignancies.
– Neurofibromatosis type 1 (von Recklinghausen’s disease).
– Incidence approx. 35/100,000.
– Autosomal dominant inherited multiorgan disease.
– Mainly involvement of the skin and nervous system (phacomatosis).
– including benign neurofibromas.
– Lifetime risk of about 5–10% for malignant transformation of a
(plexiform) neurofibroma into a malignant peripheral nerve sheath
tumor (MPNST).
Acquired Risk Factors
– Postradiogenic sarcomas (latency of up to 20 years).
– Steward-Treves syndrome (angiosarcoma originating from chronic
lymphedema).
– Viral infections in immunocompromised patients causally responsible for
STS development:
– HHV8 for Kaposi’s sarcoma.
– EBV for leiomyosarcoma.

Caution
Etiology of soft tissue sarcomas mostly unclear; rare hereditary and
acquired risk factors known.

14.2.2 Clinical Presentation

– Leading symptom = mostly painless swelling of the extremities.
– Duration of symptoms often not ascertainable.
– Frequent anamnestic association with minor trauma (patient’s need for
causality).
– Rarely specific symptoms with peripheral functional deficits – vascular-
nervous (blood flow, motor function, sensory function) deficits often
only in advanced local stages.
– B-symptomatics only present in exceptional cases.

Caution
Increased delay in diagnosis:
– Due to the rarity of soft tissue sarcomas (often the possibility of a
malignant tumour is not considered).
– Diagnosis in many cases in the context of an inadequate excision
without prior diagnosis.

Anamnesis
– Despite oligosymptomatic: careful history and examination obligatory.
– Documentation of extent and duration of swelling/discomfort.
– Querying risk factors (see above).

Investigation
– Inspection of the extremities.
– Differences in side circumference, swellings.
– Sensory and motor function deficits.
– Palpation of the lymph node stations (rarely affected).

Caution
Swelling of the extremities resistant to therapy for longer than 4 weeks
→ further investigation needed.

14.2.3 Diagnosis
– Goals
– Determination of the histology of the tumor.
– Determination of tumor size + tumor location with reference to
surrounding anatomical structures.
– Staging: detection of distant metastases (mostly haematogenous).
– Staging of children with soft tissue sarcomas: more complex +
extensive than in adults.

Local Diagnosis
Ultrasound (Unclear Swelling of the Extremity)
– Helpful for initial descriptive purposes.
– Can be repeated without complications.
– Disadvantages: Investigator dependence and relative nonspecificity.
Native X-Ray Imaging in 2 Planes
– possible calcifications in case of extraosseous bone tumours.
Magnetic Resonance Imaging (MRI): Gold Standard
– Local presentation of a soft tissue tumor of the extremity.
– MRI is clearly superior to computed tomography (CT).
– Always with and without contrast medium.
– Provides valuable information about the size, location and contents of the
tumour (necrosis, myxoid areas, haemorrhages, etc.).
– Assessment of the technical operability and evaluation of a necessary
“down-sizing”.
Fluorodeoxyglucose (FDG)-PET
– Not generally recommended.
Diagnosis of Local and General Spread
Pathophysiology
– Adult STS: Preferential metastasis = hematogenous-pulmonary.
Diagnosis
– MRI: for local extension.
– CT chest/(abdomen): Staging.
– Lymph node diagnosis: No evidence.
– Sentinel lymph node biopsy: No evidence.
– FDG-PET: Only useful in individual cases.
– Tumor markers: No evidence.
Biopsy
Caution
Suspicion of malignant soft tissue tumor: histological diagnosis is
indicated and early referral to a specialized center indicated.

General
– Differentiation malignant vs. benign with imaging techniques: not
possible with certainty.
– Multiple tumor characteristic features correlatable with ex post diagnosis
of STS:
– Tumour diameter > 5 cm.
– Size increase.
– Painfulness.
– Deep localization.

Indication for Biopsy

– Any tumor = malignant until proven otherwise.
– Slow tumor growth = no definite evidence of benign disease.
– Only every 100th–200th soft tissue tumour = malignant.
– Conclusion: Therefore very generous indication for biopsy.

Excisional Biopsy
– Only if the tumour is definitely epifascial.
– Only for small (<5 cm) tumors.
– Only for tumors without high suspicion of malignancy!
– In case of malignancy:
– Oncologic resection in these cases usually technically unproblematic.
– No influence on prognosis.

Caution
Send every specimen for histopathologic examination!
Open Incisional Biopsy
– Open incisional biopsy = procedure of choice.
– In case of suspected malignancy always at the center.
– Ideally by the surgeon scheduled for oncologic resection.
– Principle: Obtaining sufficient material including the so-called
pseudocapsule for:
– Conventional + immunohistochemical examinations.
– Molecular genetic examinations: Fluorescence in situ hybridization
(FISH), polymerase chain reaction (PCR).
– If necessary, pathology reference center (indicate generously).

Caution
Possible mistakes:
– Incision, which complicates a later ideal reconstruction (access path
and drainage outlets have to be resected during the second
operation).
– Insufficient haemostasis = haematoma formation + risk of cell
spreading.
– Unfavorable drainage outlets far away from the surgical area: need
for larger excision during second operation.

Punch Biopsy
– Possible in centres with sufficient expertise under sonographic control.
– Diagnostic certainty: punch biopsy > fine needle aspiration; punch
biopsy = open incision biopsy.
– Molecular pathology also feasible with little tissue.
14.2.4 Classification
Histological Classification
– Overview: Internationally valid classification for soft tissue tumors:
– WHO classification of tumours of the soft tissues and bone (Fletcher
et al. 2002a, b).
– Diagnostic tools PCR and FISH: detection of numerous specific
chromosomal translocations = supplementation of the classical
pathomorphological diagnosis.
– Unchanged classification according to detectable line of differentiation.
– First classification principle = descent from probable tissue of origin
(lipomatous, fibroblastic etc.)
– Second classification principle = within the entities with regard to their
dignity: benign – intermediate (locally aggressive) – intermediate (rarely
metastatic) – malignant.
Most Common Entities
– Leiomyosarcomas (15–25%).
– Liposarcomas (10–15%).
– Pleomorphic sarcoma (formerly called myxoid fibrous histiocytoma,
MFH, 15–25%).

Staging and Grading

Staging
– TNM classification of the American Joint Committee on Cancer (AJCC)
and the Union Internationale contre le Cancer (UICC; see below).
– Criteria (update 2017).
– 4 tumor sizes (T1: ≤5 cm; T2: >5 ≤ 10 cm; T3: >10 ≤ 15 cm; T4: >15
cm).
– Tumor site (formerly a = superficial, b = deep): omitted!
– Lymph node metastasis.
– Distant metastasis.
Grading
– 3-level scoring system of the FNCLCC (French Federation of Cancer
Centers Sarcoma Group):
– G1 = Low-grade tumours.
– G2 and G3 = High-grade tumours.
UICC Stages According to the TNM Classification
– TNM classification of adult soft tissue sarcomas (AJCC/UICC, eighth
amendment 2017).

IA T1 N0 M0 G1
IB T2, T3 N0 M0 G1, GX
II T1 N0 M0 G2, G3
IIIA T2 N0 M0 G2, G3
IIIB T3, T4 N0 M0 G2, G3
IIIC Each T N1 M0 Each

14.2.5 Prognosis
Main Prognostic Determinants for Non-Metastatic Tumours
– Tumor size.
– Grading.

5-Year Survival for Patients with STS of the Extremities

– Allover approx. 70–75%.
– Depending on tumor size: <5 cm: 85%, 5–15 cm: 68%, >15 cm: 52%.
– Depending on the grading: G1: 80–90%, G2: 65–77%, G3: 42–50%.
– Depending on the UICC/AJCC stage: stage I: 85–96%, stage II: 72–78%,
stage III: 50% and stage IV: 10%.

14.2.6 Therapeutic Principles

Sarcoma Centre: Tumour Board—Interdisciplinary, Multimodal
Therapy
Key Points
– Oncosurgical resective surgery = central element of therapy.
 – Reconstructive surgery afterwards.
– Radiotherapy = additional local therapy.
– Combination of pre- and postoperative chemotherapy with regional
hyperthermia (± radiotherapy) for locally advanced tumor.
– Primary treatment at a certified STS centre = improved oncological
outcome.
– Specialized certified sarcoma tumor board (general surgery,
orthopedics, plastic surgery, radiotherapy, oncology, radiology,
pathology).
– Pre- and postoperative presentation.
– Diagnosis and complete staging.
– Multimodal therapy concepts (also for metachronous recurrences or
metastases).

Surgery
Oncosurgical Resective Surgery
Wide Excision
– Goal = local tumor control to prevent local recurrence.
– Outcome: strongly dependent on initial metastasis.

Surgical Procedure
Wide Sarcoma Excision
– Resection of the tumor with sufficient safety margin (a few mm).
– Remove biopsy access and drainage of previous operations as well.
– Orientation based on preoperative MRI and intraoperative palpation.
– Consider relation of tumor to anatomical boundary structures (fasciae,
intermuscular septa, epineurium, adventitia, periosteum) (achieve R0).
– Correct thread marking of the preparation at critical sites and
correlating clip marking in the tumor bed.
– Photographic documentation.
– No general recommendation for frozen section.

No STS resection of the limb possible without knowledge of reconstructive

options. Combination of the above techniques + radiotherapy allows limb
preservation in 90–95% of cases.
Caution
– Enucleation at the edge of the pseudocapsule (marginal excision) is
not an oncologic resection in sano!
– measurement of sufficient safety margin of a wide excision in sano is
not clearly defined.
– Substantial = resection of the tumor with a sufficient margin of
healthy tissue (= seeing the tumor itself).
Compartment Resection
– Radical removal of a complete compartment.
– largely abandoned (exaggerated radicality).
– Significant loss of function.
Limb Amputation
– Individual cases (“life before limb”).
– Reconstructive techniques (e.g. Borggreve reversal plastic or stump
lengthening using fillet flaps).
– Future inovations to be expected (bionic prosthetics).
Reconstructive Surgery
– Definition
– Reconstructive-restorative measure = everything that goes beyond
primary suture.
– Goal
– Undisturbed wound healing.
– Important for general rehabilitation and undelayed initiation of
adjuvant therapies.
Surface Restoration
– Indication
– Restoration of surface continuity = reconstruction of form and surface
as accurately as possible under functional-aesthetic aspects.
– Technique/Strategy
– Split-thickness skin grafting.
 – Flap plasty.
– Random SkinFlaps.
– Displacement-swivel-plasty for smaller defects.
– Axial flap plasty for larger defects.
– Microsurgical flap plasty with negligible lifting defect morbidity =
indispensable component of modern extremity reconstruction.
– Modern perforator flap plasties.
Functional Restoration
– Principle:
– Functional reconstructions = simultaneous to surface reconstruction.
– Nerve interposition (usually suralis cable graft interposition): For
sensitive reconstruction.
– Vascular replacement (proximal to elbow and knee by vascular
surgeons).
– Motor Reconstructions:
– Tendon interposition (palmaris longus or plantaris longus muscles).
– Free functional muscle transfers rarely indicated (e.g. free gracilis
transfer as motor long finger flexor replacement).

Caution
– Resection and reconstruction often possible in a single stage.
– MRI-guided surgical planning on the extremities = essential because
of possible defects that are difficult to close.

Tumor Recurrence Surgery

– Surgical treatment of local recurrence = same principles as treatment of
primary tumor.
– Renewed staging + renewed interdisciplinary discussion obligatory.
Tumor Metastases Surgery
– Lung = most frequent target organ of STS metastases.
– Resection of lung metastases = established procedure.
– Complete surgical resection of lung metastases = positive prognostic
factor.
Radiotherapy
Principle
– Without surgery no cure.
– Palliative radiotherapy only in inoperable patients.
– Additive or adjuvant radiotherapy = in addition to surgery.
– In R1 situation, reoperation to be preferred if technically possible.
Application
– Total dose 60–66 Gy with conventional fractionation.
– Radiation field = tumor site + safety margin + all scars + drainage exit
sites.
– Improved local control for G2 and G3 STS proven.
– Influence on overall survival = controversial.
– Rradiation therapy not indicated in R0-resected G1-STS.
– Do not irradiate complex reconstructions
Neoadjuvant Radiotherapy
– Equivalent to adjuvant radiotherapy in terms of local control.
– Smaller field size and lower dose (50 Gy).
– Fewer long-term consequences (fibrosis, edema) for soft tissue.
– For irresectable tumors: Consider multimodal combinations
(radiochemotherapy or limb perfusion).

