Date: 30.07.2016
Venue: Classroom, Paediatric Surgery
Department, BSMMU
Presented by:
Dr. Mominul Haider
Phase-A Resident
MS (Urology)
Objectives
To discuss and understand -
• Normal rotation of gut
• Definition of Malrotation
• Presentation
• Investigation
• Treatment
• Complication
Introduction
• Malrotation refers to a group of congenital
anomalies resulting from aberrant intestinal
rotation and fixation.
• Incidence : 1/6000 live births
• No sex/race predilection
Normal rotation of gut
Stages of Normal Rotation
Herniation
Rotation
Retraction
Fixation
Physiologícal Herniation
• Development of the primary intestinal loop is
characterized by rapid elongation, particularly
of the cephalic limb.
• The abdominal cavity temporarily becomes
too small to contain all the intestinal loops,
and they enter the extraembryonic cavity in
the umbilical cord during the sixth week of
development
Rotation
• The primary intestinal loop rotates around an
axis formed by the superior mesenteric artery
• When viewed from the front, this rotation is
counterclockwise, and it amounts to
approximately 270° when it is complete
• Rotation occurs during herniation (about 90°)
as well as during return of the intestinal loop
into the abdomen (Remaining 180°)
Retraction
• During the 1Oth week, herniated intestinal loops
begin to return to the abdominal cavity.
• The proximal portion of the jejunum, the first part
to reenter the abdominal cavity, comes to be on
the left side
• The caecal bud is the last part of the gut to
reenter the abdominal cavity.
Key points in embryology
• Intestinal rotation starts at 5th week and
completes by 11th week
• Midgut is supplied by SMA
• Rotation takes place around SMA axis
• 270 degree counterclock wise rotation of
prearterial and post arterial limb.
• Ladds bands attach to the cecum irrespective
of its postion at the end of rotation from right
paracolic region.
Rotational disorders
Non rotation
Incomplete rotation
Reverse rotation
Nonrotation
• Neither colon or
duodenum undergo
rotation
• Most common form of
malrotation.
• M:F=2:1
Incomplete rotation
• Counter clock wise
rotation of only 180
• Caecum in the
epigastrium overlying
3rd part of duodenum.
• Most common form of
surgically treated
malrolation.
REVERSE ROTATION • Rotates clockwise.
• DJ loop anterior to SMA
and transverse colon
posterior to SMA.
• Causes compression of
colon by SMA -
obstruction.
• Ileocecal volvulus- due to
inadequate fixation of
right colon.
CLINICAL MANIFESTATIONS
• Asymptomatic
• Acute Midgut Volvulus – First month of life
• Chronic Midgut volvulus - children older than 2
years
• Acute duodenal obstruction secondary to
congenital bands - common in neonates and
infants
• Chronic duodenal obstruction secondary to
congenital bands
• Reverse rotation with colonic obstruction –
Rare, usually seen in adults
• Internal Hernia (Mesocolic hernia)
• Volvulus of the Caecum – seen in old patients
Presentation
 Usually in early age, but may present later in
life.
 75% present during 1st month of life.
 15% present within the 1st year.
• Bilious vomiting remains the cardinal sign of
neonatal intestinal obstruction, and
malrotation must be the presumed diagnosis
until proven otherwise.
• Other signs in the neonate include abdominal
pain and distention.
• The inconsolable infant may rapidly
deteriorate as metabolic acidosis quickly
advances to hypovolemic shock.
• Late signs include abdominal wall erythema
and hematemesis or melena from progressive
mucosal ischemia.
Acute Midgut Volvulus
• Sudden onset of bilious vomiting in a
previously healthy, growing infant.
• With the onset of proximal intestinal
obstruction, the distal colon empties; lower
abdomen may appear scaphoid.
• As vascular compromise progresses,
intraluminal bleeding may occur and blood is
often passed per rectum.
• Crampy abdominal pain is common.
Acute duodenal obstruction
• An infant or newborn usually presents with
forceful, bilious vomiting.
• Abdominal distention may or may not be
present
• The obstruction may be complete or
incomplete, so meconium or stool may have
been passed.
• Jaundice may be seen
Radiologic Diagnosis of Abnormalities of
Rotation and Fixation
• Radiologic studies play a critical role in
establishing a diagnosis of intestinal
malrotation.
Plain radiograph
• Right-sided jejunal markings
• Absence colonic shadow in RIF
• Features of complications
- Dilated bowel loops
- Air fluids levels
- Pneumoperitoneum
Contrast X Ray
• Delineation of the duodenojejunal junction
remains the most important diagnostic tool.
