Wilkie's syndrome: an uncommon cause of intestinal obstruction.

A 10-year-old boy presented with 9 months history of gradually worsening, recurrent postprandial upper abdominal pain, bilious vomiting and loss of weight. On examination the child was undernourished, had epigastric fullness and succusion splash was positive. Ultrasonography of the abdomen suggested a massively distended stomach, while an upper gastrointestinal contrast study showed a hugely dilated stomach along with dilated first and second parts of the duodenum with abrupt cut off at the level of third part of duodenum. Contrast enhanced CT scan of the abdomen revealed dilatation of the second part of the duodenum without any obvious abnormality of the aorta-superior mesenteric artery angle. Upper gastrointestinal endoscopy showed retained fluid and food material within a dilated stomach and second part of the duodenum; scope could not be negotiated into the third part because of an extrinsic compression. The child was diagnosed to be suffering from Wilkie's syndrome. Exploratory laparotomy, performed when conservative management failed, revealed compression of the third part of duodenum by a shortened ligament of Trietz and dense peritoneal bands near the third part of duodenum. The duodenal obstruction was bypassed by performing duodenojejunostomy. The child had an uneventful postoperative recovery. He gained around 6.8 kilograms within next five months.