Hypersplenism ;its surgical management

HYPERSPLENISM:
SURGICAL
MANAGEMENT
By Dr. DEV RAJ PATEL
IMS BHU VARANASI

INTRODUCTION
 The spleen was regarded by Galen as “an organ of
mystery,” ; By Aristotle as unnecessary, and By
Pliny as an organ that might hinder the speed of
runners
 The term ‘hypersplenism’ first appeared in the
thesis of Anatole Chauffard in 1907 and
subsequently in the study of Morawitz and
Denecked

ANATOMY
 Spleen is located in the Left Upper Quadrant(LUQ).
 Lies between the 9th -11th rib
 weighs about 150gm( 75-250gm ).
 Measures about 13 x 7 x 3 cms.

Attachement :-
 Laterally- Lienorenal
Ligament
 Anteriorly-
Gastrosplenic
Ligament (Contains
Short Gastric Arteries
and Left Gastro
Epiploic artery)
 Superiorly-
Splenophrenic
Ligament
 Inferiorly- Splenocolic
Ligament

RELATIONS
 Anterior-fundus of
the stomach
 Medially- tail of
the pancreas
 Inferiorly- splenic
flexure
 Superiorly-
diaphragm
 Posteriorly- upper
part of the kidney

Arterial supply- Splenic artery,
Short Gastric arteries
 Distributed type- the most
common (70%) and is
distinguished by a short trunk
with many long branches.
 Magistral type of splenic
artery (30%) has a long main
trunk dividing near the hilum
into short terminal branches.
Splenic vein joins the Superior
Mesenteric Vein to form the
Portal Vein and
accommodates the major
venous drainage of the
Spleen.

EMBRYOLOGY
 Spleen is the largest reticulo-
endothelial organ in the body.
 Developed from the primitive
mesoderm of dorsal mesogastrium by
fifth week of gestation.
 The most common variation of
Splenic embryology is the
accessory spleen (10-29 % of
the population.)

HISTOLOGY AND FUNCTION OF SPLEEN
Red pulp(90%)- Cords and sinuses - Phagocytosis
 White pulp- Periarticular lymphatic sheets -
Immunoglobulins.

 Reservior for platelets,monocytes,Factor VIII etc.
 Haematopoiesis in fetus
 Repairs and destruction of RBC’s by pitting & culling.
 Immune function: produces IgM ,properidin,tuftsin
 Prevention of infection- By capsulated organism
(H.influenza etc) , role in phagocytosis.

HYPERSPLENISM
Clinical syndrome characterized by:
 Splenomegaly
 Pancytopenia or a reduction in the number of one or
more types of blood cells
 Improvement of cytopenias Post-Splenectomy
 Hyperplasia of the precursor cells in the marrow or so
called maturation arrest
 Decreased RBC/platelet survival

 In Hypersplenism, Splenic function accelerates,
and begins automatically to remove cells that may
still be normal in function.
 Sometimes, the spleen will temporarily sequestrate
90% of the body platelets and 45% of the red cells.

Classification of Hypersplenism
 Hypersplenism can be classified into three categories by its
etiology as follows.( Yunfu et al., 2016)
Primary hypersplenism
Cause is not clear.
1. Primary splenic hyperplasia
2. Non-tropical idiopathic splenomegaly
3. Primary splenic granulocytopenia
4. Primary splenic pancytopenia
5. Splenic Anemia or thrombocytopenia

Secondary hypersplenism
Cause is clear
A. Infections - viral hepatitis, brucellosis, subacute or chronic diseases,
infectious mononucleosis syndrome and malaria.
B. Alcohol
C. Portal hypertension (PH) - liver cirrhosis of various causes including
Post-hepatitic Cirrhosis, Alcoholic Cirrhosis, Biliary Cirrhosis, Fatty Liver
Cirrhosis, Post-hepatitic Autoimmune Cirrhosis, Schistosomiasis-induced
Cirrhosis, & Drug-induced Cirrhosis, as well as Hemosiderosis And
Portal Vein Thrombosis.
D. Granulomatous inflammation - Systemic Lupus Erythematosus,
Rheumatoid Arthritis, Chronic Syphilis, Chronic Tuberculosis, Felty's
Syndrome, & Sarcoidosis.

