Wilkie's Syndrome: An Unexpected Finding

Wilkie's syndrome is a rare pathology caused by extrinsic compression of the third part of the duodenum by the superior mesenteric artery (SMA) at its origin. The symptoms are variable and non-specific consisting of postprandial abdominal pain, nausea and vomiting, early satiety, anorexia, and weight loss. A contrast-enhanced CT scan is the standard imaging modality. Surgery is reserved for severe cases or those unresponsive to pharmacological treatment. We present a case of a 66-year-old woman with a history of prolonged postprandial abdominal pain, nausea, and substantial weight loss (30 kg in four months). Laboratory tests revealed acute renal failure with hypokalemia due to severe dehydration. She underwent an abdominal-pelvic CT scan that showed enlargement of the second and third parts of the duodenum, without an identifiable cause, followed by an upper gastrointestinal (GI) endoscopy that showed a dilated duodenum until D2 and inability of progression to D3, without mucosal abnormalities. Her MRI revealed considerable gastric and proximal duodenum distension with compression of D3 by the SMA. The patient underwent a laparoscopic duodenojejunostomy with intra-operative findings consistent with the diagnosis. The procedure and the postoperative period were uneventful, and the patient was discharged on the ninth postoperative day. Gastrografin study made at day six postop showed normal progression of the oral contrast. At the outpatient reevaluation one month postop, she remained asymptomatic and with progressive weight gain. Wilkie's syndrome is a rare form of intestinal obstruction, which is commonly disregarded. Its non-specific symptoms make it a challenging diagnosis and imply a high clinical suspicion. Among the different surgical options, duodenojejunostomy presents the best outcomes.