Caution
With neoadjuvant radiotherapy, the rate of early postoperative
(sometimes severe) wound healing disorders of the lower extremity is
significantly increased!

Intraoperative Radiotherapy
– Limited availability.
– Targeted application of a single dose of 12–20 Gy into tumor bed.
– Always in combination with neoadjuvant or adjuvant radiotherapy.
– Reduces percutaneous residual dose.

Chemotherapy
– STS generally little chemosensitivity.
– Exception: small, blue, round cell sarcomas such as extraosseous Ewing
sarcoma, rhabdomyosarcomas, primitive neuroectodermal tumors
(PNET) and desmoplastic, small and round cell tumors with a clear
recommendation for neoadjuvant chemotherapy.
– “Molecular targeted therapy” very limited (Imatinib for
dermatofibrosarcoma protuberans, Sorafenib for angiosarcoma).
– Limited benefit of chemotherapy: No general recommendation.
Hyperthermia/Isolated Limb Perfusion
– Combination of pre + postoperative chemotherapy with regional
hyperthermia (± radiotherapy).
– In locally advanced soft tissue sarcomas: improvement of local tumor
control and progression-free survival.
– Isolated limb perfusion (ILP) with TNF-α and melphalan.

Tumor Follow-up
– Department of General Surgery, University Hospital Basel,
BaselIndividualized, risk-adapted follow-up care for at least 10 years.
Local Tumor Follow-up
– Regular clinical examination.
– MRI.
Systemic Tumor Follow-up
– Regular oncological follow-up.
– every 6 months Chest Imaging.
– FDG-PET in individual cases.

14.2.7 Guidelines
AWMF soft tissue sarcoma guideline registry number 025–007; 3/2017; ►
https://​www.​awmf.​org/​leitlinien/​detail/​ll/​025-007.​html.
Kandel R, Coakley N, Werier J, Engel J, Ghert M, Verma S; Sarcoma
Disease Site Group of Cancer Care Ontario’s Program in Evidence-Based
Care (2013) Surgical margins and handling of soft-tissue sarcoma in
extremities: a clinical practice guideline. Curr Oncol 20: e247-e254.
 Schütte J, Hartmann JT, Reichardt P, Issels RD, Tunn PU, Budach V
(2011) Soft tissue sarcomas, DGHO guideline. ► https://​www.​dgho-
onkopedia.​de/​de/​onkopedia/​leitlinien/​weichteilsarkome​.

14.3 Retroperitoneal Sarcomas

A. Tampakis and C. Kettelhack

14.3.1 Epidemiology and Prognosis

Epidemiology
– 0.2–0.3% of all malignancies in adults.
– Annual incidence = 0.3/100,000 population (estimated).
– 15% of all soft tissue sarcomas.
– 1/3 of malignant retroperitoneal tumors.
– Associated genetic diseases:.
– Li-Fraumeni Syndrome
– Familial adenomatous polyposis.
– Gardner Syndrome.
– Carney-Stratakis Syndrome.

Prognosis and Prognostic Factors

– 5-year survival rate = 64.6%.
– Prognostic factor for overall survival, 5-year survival, and survival after
relapse = histologic subtype: atypical lipomatous tumor (ALT, well-
differentiated liposarcoma) vs. non-ALT liposarcoma vs. other subtypes.
– Median time to locoregional recurrence (23% at 5 years) = 41 months.
– Prognostic factors for local recurrence = size, histologic grade, and
completeness of surgical resection.
Tumor size + malignancy grade = most important prognostic factors for
overall survival.

14.3.2 Pathology
– Histology of retroperitoneal sarcomas (RPS): well-differentiated
liposarcomas, malignant fibrous histiocytomas, dedifferentiated
 liposarcomas, leiomyosarcomas, malignant peripheral nerve sheath
tumors, solitary fibrous tumors, and other sarcomas (◘ Table 14.2).
Table 14.2 Pathology of retroperitoneal sarcomas. (According to Nathan et al. 2009)

Histology Number (n) Share (%)

Liposarcoma 682 50
Leiomyosarcoma 358 26
Malignant fibrous histiocytoma 146 11
Fibrosarcoma 24 2
Rhabdomyosarcoma 21 2
MPNST 15 1
Hemangiopericytoma 13 <1
Hemangiosarcoma 10 <1
Malignant mesenchymoma 5 <1
Sarcoma NOS 91 7
Total 1365 100

MPNST malignant peripheral nerve sheath tumors, NOS not otherwised

specified

14.3.3 Classification
AJCC/UICC
– TNM and staging.
– Malignancy grade: High significance in sarcomas.
– Pathology of retroperitoneal sarcomas (AJCC Soft Tissue Sarcoma
Staging System; Feig and Ching 2012).

TNM Classification
– Primary tumor (T).
– Tx Primary tumor cannot be assessed.
– T0 No evidence of primary tumor.
– T1 tumor ≤5 cm in the largest diameter.
– T1a Above and no invasion of the fascia.
– T1b Invasion of the fascia or below the fascia.
 – T2 Tumor >5 cm in largest diameter.
– T2a Above and no invasion of the fascia.
– T2b Invasion of the fascia or below the fascia.
– Regional lymph nodes (N).
– Nx Regional lymph nodes cannot be assessed.
– N0 No regional lymph node metastases.
– N1 Regional lymph node metastases.
– Distant metastases (M).
– Mx distant metastases cannot be assessed.
– M0 No distant metastases
– M1 distant metastases.
– Histopathological grading (G).
– Gx: Grading cannot be assessed.
– G1: Grade 1.
– G2: Grade 2.
– G3: Grade 3.

UICC Stages According to the TNM Classification

Stage IA T1a, T1b N0 M0 G1, Gx
Stage IB T2a, T2b N0 M0 G1, Gx
Stage IIA T1a, T1b N0 M0 G2, G3
Stage IIB T2a, T2b N0 M0 G2
Stage III T2a, T2b N0 M0 G3
Each T N1 M0 Each G
Stage IV Each T Each N M1 Each G

14.3.4 Molecular Genetics

– Mesenchymal origin.
– Dysregulation of gene expression by aberrant chimeric transcription
factors.
– Genetic-etiological 2 main categories:
– Tumor-specific translocation.
 – Complex karyotypes (= characteristic feature of severe genetic +
chromosomal instability).

14.3.5 Clinical Presentation

Early Symptoms
– Mostly missing.
– Due to large expansion potential of the retroperitoneum and the
abdominal cavity (= progressive growth of retroperitoneal sarcomas
unnoticed until advanced stages).
– At diagnosis: Retroperitoneal sarcomas >20 cm = 50%.

Clinical Symptoms (If Present)

– Mostly due to compression/invasion of neighbouring structures.
– abdominal distension.
– Changes in bowel movements.
– Abdominal pain.
– Weight loss/anemia (occasionally).

Caution
– In case of fever + night sweats: think of lymphoma as differential
diagnosis.
– Testicular examination (clinical examination + ultrasound if
necessary) in men = important, since retroperitoneal metastases
from a primary gonadal tumor often present similarly.

Guidelines (main guidelines)

– NCCN(The National Comprehensive Cancer Network) Guidelines from
2014.
– European Consensus Guidelines of ESMO (European Society for
Medical Oncology) 2014.

14.3.6 Diagnosis
Medical History and Clinical Examination
– Exclusion of lymphoma signs (see above).
– Clinical Examination:
– Draining lymph node groups.
– Testis: Exclusion of metastatic/advanced testicular tumor…
– Laboratory test: LDH + AFP + βHCG to exclude lymphoma/germ cell
tumour.

Radiology
Multiphase Spiral CT scan
– Abdomen/Retroperitoneum/Pelvis: Most important diagnostic procedure.
– Thorax: for the detection of lung metastases/staging.
MRI
– Essential for vascular assessment, delineation of compartments, fasciae,
nerves.
PET/PET-CT
– No required as standard examination.
– Potential use for response assessment in preoperative therapy.
– Possible use to exclude distant metastases.
Renal Scintigraphy
– Examination of the split renal function.
– Preparation for possible nephrectomy for en bloc multivisceral
resections.

Biopsy
– Ideally preoperative image-guided punch biopsy.
– If large tumor heterogeneity on CT: Multiple biopsies.
No evidence for worsening of prognosis by percutaneous punch biopsy:
In retroperitoneal sarcomas no influence of biopsy on local recurrence,
disease-free survival, overall survival.
NCCN Guidelines
– In case of clearly resectable tumor: Surgery also justifiable without prior
biopsy to confirm the diagnosis.

Caution
Surgical resection without prior biopsy: Only after interdisciplinary
discussion in the sarcoma tumor board.

– Biopsy necessary before starting preoperative radio- or chemotherapy.

– Open incisional biopsy by laparoscopy or laparotomy not indicated.

14.3.7 Therapy
Surgical Therapy
– Surgical tumor resection with tumor-free resection margins = only
curative therapy option.
– Incomplete resection = direct effect on outcome:
– Increased risk of recurrence (mortality usually associated with local
recurrence).
– Increased risk of distant metastasis.
– Worse overall survival.
Macroscopic complete resection possible in 40–60% of cases: Due to
narrow anatomical relationship + locally advanced tumor extension.
En Bloc Organ Resection
– Traditionally only in case of direct infiltration through the tumor
Principle En Bloc Resection
– Tumors + adjacent organs (e.g. kidney, colon, psoas muscle, small
intestine, pancreas tail, diaphragm).
– Critical structures: resection only in case of direct infiltration
(duodenum/pancreas head, liver, stomach, large abdominal vessels and
nerves, bones).
– Results: Significantly fewer locoregional tumor recurrences, no effect on
survival rates.
Palliative resections to improve quality of life possible (e.g. for
symptoms such as ileus, bleeding).
Morbidity and Mortality
– Morbidity 22–26% and perioperative mortality 1.7–3%.
– Most common complications:
– Anastomotic insufficiencies.
– Paralytic ileus.
– Retroperitoneal fluid collections.
– Bleeding.

Independent Predictive Factors Associated with a Lower

Locoregional Tumor Recurrence Rate
– Complete tumor resection without intraoperative tumor rupture.
– Low-grade tumor.
– Histologically confirmed negative resection margins.
– Number of cases treated annually per institution; so-called hospital
volume.
– Radical surgical strategy.
For completely resected RPS: 5-year local recurrence-free survival
(LRFS) = 55%; 5-year distant metastasis-free survival = 66–79%.

Radiotherapy
Preoperative Radiotherapy (PrR)
Main Objectives of the PrR
– Increase in resectability.
– Local control.
– Lack of level 1 evidence for PrR
– Incidence of PrR use for RPS: 3% in 2005; 10% in 2011.
Theoretical Advantages of PrR (Nussbaum et al. 2014)
– Irradiation field aligned more precisely.
– Thickness of the tumor pseudocapsule increases after irradiation = better
imageable margin layer between healthy tissue and tumor.
– Reduction of tumor size = higher probability of R0 resection.
– Morbidity and mortality after RPS resection + previous PrR: Not
significantly increased.
– Mean dose of PrR in studies: from 45 to 50.4 Gy
– PrR tolerated with selective dose increase in the tumor area (“boost”)

Intraoperative Radiotherapy (IORT)

Principle
– Displacement/Protection of sensitive normal tissue.
– Use of individualized shields.
– Limited penetration depth of electrons: Use of a biologically effective
single large dose of radiation by IORT.
Limits of the IORT
– Limited availability of technology.
– Lack of data.
– ESMO guidelines: Efficacy of intraoperative irradiation not sufficiently
proven so far, therefore not recommended outside studies
– NCCN Guidelines: IORT with or without external beam radiotherapy
effective in local control and survival in patients with primary and
recurrent RPS
Postoperative Radiotherapy (PostR)
– Indications
– Narrow (Rx) or positive (R1) resection limits.
– Especially recommended for high-grade tumors.
– General efficacy in retroperitoneal tumors = not sufficiently documented.
– Not indicated in the presence of metastases.
– ESMO Guidelines (2014): Adjuvant radiotherapy of limited value +
significant short- and long-term toxicity: therefore use only in very
selective cases.
– If PostR planned: Recommendation to use omentum or other
placeholders to move the bowel out of the tumor bed = reduction of the
risk of radiation-induced bowel toxicity.