 Malignancies - Splenic lymphosarcoma, leukemia, and cancer
metastasis.
 Chronic hemolytic diseases - hereditary spherocytosis,
autoimmune hemolytic anemia and thalassemia.
 Lipidosis - Gaucher's disease, and Niemann-Pick disease.
 Myeloproliferative disorders- Polycythemia Vera, Chronic Myeloid
Leukemia, Myelofibrosis

OCCULT HYPERSPLENISM
 Sometimes due to benign bone marrow hyperplasia and
sufficient bone marrow compensation, peripheral
cytopenias may not occur.
 In this case, hypersplenism becomes occult with no
symptoms.
 Bone marrow hematopoietic function is suppressed by
factors such as infection or drugs, peripheral cytopenia
occurs, accompanied by clinical symptoms, which is not
classified as occult hypersplenism.

DEGREES OF SPLENOMEGALY BASED ON CRANIO-CAUDAL LENGTH
ON CT OR POST-RESECTION WEIGHT
SPLENIC LENGTH (CM) SPLENIC WEIGHT
(GM)
Normal spleen Up to 13 <300
Mild splenomegaly 13–15 300–500
Moderate splenomegaly 16–20 500–1000
Massive splenomegaly >20 >1000
gm with etiological di
agnosis

HACKETT’S GRADING SYSTEM FOR PALPABLE
SPLENOMEGALY
MILD-palpable <3cms below LCM
MODERATE-4-7 below LCM
SEVERE- >7cms below LCM

MASSIVE SPLENOMEGALY ( >20CM, >1000GM
)
Causes
 Leishmaniasis
 Malaria
 Myeloproliferative disease
 Portal vein obstruction/portal hypertension
 Schistosomiasis
 NiemannPick disease
 Mucopolysaccharidosis
 Lymphomas
 Gaucher disease
 Hereditary spherocytosis

CLINICAL FEATURES
 Abdominal pain/tenderness.
 Early satiety due to splenic encroachment.
 Symptoms of anemia due to accompanying cytopenia.
 Febrile illness (infectious).
 Pallor, dyspnea, bruising, and/or petechiae (hemolytic
process).

 History of liver disease (congestive).
 Weight loss, constitutional symptoms (neoplastic).
 Pancreatitis (splenic vein thrombosis).
 Alcoholism, hepatitis (cirrhosis).
Examination
 Inspection may reveal fullness in the LUQ.
 Palpation.
 Percussion- Nixon, Castell, Percussion of Traube's
semilunar space.
 Auscultation- may reveal a venous hum or friction rub

INVESTIGATION
 Ultrasound -
The spleen is considered to be normal in size if its length is
<13 cm or its thickness is ≤5 cm
Plain film
The spleen is normal in size if it is
not seen on the abdominal plain
film.
It is considered enlarged
( if >6cm wide or >13.6 cm long) .

CT Scanning-
In general, the
spleen can be
considered
enlarged if its
craniocaudal length
is more than 10cm .
Spleen that extends b
elow the lower th
ird pole of the kidn
ey
is also indicative of
splenomegaly

 LiverSpleen Colloid Scanning-
A splenic length of greater than 14 cm is considered enlarged on
liverspleen scan .
Erythrocytes are labeled with chromium51, mercury197 ,
rubidium81
 Bone marrow examination is useful in diagnosis of
histiocytoses, lysosomal storage disorders, and some
infections(e.g., disseminated histoplasmosis).
.

 MRI/ Doppler USG- portal/splenic vein thrombosis -
cavernomas
 MRI scan- liver hemangiomas hemochromatosis
erlenmeyer flask sign(Gaucher)
 PET scan - Dx & staging of lymphomas - determine
metabolic cells in spleen
 Splenectomy and Splenic Biopsy

LABORATORY STUDIES
 Complete blood cell count (CBC) with differential.
 Liver function testing
 Hepatitis B and C testing
 Lactate dehydrogenase (LDH)
 Erythrocyte sediumentation rate (ESR)
 Peripheral blood smear for RBC morphology & signs of myelopr
oliferative disorders underlying bone marrow disorders.
 Prothrombin time (INR) and activated partial
thromboplastin time (aPTT)

TREATMENT
 Medical treatment
 Partial splenic embolisation
 Splenectomy
1.partial splenectomy
2.Total splenectomy:-
-Open splenectomy
-Laparoscopic splenectomy
-Robotic splenectomy

MEDICAL TREATMENT
 Whole blood transfusion
 For anemia and leucopenia
 Platelet transfusion
 Correction of coagulopathy
 Pre-operative nutritional optimisation
 Treatment of underlying cause- portal hypertension, liver
transplant for liver cirrhosis, anti-malarials for tropical
splenomegaly

