Introduction Portal hypertension isdefined as an elevation of portal pressure >10–12 mm Hg.1 There are manycauses of portal hypertension including heterogeneous group of diseases due to intrahepatic or extrahepatic etiologies.2
Hepatic vascularmalformations usually occur secondary totrauma, percutaneous interventions, neoplasms and cirrhosis. Arteriovenousfistulae may also be congenital.3
Patients withDown syndrome might have a reduced riskof vascular anomalies compared with the general population.4 Althoughvarious vascular abnormalities were described in patients with Down syndrome,arteriovenous fistulae between hepatic artery and portal vein is rarely not reported.
Case report We describe a three month old infant with trisomy 21 (Down syndrome) andarterioportal fistula (APF) associated with extrahepatic portal hypertensionand massive ascites.
Sonographic examination of the upper abdomen showed severe ascites and hepatosplenomegaly. Multiple aneurismatic arteriovenous fistulae betweenhepatic artery and left portal vein were demonstrated by portal Doppler sonography. Celiacarteriography was performed for further evaluation and coil embolization;demonstrating that there were diffuseconnections between left portal vein and main hepatic artery, gastroduodenal artery,second and third branches of right and left hepatic artery (Figure 1A and 1B).
In addition to transarterial interventionpercutaneus transhepatic access to the left portal vein was performed and lefthepatic artery to left portal vein fistulae were embolized with n-butyl 2cyanoacrylate which is a liquid embolic agent. Right hepatic artery and mainhepatic artery were embolized with multiple metalic coils bymicrocatheterization technique. Following the embolization procedure celiacarteriography revealed only partialocclusion of APF due to diffuse involment (Figure 2).
Discussion Our case isunique as it is the first case who wasattempted to be treated with percutaneous transvascular coil embolization of the APF. Hepaticangiography is the gold Standard to confirm the diagnosis and to demonstratethe vascular anatomy (5).
References 1 Suchy FJ. Portal Hypertension and Varices, Nelson Text Book Of Pediatrics, 18 th ed. Philadelphia, WB Saunders,2007, pp:1709–1712
2 Sarin SK, Kumar A. Noncirrhotic portal hypertension. Clin Liver Dis. 2006;10(3):627–51
3 Landau YE, Schwarz M, Belenky A, Shapiro R, Amir J. Arteriovenous Fistula and Portal Hypertension in a Child with Down Syndrome. IMAJ 2007;9:825–826
4 Greene AK, Kim S, Rogers GF, Fishman S, et al . Risk of Vascular Anomalies With Down Syndrome. Pediatrics 2008;121(1):135–140
5 Karnak I, Cil BE, Akay H. Congenital Intrahepatic Arterioportal Fistula:An Unusual Cause of Portal Hypertension Treated by Coil Embolization in an Infant. Eur J Pediatr Surg. 2009;19:251–271