Wilkie’s Syndrome: A Rare Cause of Duodenal Obstruction

Abstract

Introduction: Wilkie’s syndrome, or superior mesenteric artery (SMA) syndrome, is a rare cause of partial or complete duodenal obstruction due to exterior compression of the third portion of duodenum by the SMA. Loss of retroperitoneal fatty tissue as a result of a variety of debilitating conditions is believed to be the etiologic factor causing the acute angulation between SMA and aorta. This is a true syndrome with characteristic clinical picture, which troubles patients suffering from it before a firm diagnosis is established. Case Report: A 17-year-old girl presents with abdominal pain and intractable vomiting following a significant weight loss after a hospitalization for pneumonia and 2 years of extensive investigation to no avail. CT of abdomen showed a narrow SMA-Aorta angle of 10 degrees (Figure 1), gastric dilatation, and a short SMA-Aorta distance of 5.5 mm (Figure 2), consistent with SMA syndrome. GI series showed severely delayed transit through the third portion of duodenum. Conservative management failed and the patient did well after duodenojejunostomy.Figure 1: CAT image showing the entrapment of the duodenum between the aorta and the SMA with an angle of 10 degrees.Figure 2: CAT scan showing the SMA and the aorta separated by 5.5 mm and severe gastric distension.Discussion: SMA syndrome is an atypical cause of proximal intestinal obstruction. Identification of this underestimated syndrome can be a diagnostic dilemma, and is frequently delayed, as in our case. Conservative approach is the first-line treatment. Should it fail, duodenojejunostomy is considered the operation of choice. Increased awareness is required to improve early recognition in order to reduce irrelevant tests and unnecessary treatments.