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Misdiagnosis of primary aortoenteric fistula (PAEF) frequently occurs in clinical practice owing to the rarity of this condition. Herein we present the experience of diagnosis and management for PAEF.Eighteen patients with PAEF at 2 medical centers in China were reviewed. The clinical data, diagnostic procedures, treatment options, and patient outcomes were evaluated.The fistulas were located at esophagus (5), duodenum (8), jejunum (3), ileum (1), and transverse colon (1). The etiologies include atherosclerotic aneurysms and foreign body. Typical abdominal triad (pain, upper GI bleeding, and abdominal pulsating mass) was found in 27.8% of patients, and Chiari's triad (mid-thoracic pain, sentinel hemorrhage, and massive bleeding after a symptom-free interval) was present in 3 of 5 cases with thoracic aortoesophageal fistulas. All patients had an average of 3.6 (1–9) episodes of gastrointestinal bleeding. The interval between the first sentinel hemorrhage and ultimate exsanguination ranged from 5 hours to 5 months (median, 4 days). Six patients (33.3%) were diagnosed or suggested by diagnostic tools including endoscopy, computerized tomography, and arteriography. Others were diagnosed by surgical exploration (7) and autopsy (5). One to 5 rounds (mean 1.8) of misdiagnosis occurred in 15 patients. Six patients recovered from surgery and remained well during a 36-month follow-up. The surgical options used included in situ replacement with vascular graft (3), aneurysmorraphy and closure of fistula (1), and endovascular stenting (2).A high index of suspicion is necessary for correct diagnosis and prompt management of PAEF, especially in patients with aortoiliac aneurysms presenting with gastrointestinal bleeding. In situ graft replacement and endovascular stent-graft may be the preferred therapeutic options.