Congenital Extrahepatic Portosystemic Shunt Complicated by the Development of Hepatoblastoma: A Case Report and Review of Literature.

ABSTRACT

Congenital portosystemic shunts (CPSS) or congenital extrahepatic portosystemic shunts (CEPS) is a rare malformation. This congenital anomaly presents with a diverse array of clinical manifestations, ranging from asymptomatic to severe complications such as cardiac failure, pronounced pulmonary hypertension, and widespread pulmonary arteriovenous malformations. CPSS increases the risk of developing benign or malignant liver tumors, including nodular regenerative hyperplasia, focal nodular hyperplasia, hepatic adenoma, hepatocellular carcinoma, and hepatoblastoma. We report a case of a 15-month-old boy, identified with Abernethy's malformation type Ib, who presented with an abdominal mass during a follow-up. A comprehensive assessment established a diagnosis of hepatoblastoma. The patient was transferred to a specialized liver transplant center for further treatment and management. This is a review of literature highlighting the complexity of Abernethy malformation and its associated risk of liver tumors.