Chemotherapy
Preoperative Chemotherapy
– No recommendation for neoadjuvant therapy.
 Phase 3 multicenter randomized trial (Gaspar et al. 2015): Neoadjuvant
chemotherapy (etoposide + ifosfamide + doxorubicin with/without regional
hyperthermia) = better local tumor control in patients at high risk of tumor
recurrence (grading 2 and 3, tumor diameter > 5 cm).
Adjuvant Chemotherapy
– No indication for adjuvant chemotherapy in retroperitoneal sarcomas.

14.3.8 Management of Recurrences

Incidence
– Recurrence = in 2/3 of patients.
– Local in tumor bed.
– Metastases: Lung, liver.
– Sarcomatosis: Diffuse recurrence in the peritoneal cavity.
– In up to 40% of patients: Recurrence later than 5 years after initial
surgery.

Treatment of the Recurrence

– If resectable: goal should be R0 resection.
– Isolated liver metastases: Resection/RFTA (radiofrequency
thermoablation)/chemoembolization possible (if stable for months).
Resectability of local recurrence = decreasing with each recurrence:
57% of patients at first recurrence, 20% at second recurrence, 10% at third
recurrence.
– Most important outcome predictor in local recurrence = resectability of
recurrent tumors: median survival = 60 months in operated patients vs.
20 months in non-operated patients.

14.3.9 Follow up
Evidence
– Recommendations for follow-up = not sufficiently evidence-based.
– No evidence of improvement in recurrence-free or overall survival with
intensive follow-up.
 – Risk of recurrence and metastasis: highest in the first 2 years after
primary surgery.

Strategy
– In high-risk patients:
– CT (MRI) scan of the abdomen (and thorax) recommended at 3- to 4-
month intervals for 2 years.
– 6 months in the third year, then annually.
– After chemotherapy:
– Laboratory examination every 3 months for the first 2 years, every 6
months in the third and fourth year and annually from the fifth year
onwards.

14.3.10 Guidelines
AWMF soft tissue sarcoma guideline registry number 025–007; 3/2017; ►
https://​www.​awmf.​org/​leitlinien/​detail/​ll/​025-007.​html
ESMO (2014) European Consensus Guidelines. ► https://​www.​nccn.​
org
NCCN (2014) Guidelines. ► http://​oncologypro.​esmo.​org/​Guidelines-
Practice/​ESMO-Consensus-Conferences

References
Feig BW, Ching CD (2012) The MD Anderson surgical oncology handbook, 5th edn. Lippincott,
Williams and Wilkins, Philadelphia/Baltimore/New York/London

Fletcher CD, Berman JJ, Corless C et al (2002a) Diagnosis of gastrointestinal stromal tumors: a
consensus approach. Hum Pathol 33:459–465
[Crossref][PubMed]

Miettinen M, Lasota J (2001) Gastrointestinal stromal tumors – definition, clinical, histological,

immunohistochemical, and moleculargenetic features and differential diagnosis. Virchows Arch
438:1–12
[Crossref][PubMed]

Fletcher CDM, Unni KK, Mertens F (2002b) Pathology and genetics of tumours of soft tissue and
bone. In: WHO classification of tumours, international agency for research on cancer. IARC Press,
Lyon
Gaspar N, Hawkins DS, Dirksen U et al (2015) Ewing sarcoma: current management and future
approaches through collaboration. J Clin Oncol 33(27):3036–3046
[Crossref][PubMed]

Nathan H, Raut CP, Thornton K, Herman JM et al (2009) Predictors of survival after resection of
retroperitoneal sarcoma: a population-based analysis and critical appraisal of the AJCC staging
system. Ann Surg 250:970–976
[Crossref][PubMed]

Nussbaum DP, Speicher PJ, Gulack BC et al (2014) The effect of neoadjuvant radiation therapy on
perioperative outcomes among patients undergoing resection of retroperitoneal sarcomas. Surg Oncol
23:155–160
[Crossref][PubMed][PubMedCentral]

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_15

15. Perioperative Medicine

Maren Rudat1 and Sebastian Stehr2
(1) Interdisciplinary Intensive Care Unit, Sana Kliniken Ostholstein, Eutin
Hospital, Eutin, Germany
(2) Department of Anesthesiology and Intensive Care Medicine,
University Hospital Leipzig, Leipzig, Germany

Sebastian Stehr
Email: [email protected]

15.1 Preoperative Phase

Key Points
– Risk stratification (patient-related, surgery-related risk factors,
medication, laboratory chemistry).
– Determination of perioperative anticoagulation and medication
management.

15.1.1 Risk Stratification

– Patient-related surgical risk depending on the patient’s state of health and
the invasiveness of the operation.

Identification of Patient-Related Risks

– Careful anamnesis, thorough examination.
– Presentation in the anaesthesia department (ideally max. 6 weeks before
surgery).
– Initiate further diagnosis.
– General condition of the patient according to ASA classification:
– Description of preoperative health status, 1 (healthy patient) to 6
(brain-dead patient at organ removal ◘ Table 15.1).
Table 15.1 ASA (American Society of Anesthesiologists) classification

Class Criteria
ASA 1 Normal, healthy patient
ASA 2 Patient with mild general illness
ASA 3 Patient with severe general illness
ASA 4 Patient with a severe general illness that is a constant threat to life
ASA 5 Moribund patient unlikely to survive without surgery
ASA 6 Brain-dead patient whose organs are removed for organ donation

Systemic Diseases with High Postoperative Risk

– Cardiovascular System:
– Various scores: RCR (Revised Cardiac Risk) index according to Lee
(Lee et al. 1999), NYHA (New York Heart Association) classification,
MET (metabolic equivalent).
– Risk factors for cardiovascular complications: Coronary artery disease
(CAD), heart failure, fresh myocardial infarction, chronic venous
insufficiency (CVI), insulin-dependent diabetes mellitus (IDDM),
chronic renal failure, cardiac arrhythmia (CAr), fresh insult.
– Diagnosis: 12-lead ECG, echocardiography, exercise ECG, coronary
angiography, carotid Doppler, chest X-ray.
– Pulmonary disease:
– Different scores for the prediction of postoperative ventilation risk and
re-intubation.
– Increased risk in chronic obstructive pulmonary disease (COPD),
asthma, obesity, obstructive sleep apnoea syndrome (OSAS) and
smokers.
– Diagnosis: chest X-ray, blood gas analysis BGA, lung function test.
– Diabetes mellitus:
– Perioperative normoglycemic setting: blood glucose (BG) = 140–180
mg/dl and close-meshed BG controls.

Perioperative Risk (◘ Table 15.2)

– Depending on invasiveness, duration and possible blood loss.
– Mild: minor endoscopic and outpatient procedures, breast surgery.
– Moderate: intraperitoneal, intrathoracic surgery, orthopedics.
– High: Aortic surgery, vascular surgery.
Table 15.2 Risk of perioperative myocardial infarction or death within 30 days after surgery

Low <1% Mean 1–5% High >5%

Chest Visceral surgery Aorta
Tooth Carotid Major vascular surgery
Eye Angioplasty PAOD
Gynecology Endovascular aneurysms Esophagectomy
Minor orthopedic surgery (knee) Head and neck Cystectomy
Urology Major orthopedic surgery Pneumonectomy
Transplant
Major urological surgery

PAOD peripheral arterial occlusive disease

Intubation Conditions
– To estimate a possible difficult airway.
– Mallampati score (relation of tongue size to pharynx).
– Head reclination.
– Mouth opening.
– 3-3-2 rule (mouth opening >3 fingers, os hyoideum chin distance ≥3
fingers, thyromental distance >2 fingers).
– Aspiration risk.

15.1.2 Laboratory and Blood Products

Blood Management
– Preoperative anemia prevalence: approx. 30%.
– Risk factor for perioperative morbidity and mortality.
– Preoperative improvement through “patient blood management” by
increasing erythropoiesis (erythropoietin, iron substitution).
– Preoperative blood transfusion.
– Preparation of packed red blood cells (PRBCs) depending on the planned
procedure.
– Caution: PRBCs administration: increased morbidity, mortality and risk
of complications.

Laboratory Diagnosis
– Blood tests dependent on:
– Age.
– ASA classification (see above).
– Operation.
– Risk profile of the patient.
– Small blood count, electrolytes, coagulation, BG, creatinine,
transaminases.
– No routine screening.

15.1.3 Additional Investigations

ECG
– Preoperative ECG not necessary in asymptomatic and anamnestically
unremarkable patients.
– ECG recommended for
– Patients with cardiac symptoms and/or
– Abnormal cardiac history.

Chest X-Ray
– Indicated for new onset or acutely symptomatic respiratory symptoms.

Pulmonary Function Diagnosis

– Indicated for severity assessment in new-onset or acutely symptomatic
pulmonary disease, lung surgery.
15.1.4 Perioperative Anticoagulation
Coronary Artery Disease and Stent Implantation
– Metal stents: dual platelet aggregation inhibition up to 3 months.
– DES (Drug Eluting Stent): Time interval up to 12 months.
– If possible, no elective operations during this period.
– Commonly used: Acetylsalicylic acid (ASA), dipyridamole, clopidogrel,
prasugrel, ticagrelor.
– Perioperative discontinuation: Increased rate of cardiovascular events
due to rebound phenomenon = continue ASA.
– Bleeding risk:
– ASA only moderate bleeding risk, exceptions: NCH (neurosurgery),
prostate resection…
– Dual platelet aggregation = high risk of bleeding: discontinue 7–10
days prior to major procedures, procedures in closed body cavities,
and spinal anesthesia close to the spinal cord.

Perioperative Thrombosis Prophylaxis

– Venous thromboembolism = still clinically relevant complication.
– The more morbid the patient, the higher the risk of thrombosis.
– Incidence can be reduced by 50% through prophylaxis.
– Parenteral: Unfractionated heparin (UFH), low-molecular-weight heparin
(LMWH), Fondaparinux.

Caution
Heparin-induced thrombocytopenia type II (HIT II) in UFH and
LMWH.

– Alternative anticoagulants:
– Argatroban (Agarta): for HIT II.
– Bivalirudin (Angiox): Alternative to UFH for coronary intervention.
– Phenprocoumon.

New Oral Anticoagulants (NOACs/DOACs)

– Pradaxa (dagibatran etexilate) and Xarelto (rivaroxaban) for:
– Knee and hip joint replacement.
– Stroke prophylaxis in atrial fibrillation.
– Therapy for venous thromboembolism.
– Eliquis (apixaban) approved for knee and hip replacements.

15.1.5 Medication Management

Continue
– Antianginal, antihypertensive and antiarrhythmic medication:
– Beta-blockers: otherwise increase in mortality due to rebound
phenomenon; preoperative new medication can be considered with
sufficient distance to surgery and high-risk patients.
– Calcium antagonists: Otherwise possible preoperative blood pressure
increase.
– Nitrates: risk of myocardial ischemia.
– Antiarrhythmic drugs: risk of arrhythmias.
– Corticosteroids:
– Continue substitution for longer than 5 days if substitution is already
in place.
– In addition, 50–200 mg hydrocortisone perioperatively over 48 h in
patients with long-term medication above the Cushing’s threshold,
depending on the severity of the procedure.
– Statins:
– Continue perioperatively.
– New prescription after vascular surgery.
– Reduce perioperative risk of infarction.
– Anticonvulsants: triggering seizures.
– Parkinson’s drugs: enhancement of extrapyramidal symptoms.
– Thyroid hormones.
– Psychotropic drugs:
– Tricyclic antidepressants.
– Neuroleptics.
 – Selective serotonin reuptake inhibitors.
– Third generation MAO (monoamine oxidase) inhibitors.

