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Intestinal malrotation is a congenital anomaly where the midgut fails to complete its normal 270-degree counterclockwise rotation during fetal development, resulting in abnormal positioning of the small bowel and colon. This condition predisposes patients to midgut volvulus, a life-threatening complication where the small bowel twists around the superior mesenteric artery, causing intestinal obstruction and potential bowel ischemia.
During normal embryologic development, the midgut undergoes a 270-degree counterclockwise rotation around the superior mesenteric artery between the 6th and 10th weeks of gestation. In malrotation, this process is incomplete, leading to abnormal fixation of the mesentery with a narrow base and abnormal positioning of the cecum and duodenum. The shortened mesenteric base creates a predisposition for the entire small bowel to twist around the superior mesenteric vessels, compromising blood flow and causing mechanical obstruction.
The clinical presentation varies by age, with neonates typically presenting with bilious vomiting and signs of intestinal obstruction, while older children may have chronic symptoms including intermittent abdominal pain, failure to thrive, or acute episodes of volvulus. The key diagnostic clue is bilious vomiting in a newborn, which should prompt immediate imaging with upper GI series showing an abnormal position of the ligament of Treitz or a 'corkscrew' appearance of the duodenum. Emergency surgical intervention with Ladd's procedure is indicated to detorse the bowel, divide adhesive bands, widen the mesenteric base, and perform prophylactic appendectomy due to the abnormal cecal position.