Superior mesenteric artery syndrome (Wilkie Syndrome) with unusual clinical onset: Description of a rare case

Wilkie''s Syndrome is a very rare disease caused by reduction of aorto-mesenteric space with consequent duodenum compression. It can combine with left renal vein stenosis which, when symptomatic, is known as "Nutcracker Syndrome". We describe a clinical onset case with epigastric pain without vomiting in a normal weight patient. 28-year-old woman who came to our observation for intense epigastric pain after a weight loss of 14 kg in 4 months. Multidetector Computed Tomography and Ultrasound revealed gastric and duodenal overdistension with hydro-air levels, severe duodenum stenosis, and left renal vein compression. Wilkie''s Syndrome is common in anorexic individuals suffering from recurrent postprandial vomiting, onset with severe epigastric pain, without vomiting, is quite unusual. High-calorie diet must be first therapeutic approach, in case of failure treatment of first choice should be endovascular stenting and, only in selected cases, surgical treatment should be used because it is very invasive and burdened with numerous complications. Failure to diagnose this disease can expose patients to serious health risks.