Caution
Perioperative drug interaction.

Convert
– Phenprocoumon: convert to heparin 3–5 days before surgery.
– MAO inhibitors: Switch to reversible and selective third generation
MAO inhibitors 2 weeks prior to surgery.

Discontinue
– Diuretics
– Otherwise risk of hypovolaemia with hypotension.
– Immediate restart after surgery in stable patients.
– ACE (angiotensin converting enzyme) inhibitors and AT II (angiotensin
II) blockers
– Danger of perioperative hypotension in operations with high volume
shifts, otherwise due not discontinue.
– Digitalis
– Controversially discussed.
– Long half-life, short-term discontinuation associated with little
benefit.
– Continue in patients with normofrequency absolute arrhythmia.
– OAD (oral antidiabetic drugs)
– Risk of hypoglycaemia: Regular perioperative BG measurements.
– Metformin: Risk of lactic acidosis, discontinue 48 h before surgery.

Endocarditis Prophylaxis
– Depends on operation and patient-related risk
– Patients with valve replacements (mechanical and biological
prostheses), patients with reconstructed valves using grafts for 6
months after surgery.
 – Patients after endocarditis.
– Patients with congenital heart defects (cyanotic, postoperative).
– Patients after heart transplant, with cardiac valvulopathy.
– For interventions in the gastrointestinal tract or urinary tract
– Prophylaxis only in cases of an infection of these organs.
– Amoxicillin: 2 g single dose 60 min before surgery.
– In case of penicillin or ampicillin allergy: Clindamycin 600 mg.

15.1.6 Information from the Anaesthetist’s Point of View

Legal Situation (Germany)
– Any medical interference with bodily integrity: criminal offence of
bodily harm.
– Consent of the patient only legal after detailed explanation and
documentation.
– If possible, 24 h before planned surgical intervention.

Prerequisite
– The patient has to understand and decide, voluntariness.
– Patients who have reached the age of majority and have the capacity to
consent and make decisions.
– In the case of minors and persons incapable of giving consent: Parents,
legal guardians.

Requirement
– Explanation of the relevant information.
– Procedure with risks typical of procedures and anaesthesia.
– Various therapy options with risk-reward consideration.
– Understanding the patient.
– 3 phases of enlightenment according to hick:
– Comprehensive information.
– Summary.
– Decision of the patient.

Elements of Consent
– Decision for a course of action (alternatives).
– Placement of the treatment order.
– Caution: Documentation is obligatory (in writing).

Outpatient Interventions
– For minor surgery: Consent is possible directly prior to surgery (without
premedication).

Living Will or Health Care Proxy

– For major procedures or anticipated intensive care stays, inquire about.

15.2 Intraoperative Phase

Key Points
– Essential intraoperative monitoring.
– Central importance of volume management, thermal homeostasis and
hemodynamics.

15.2.1 Intraoperative Monitoring According to AAGBI and

BDA Guidelines
– WHO checklist, team time-out before start of surgery.
– Essential Equipment:
– Ventilation system with CO2 –, O2 – and ventilation pressure
measurement.
– Pulse oximetry.
– NIBD (non-invasive blood pressure measurement).
– ECG.
– Relaxometry.
– Temperature measurement.
– Defibrillator and cardiopulmonary resuscitation equipment.
– Infusion pumps.
– In addition, according to the severity of the intervention and the
morbidity of the patient:
– Invasive blood pressure measurement and haemodynamic monitoring.
– Transesophageal Doppler.
– Cerebral measurement of oxygen saturation.
– Blood glucose meter.
– BIS (Bispectral Index) Monitor.

15.2.2 Volume Management

– Avoid preoperative exsiccosis and malnutrition.
– Hemostasis and coagulation management.
– Surgical technique and careful haemostasis decisive.
– Implementation of an evidence-based perioperative transfusion regime.
– Measures to save foreign blood.
– Normothermia and avoidance of acidosis.
– if necessary, use of hemostatic drugs (tranexamic acid, Minirin).

15.2.3 Hemodynamics
Pathophysiology
– MAP (mean arterial pressure) <60 mmHg: decrease in cerebral and renal
blood flow.
– Critical perfusion pressure of the coronaries dependent on cardiac output
(CO).

Risk Factors for Hypotension

– Age.
– ASA classification.
– Duration of the operation.
– Combined regional and general anaesthesia.
– Premedication.
– Storage.
– Intraoperative hypotension associated with increased 1-year mortality.

Principles/Goals
– MAP >60 mmHg, in hypertensive patients >80 mmHg.
– Early initiation of volume and catecholamine therapy, if necessary with
hemodynamic monitoring.

15.2.4 Heat Retention

– Perioperative hypothermia = risk factor for
– Worsened outcome.
– Wound healing disorder.
– Extended length of stay in hospital.

15.2.5 Perioperative Antibiotic Therapy

Incidence of SSI (“Surgical Site Infection”)
– Wound healing disorders in approx. 10% of all operations.
– 16% of all nosocomial infections.
– Up to 24.5% after gastrointestinal surgery.
– Lead to longer hospital stays.
– Additional costs.

Risk Factors
– Patient-Related:
– Diabetes mellitus.
– Obesity.
– Clotting disorder.
– Age.
– Malnutrition.
– Medication.
– Patient-independent:
– Hygiene standards.
– Operating time.
– Inadequate perioperative antibiotic therapy.

Pathogen Spectrum
– According to the type and location of the intervention
– Frequently mixed infections with enterobacteria, approx. 2/3 of all
infections by: E. coli, Enterococcus spp., Bacteroides spp.,
Pseudomonas aeruginosa.
– Multi-resistant germs:
– MRSA (methicillin-resistant Staphylococcus aureus).
– MRSE (coagulase-negative staphylococci with methicillin/oxacillin
resistance).
– VRE (vancomycin-resistant enterococci).
– ESBL (extended spectrum beta-lactamases).

Prevention
– Avoid preoperative medications: NSAIDs (non-steroidal anti-
inflammatory drugs), chemotherapy, phenprocoumon.
– Optimize concomitant diseases.
– Perioperative antibiotic administration.
– Hygiene measures: Hand disinfection, area clothing, asepsis.
– Wound closure without impairment of local blood circulation.
– Drains as short as possible in situ.

Caution
No recommendation for irrigation of the abdominal cavity before
wound closure.

Perioperative Antibiotic Prophylaxis (PAP)

– Requirement: bactericidal, i.v. application, tolerable
– Two goals:
– Reduction of bacteria introduced into the surgical area.
– Prevention of systemic germ introduction.
– Antibiotic of choice: aminopenicillins plus beta-lactam inhibitor or first
or second generation cephalosporins.
– Second choice antibiotic: third or fourth generation cephalosporins in
combination with metronidazole or carbapenem.
– Time of application: 1 h before to 2 h after skin incision.
Caution
Vancomycin or fluoroquinolones have a longer infusion duration (60
min).

– In case of bacteriological sample collection (e.g. blood culture),

administration after sample collection.
– 1–2 doses only for 24 h after surgery, if necessary only single dose.
– For long operations second dose intraoperatively.

15.3 Postoperative Phase

Key Points
– Determination of a strategy for postoperative analgesia.
– Prevention/treatment of PONV, delirium, POCD.
– Principles of fast-track surgery.

15.3.1 Analgesia
Pathophysiology
– Prevention:
– Delirium.
– Chronification.
– Cardiorespiratory problems.
– Delayed mobilization.

Evidence-Based Analgesia
– Evidence-based analgesia positive for:
– Earlier hospital discharge.
– Reduce morbidity.

Pain Measurement
– Measurement of pain by:
– VAS (visual analogue scale).
– NRS (numerical rating scale).
 – If possible 2-hourly in the first 24 h.

Caution
Increase in pain or new onset of increased analgesic consumption:
indication of complications.

Principles
– Individual adaptation to patient, comorbidity.
– Stepwise therapy according to WHO analgesic ladder.
– Administration of opioids as sparingly as possible.
– Use coanalgesics such as clonidine, spasmolytics.
– Prefer perioperative epidural anesthesia.

15.3.2 Postoperative Nausea and Vomiting (PONV)

Forecasting Systems
– For the assessment of postoperative nausea and vomiting.
– E.g. Apfel Score:
– Female.
– History of PONV/kinetosis.
– Non-smoker.
– Opiate administration.

Prophylaxis
– Regional anaesthesia, no volatile anaesthetics, avoid opiates.
– Medications:
– Corticosteroids (dexamethasone).
– 5-HT3 antagonists: administration at the end of surgery.
– No butyrophenones or benzamines because of possible
extrapyramidal motor effects.
– Adjuvants: Acupuncture/acupressure on the wrist, aromatherapy, ginger.

Therapy
– Quick action.
– 5-HT3 antagonists as first-choice drugs.
– Dexamethasone only slow onset of action, only in combination.
– Alternative: haloperidol, dimenhydrinate, promethazine.

15.3.3 Delirium/Postoperative Cognitive Deficit (POCD)

Epidemiology
– Prevalence 15–50%, ventilated patients up to 80%.
– Longer hospital stay, increased mortality, and cognitive late effects on
long-term follow-up.

Division
– Three types:
– Hyperactive.
– Hypoactive.
– Mixed type.
– Three forms of postoperative cognitive deficit:
– Emergence Delirium: at discharge.
– Postoperative delirium.
– Transient cognitive impairment.

Preoperative Evaluation of Risk Factors

– Age.
– Morbidity.
– Cognitive skills.
– Severity of the surgical procedure.
– Hypoxia.
– Infection.

Prevention
– Avoid preoperative food restriction and fluid deficit.
– Stress avoidance (isolation, lack of daylight, restraint).
– Communication aids (glasses, hearing aid).
– Early mobilization.
– Avoid prodelirant drugs (e.g. benzodiazepines, opiates, sedative
hypnotics).

Early Screening
– CAM-ICU (Confusion Assessment Method/Intensive Care Unit), ICU:
– Acute onset or fluctuating course.
– Attention Deficit Disorder.
– Changes in awareness.
– Disorganized thinking.
– Nu-DESC (Nursing Delirium Screening Scale), PACU.

Therapy
– Most important tool: Recognition of delirium.
– Reduce risk factors.
– Strengthen reorientation.
– Drug therapy:
– Haloperidol.
– Atypical neuroleptics.
– Dexmedetomidine.
– Caution: Haloperidol: QT time, extrapyramidal symptoms at more than
4.5 mg/day.

15.3.4 Recovery Room (PACU)

– Regular documentation of vital parameters.
– Surveillance:
– State of alertness according to AVPU (“alert, voice, pain,
unresponsive”) scheme, protective reflexes present.
– Circulatory situation: blood pressure, heart rate, ECG.
– Airway: pulse oximetry, oxygen supply and if necessary airway
protection e.g. by Wendl tube.
– Assessment of dressings and drains.
– Recognize and Treat:
– PONV.
 – Shivering.
– Restlessness.
– Postoperative pain.
– Transfer if:
– Patient awake, cooperative, preserved protective reflexes.
– No more risk from anaesthesia and perioperative respiratory or
circulatory problems.
– Discharge criteria met.
– Responsibility for discharge lies with anaesthetist.
– Transfer to another ward/home.

15.3.5 Intensive Care Unit (ICU)/Intermediate Care (IMC)

– In addition to the tasks of the PACU listed above:
– Ward with monitoring and treatment of patients after extensive
operations.
– Monitoring and treatment of patients with high morbidity/mortality
after minor surgery.
– Circulatory the rapy.
– Weaning.
– Pre-operative stabilisation and preparation for surgery.
– Organ-specific support.

15.4 Fast Track Surgery

Key Points
– Evidence-based multimodal interdisciplinary perioperative treatment
concept.
– Goals = Shortening of treatment duration + Reduction of perioperative
complications.

15.4.1 Definition
– Multimodal interdisciplinary perioperative treatment concept according
to defined clinical treatment algorithms.
– Objectives
– Shortening the duration of treatment.
– Reduction of perioperative complications.

15.4.2 Preoperative Management

– Short preoperative food abstinence.
– Premedication with short-acting substances.

