The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification.
Congenital intrahepatic arterioportal fistula is a rare but treatable cause of portal hypertension for which early recognition may lead to successful radiological management. We report an infant presenting with severe failure to thrive, melena and splenomegaly due to a congenital intrahepatic arterioportal fistula, successfully ablated after multiple trials of superselective transarterial embolization. Comprehensive review of congenital cases provides an understanding of the key clinical features defining this syndrome. A classification system is proposed, upon which treatment decisions may be based.
Norton
Seamus P
SP
Department of Pediatrics, British Columbia's Children's Hospital, Faculty of Medicine, University of British Columbia, Vancouver, Canada.
Jacobson
Kevan
K
Moroz
Stanley P
SP
Culham
Gordon
G
Ng
Vicky
V
Turner
Justine
J
John
Philip
P
D015746:Abdominal Pain / Q000209:etiology; D001164:Arteriovenous Fistula / Q000145:classification* / Q000151:congenital* / Q000628:therapy*; D002675:Child, Preschool; D004621:Embolization, Therapeutic; D005183:Failure to Thrive / Q000209:etiology; D006499:Hepatic Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007223:Infant; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome