Splenic arteriovenous fistula.

Splenic arteriovenous fistulas are uncommonly encountered, yet they can invoke serious pathophysiological consequences. Four patients treated for splenic fistulas at our institution over the last decade were combined with all previous case reports to produce the most extensive review yet reported, totalling 91 patients. Common etiologies included atherosclerotic aneurysm rupture (44%), congenital malformation (20%), and prior splenectomy (13%). The most frequent manifestation was gastrointestinal hemorrhage (48%), which was most prevalent with congenital fistulas (61% p < 0.05) or fistulas located proximally (54%, p < 0.005). Chronic portal hypertension that was usually characterized by preservation of hepatic function developed in 65%. Management included splenectomy (70%), fistula excision (59%), and occasionally required distal pancreatectomy (10%). Persistent portal hypertension necessitated operative portosystemic shunting in select cases (9%), with an overall 30 day operative mortality of 9%. While percutaneous embolization provided an additional therapeutic option in 4%, optimal management continues to include operative intervention.