15.4.3 Intraoperative Management

– Atraumatic surgical technique.
– Anaesthetic guidance with short-acting substances.
– PONV prophylaxis.
– Peridural analgesia: Improves perioperative mortality and reduces tumor
recurrence rate.
– Balanced volume therapy.
– Caution: Intestinal edema.
– Avoid hypothermia.
– Periopertiave antibiotic prophylaxis.

15.4.4 Postoperative Management

Analgesia
– Peridual anesthesia instead of systemic opiate administration.
– Analgesia according to WHO stage scheme.

Caution
Gastrointestinal bleeding is possible with NSAIDs.

Early Mobilization
Optimized Diet
– Epidemiology: mortality rate in ICU patients with gastrointestinal failure
43.7% vs. 5.3% without gastrointestinal failure.
– Pathophysiology:
– Operation = motility disorder.
 – Motility disorder = passage disorder (bacterial density increased) +
barrier function impaired.
– Causes of motility disorder:
– Drugs (opiates).
– Immobilization.
– Electrolyte derailments.
– Shock.
– Inflammation of the intestinal wall due to cytokine release also
during surgical interventions.
– Bowel wall edema.
– Increased sympathetic tone with vasoconstriction in the splanchnic
area.
– Fast track therapy.
– Keep alimentation interruption as short as possible.
– Start enteral nutrition early.
– Laxatives (lactulose, macrogol).
– Prokinetics (metoclopramide, erythromycin, neostigmine).
– Opiate receptor antagonist (Relistor).

15.4.5 Guidelines
ESC/ESA (2014) Guidelines on non-cardiac surgery: cardiovascular
assessment and management. Eur Heart J 35:2383–2243.

Reference
Lee TH et al (1999) Derivation and prospective validation of a simple index for prediction of cardiac
risk of major noncardiac surgery. Circulation 100:1043–1049
[Crossref][PubMed]

OceanofPDF.com
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery
https://doi.org/10.1007/978-3-662-66735-4_16

16. Emergency Surgery

Benjamin Weixler1 , Raoul A. Droeser2 , Robert Mechera2,
Christian A. Nebiker3 , Debora Nowakowski4 , Heidi Misteli5 and
Henry Hoffmann6
(1) Department of General and Visceral Surgery, Campus Benjamin
Franklin, Charité—University Hospital Berlin, Berlin, Germany
(2) Clarunis—Universitäres Bauchzentrum Basel, University Hospital
Basel, Basel, Switzerland
(3) Department of General Surgery, Hospital Aarau, Aarau, Switzerland
(4) Department of Surgery, Hospital Baselland, Basel, Switzerland
(5) Department of Surgery, Hospital Uster, Uster, Switzerland
(6) Center for Hernia Surgery and Proctology, ZweiChirurgen GmbH,
Basel, Switzerland

Benjamin Weixler (Corresponding author)

Email: [email protected]

Raoul A. Droeser
Email: [email protected]

Christian A. Nebiker
Email: [email protected]

Debora Nowakowski
Email: [email protected]

Heidi Misteli
Email: [email protected]
 Henry Hoffmann
Email: [email protected]

16.1 Polytrauma: Abdominal Trauma

B. Weixler and R. A. Droeser

Key Points
– Abdominal trauma (in Europe): ≥80% blunt, ≤20% penetrating; mainly
due to traffic and occupational accidents.
– Stabilization according to ATLS (Advanced Trauma Life Support)
criteria: Airways, Breathing, Circulation, Disability, Exposure
(ABCDE).
– FAST (Focused Assessment with Sonography in Trauma) examination:
for all patients.
– Contrast-enhanced computed tomography (CT): only for patients
hemodynamically stable.
– Circulatory unstable patients with positive FAST: immediate
laparotomy.
– Circulatory stable patients without evidence of hollow organ
perforation: non-operative approach.

16.1.1 Anatomy of the Abdomen

External Divisions
Anterior Abdominal Wall
– From costal margin to symphysis.
– On both sides from midline to anterior axillary line.
Lateral Abdominal Wall (= Flank)
– Between anterior and posterior axillary line.
– 6. intercostal space to iliac crest.
Abdominal wall = anterior +2 lateral abdominal walls.
Quadrant/Sector Breakdown
– Regional structure of the abdominal wall (◘ Fig. 16.1).
– Sector breakdown:
– Epigastric region.
– Umbilical region.
– Pubic region.
– Left and right hypochondrium.
– Left and right lateral abdominal region (= Regio hypogastrica =
Flank).
– Left and right inguinal region (= groin).
Fig. 16.1 Regional division of the abdominal wall

Internal Divisions
Abdominal Cavity
– Definition: Abdominal cavity = peritoneal cavity + preperitoneal and
retroperitoneal cavity.
– Peritoneal cavity.
– With liver, pancreas, spleen, small and large intestine, uterus (in
pregnant women), filled urinary bladder.
– Intracostal abdomen: special subdivision of the abdominal cavity, in
the rib cage, with diaphragm, liver, pancreas, spleen.
– Retroperitoneal space (= retroperitoneum).
– With kidneys, ureters, pancreas, aorta and v. cava.
– Continuity with preperitoneal space and infraperitoneal space.
Pelvis
– Definition: pelvic cavity = true pelvis = peritoneal + infraperitoneal
cavity.
– With urinary bladder, urethra, rectum, small intestine, ovaries and uterus.

16.1.2 Injury Mechanisms (Aetiology and Pathophysiology)

Blunt Abdominal Trauma
– Approx. 80% of abdominal injuries in Central Europe.
– Mainly: traffic and work accidents.

Deceleration Trauma
– Shear forces with traction on organs and vascular trunks.
– Injuries: Spleen (40%), liver (35%), small intestine (10%).

Crush Injury
– Between abdominal wall and spine and posterior thoracic wall.
– Especially vulnerable = solid organs (liver, spleen, kidneys).

Compression Injury
– Due to impacts, external compression (e.g. seat belt).
– Abrupt increase in intra-abdominal pressure: rupture of a hollow organ.

Penetrating Abdominal Trauma

– Often criminal/suicidal intent.
– Mostly single cavity, rarely double cavity injuries.

Stab Wound
– Prognostically most favourable form of injury.

Gunshot Wound
– Different injury patterns.
– Impact/Prognosis dependent on:
– Velocity of projectile.
– Type of projectile.
– Firing distance.

Impalement Injury (Due to Accidents)

– Degree of injury depending on shape and penetration depth.
– Combination injuries (impalement + blunt trauma) possible.

16.1.3 Management and Diagnosis

Primary Management

Treatment According to ABCDE Rules (ATLS): Simultaneous

Identification + Stabilization of Life-Threatening Injuries
– Airway/cervical spine protections (“Airway, with cervical spine
protections”).
– Ventilation, ensure gas exchange (“Breathing”).
– Hemodynamic stabilization (“Circulation”).
– Neurological status (“Disability”).
– Exposure, complete undressing (“Exposure”).

Anamnesis
– Rapid investigation of accident mechanism and timing.
– Allergies, medications, etc.
Clinical Presentation
– Inspection (ecchymosis, “seat belt sign”, eviscerations, foreign bodies).
– Palpation, percussion, auscultation.
– Rectal examination (prostate protrusion for urethral lesion, evidence for
bleeding, sphincter tone for neurological status).

Caution
– Intra-abdominal blood loss and/or small bowel rupture may remain
asymptomatic for a prolonged period; development of peritonism
takes several hours!
– Overlooking blunt abdominal trauma in unconscious patients.

Laboratory Tests
– Haemoglobin + haematocrit, electrolytes, creatinine and urea levels,
blood coagulation, blood gas analysis, glucose, serum amylase, alcohol
level, urinalysis, drug screening in urine and pregnancy test if necessary.
– Blood grouping + irregular antibody search; in case of circulatory
instability, crossmatch red cell concentrates.

Diagnostic Imaging
X-ray
– Conventional images = low significance in abdominal trauma.
Ultrasound Examination
– FAST examination (Focused Assessment with Sonography for Trauma).
– Detection of free fluid and possible organ rupture (liver, spleen, kidney).
– Poor sensitivity compared to CT (82%).
Computer Tomography
– Whole-body spiral CT “Trauma spiral CT” in polytrauma; abdominal and
pelvis CT scan in isolated abdominal trauma.
– No oral contrast agent necessary.
– Sensitivity for intra-abdominal injuries 98%, specificity 99%.

Caution
– Repeated clinical examination of the abdomen in addition to
ultrasound + CT in the presence of a significant trauma mechanism.
– CT only if patient is hemodynamically stable.
– Diaphragmatic ruptures, intestinal perforations and pancreatic
injuries often not visible at the beginning; in case of suspicion:
repeat CT after 36–48 h.

16.1.4 Therapeutic Procedure

Conservative Therapy
Key Points
– Conservative therapy only in stable patients without relevant
coagulation disorder.
– Conservative therapy never in case of hollow organ injury!

Blunt Abdominal Trauma

– Continuous (intensive medical) monitoring; also possible in the case of
major injuries to parenchymatous organs.
– For liver and spleen injuries:
– Regular clinical, laboratory and ultrasound control.
– Conservative approach successful in over 80% of cases.
– Selective arteriography: for liver/spleen/vascular injury.
– Rarely also other interventional measures (drainage, stent placement,
etc.)

Penetrating Abdominal Trauma

– Exploration of the wound:
– Deep fascia intact: Conservative management possible.
– Deep fascia injured: Diagnostic laparoscopy with evidence/exclusion
of penetration of the parietal peritoneum, laparoscopic bowel revision
if necessary (only reliable with sufficient overview).

Surgical Therapy
Circulatory Instability
– Patients with positive FAST: emergency laparotomy.
– If FAST not conclusive: If possible, stabilize patient so that CT can be
performed…
Circulatory Stability
– Negative FAST and/or CT: Think of other sources of bleeding/shocks.
Major Visceral Trauma/Complex Surgery
– High mortality: due to intraoperative metabolic disturbances.
– Avoid lethal triad at all costs:
– Coagulopathy.
– Hypothermia.
– Metabolic acidosis.
– Strategy:
– Damage-control laparotomy: find source of bleeding quickly + stop
bleeding; stop leakage of bowel contents, close bowel with GIA (blind
closure), remove perforated sections.
– Stabilization in the intensive care unit with the goal of euvolemia +
coagulation recovery + warming up of the patient.
– Planned second-look relaparotomy after 24–48 h for definitive
treatment of the abdominal injuries, stoma creation if necessary.

Surgical Procedure
Damage Control Concept (Damage Control Laparotomy)
– Access always via median laparotomy.
– Abdominal packing: Packs inserted in all 4 quadrants, systematic
exploration of all 4 quadrants.
– Emergency subdiaphragmatic clamping of the aorta, if necessary.
– Identification of the source of bleeding.
– In case of bleeding from liver parenchyma: possibly Pringle
manoeuvre (temporary clamping of the hepatoduodenal ligament),
debridement of avital parts of the parenchyma, possibly perihepatic
packing (especially in case of coagulation disorder).
– For bleeding from the spleen: splenectomy.
 – Bleeding from aorta and iliac vessels: sutures for small lesions, intra-
arterial shunt for larger injuries.
– Injury to the vena cava: direct suturing, packing retrohepatic vena
cava.
– After hemostasis: look for hollow organ perforations: Direct suture for
small perforations; resect larger ones using linear stapler; no
anastomosis, no enterostomy! Stomas are only created on the occasion
of the second-look laparotomy.
– Leave the abdomen open (= avoid compartment syndrome; ► Sect.
16.3).
– Possibly postoperative angiography for interventional embolisation.

16.1.5 Guidelines
Como JJ, Bokhari F, Chiu WC, Duane TM, Holevar MR, Tandoh MA,
Ivatury RR, Scalea TM (2010) Practice management guidelines for
selective nonoperative management of penetrating abdominal trauma. J
Trauma 68:721–733.
AWMF Guideline Polytrauma/Serious Injury Treatment, Register
Number 012–019; ► https://​www.​awmf.​org/​leitlinien/​detail/​ll/​012-019.​
html.
► https://​www.​wses.​org.​uk/​guidelines.
Benz D, Balogh ZJ (2017) Damage control surgery: current state and
future directions. Curr Opin Crit Care 23(6):491–497. ► https://​doi.​org/​10.​
1097/​MCC.​0000000000000465​.

