Wilkie syndrome, a rare chronic intestinal occlusion syndrome, to be considered in the differential diagnosis of gastrointestinal neoplasms

Abstract

Wilkie syndrome, also known as Cast syndrome, is a very rare vascular disease with an incidence of 0.2-0.7% and most often occurs in women. The decrease of the retroperitoneal perivascular fatty cushions which causes the decrease of the aortomesenteric angle and extrinsic compression of the third portion of the duodenum which generates symptoms of intestinal occlusion and weight loss, in most of the patients this pathology can have a chronic course. We present the case of a 58-year-old male patient, with no relevant history, who presents to the emergency department due to dehydration, involuntary weight loss, abdominal distension, oral intolerance, nausea and vomiting. Medical management was started to improve general conditions, diagnostic protocol was initiated due to clinical picture of intestinal occlusion, and finally a diagnosis of Wilkie syndrome was established with the help of abdomino-pelvic tomography with intravenous contrast, surgery was performed with exploratory laparotomy, and laterolateral duodenojejunal anastomosis in two planes, the patient had an adequate postoperative evolution with adequate tolerance to the oral route, and was discharged home without complications.