INTRODUCTIONCongenitalhepatoportalarteriovenousfistulasareararecause of segmental portal hypertension and splenic veinthrombosis (1–4). These may be intrahepatic or extrahe-patic in location and present during infancy or earlychildhood with portal hypertension and gastrointestinalbleeding secondary to varices, ascites, malabsorption,anemia, and congestion of the bowel (5). In childhood,arterioportal fistulas are usually intrahepatic andregarded as congenital. The congenital fistulas aregenerally due to cavernous hemangioma, hereditarytelangiectasia,andEhlers-Danlossyndrome.Theacquiredarterioportal fistulas are usually traumatic, iatrogenic(following liver biopsy), or a result of rupture of a hepaticartery aneurysm (6).CASE REPORTA 16-year-old boy presented with a history ofabdominal pain and distension for the previous 3 months.There were no complaints of fever, loss of weight, loss ofappetite,orgastrointestinalbleeding.Therewasnohistoryof trauma, liver biopsy, or any other predisposingcondition, nor any cutaneous stigmata to suggest Ehlers-Danlos syndrome or hereditary telangiectasia. Thehemogram and liver function tests were within normallimits. Ultrasound examination revealed evidence ofmassive splenomegaly. On color Doppler imaging, therewas gross dilation of the left portal vein and the hepaticartery.High-velocitypulsatileflowwasencounteredintheportal vein, which showed a hepatofugal flow pattern.Thesplenic vein was visualized only at the splenic hilum.Computed tomography confirmed the aforementionedfindings and showed a thrombosed splenic vein withmultiple collateral channels at the splenic hilum, fundusofstomach,andaroundthegastroesophagealjunction.Theportal vein was dilated with gross ectasia of its leftbranch (Figs. 1 and 2). The celiac axis and the hepaticartery were also dilated. These findings were conclusiveforportalhypertensionasaresultofarterioportalfistula,soendoscopy was not performed and the patient was takendirectly for angiography. Intraarterial catheter angiogra-phy demonstrated the fistulous communication betweenthe right hepatic artery and left portal vein (Figs. 3 and 4).On selective superior mesenteric artery injection in thevenous phase, there was opacification of the superiormesenteric vein but the portal vein was not filling up,which was attributed to reversed flow in the portal vein.The mean pressure measurement taken at the time ofangiography in the right hepatic artery and left portal veinwas85mmHg,withnopressuregradient.Becausethiswasthought to be a congenital fistula, the surgical option wasconsidered and the child underwent splenectomy withhepatic artery ligation. He did well postoperatively, andfollow-upDopplerexaminationrevealedthrombosisofthehepatic artery with restoration of antegrade flow in theportalvein.Thechildhadnonewcomplaintsduring1yearof follow-up.DISCUSSIONThe earliest description of a hepatoportal arteriovenousfistula was made by Sachs in 1892 in a 60-year-old manwho died of bleeding esophageal varices secondaryto portal hypertension (7). Congenital intrahepatic hepa-toportal arteriovenous fistulas are extremely rare and aregenerally attributed to hereditary hemorrhagic telangiec-tasia(ie,Rendu-Osler-Weber syndrome) orEhlers-Danlossyndrome (8). The acquired fistulas are encountered morefrequently than congenital fistulas and have beendescribed after blunt abdominal trauma, penetrating liverinjury (including liver biopsy), or in association withhepatocellular carcinoma (5,9).Patients with hepatoportal arteriovenous fistulas maybe asymptomatic or can present with varying clinicalmanifestations depending on the size, location, andduration of the arterioportal communication. The patients