title: Congenital, solitary, large, intrahepatic arterioportal fistula in a child: management and review of the literature.
abstract: Congenital intrahepatic arterioportal fistula (APF) is a rare condition. In most cases, the symptoms and complications develop during infancy. We report here the incidental finding of a large and solitary congenital APF in a 13-year-old boy, with subsequent related clinical complications. At angiography, an APF connecting the left hepatic artery and the left branch of the portal vein (PV) was demonstrated with reversed flow in the left and main PV. The fistula was successfully occluded, in a single embolisation session, using an Amplatzer occlusion device. This was associated with immediate restoration of normal hepatopetal flow in the PV and followed by resolution of the clinical signs of portal hypertension. This patient is the oldest child with congenital intrahepatic APF to be reported. We emphasise the interest of using a large device (Amplatzer) to occlude a solitary large APF in a single session and, more importantly, to avoid other possible complications related to embolisation.
authors.affiliation: Liver Unit, Birmingham Children's Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH, UK.
authors.forename: Nagappan
authors.initials: N
authors.lastname: Kumar
authors.forename: Jean de Ville
authors.initials: Jde V
authors.lastname: de Goyet
authors.forename: Khalid
authors.initials: K
authors.lastname: Sharif
authors.forename: Patrick
authors.initials: P
authors.lastname: McKiernan
authors.forename: Philip
authors.initials: P
authors.lastname: John
mesh_terms: D000293:Adolescent; D001164:Arteriovenous Fistula / Q000151:congenital* / Q000175:diagnosis* / Q000628:therapy; D004621:Embolization, Therapeutic; D006499:Hepatic Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011856:Radiographic Image Enhancement; D018615:Ultrasonography, Doppler, Color