Primary aortojejunal fistula: a case report.

Primary aortoenteric fistulae (AEFs) are extremely rare vascular entities, with fewer than 250 cases reported in the world medical literature as of 1996. Incidence is less than 1 per cent, with a mortality ranging from 33 to 85 per cent. Atherosclerosis remains the most common etiology, accounting for more than two-thirds of the cases reported. Other etiologies include carcinoma, ulcers, gallstones, diverticulitis, appendicitis, and foreign bodies. Early diagnosis is crucial for survival and mandates recognition of the typical "herald bleed." Additional findings on initial presentation frequently include flank pain, abdominal pain, hematemesis, melena, and an abdominal mass. More than 80 per cent of primary AEFs involve the duodenum, with the overwhelming majority located in the third or fourth portion. Successful management of primary AEF requires a high index of suspicion for diagnosis and prompt surgical intervention for survival.