Endovascular Treatment of Congenital Arterioportal Fistulas

To characterize anatomy of congenital arterioportal fistulas (CAPF) and correlate this with technique and outcomes of transcatheter embolization (TCE). Retrospective review was conducted of children with CAPF that underwent TCE in a 10-year period. Medical records, imaging and procedure details were reviewed. TCE was performed via transarterial (n = 5), portal (n = 5) or patent ductus venosus (n = 1) approach. Embolic agents used were coils (n = 10), Onyx (n = 1) and Amplatzer septal occluder (n = 1). A total of 7 patients were included (4 female). Median age at treatment was 4 months (2 month-3 year). Most common symptoms were GI bleeding (n = 4), anemia (n = 4) and ascites (n = 3). Hepatopetal flow was seen in all on pre-procedure sonography. On angiography, a simple lesion, with direct fistulous connections, from hepatic arterial (HA) branches to portal venous (PV) system was seen in 3. A complex lesion with multiple connections was identified in 4. In simple lesions, cessation of shunting was achieved in 1 procedure, with embolization of afferent vessels. In complex lesions, multiple procedures were required in 3 of 4 patients. All 4 with complex connections required embolization of the aneurysmal PV segment. There were no major complications. Two minor complications were noted: localized biliary leak in 1 and femoral pseudoaneurysm in 1. There was resolution of symptoms in 6. TCE is effective as primary treatment of CAPF. Patients with simple arteriovenous connections can be treated with embolization of afferent vessels, but children with multiple complex connections usually require embolization of the dilated PV segment.