WILKIE'S SYNDROME: A RARE CAUSE OF INTESTINAL OBSTRUCTION

INTRODUCTION

Superior mesenteric artery (SMA) syndrome or Wilkie's syndrome is a rare but potentially life threatening gastrointestinal condition. This syndrome is a clinical phenomenon believed to be caused by compression of the third part of the duodenum between the SMA and the aorta, leading to obstruction. Patients may present symptoms of gastrointestinal obstruction, such as with recurrent episodes vomiting, upper abdominal distension and epigastric tenderness⁸. Various etiology theories, clinical course and treatment options have hitherto been discussed⁵. An interdisciplinary teamwork provides the most beneficial diagnostic and therapeutic result in this often underestimated disease.

CASE REPORT

A 27 years old woman was referred to our hospital, with recurrent episodes of profuse vomiting and upper abdominal pain associated with loss of appetite and dyspepsia since two years. She had no other comorbidities. Had been treated at another hospital with proton pump inhibitors, analgesics and intravenous fluids. She had a history of chronic anorexia and progressive loss of weight along with recurrent episodes of vomiting and upper abdominal pain. Clinical examination revealed dehydration, asthenicity (body mass index 19,5 kg/m², weight: 50 kg, length:160 cm), abdominal distension, epigastric tenderness. Laboratory investigations showed a total white cell count of 9 500 mm³ and hypokalaemia (serum potassium: 3 mEq/l). Plain radiograph of the abdomen revealed gastric dilation. Ultrasonography was unremarkable. Upper gastrointestinal endoscopy showed dilated stomach and duodenum. Contrast-enhanced computerized tomography scan revealed grossly distended stomach and duodenum proximal to the third part of the duodenum at the level of the origin of superior mesenteric artery with abrupt narrowing at this level, suggestive of Wilkie's syndrome. While, normally, the angle between the SMA and the aorta is 22° to 60°, in this case, the aortomesenteric angle was 13,5°(Figure 1). In this case, conservative management was inefficient, so surgical treatment aiming to bypass the obstruction by an anastomosis between the jejunum and the proximal duodenum (duodenojejunostomy) was successful.