MALROTATION
Embryology
• The intestinal development proceeds through four key stages:
1. Herniation (around 4 weeks)
2. Rotation
3. Retraction (by 10 weeks)
4. Fixation (from 12 weeks to birth)
• Initially, the midgut undergoes a 90° rotation outside the abdomen, followed
by a second 90° rotation as the intestine returns at the 10th week—placing the
duodenojejunal (DJ) junction to the left of the superior mesenteric artery (SMA) and
the cecum (CC) to the right. The total rotation of 270° counterclockwise positions
the intestines correctly.
Rotational Disorders
1. Nonrotation:
• Failure to complete the normal 270° counterclockwise
rotation.
• Results in the DJ junction lying in the right hemiabdomen and
the cecum in the left hemiabdomen.
• Risks include midgut volvulus due to a narrow mesenteric
pedicle and extrinsic duodenal obstruction from abnormal cecal
attachments.
2. Incomplete Rotation:
• Rotation stops around 180°.
• The cecum is often found in the right upper abdomen and may
be associated with obstructing peritoneal bands.
3. Reversed Rotation:
• Clockwise rotation occurs instead of counterclockwise.
• The duodenum takes an anterior position and may lead to
volvulus or obstruction of the transverse colon.
Clinical Presentation
• Incidence: Estimated at about 1 in 6,000 live births.
• Midgut Volvulus:
• Typically presents acutely in neonates (with up to 75% during
the first month, 15% within the first year, and 10% later).
• Characterized by acute bilious vomiting, abdominal pain,
distention, and rapid progression to metabolic acidosis and shock.
• Duodenal Obstruction:
• Can occur from kinking, torsion, or extrinsic compression by
Ladd’s bands.
• May cause bilious or even non-bilious vomiting with metabolic
imbalances.
• Chronic/Intermittent Symptoms:
• Some patients experience intermittent abdominal pain,
vomiting, failure to thrive, weight loss, and diarrhea.
• Incidental Findings:
• In some cases, malrotation is discovered during contrast
studies or surgery for unrelated conditions.
Diagnosis
• Plain Abdominal X-ray:
• May show a “double bubble” sign and a paucity of distal gas;
however, a normal study does not exclude malrotation.
• Contrast Studies:
• Upper GI Series: Can reveal high-grade duodenal obstruction
with a “bird’s beak” appearance or a corkscrew pattern in partial
obstructions.
• Contrast Enema: Used to locate the position of the cecum.
• Ultrasound/CT Scan:
• Look for inversion of the superior mesenteric artery and vein,
often described as the “whirlpool sign.”
Management
• Immediate Resuscitation:
• Critical in acute presentations with complete intestinal
obstruction.
• Surgical Intervention:
• In cases with volvulus or complete obstruction, urgent
laparotomy is required.
• For malrotation without volvulus, the timing of surgery
(typically a Ladd’s procedure) depends on the patient’s symptoms and
age:
• Symptomatic cases should be addressed
promptly.
• Elective surgery may be considered in
asymptomatic patients, especially in those under 1
year of age (older patients present a more
controversial scenario).
• Operative Technique:
• The Ladd’s procedure is the standard operation, with noted
mortality rates ranging from 3% to 9%.
• Potential Complications:
• Recurrent volvulus, intestinal dysmotility, prolonged ileus,
postoperative adhesions, and in severe cases, short gut syndrome.
