Malrotation of the bowel is failure of the bowel to assume its normal place in the abdomen during intrauterine development.
During embryonic development, the primitive bowel protrudes from the abdominal cavity. As it returns to the abdomen, the large bowel normally rotates counterclockwise, with the cecum coming to rest in the right lower quadrant. Incomplete rotation, in which the cecum ends up elsewhere (usually in the right upper quadrant or midepigastrium), may cause bowel obstruction due to retroperitoneal bands (Ladd bands) that stretch across the duodenum or due to a volvulus of the small bowel, which, lacking its normal peritoneal attachment, twists on its narrow, stalk-like mesentery. Other malformations occur in 30 to 60% of patients, most commonly other GI malformations (eg, gastroschisis, omphalocele, diaphragmatic hernia, intestinal atresia).
Patients with malrotation can present in infancy or in adulthood with acute abdominal pain and bilious emesis, with an acute volvulus, with typical reflux symptoms, or with chronic abdominal pain. Bilious emesis in an infant is an emergency and should be evaluated immediately to make sure the infant does not have malrotation and a midgut volvulus; untreated, the risk of bowel infarction and subsequent short bowel syndrome or death is high.
Plain films of the abdomen should be done immediately. If they show dilated small bowel, a paucity of bowel gas distal to the duodenum, or both (suggesting a midgut volvulus), further diagnosis and treatment must be done emergently. Barium enema typically identifies malrotation by showing the cecum outside the right lower quadrant. If the diagnosis remains uncertain, an upper GI series can be done cautiously.
The presence of malrotation and midgut volvulus is an emergency requiring immediate surgery, which is a Ladd procedure with lysis of the retroperitoneal bands and relief of the midgut volvulus.
Last full review/revision March 2013 by William J. Cochran, MD
Content last modified May 2013