Recurrent superior mesenteric artery (Wilkie's) syndrome: a case report

Superior mesenteric artery syndrome (SMAS) is a rare condition. The diagnosis is usually made by exclusion. A previously healthy 20-year-old woman who had recurrent SMAS is described. Diagnosis of the condition was difficult. Initially, small-bowel enteroclysis, upper gastrointestinal series and endoscopy, biopsy of gastric and duodenal mucosa, abdominal computed tomography (CT) and ultrasonography were used to make the diagnosis. Abdominal CT suggested pancreatitis causing compressive obstruction of the superior mesenteric artery. Conservative management was helpful at first, but cramping and projectile emesis recurred. Upper gastrointestinal series suggested duodenal distension and a filling defect in the region of the superior mesenteric artery. Repeat endoscopy showed a lateral pulsatile compression in the region of the distal duodenum and SMAS was diagnosed. Medical therapy was not helpful so duodenojejunostomy was carried out. The operation was successful and the patient was symptom-free for 1 year, when the syndrome recurred, with symptoms of periumbilical pain, intermittent episodes of vomiting and loose stools. At reoperation the duodenojejunal anastomosis was found to be displaced to the left of the superior mesenteric artery pedicle causing recurrent obstruction. The duodenojejunostomy was converted to a Roux-en-Y duodenojejunostomy. The patient has since remained well. A MEDLINE search of the literature for the period 1961 to October 1994 revealed that there were no reported cases of a recurrence of SMAS in an otherwise healthy adult patient. In spite of the difficulty in diagnosing this condition, heightened awareness can lead to early diagnosis and avoid unnecessary suffering for the patient.