Superior mesenteric artery syndrome (Wilkie’s syndrome): acute severe presentation in a 10 year old boy and role of early surgical management

Abstract

Superior mesenteric artery syndrome is an atypical condition which was first described in 1861 by von Rokitansky.1 It is an unusual form of upper GI obstruction, also synonymous with Wilkie’s syndrome or cast syndrome or arteriomesenteric duodenal compression or chronic duodenal ileus which occurs due to compression of the third part of duodenum between abdominal aorta posteriorly and superior mesenteric artery anteriorly.2,3 It is mostly due to loss of retroperitoneal fat that normally acts as cushion around the SMA. Patient usually presents with chronic intermittent pain abdomen, bilious vomiting, nausea, early satiety and anorexia. A history of weight loss is usually seen but it is not a necessity.3 The diagnosis is basically by exclusion of other causes of upper GI obstruction and confirmed by upper gastrointestinal contrast study and contrast enhanced CT abdomen. A trial of initial conservative management can be given to all patients but surgical intervention in the form of duodenojejunostomy gives early and complete relief of the symptoms.