YMJ. 2016; 12(-37-38): 989-992

ABERNETHY SYNDROME; REPORT OF A CASE

Fatma Tuba Coşkun¹, Meltem Ugras¹, Endi Romano¹, Mert Levent Barut², Ercan Kocakoç^3
1Yeditepe University Hospital, Department of Pediatrics, Istanbul, Turkey
²Yeditepe University Hospital, Istanbul, Turkey
³Yeditepe University Hospital, Department of Radiology, Istanbul, Turkey.

Congenital extrahepatic portosystemic shunt known as Abernethy malformation is a rarely seen disorder. It is classified into two types based on the pattern of anastomosis between the portal vein(PV) and inferior vena cava(IVC), and the presence or absence of an intrahepatic portal venous supply. Patients with Abernethy malformations have cardiac and hepatic abnormalities. It rarely can lead to hepatocellular carcinoma in the long term. Here we report a 16- years- old girl who is diagnosed as Abernethy malformation as a result of investigations of early puberty.

Keywords: Abernethy syndrome, portosystemic shunt, child.

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ABERNETHY SENDROMU; BİR OLGU SUNUMU

Fatma Tuba Coşkun¹, Meltem Ugras¹, Endi Romano¹, Mert Levent Barut², Ercan Kocakoç^3
1Yeditepe University Hospital, Department of Pediatrics, Istanbul, Turkey
²Yeditepe University Hospital, Istanbul, Turkey
³Yeditepe University Hospital, Department of Radiology, Istanbul, Turkey.

Konjenital ekstrahepatik portosistemik şant (Abernethy malformasyonu) çok nadir görülen bir bozukluktur. It is classified into two types based on the pattern of anastomosis between the portal vein(PV) and inferior vena cava(IVC), and the presence or absence of an intrahepatic portal venous supply. Patients with Abernethy malformations have cardiac and hepatic abnormalities. It rarely can lead to hepatocellular carcinoma in the long term. Here we report a 16- years- old girl who is diagnosed as Abernethy malformation as a result of investigations of early puberty.

Anahtar Kelimeler: Abernethy sendromu, portosistemik şant , çocuk.

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