16.2 Ileus/Obstruction
R. Mechera and C. A. Nebiker

16.2.1 Definition—Classification
Definition
– Intestinal ileus = intestinal obstruction = (small/large) bowel obstruction.
– Interruption of the flow of gastrointestinal contents from oral to aboral
direction.
– Distinction:
– Functional ileus = paralysis.
– Mechanical ileus = mechanical obstruction.

Classification
– By course: Acute vs. subacute vs. chronic.
– By completeness: Complete vs. incomplete.
– According to localization: high vs. deep small bowel ileus vs. large
bowel ileus.

16.2.2 Epidemiology
– Common clinical picture.
– Approx. 300,000 laparotomies in USA/year due to small bowel
obstruction = approx. 850,000 hospitalization days = 1.3 billion USD per
year.

16.2.3 Pathophysiology
“Circulus Vitiosus” of Ileus
– Numerous feedbacks.
– Based on intestinal distention.

Intestinal Distention
– Increase in wall tension → consecutive microcirculatory disturbance:
intestinal wall edema + hypoxia of the intestinal wall.
– Consequences of bowel wall edema and bowel wall hypoxia:
– Transmigratory peritonitis: hypoxia → impaired mucosal barrier and
additional stasis → bacterial overgrowth + transmigration.
– Increase in fluid sequestration into the intestinal lumen, intestinal wall
and abdominal cavity.
– Activation of biogenic amines, kinins and release of interleukins →
formation of prostaglandin E with protein degradation, leukocyte
increase and fever.
– Ileus disease = hypovolemic, septic-toxic shock up to multi-organ
failure (as final stage).
 Normal postoperative normalization of intestinal transit
– Small intestine: 5–24 h.
– Stomach: 1–2 days.
– Colon: 2–3 days (from oral to aboral).

16.2.4 Diagnosis
Key Points
– Previous surgery as a clue of possible adhesions.
– Clinical presentation: palpation + auscultation.
– Abdominal CT scan: increasingly imaging of choice.
– Electrolyte and fluid balance: In the time interval between diagnosis
and surgery.

Medical History and Symptoms

– Previous surgery as a clue of possible adhesions (bands).
– Previous changes in bowel habit (more often than complete
ileus/obstruction) as the first symptom.
– Pencil thin stool.
– Weight loss.
– Family history.
– Fever.
– Night sweats.
– Loss of appetite, nausea, vomiting.
– Stool and wind retention.
– Acute onset pain: spasmodic/nonspecific.
– Increase in abdominal girth (meteorism).

Clinical Examination: Status Survey

– Fluid status and vital signs.
– Inspection: Surgical scars, hernias.
– Palpation:
– Abdominal point tenderness.
– Blumberg’s sign/rebound tenderness.
– Abdominal guarding (“défense musculaire”).
 – Resistances.
– Percussion: Meteorism
– Auscultation:
– Hyperperistalsis in mechanical ileus (high-pitched, metallic bowel
sounds).
– Intestinal sounds sparse or absent (so-called dead silence) in paralytic
ileus.
– Digital rectal examination obligatory:
– Stenoses.
– Rectal Tumor.
– Impacted stool.
– Blood on the glove, if any.

Lab
– Signs of dehydration (hematocrit, creatinine, urea, electrolytes, acid-base
balance)
– Infection parameters (CRP, leucocytes).
– Lactate and LDH: sign of vascular ileus (caution: normal in 40%).
– Additional parameters to exclude other causes and differential diagnoses:
amylase, lipase.

Quantity/Quality of Gastrointestinal Contents Discharged

– Quantity/quality of gastrointestinal contents drained (gastric tube).

Imaging
Ultrasound
– Dilated fluid-filled intestinal loops.
– Intestinal peristalsis.
– Stenoses/strictures.
– Incarcerated hernias.
– Small bowel intussusception.
– intestinal wall thickening.
– Extraluminal free fluid with ineffective peristalsis (in severe ileus) (“to-
and-fro” or “whirling” appearance of intra-luminal contents).
– Detection of obstruction: sensitivity 83%, specificity 100%, localization:
70%, etiology: 23%.
Conventional Abdominal Radiograph
– Supine and standing or left lateral position (abdominal overview).
– Obstruction sign:
– Gas-fluid levels if the study is erect: interface between air/liquid in the
intestine.
– Free air, aerobilia (in 50% with gallstone ileus), shadowing
concretions, foreign body.
– Obstruction localization: eventually possible using gas-fluid level
arrangement.
– Hyperinflation/distention of the caecum in colonic ileus.
– Detection of obstruction: sensitivity 77%, specificity 50%, localization
60%, etiology: 7%.
Contrast-Enhanced (Gastrographin) Conventional X-Ray
– 100 mL gastrographin p.o./gastric tube, abdominal X-ray after 4–6 h.
– Statement possible about the level and extent of ileus.
– ileus resolved if contrast medium in colon in 24 h (sensitivity 97%,
specificity 96%).
– Important in “high” proximal small bowel ileus, as no gas-fluid level
formation in the normal abdominal radiograph.
– In addition, therapeutic laxative effect.

Caution
Contrast agent can convert a subileus into a complete ileus.

Computer Tomography (CT scan)

– Increasingly imaging modality of choice.
– Pathognomonic ileus signs = local wall thickening due to wall edema and
caliber jump (dilated small bowel loops >2.5 cm up from outer wall to
outer wall proximal to obstruction and normal caliber or collapsed loops
distally) ± evidence of free abdominal fluid.
– Detection of obstruction: Sensitivity 93%, Specificity 100%,
Localization: 93%, Etiology: 87%.
16.2.5 Mechanical Ileus (Mechanical Obstruction)
– Important measure: Balancing the electrolyte and fluid balance: In the
time interval between diagnosis and surgery.

Etiology and Pathogenesis

Etiology (◘ Table 16.1)
Table 16.1 Causes of mechanical obstruction

Mechanical small bowel obstruction

External cause Adhesions, Single band
Internal and external hernias
Tumors (pancreas, bile duct, etc.)
Volvulus
Intraabdominal abscess
Intraabdominal hematoma
Pancreatic pseudocyst
Superior mesenteric artery compression syndrome
Intra-abdominal drains
Peritoneal carcinomatosis
Intraluminal causes Neoplasms
Gallstones
Foreign bodies
Bezoar
Intramural causes Neoplasms
Strictures (Crohn’s disease, etc.)
Hematomas
Intussusception
Actinic enteritis
Regional enteritis
Mechanical large bowel (colon) obstruction
Common causes Malignancies
Volvulus
Diverticulitis
Pseudo-obstruction (Ogilvie syndrome)
Hernia
Anastomotic stricture
Rare causes Intussusception
Stool impaction
Strictures
Foreign bodies
External pressure

Pathogenesis
– Classification according to the type of obstruction:
– Luminal obstruction (from inside).
– Compression (from outside).
– Strangulation (single band, adhesion, volvulus, abdominal wall
hernias).
– Classification according to localization:
– Small bowel obstruction (80% of all intestinal peristalsis disorders;
high vs. low); bands, malignancies, hernias, adhesions (= 90% of the
etiologies).
– Large bowel (colonic) obstruction (10–15% of all intestinal peristalsis
disorders): Stenosing cancer, diverticulitis, volvulus, etc.

Specific Symptoms
High Small Bowel Ileus
– Colicky pain.
– Severe biliary or clear vomiting.
– But: meteorism may be absent.
Low Small Bowel Ileus
– Colicky pain.
– Meteorism.
– Fecal vomiting (Miserere).
Large Bowel Obstruction
– Meteorism.
– Less pain.

Complications
– Ischemia.
– In case of strangulation with vascular involvement.
– Especially in the case of mobile small intestine (e.g. in the context of a
single band obstruction, in the case of incarcerated hernia, etc.).
– Paralysis

16.2.6 Paralytic Ileus/Functional Obstruction

Etiology and Pathogenesis
Etiology (◘ Table 16.2)
Table 16.2 Causes of paralytic ileus

Primary forms
Myopathic chronic familial pseudoobstruction
Neuropathic chronic pseudoobstruction
Secondary forms
Intra-abdominal Inflammatory Peritonitis, abscess, colitis
pathologies
Mechanical Operation, Foreign Body
Chemical Gastric juice (perforated gastric ulcer), bile,
blood
Autoimmune Serositis, vasculitis
Intestinal ischemia Arterial, venous
Retroperitoneal Pancreatitis
pathologies Haematoma
Trauma, e.g. vertebral body fracture
Urolithiasis
Pyelonephritis, etc.
Extraabdominal disease Thoracic pathologies Myocardial infarction
Pneumonia
Decompensated heart failure
Rib fractures
Metabolic changes Electrolyte imbalance, e.g. hypokalemia
Porphyria
Hypothyroidism
Hypoparathyroidism
Uremia
Lead poisoning
Drugs Opiates
Anticholinergics
Antihistamines
Catecholamines
Antidepressants
Sepsis
Chemotherapy or
radiotherapy
Trauma Craniocerebral trauma
Thoracic trauma
Spinal cord injuries

Pathogenesis
– Impairment of the muscular function of the intestinal wall (motility
disorder).
– Reaction to various organ diseases, consequences of inflammation,
injuries, circulatory or metabolic disorders.
– Reflective:
– After major abdominal surgery.
– For intra-abdominal pathology.
– Response to acute extra-abdominal diseases.
– Special form: Intestinal pseudoobstruction (Ogilvie syndrome) =
peristalsis disorder of the colon due to various causes with distension of
the caecum.
Specific Symptoms
– Symptoms of the underlying pathology → Symptoms of obstruction →
Symptoms of “ileus disease.”

16.2.7 Therapy
Key Points
– Always general measures ± antibiotic therapy.
– Absolute indication for surgery:
– Complete mechanical ileus.
– High small bowel ileus.
– Peritonitis with/without paralysis.
– Strangulation Ileus.
– Vascular ileus.
– Gallstone ileus.

Conservative Therapy
General Measures
– Parenteral nutrition therapy (caloric intake).
– Monitoring and compensation of the fluid/electrolyte loss.
– Gastric tube (relief of gastrointestinal tract distension, electrolyte
balance, reduction of aspiration risk).
– Regular clinical examination by experienced surgeon.
Antibiotic Therapy
– In the case of bacterial translocation.
Drug Stimulation in Paralytic Ileus
– Sympathicolysis (e.g. peridural catheter).
– Parasympatheticomimetic agents (e.g. neostigmine).
– Prokinetics (e.g. metoclopramide, cisapride).
– Erythromycin: stimulation of gastric peristalsis.
– Hyperosmolar substances.
– If Emergency surgery unlikely; hyperosmolar gastrographin:
– Reduces bowel wall edema.
– Promotes peristalsis.
Surgical Therapy
Goals
– Intestinal Decompression.
– Restoration of gastrointestinal patency and blood circulation.
Emergency Indications
– Complete mechanical ileus.
– High small intestine ileus.
– Peritonitis with/without paralysis.
– Strangulation ileus.
– Vascular ileus.
– Gallstone ileus.
Relative Indications
– Chronic recurrent ileus (abdominal adhesions).
– Subileus due to chronic inflammatory bowel disease.
– Peritoneal carcinomatosis.
– Ogilvie’s syndrome.

Surgical Procedure
Surgery for Ileus
– Perioperative antibiotic therapy.
– Median laparotomy (extended, if necessary); eventually laparoscopy if
single band expected.
– Access to the peritoneal cavity.
– Adhesiolysis: to localize obstruction + to treat obstruction.
– Possibly bowel segment resection (injury, stenosis, tumor, ischemia).
– Careful bowel decompression (caution: serosal lesions, endotoxin
washout, postoperative atony).
– Assessment of blood flow/vitality of the intestine.
– Schedule second-look laparotomy if needed.

