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 fifth 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°)
• Anticlockwise rotation by 90°  now loop lies
in a horizontal plane
• Pre- areterial segment lies on right and post
arterial segment on left
• Prearterial segment now undergoes great
increase in length to form jejunum and ileum .
• The coils of jejunum and ileum [ prearterial
segment ] now return to abdominal cavity .
As they do so midgut loop undergoes a
further anticlockwise rotation

• Coils of jejunum and ileum pass behind the

SMA into left half of abdominal cavity.
Duodenum comes to lie behind SMA , coils
of jejunum and ileum lie posterior and to
left of abdominal cavity
• Finally post arterial segment of midgut loop
returns to abdominal cavity , as it does so it
rotates anticlockwise within the result that
transverse colon lie anterior to SMA and caecum
comes to lie on right side.
• At this stage caecum lies below the liver.
Gradually the caecum descends to right iliac
fossa and the ascending , transverse and
descending parts of colon become distinct.
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• 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.
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 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
Non
rotation
• Neither colon or
duodenum undergo
rotation
• Subtype of
malrotation.
• Risk of volvulus is
lower.
• Most common form
of malrotation.
• M:F=2:1
Incomplete rotation/malrotation
•Counterclockwise rotation of
only 180 °.

•Caecum is in epigastrium
overlying 3rd part of duodenum

•Most common form of surgically

treated malrotation
 TABLE 31-1 Incidence of
associated anomalies with
malrotation
Associated Anomaly
Incidence
Intestinal atresia 5-26%
Imperforate anus 0-9%
Cardiac anomalies 7—
Duodenal web 13%
Meckel diverticulum 1—2%
Hernia 1—4%
Trisomy 21 0—7%
3—
Rare: esophageal atresia,
10OzL
biliary atresia, mesenteric cyst,
craniosynostosis, Hirschsprung disease, intestinal
duplication.
REVERSE ROTATION • Rotates clockwise.
• DJ loop anterior to SMA
and transverse colon
posterior to SMA.
• Causes compression of
colon by SMA -
obstruction.
• Small intestine lie on left
and large intestine on
right . Caecum is found in
center.
• Ileocecal volvulus- due to
inadequate fixation of
right colon.
 CLINICAL MANIFESTATIONS
• Acute Midgut Volvulus – First month of life
• Asymptomatic
• Chronic Midgut volvulus - children older than
2 years
• Acute duodenal obstruction secondary to
congenital bands - common in neonates and
infants
• Ladd bands peritoneal bands extend from
abnormally positioned caecum to peritoneum
and liver crossing deodenum.
• 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
 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.
 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
Figure 6. Malrotation with obstructing duodenal bands. Radiographs show marked
distention and obstruction of proximal part of the duodenum (a) and an abnormal location of
the duodenojejunal junction and the proximalpart of the jejunum in the right upper
abdominal quadrant
“Double bubble” in
duodenalobstruction
Figure 9. Malrotation with volvulus in a neonate. Radiographs obtained with
barium administered via a nasogastrictube show a corkscrew appearance of the
duodenum and an abnormal position of the duodenojejunal junction on
both frontal (a) and lateral (b) views, features diagnostic of volvulus, which
constitutes a surgical emergency. Twisted on there short mesentery.
 Ultrasound
• Reversal of the normal anatomic
relationship between the SMA and SMV

• [Normal : SMV lies on right of SMA]

• “whirlpool sign” – midgut volvulus.

 CT Abdomen
• Anatomic location of small bowel on right
and colon on left
• Relationship of the superior mesenteric
vessels–“verticallyplacedorinvertedsides”
• Aplasia of the uncinate process
• Features of volvulus / obstruction / gangrene
• Other associated anomalies
Reversal of SMA and Whirlpool sign
SMV
 Treatment
Aim of treatment- Reduce the recurrence of
volvulus, not the position.
Surgery(Ladd’s procedure)
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