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Dr Chor Ath Malrotation of the gut results when the embryologic sequence of gut herniation, rotation and return to the abdomen is disturbed Development of the human gut takes place during the first months of fetal life. In the normal embryo, physiologic herniation of the gut through the umbilicus at 6 weeks is accompanied by a 270 degree counterclockwise rotation of the developing intestine around the superior mesenteric artery (SMA). By 10 to 12 weeks, the intestine returns to the abdomen, and assumes its normal adult anatomic position.1 2 The normal small bowel mesentery has a broad attachment stretching diagonally from the duodenojejunal junction (in the left upper quadrant) to the cecum (in the right lower quadrant). The point of attachment at the duodenojejunal junction is referred to as the Ligament of Treitz. Malrotation predisposes to two problems: midgut volvulus and small bowel obstruction. The close proximity of the cecum to the duodenum is associated with a narrow stalk of mesentery about which the gut may twist, resulting in midgut volvulus. Accompanying superior mesenteric vascular compromise (first venous followed by arterial) can lead to life-threatening ischemia of the small bowel and gangrenous necrosis Coiling of the duodenum with the ascending colon will produce complete or partial duodenal obstruction. Abnormal bands of peritoneum, called Ladd bands, are often found connecting the duodenum and cecum anteriorly; these may (rarely) cause duodenal obstruction.2 5 Malrotation with midgut volvulus classically presents in the neonate with bilious vomiting and high intestinal obstruction. While most neonates with bilious vomiting will not have midgut volvulus, this diagnosis must be ruled out. Volvulus is a surgical emergency with a mortality rate of at least 15%.2 Malrotation, due to the danger of volvulus, is also treated as an emergency. An upper GI series is the diagnostic test for malrotation with midgut volvulus, and must be performed unless delay in surgery would further compromise outcome, as in the case of a moribund child.5 8 This is performed with barium, either administered by bottle or through a nasogastric tube. The normal duodenojejunal junction lies to the left of the left-sided spinal pedicle at the level of the duodenal bulb on a true frontal view.5 The duodenal C-sweep courses posteriorly, inferiorly, anteriorly, then superiorly. Findings on Upper GI in malrotation include:2 5 the duodenojejunal junction is displaced downward and to the right on the frontal view abnormal course of duodenum on lateral view abnormal position of jejunum: lying on the right side of the abdomen In malrotation with midgut volvulus, findings also include:9 dilated fluid-filled duodenum proximal small bowel obstruction "corkscrew" pattern: proximal jejunum spiraling downward in the right or mid upper abdomen in midgut volvulus (rare) mural edema, thick folds Malrotation is very rare in the absence of all of these signs. Sensitivity of the UGI is 85-95%,1 10 with a higher specificity (false positives are rare). Although a displaced duodenojejunal junction is a very sensitive indicator of malrotation, this finding can also occur secondary to distended bowel, masses, and splenomegaly.5 Scoliosis makes it difficult to rule out malrotation because the normal bony landmarks are lost. Barium enema, once used to detect an abnormally positioned cecum, is felt to lack sensitivity (15-20% of malrotations are associated with a normally positioned cecum)5 and a high or mobile cecum can be normal in infancy).1 9 Ultrasound and CT may suggest the diagnosis of malrotation but the sensitivity and specificity are low compared to the upper GI series, and therefore an upper GI examination is mandatory to confirm the diagnosis if suspected on CT or ultrasound.11 12 If the SMV lies to the left of, or posterior to the SMA, malrotation is suggested. However, normal vascular positioning - a SMV slightly ventral and to the right of the SMA - can be found in approximately 30% of malrotation cases. The "whirlpool sign" on color Doppler shows mesentery and flow within the superior mesenteric vein (SMV) wrapping around the SMA, indicating malrotation with volvulus.13 14 A dilated, fluid-filled duodenum is frequently seen in cases of obstruction without volvulus.