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Paper Details

An Unexpected Outcome of Congenital Intrahepatic Arterioportal Fistula with Trisomy 21

Aktas Selma, and Nur Seyma Zengin.

Journal Title:Acta Scientific Paediatrics

Congenital intrahepatic arterioportal fistula is a rare condition and presents with symptoms of portal hypertension. Congenital cardiac anomalies are common among children with Trisomy 21 and there is also an association between Trisomy 21 and congenital vascular malformations of the liver. We discuss a patient with Trisomy 21 that presented with continuous murmur in the right upper quadrant of abdomen, thrombocytopenia and mild pulmoner hypertension and diagnosed as congenital intrahepatic arterioportal fistula by doppler USG and angiography. Surprisingly the shunt regressed spontaneously without any intervention. Keywords: Trisomy 21; Congenital İntrahepatic Arterioportal Fistula