TY - JOUR AB - Abernethy malformation is a rare congenital malformation defined by an extrahepatic portosystemic shunt. The majority of affected patients are young (<18 years of age) and experience various symptoms, including vomiting, jaundice, dyspnea and coma. The current study presents a case of Abernethy malformation in an asymptomatic adult male patient. The patient exhibited congenital absence of the portal vein, congenital heart disease (postoperative ventricular septal defect status), and multiple liver lesions, confirmed to be focal nodular hyperplasia by biopsy. Ultrasonography and magnetic resonance imaging findings revealing the liver lesions, type II congenital absence of the portal vein and the portosystemic shunt are presented. In addition, the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder are investigated by reviewing 101 previously reported cases. AD - Department of Endocrinology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, P.R. China Department of Hepatology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, P.R. China AU - Hao,Yabin AU - Hong,Xu AU - Zhao,Xinyan DA - 2015/02/01 DO - 10.3892/ol.2014.2767 EP - 700 IS - 2 JO - Oncol Lett KW - focal nodular hyperplasia ventricular septal defect portal vein portosystemic shunt PY - 2015 SN - 1792-1074 1792-1082 SP - 695 ST - Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review T2 - Oncology Letters TI - Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review UR - https://doi.org/10.3892/ol.2014.2767 VL - 9 ER -