 Transcatheter embolisation method
 It includes embolisation of certain
branches of splenic artery
leading to infarction of part of spleen.
 PVA, gelatin sponge are used for
embolisation
 Hypersplenism is relieved if
>50% of splenic area is embolised
Partial splenic embolisation

 Role in reducing portal pressure in
hypersplenism due to portal
hypertension
 Contraindications - pyemia,
splenic abscess, coagulopathy
 Complications- post-embolisation
syndrome (fever, abdominal pain,
vomiting), lung atelectasis,
pleural effusion, splenic abscess,
deranged liver or renal function,
portal vein thrombosis

SPLENECTOMY
Preoperative Planning
•Vaccination against pneumococcus, meningococcosis, and H.
influenzae should be administered to reduce the risk of OPSI

 Blood grouping and Cross matching
 Platelets should not be administered preoperatively in
patient with ITP
 In myeloproliferative disorders - Low-dose heparin
and aspirin on the day before surgery upto 5 days
postoperatively.
 Orogastric tube is used during the operation.
 Preoperative embolization(massive spleen)
 Perioperative steroids are usually given if a patient
had prolonged steroid treatment

INDICATION OF SPLENECTOMY
Absolute
 Bleeding varices due to splenic vein thrombosis
 Hereditary spherocytosis
 Massive splenic trauma
 Primary splenic malignancy
Relative
 Autoimmune hemolytic anemia
 Hypersplenism due to portal HTN
 Idiopathic thrombocytopenic purpura (ITP)
 Leukemia (chronic myeloid leukemia )

 Lymphoma
 Primary hypersplenism
 Myelofibrosis
 Sickle-cell disease
 Splenic abscess
 Staging for hodgkins lymphoma
 Thalassemia
 Thrombotic thrombocytopenic purpura
 Radical gasterctomy involving removal of spleen
Splenectomy not indicated
 Hereditary hemolytic anemia of modrerate degree
 Acute leukemia
 Agranulocytosis

SURGICAL TECHNIQUE
 Open Splenectomy
- in blunt abdominal trauma, staging of Hodgkin disease
an upper midline incision given.
- In hematological disorder, a left oblique subcostal
incision approximately two finger breadths below the costal
margin given.
-Preoperative angiographic embolization can be
considered to reduce bleeding in cases of massive
splenomegaly

 Splenectomy starts with mobilization and dissection down to
an ultimate pedicle of Splenic Artery and Vein.
 Transection of the ligamentous attachments, including the
splenophrenic ligament at the superior pole and the
splenocolic and splenorenal ligaments at the inferior pole.


After the ligamentous attachments are transected, two to six
gastric vessels should be ligated in continuity and divided

 After these maneuvers are completed, the spleen can be
delivered into the wound by blunt dissection of the posterior
attachments.
 Care should be taken not to divide the posterior
attachments too far medially to avoid entering the splenic
vein.
 Dissection is carried out at the hilus as close to the spleen
as possible to avoid injury to the Pancreas.

 Splenic artery ligation is managed by double ligation and
suture ligature, where as the splenic vein can be doubly
ligated and divided.
Ligation of the splenic artery and splenic vein in relation to the hilus

 Three major areas to be inspected for bleeding:-
(a) the inferior surface of the diaphragm.
(b) the greater curvature of the stomach and region of the
short gastric vessels.
(c) the region of the hilus.
 An integral part of splenectomy for haematological disease is
a thorough exploration to detect any accessory spleens.

PREOPERATIVE SPLENIC ARTERY EMBOLIZATION
(SPIGOS ET AL, 1979, )
 Embolization is achieved using microcoils and/ or Gelfoam.
 To reduce vascularity and size of massive spleen in
preparation for a laparoscopic approach.
 Applied in the treatment of PH and bleeding
esophagogastric varices.

Merit
 Faster Increase in platelet and leukocyte counts.
 Reduces splenic size, improves pancytopenia, and
stimulates the immune system
RISKS
•Post-embolization syndrome: pain, fever, ileus, pleural
effusion
•Pancreatitis
•Splenic abscess or rupture
•Peritonitis

LAPAROSCOPIC SPLENECTOMY
 Laparoscopic techniques is mostly preferred for elective
splenectomy.
 The complicating factors are a large spleen (>500 g),
suspected perisplenitis ( infections or portal hypertension)
and previous gastric surgery.
 ITP patients and staging laparotomy is suited ideally for
laparoscopic approaches as well.

