SUPERIOR MESENTERIC ARTERY SYNDROME WILKIE SYNDROME

REPORT OF TWO CASES

Abstract

Introduction: Superior Mesenteric Artery Syndrome / Wilkie Syndrome is characterized by the compression of the third portion of the duodenum, due to the reduction of the aorta angulation with the superior mesenteric artery. A rare disease, with few cases described in the medical literature. Case 01: A 13-year-old male patient, diagnosed with Blackfan-Diamond Anemia and with acute intestinal occlusion for 2 days with previous recurrent episodes of intestinal occlusion. CT showing gastrectasis and dilation of slender loops up to the third portion of the duodenum. Case 02: 18-year-old female patient, without previous comorbidities, with high intestinal obstruction for 7 days, with CT showing duodenal obstruction and reduction of aorto-mesenteric angulation. Discussion: The most prevalent age group occurs between 18 and 35 years. Associated with mainly weight loss and Cast Syndrome. The diagnosis is given by clinical suspicion followed by imaging tests such as upper gastrointestinal tract radiography and contrast abdominal CT. Treatment may be conservative with weight gain or surgical measures, the duodenojejunoanastomosis is the technique of choice. Blackfan-Diamond anemia despite being related to staturo-ponderal alterations no association was identified in the medical literature with Wilkie Syndrome. Conclusion: Differential diagnosis should be made in young patients with repetitive vomiting. Upper Gastrointestinal Endoscopy may delay the diagnosis.