16.2.8 Specific Therapy for Certain Types of Ileus

Paralytic Ileus
Conservative Therapy
– Basic therapy as outlined (► Sect. 16.2.7 “Conservative therapy”).
– Drug stimulation.
– Elimination of triggering factors.
Surgical/Endoscopic Therapy
– Only in case of severe intestinal distension with consecutive risk of wall
ischemia and rupture (especially in the cecal region).
– Endoscopic decompression, decompression tube.
– Surgical creation of a fistula/stoma.

Vascular Ileus
– Revascularization.
– Embolectomy.
– Thrombectomy.
– Aortomesenteric bypass.
– Resection of avital bowel segments.

Strangulation Ileus
– Mechanical ileus with impaired blood circulation.
– Adhesiolysis, single band resection (possibly laparoscopic), hernia repair.
– Resection of avital bowel segments.

Postoperative Ileus
– Wait and see.
– Supportive therapy.
Caution: make the difference between postoperative ileus and ileus due
to surgical complications:
– E.g. anastomotic insufficiency, intra-abdominal abscess.
– In case of surgical complications: Revisional surgery/intervention =
necessary.

Ogilvie’s Syndrome
– Indication for surgery: In case of hyperinflation of the caecum >10 cm
– Interventions:
– Ileostomy creation.
– Colon resection.
– Possibly endoscopic decompression.

Large Bowel Obstruction Due to Colon Cancer

– Procedure dependent on:
– Localization of the cancer.
– General condition of the patient.
– Local intraoperative findings.
Conservative Therapy
– Palliative.
– Colonoscopic stent insertion.
Bridge-to-Surgery
– Goal = staged surgery (tumor operation in a second step).
– Indications:
– Compensated ileus (= no distension of the small intestine).
– Obstruction in the left hemicolon/rectum + mild symptoms.
– Methods:
– Colonoscopic stent insertion.
– Creation of ileostomy or colostomy.
– Colonoscopic decompression tube.
Primary Resection
– Indication = Decompensated ileus.
– On the Right side (caecum to mid-transverse): Single-stage resection
with simple or extended hemicolectomy and aspiration of the contents of
the small intestine.
– On the Left side:
– Resection with primary anastomosis, possibly insertion of a
defunctioning ileostoma.
 – Discontinuity resection (Hartmann’s procedure).

Gallstone Ileus
– Removal of stone via enterotomy, cholecystectomy.
– Resection of the cholecystoduodenal fistula controversial (high
morbidity).

Volvulus
– Attempt at colonoscopic derotation/decompression.
– If necessary staged surgical colopexy.

16.3 Abdominal Compartment Syndrome

D. Nowakowski and H. Misteli

16.3.1 Definitions
Key Points
– Abdominal compartment syndrome (ACS) = multiorgandysfunction.
– Intra-abdominal hypertension = origin of dysfunction.
– Mostly in critically ill patients.
– Wide range of medical and surgical clinical pictures.

Intra-Abdominal Pressure (IAP)

– Abdomen = polycompartment model.
– Bounded by rigid, bony (ribs, pelvis, spine) and flexible (abdominal
wall, diaphragm) structures.
– Closed space with physiological pressure = intra-abdominal pressure
(IAP).
– IAP:
– Normally in a “steady state”.
– Changes depending on wall characteristics (external influencing
factors)/filling state of the abdominal cavity (internal influencing
factors).
– Measurement (gold standard):
– Indirect measurement: bladder pressure (in mmHg) (► Sect. 16.3.4
Bladder pressure measurement).
– Normal levels: 5–7 mmHg in a healthy person, up to 10 mmHg in an
intensive care patient.
IAP:
– Good correlation between bladder pressure and IAP.
– Aterated level in: Adhesions, pelvic hematoma or fracture, pelvic girth,
neurogenic bladder.

Abdominal Perfusion Pressure (APP)

– Calculation: APP = mean arterial pressure (MAP) minus IAP (APP =
MAP – IAP).
– Provides conclusions about perfusion of the abdominal organs.
– APP >60 mmHg: correlation with better survival in ACS.

Intra-Abdominal Hypertension (IAH)

– IAP continuous or over a prolonged period ≥12 mmHg.
– Classification:
– Grade I: IAP = 12–15 mmHg.
– Grade II: IAP = 16–20 mmHg.
– Grade III: IAP = 21–25 mmHg.
– Grade IV: IAP > 25 mmHg.

Abdominal Compartment Syndrome (ACS)

– Prolonged elevation of IAP >20 mmHg + new onset of organ
dysfunction/failure.
– Distinction: Acute vs. chronic ACS.
– Destructive course of the disease comparable to ACS of the extremities.

16.3.2 Aetiology
Classification of Abdominal Compartment Syndrome (ACS, ◘
Table 16.3)
– Primary ACS: Due to intra-abdominal injury/disease (abdominal trauma,
hematoperitoneum, pancreatitis).
– Secondary ACS: Without initial abdominal focus (hemorrhagic shock,
infusion therapy, mesenteric ischemia, and reperfusion).
Table 16.3 Classification and causes of abdominal compartment syndrome (ACS)

Division Etiology
Primary ACS (= acute) Penetrating abdominal trauma
Intraperitoneal bleeding
Pancreatitis
External compression: e.g. after polytrauma,
traffic accident, explosion
Pelvic fracture
Ruptured abdominal aortic aneurysm
Perforated gastric ulcer
Secondary ACS (no acute event, subacute fluid Large volume administration during resuscitation
accumulation with IAH) (>3 L)
Large-scale combustion (especially third degree
combustion)
Penetrating/blunt abdominal trauma without
visible injury
Postoperative
After packing and fascial closure
Sepsis
Chronic ACS Peritoneal dialysis
Morbid obesity/extreme adiposity
Cirrhosis
Meigs’ syndrome
Intraabdominal mass

IAH intra-abdominal hypertension

Pathophysiology
– IAH = Restriction of organ function → ACS.
– At the cellular level: swelling, hypoxia, dysfunction.
– Cardiovascular IAH = diaphragmatic elevation induced:
– Decreased cardiac output.
 – V. cava compression (reduced backflow, blood pooling to the pelvis
and lower extremities).
– Decreased compliance and contractility due to direct compression.
– Pulmonary IAH = decreased thoracic volume induced:
– Atelectasis + oedema (reduced oxygen diffusion).
– Increased ventilation pressures needed (parenchymal lesion).
– Ventilation-perfusion mismatch (increased intrapulmonary shunt
fraction, increased alveolar dead space volume).
– Renal IAH = venous compression induced:
– Decreased venous outflow.
– Arterial vasoconstriction (activation of the renin-angiotensin-
aldosterone system).
– Decrease in glomerular perfusion and diuresis.
– Gastrointestinal IAH = decreased mesenteric blood flow induced:
– Decreased mucosal perfusion.
– Compression of the mesenteric veins.
– Wall Edema.
– “Circulus vitiosus” with end result of intestinal ischemia, lactic
acidosis and possible bacterial translocation, sepsis, multiple organ
failure.
– Liver IAH = decreased portal return induced by compression:
– Decreased degradation of lactate.
– Cerebrovascular IAH = increased intracranial pressure:
– Critical cerebral perfusion → cerebral ischemia.
IAH:
– Increased risk of thrombosis due to blood pooling in the lower
extremities.
– Visceral “Circulus vitiosus”: End result = intestinal ischemia.

16.3.3 Clinical presentation

Key Points
 – Key role: compliance of the abdominal wall + intra-abdominal volume.
– Mostly lack of communication with severely ill patients.

Risk Factors
– Key role: compliance of the abdominal wall + intra-abdominal volume.
– “Deadly triad”: acidosis + hypothermia + coagulopathy.
– Massive fluid substitution with crystalloids (hemorrhage, sepsis, burns).
– Anthropomorphology:
– Male (intra-abdominal fat distribution).
– Higher age.
– Obesity.
– Small size.
– Comorbidities and/or increased intrabdominal volume:
– Ascites.
– Fluid-filled intestinal loops (ileus, mesenteric ischemia).
– Hepato−/splenomegaly.
– Pancreatitis.
– Increased tension of the abdominal wall/diaphragm.
– Fascia closure after damage-control laparotomy.
– Active muscle contraction (pain).
– “Body Builder”.
– Anasarca.
– Abdominal wall hematoma (especially rectus sheath hematoma).
– COPD (chronic obstructive pulmonary disease), mechanical
ventilation (PEEP, “positive end-expiratory pressure”), pneumonia.
– Burns.

Symptoms
– Distended abdomen.
– Oliguria to anuria.
– Hypercapnia and hypoxia (increase in ventilation pressure).
– Circulatory insufficiency.
– Decreased organ perfusion to lactic acidosis.
– Decreased cerebral and limb perfusion.
 – Hypotension, tachycardia, increased jugular venous pressure, peripheral
oedema, diffuse abdominal pain.
If ACS is imminent = usually lack of communication in a seriously ill
patient.

16.3.4 Diagnosis
Key Points
– Bladder pressure = gold standard.
– Mortality in abdominal compartment syndrome = 40–100%.

Bladder Pressure Measurement (Gold Standard)

– Measurement in mmHg, at the end of expiration, in supine position.
– Reference point = mean axillary line.
– Instillation of 25 ml saline solution into an empty bladder.
– Regular measurement in intensive care patients with risk factors for ACS
(4 to 6 h).

Clinical Presentation
– Poor predictor (► Sect. 16.3.3).

Imaging
– Not helpful for diagnosis; mostly CT.
– Signs on imaging: Visible IAH.
– Diaphragmatic Protrusion.
– Vein compression (especially inferior vena cava).
– Abdominal distension.
– Renal compression.
– Bilateral inguinal hernias.

Caution
– Clinical diagnosis (detection) should be made before the formation of
ACS at the stage of IAH.
– Mortality of ACS = 40–100%.
16.3.5 Therapy
Key Points
– Supportive measures + surgical pressure relief.
– Temporary → definitive abdominal closure.

General Principles of Therapy

WSACS Guidelines
– WSACS = “World Society of the Abdominal Compartment Syndrome”:
Recommendation of a Therapy Algorithm, Update 2013 (► Sect.
16.3.6).
Supportive Measures
– To lower the IAP ± surgical abdominal decompression.
Goals
– Improvement of abdominal wall compliance.
– Reduction of intra-abdominal volume.

Conservative Measures
Improvement of Abdominal Wall Compliance
– Analgesia.
– Sedation.
– Relaxation + anxiolytic therapy, respiratory support.
– Neuromuscular blockade.
Decrease in Intra-Abdominal Volume
– Balanced fluid management, fluid restriction if possible.
– Gastric/colonic compression:
– Gastric tube/rectal tube (intestinal tube).
– Enemas.
– Prokinetics (metoclopramide, erythromycin, neostigmine).
– Endoscopy: decompression of hollow organs.
– Drains:
– Percutaneous drainage for obvious intraperitoneal fluid accumulation
(ascites, hematoperitoneum).
– Interventional drainage of an intra/retroperitoneal collection (abscess,
pseudocyst).

Surgical Measures
– Surgical decompression = definitive therapy.
– Relevant complications of surgical therapy.
– Mortality up to 50% (depending on the etiology of ACS).
Decompressive Laparotomy
– Standard procedure.
– Median laparotomy (opening the linea alba + abdomen).
New Alternatives
– Minimally invasive percutaneous endoscopic component separation
technique.
– Subcutaneous linea alba fasciotomy (SLAF).
Temporary Abdominal Closure
– Initially: leaving open (the fascia) of the abdomen.
– Abdominal wound dressing with bridging of the fascial ends (=
prevention of evisceration/heat loss).
– Vicryl mesh.
– “Bogota bag” (sterile plastic bag).
– “Packing” with wet cloths.
– Vacuum dressing (= gold standard).

Caution
Operative measures for all strategies: risk = small bowel fistula.

Definitive Abdominal Closure

– Recommendations:
– Early.
 – If possible in the same hospitalization (usually within 5–7 days).
– For long-term open abdomen:
– Often “loss of domain” of the abdominal organs (= covering with
granulation tissue).
– Needed closure with “mesh graft”: transplantation or cutaneous
displacement flap.
– Subsequent fascial closure: only aim for after 9–12 months after.