• The duodenum should be seen traveling across
the spine to the left.
• Additionally, the lateral film will show the
duodenum obtaining a retroperitoneal, posterior
position.
• Abnormal findings include
– positioning of the duodenojejunal flexure to the right
of the spine, obstruction of the duodenum
– The “coil spring,” “corkscrew,” or “beak” appearance
of the obstructed proximal jejunum
• Double-bubble sign in acute duodenal
obstruction.
• Gasless abdomen in midgut volvulus (gut is filled
with fluid)
Normal position and fixation of Duodenum
“Double bubble” in duodenal obstruction
Ultrasound
• Reversal of the normal anatomic relationship
between the SMA and
• “whirlpool sign” - midgut volvulus.
CT Abdomen
• Anatomic location of small bowel on right and
colon on left
• Relationship of the superior mesenteric
vessels – “vertically placed or inverted sides”
• Aplasia of the uncinate process
• Features of volvulus / obstruction / gangrene
• Other associated anomalies
Reversal of SMA and SMV Whirlpool sign
Treatment
Aim of treatment- Reduce the recurrence of
volvulus, not the position.
Preoperative preparation
• Aggressive resuscitation with fluid &
electrolyte.
• Intravenous broad spectrum antibiotics.
• Taken to the operating room for immediate
exploration.
• Placement of a Nasogastric tube.
Surgery (Ladd’s procedure)
• 2nd look operation is usually performed when
there are multiple areas of bowel of
questionable viability, when the entire midgut
appears nonviable, or when clinical signs &
symptoms suggest progressive loss of
intestine
Post operative care
• Nasogastric decompression
• Total parenteral nutrition until return of bowel
function.
Complications
• Diarrhea & dehydration in short bowel
syndrome
• Postoperative intussusception
• Postoperative adhesion
• Recurrent volvulus
References
• Pediatric surgery. —7th ed. / editor in chief,
Arnold G. Coran ; associate editors, N. Scott
Adzick . . [et al.]
• Ashcraft’s pediatric surgery / [edited by]
George Whitfield Holcomb III, J. Patrick
Murphy ; associate editor, Daniel J. Ostlie. —
5th ed.
Malrotation of gut

Malrotation of gut

  • 2.
    Date: 30.07.2016 Venue: Classroom,Paediatric Surgery Department, BSMMU Presented by: Dr. Mominul Haider Phase-A Resident MS (Urology)
  • 3.
    Objectives To discuss andunderstand - • Normal rotation of gut • Definition of Malrotation • Presentation • Investigation • Treatment • Complication
  • 4.
    Introduction • Malrotation refersto a group of congenital anomalies resulting from aberrant intestinal rotation and fixation. • Incidence : 1/6000 live births • No sex/race predilection
  • 5.
    Normal rotation ofgut Stages of Normal Rotation Herniation Rotation Retraction Fixation
  • 6.
    Physiologícal Herniation • Developmentof the primary intestinal loop is characterized by rapid elongation, particularly of the cephalic limb. • The abdominal cavity temporarily becomes too small to contain all the intestinal loops, and they enter the extraembryonic cavity in the umbilical cord during the sixth week of development
  • 8.
    Rotation • The primaryintestinal loop rotates around an axis formed by the superior mesenteric artery • When viewed from the front, this rotation is counterclockwise, and it amounts to approximately 270° when it is complete • Rotation occurs during herniation (about 90°) as well as during return of the intestinal loop into the abdomen (Remaining 180°)
  • 12.
    Retraction • During the1Oth week, herniated intestinal loops begin to return to the abdominal cavity. • The proximal portion of the jejunum, the first part to reenter the abdominal cavity, comes to be on the left side • The caecal bud is the last part of the gut to reenter the abdominal cavity.
  • 16.
    Key points inembryology • Intestinal rotation starts at 5th week and completes by 11th week • Midgut is supplied by SMA • Rotation takes place around SMA axis • 270 degree counterclock wise rotation of prearterial and post arterial limb. • Ladds bands attach to the cecum irrespective of its postion at the end of rotation from right paracolic region.
  • 17.
  • 19.
    Nonrotation • Neither colonor duodenum undergo rotation • Most common form of malrotation. • M:F=2:1
  • 21.
    Incomplete rotation • Counterclock wise rotation of only 180 • Caecum in the epigastrium overlying 3rd part of duodenum. • Most common form of surgically treated malrolation.
  • 22.