Position- right side down
 Ports-
1. At midline and 4 cm below the spleen tip,
2. Near the tip of the 11th rib along the posterior axillary line
3. Half way between the other two, along the anterior axillary
line. Occasionally, a fourth port may be required.
 Scissors with cautery or preferably the harmonic Scalpel can
be used to take down the lateral peritoneal attachments and
can be used to ligate short gastric vessels.

 Ligation and division of the short gastric vessels then splenic
artery and vein secured
 Specimen delivery - morselization of the spleen in a bag or port
site can be enlarged to facilitate removal
 If the spleen is too large, a small Pfannenstien incision and
removing the spleen through a suprapubic area may be more
cosmetically satisfactory.

HAND-ASSISTED SPLENECTOMY
Hand-assisted laparoscopic surgery (HALS )
 As an alternative to the LS approach with same positioning
 Spleen greater than 22 cm in craniocaudal length or 19 cm in
width may benefit.
Merit
 Marked reduction in average operative time.
 This technique allows for a tactile feedback and atraumatic
manipulation of the enlarged spleen.
Demerit
Require a small incision (7–8 cm) for hand insertion and
specimen extraction.

SINGLE-INCISION LAPAROSCOPIC SURGERY
(SILS)
 One small transabdominal incision
 Incision -periumbilical and is used as the specimen
extraction site.
 Theoretical benefits of less pain and better cosmetic.
 Technical challenging for solid organs- since all
instruments are closely aligned together.
 Limited degrees of movement

ROBOTIC SPLENECTOMY
 Unique three-dimensional visualization of the surgical field.
 Facilitates movement with higher precision than standard
laparoscopy.
 Robotic splenectomy is very similar to standard laparoscopy,
although not as cost effective.
 No clear benefit of robotic versus laparoscopic splenectomy.

POST OPERATIVE MANAGEMENT
 Remove NG tube and suction drain when drainage
is minimal (usually 24 - 48hours)
 Commence oral when bowel activity resumes.
 Long term oral penicillin 250mg daily.
 Pneumococcal vaccine 2 weeks post op.
 Anti-malaria prophylaxis.

COMPLICATIONS
Early
 Acute gastric dilatation
 Fundal ischemia- hematemesis, perforation
 Pancreatic fistula
 Portal vein thrombosis
 Reactionary hemorrhage from splenic vessel 4% to 16% of
patients
 The most common site of bleeding is the short gastric
vessels
Late
 Infection; pneumococcal, viral, OPSI
 Thrombocytosis

OVERWHELMING POSTSPLENECTOMY INFECTION (OPSI)
 Incidence - 4%.
 Due to reduced IgM, tuftin, properdin and other antibodies,
phagocytosis of encapsulated bacteria is defective.
 Post-splenectomised patient is more prone for
Pneumococcal septicaemia (commonest), N. meningitides,
H. influenzae, Babesia microti infections.
 Common in first two years after splenectomy but life long
risk present.

Clinical Features-
 Prodromal phase—fever, chills, sore throat.
 DIC ,Hypotension, shock.
 Respiratory distress, coma, death.
 Mortality for fully developed OPSI—50-70%.
.

Treatment of OPSI
 Antibiotics like Cefoperazone, Ceftazidime, Amikacin
 Ventilatory support—ICU care.
 Blood transfusion.
 Immunoglobulin transfusion.
 Nutrition (TPN) and maintaining of urine output.

PREVENTION
 Pneumococcal vaccine should be given to all
splenectomised patients.
 Polyvalent pneumo-vac is given 2-3 weeks prior to surgery
and repeated once in 5 years (>2 yr of age).
 meningococcal vaccine (only to those who travel with high-
risk), H. influenzae ‘B’ vaccine (to all whatever the age,
once in 10 years).
 In malaria endemic areas, anti-malarial prophylaxis is given
for patients after splenectomy

 Management and treatment should therefore be
administered taking into account the specific etiology and be
individualized for each patient.
 Surgical outcome following Splenectomy is usually
satisfactory.
 Continuous basic and clinical studies will advance our
understanding of the underlying mechanisms of the
development of hypersplenism, and provide better
management strategies for the treatment of patients with
hypersplenism.
CONCLUSION

Hypersplenism ;its surgical management

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