The primary aortoenteric fistula in The Netherlands--the unpublished cases

OBJECTIVE: Primary aortoenteric fistula is a rare disorder of which only four patients have been reported in the Dutch literature so far. The objective of our study was to obtain more realistic figures on the incidence of this condition, with data on the clinical presentation, diagnostic procedures, treatment and results in a group of patients not previously reported as "case histories". METHODS: A questionnaire was sent to all surgical clinics in The Netherlands. Out of 180 questionnaires, 102 have been returned reporting 27 patients to which data of eight others treated in our own institution were added. RESULTS: In all but one of these 29 patients the fistula was caused by an atherosclerotic aneurysm, the one exception being caused by an ingested cocktail pin. Gastrointestinal haemorrhage was the predominant symptom, being present in 28 of the patients, while the complete triad of haemorrhage, pain and a pulsating mass was found in only eight patients. Twenty-seven patients were treated with an in situ graft of which 14 are doing well at long term follow-up. CONCLUSIONS: Primary aortoenteric fistula is far more common than one would expect from the number of patients reported in literature. A high index of suspicion based on a complete physical examination remains the key to a correct diagnosis. Direct closure of the intestine and in situ grafting of the aorta is the treatment of first choice.