Wilkie's syndrome: A rare cause of vomiting and weight loss

Nazif Erkan,Ali Coşkun ,Deniz A Uçar ,Erdem Barış Cartı ,M Zeki Yıldırım ,Erkan Oymacı

doi:10.5348/ijcri-2014-02-461-cr-13

Abstract

Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is extremely rare and is characterized by postprandial epigastric pain, nausea, vomiting and loss of appetite, with subsequent weight loss, which aggravates the condition of the patients. The syndrome is caused by compression of the third part of the duodenum in the angle between the aorta and the superior mesenteric artery. Herein, we presented a patient with Wilkie's syndrome and discussed the diagnostic difficulties and surgical treatment options. We report a case of 13-year-old female who was diagnosed with SMA syndrome. There was no history of recent trauma, surgery, prolonged immobilisation or neurological illness. Her weight loss was gradual. The patient was initially diagnosed as anorexia nervosa due to her symptoms of vomiting and anorexia without any clinical cause, but later on, she underwent computed tomography scan and was diagnosed with SMA syndrome. Her SMA syndrome, resolved after successful nonoperative management based on accepted guidelines for nutritional therapy, thus avoiding the need for operation. One year follow-up was uneventful. Superior mesenteric artery syndrome is a rare cause of intestinal obstruction and its first-line treatment is usually conservative with jejunal or parenteral nutrition for restoration of the aortomesenteric fatty tissue. If conservative management fails, surgical options , open or laparoscopic duodenojejunostomy or duodenal mobilization. Its recognition is important because early diagnosis of a partial obstruction may allow for medical rather than surgical intervention, as exemplified by our case.

Highlights

Superior mesenteric artery (SMA) syndrome, known as Wilkie’s syndrome, is extremely rare and is characterized by postprandial epigastric pain, nausea, vomiting and loss of appetite, with subsequent weight loss, which aggravates the condition of the patients
Its recognition is important because early diagnosis of a partial obstruction may allow for medical rather than surgical intervention, as exemplified by our case
Any factor that sharply narrows the aortomesenteric angle to approximately 6–25° can cause entrapment and compression of the third part of the duodenum as it passes between the SMA and aorta, resulting in SMA syndrome

Summary

Introduction

Superior mesenteric artery (SMA) syndrome which is known as Wilkie’s syndrome, is a rare cause of upper gastrointestinal obstruction [1,2,3]. Wilkie provided a more detailed clinical and pathophysiologic description in a series of 64 patients and suggested treatment approaches This is usually associated with conditions that cause significant weight loss, such as anorexia nervosa, malabsorption, or hypercatabolic states such as burns, major surgery, severe injuries, or malignancies as fat loss causes direct compression of third part of duodenum with superior mesenteric. Endoscopic examination of the upper gastrointestinal tract revealed mild esophagitis, dilated stomach and proximal duodenum, and narrowing of the third part of the duodenum due to a pulsating extrinsic compression. She was hospitilized and total parenteral nutrition was administered with Daily 950 kcal (35 kcal/kg/d) of oliclinomel N7 (Baxter, Eczacıbası, Turkey). She gained 4 kg weight before discharge and she has been following without any complication and complain for one year

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CONCLUSION