16.3.6 Guidelines
Kirkpatric AW, Roberts DJ, De Waele J, Jaeschke R, Malbrain ML, De
Keulenaer B, Duschesne J, Bjorck M, Leppaniemi A, Ejike JC, Sugrue M,
Cheatham M, Ivatury R, Ball CG, Reintam Blaser A, Regli A, Balogh ZJ,
D’Amours S, Debergh D, Kaplan M, Kimball E, Olvera C; Peditric
Guidelines Sub-Committee for the World Society of the Abdominal
Compartment Syndrome (2013) Intra-abdominal hypertension and the
abdominal compartment syndrome: Updated consensus definitions and
clinical practice guidelines from the World Society of the Abdominal
Compartment Syndrome. Intensive Care Med 39:1190–1206.
Sosa G, Gandham N, Landeras V, Pauline Calimag A, Lerma E (2019)
Abdominal compartment syndrome. Dis Mon 65(1):5–19.
Rogers WK, Garcia L (2018) Intraabdominal hypertension, abdominal
compartment syndrome, and the open abdomen. Chest 153(1):238–250. ►
https://​doi.​org/​10.​1016/​j.​chest.​2017.​07.​023
Leppäniemi A, Hienonen P, Mentula P, Kemppainen E (2011)
Subcutaneous linea alba fasciotomy, does it really work? Am Surg
77(1):99–102.

16.4 Intestinal Ischemia

B. Weixler and H. Hoffmann

16.4.1 General
– Intestinal ischemia = mesenteric ischemia.
– Classification of intestinal ischemia:
– Due to the speed of formation/evolution.
 – Due to the severity of intestinal circulatory disorders.
– Two types of intestinal ischemia:
– Acute intestinal ischemia (= acute mesenteric ischemia).
– Chronic intestinal ischemia (= chronic mesenteric ischemia).

16.4.2 Acute Intestinal Ischaemia

Key Points
– Causes of acute mesenteric ischemia (AMI): embolus (50%), arterial
thrombosis (25%), nonocclusive (20%), venous thrombosis (5%).
– Causes of ischemic colitis: Nonocclusive (95%).
– Clinical presentation: extent of subjective abdominal pain often in
contrast to indolent abdominal examination.
– Three-phase clinical course: Acute pain interval (0–6 h); pain-free
interval with intestinal paralysis, so-called “rotten peace” (7–12 h);
renewed pain interval with peritonitis and septic shock with intestinal
necrosis (12–24 h).
– Diagnosis: abdominal CT angiography with contrast medium.
– Caution: Normal lactate does not exclude intestinal ischemia!
– Therapy: Rapid start with broad-spectrum antibiotics; always surgical
embolectomy and/or arterial thrombectomy, resection of avital
intestinal segments if necessary.

Epidemiology
– Prevalence: approx. 1% of all patients with acute abdomen.
– In >70-year-olds: Prevalence = 10%.
– 60–70% of all intestinal ischemias = acute mesenterial ischemia (AMI).

Etiology and Pathogenesis

– Mesenteric artery embolism (50%):
– Cardiac embolus (most common).
– Ruptured plaque of the proximal aorta.
– Mesenteric artery thrombosis (25%):
– Atherosclerosis (most common cause).
– Arteritis.
 – Dehydration.
– Nonocclusive Mesenterial Ischemia (NOMI; 20%): Hypoperfusion
and/or vasoconstriction in the splanchnic area (in heart failure, sepsis,
cardiac or abdominal surgery, use of vasopressors, ergotamines, cocaine).
– Mesenteric vein thrombosis (5%):
– Genetic hypercoagulability (>75%).
– Paraneoplastic.
– Cirrhosis of the liver.
– Pancreatitis.
– Pregnancy.
Ischemic colitis = most common form of intestinal ischemia: 95% of
ischemic colitis due to NOMI in the area of the vascular anastomoses of the
colonic arteries (left flexure and rectosigmoidal junction).

Clinical Presentation
Non-Specific Clinical Presentation
– Sudden onset of periumbilical abdominal pain.
– Often in discrepancy with the inconspicuous abdominal examination.
– Often accompanied by nausea, vomiting, diarrhea.
– Localized pain over affected bowel segment.
– In ischemic colitis, hematochezia/bloody diarrhea (typically only after 24
h).

Caution
– The first clinical examination of the abdomen may be completely
normal!
– Compared to acute mesenteric ischemia, the pain associated with
colonic ischemia is often not as severe.

3-Phase Clinical Course (Rarely Detectable)

– Acute pain interval: colicky pain + vomiting, diarrhoea, shock (after 0–6
h).
– Pain-free interval: intestinal paralysis, acidosis, “rotten peace” (7–12 h).
– New pain interval: peritonitis + septic shock with intestinal necrosis (12–
24 h).
Diagnosis
Anamnesis
– Previous thromboembolic events.
– Postprandial abdominal pain (= “abdominal angina”).
Laboratory Tests
– Lab levels unreliable; suggest AMI, but can never rule it out!
– Marked leukocytosis (>15,000/μl in 75% of patients).
– Elevation of lactate, LDH, CK or amylase = indicators for extent of
tissue damage.
– Lactate increase = late; a lactate that remains constant during the course
must suggest other diagnoses (sensitivity 90–96%, specificity 60–87%).
Important: If AMI is suspected (with normal laboratory) = no delay due
to further diagnosis.

Caution
Lactate levels:
– Only increased lactate = indicative.
– Normal lactate = no exclusion of mesenterial ischemia!
– Explanation for normal levels: complete circulatory arrest in the
ischemic area = no drainage of accumulated lactate = no lactate in
the peripheral circulation.

Diagnostic Imaging
– CT angiography of the abdomen with contrast medium:
– Imaging of vessel occlusion (= Contrast Medium stop).
– Exclusion of tissue necrosis (intramural gas in the GI tract-
pneumatosis intestinalis, portal venous gas: gas in the V. portae-
hepatis branches).
– Exclusion of bowel perforation (abdominal free air).

Therapy
Caution
As a principle: If there are clinical signs of intestinal necrosis =
generous indication for exploratory laparotomy!
Stabilization
– Always intensive medical monitoring/treatment.
– Oxygen administration + circulation stabilization + fluid balancing.
– Broad-spectrum antibiotic therapy (after only a few hours of ischemia =
disintegration of the mucosal barrier = bacterial translocation).
Acute Mesenteric Artery Embolism
– Emergency laparotomy (see “Operative procedure” below).
– Embolectomy.
Acute Mesenteric Artery Thrombosis
– Emergency laparotomy (see “Operative procedure” below).
– Surgical thrombectomy.
– Alternative option = angioplasty within 8 h after symptom onset in stable
patients without peritonism.
Acute Mesenteric Vein Thrombosis
– Emergency laparotomy: if evidence of bowel necrosis.
– Conservative therapy: Only in the absence of intestinal necrosis:
– Heparin bolus 80 U/kg BW, not exceeding 5000 U, then infusion at 18
U/kg BW/h.
– ICU-Monitoring.
Ischemic Colitis and Nonocclusive Mesenteric Ischemia (NOMI)
– Intensive care monitoring in the absence of gangrene or perforation:
surgery necessary in only about 20% of patients (gangrene/perforation):
– Improvement in heart function.
– Correction of hypovolemia and metabolic acidosis.
– Stop vasopressors.
– Anticoagulation = no evidence.
– Emergency laparotomy if signs of intestinal necrosis (see “Operative
procedure” below).

Surgical Procedure
Acute Mesenteric Artery Embolism
 – Access via median laparotomy.
– Exposure of the superior mesenteric artery (SMA) in the mesenteric
root: At the inferior border of the pancreas (lesser sac − bursa
omentalis)/through inframesocolic access (transverse colon).
– Vascular incision proximal to the embolus + embolectomy (Fogarty
catheter size 3/4).
– Examination of intestinal vitality (peristalsis and colour); examination
of blood flow using vascular Doppler probe.
– If embolectomy not satisfactory: mesenteric vascular bypass if
necessary.
– Resection of necrotic bowel + creation of primary anastomosis,
ileostomy if necessary.
– In case of inconclusive exploration/findings: laparostoma (leaving the
abdomen open; e.g. insertion of an abdominal vacuum dressing +
second-look laparotomy within 24–48 h).

Surgical Procedure
Acute Mesenteric Artery Thrombosis
– Access via median laparotomy.
– Exposure of the superior mesenteric artery (SMA) in the mesenteric
root: at the inferior border of the pancreas (lesser sac − bursa
omentalis)/through inframesocolic access (transverse colon).
– Identification of the affected arteries and intestinal segments
(inspection, Doppler).
– Thrombectomy + arterial reconstruction; aortomesenteric bypass if
necessary.
– Checking the vitality of the intestine/if necessary, segment resections
with primary anastomosis.
– In case of inconclusive exploration/findings: Stapler closure of the
bowel ends + laparostoma, second-look laparotomy within 24–48 h.

16.4.3 Chronic Mesenteric Ischaemia (CMI)

– Chronic mesenteric ischemia = chronic intestinal ischemia.

Etiology and Pathogenesis

Etiology
– Atherosclerosis of the mesenteric vessels (>95% of CMI).

Median Arcuate Ligament Syndrome (= MALS = Dunbar

Syndrome)
– Controversially discussed clinical entity.
– Chronic intestinal ischemia = possible clinical presentation.
– Pathogenesis: Compression of the coeliac trunk by the median arcuate
ligament (diaphragm).
– Rare: Diagnosis of exclusion, sometimes posture or respiration
dependant pain.

Pathogenesis (= Atherosclerosis)
– Lack of increase in blood flow while increased demand.
– Reduced inflow.

Clinical Presentation
– Postprandial pain (typical “abdominal angina”).
– 10 min-3 h after food intake.
– Mostly epigastric or periumbilical.
– Food aversion.
– Weight loss.

Diagnosis
– Diagnostic imaging.
– Angiography = gold standard.
– CT angiography (sensitivity 96%, specificity 94%): Additional
information (e.g., topographic relationships).
– MRI angiography (often only 25% of the course of IMA can be
visualized).
– Duplex ultrasound (SMA can be visualized in 90%, coeliac trunk in
80%).
Therapy
– Elective surgical vascular reconstruction.
– Percutaneous transluminal angioplasty (PTA).
– “Acute-on-chronic mesenteric ischemia = treat as an emergency.

16.4.4 Guidelines
Hirsch AT, Haskal ZJ, Hertzer NR, Bakal CW, Creager MA, Halperin JL,
Hiratzka LF, Murphy WR, Olin JW, Puschett JB, Rosenfield KA, Sacks D,
Stanley JC, Taylor LM Jr., White CJ, White RA, Antman EM, Smith SC Jr.,
Adams CD, Anderson JL, Faxon DP, Fuster V, Gibbons RJ, Hunt SA,
Jacobs AK, Nishimura R, Ornato JP, Page RL, Riegel B (2006) ACC/AHA
2005 Practice Guidelines for the management of patients with peripheral
arterial disease (lower extremity, renal, mesenteric, and abdominal aortic):
A collaborative report from the American Association for Vascular
Surgery/Society for Vascular Surgery, Society for Cardiovascular
Angiography and Interventions, Society for Vascular Medicine and Biology,
Society of Interventional Radiology, and the ACC/AHA TAsk Force on
Practice Guidelines (Writing Committee to Develop Guidelines for the
Management of Patients with Peripheral Arterial Disease). Circulation
113:e463–654.
American Gastroenterological Association Medical Position Statement:
guidelines on intestinal ischemia (2000) Gastroenterology 118:951–953.
Pecoraro F, Rancic Z, Lachat M, Mayer D, Amann-Vesti B, Pfammatter
T, Bajardi G, Veith FJ (2013) Chronic mesenteric ischemia: critical review
and guidelines for management. Ann Vasc Surg 27:113–122.
Bala M, Kashuk J, Moore EE, et al. (2017) Acute mesenteric ischemia:
guidelines of the World Society of Emergency Surgery. World J Emerg Surg
12:38. Published 2017 Aug 7. ► https://​doi.​org/​10.​1186/​s13017-017-0150-
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