    REVERSE ROTATION •Rotates clockwise. • DJ loop anterior to SMA and transverse colon posterior to SMA. • Causes compression of colon by SMA - obstruction. • Ileocecal volvulus- due to inadequate fixation of right colon.
  • 24.
    CLINICAL MANIFESTATIONS • Asymptomatic •Acute Midgut Volvulus – First month of life • Chronic Midgut volvulus - children older than 2 years • Acute duodenal obstruction secondary to congenital bands - common in neonates and infants
  • 25.
    • Chronic duodenalobstruction secondary to congenital bands • Reverse rotation with colonic obstruction – Rare, usually seen in adults • Internal Hernia (Mesocolic hernia) • Volvulus of the Caecum – seen in old patients
  • 26.
    Presentation  Usually inearly age, but may present later in life.  75% present during 1st month of life.  15% present within the 1st year. • Bilious vomiting remains the cardinal sign of neonatal intestinal obstruction, and malrotation must be the presumed diagnosis until proven otherwise.
  • 27.
    • Other signsin the neonate include abdominal pain and distention. • The inconsolable infant may rapidly deteriorate as metabolic acidosis quickly advances to hypovolemic shock. • Late signs include abdominal wall erythema and hematemesis or melena from progressive mucosal ischemia.
  • 28.
    Acute Midgut Volvulus •Sudden onset of bilious vomiting in a previously healthy, growing infant. • With the onset of proximal intestinal obstruction, the distal colon empties; lower abdomen may appear scaphoid. • As vascular compromise progresses, intraluminal bleeding may occur and blood is often passed per rectum. • Crampy abdominal pain is common.
  • 29.
    Acute duodenal obstruction •An infant or newborn usually presents with forceful, bilious vomiting. • Abdominal distention may or may not be present • The obstruction may be complete or incomplete, so meconium or stool may have been passed. • Jaundice may be seen
  • 30.
    Radiologic Diagnosis ofAbnormalities of Rotation and Fixation • Radiologic studies play a critical role in establishing a diagnosis of intestinal malrotation.
  • 31.
    Plain radiograph • Right-sidedjejunal markings • Absence colonic shadow in RIF • Features of complications - Dilated bowel loops - Air fluids levels - Pneumoperitoneum
  • 33.
    Contrast X Ray •Delineation of the duodenojejunal junction remains the most important diagnostic tool. • The duodenum should be seen traveling across the spine to the left. • Additionally, the lateral film will show the duodenum obtaining a retroperitoneal, posterior position.
  • 34.
    • Abnormal findingsinclude – positioning of the duodenojejunal flexure to the right of the spine, obstruction of the duodenum – The “coil spring,” “corkscrew,” or “beak” appearance of the obstructed proximal jejunum • Double-bubble sign in acute duodenal obstruction. • Gasless abdomen in midgut volvulus (gut is filled with fluid)
  • 35.
    Normal position andfixation of Duodenum
  • 36.
    “Double bubble” induodenal obstruction
  • 38.
    Ultrasound • Reversal ofthe normal anatomic relationship between the SMA and • “whirlpool sign” - midgut volvulus.
  • 41.
    CT Abdomen • Anatomiclocation of small bowel on right and colon on left • Relationship of the superior mesenteric vessels – “vertically placed or inverted sides” • Aplasia of the uncinate process • Features of volvulus / obstruction / gangrene • Other associated anomalies
  • 43.
    Reversal of SMAand SMV Whirlpool sign
  • 44.
    Treatment Aim of treatment-Reduce the recurrence of volvulus, not the position.
  • 45.
    Preoperative preparation • Aggressiveresuscitation with fluid & electrolyte. • Intravenous broad spectrum antibiotics. • Taken to the operating room for immediate exploration. • Placement of a Nasogastric tube.
  • 46.
  • 48.
    • 2nd lookoperation is usually performed when there are multiple areas of bowel of questionable viability, when the entire midgut appears nonviable, or when clinical signs & symptoms suggest progressive loss of intestine
  • 49.
    Post operative care •Nasogastric decompression • Total parenteral nutrition until return of bowel function.
  • 50.
    Complications • Diarrhea &dehydration in short bowel syndrome • Postoperative intussusception • Postoperative adhesion • Recurrent volvulus
  • 51.
    References • Pediatric surgery.—7th ed. / editor in chief, Arnold G. Coran ; associate editors, N. Scott Adzick . . [et al.] • Ashcraft’s pediatric surgery / [edited by] George Whitfield Holcomb III, J. Patrick Murphy ; associate editor, Daniel J. Ostlie. — 5th ed.