IS Case 178: Intestinal malrotation
Imaging Sciences URMC 2008
Publication Date: 2009-05-21
Intestinal malrotation is congenital abnormal position of the bowel within the peritoneal cavity. There are two main types: intestinal non-rotation and incomplete rotation around the superior mesenteric artery. Malrotation is accompanied by either abnormal bowel fixation by mesenteric bands or absence of normal fixation of the bowel. Both of these abnormalities lead to an increased risk of bowel obstruction, acute or chronic volvulus, and bowel necrosis. The most severe condition that malrotated patients are predisposed to is midgut volvulus, a surgical emergency due to twisting of the gut around the superior mesenteric artery and vein. Malrotation occurs in 1 in 500 births. Approximately 40% of patients with malrotation present within the first week of life, usually with bilious emesis. Overall, approximately 75% of malrotated patients are diagnosed in the first year of life. There are multiple associated congenital anomalies such as gastroschisis, omphalocele, duodenal atresia or congenital diaphragmatic hernia. The diagnosis is usually made by an upper GI examination demonstrating abnormal location of the duodenojejunal junction. Normal rotation is present if the duodenal C-loop crosses the midline and places the duodenojejunal junction to the left of the left-sided pedicle at a vertebral level greater than or equal to the level of the duodenal bulb. Other radiologic findings suggestive of malrotation include inversion of the superior mesenteric artery and vein and abnormal location of the cecum. The definitive treatment for malrotation is surgical. The Ladd procedure is reduction of volvulus (if present), division of mesenteric bands, placement of the small bowel on the right and large bowel on the left of the abdomen, and appendectomy.
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