Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt

Hannah Glonnegger; Maren Schulze; Simone Kathemann; Sebastian Berg; Hannah Füllgraf; Andrea Tannapfel; Patrick Gerner; Jochen Grohmann; Charlotte Niemeyer; Simone Hettmer

doi:10.3389/fped.2020.00501

Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt

Front Pediatr. 2020 Aug 25:8:501. doi: 10.3389/fped.2020.00501. eCollection 2020.

Authors

Affiliations

¹ Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany.
² Department of Transplant and General Surgery, University Hospital Essen, Essen, Germany.
³ Division of Pediatric Radiology, Department of Radiology, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany.
⁴ Department of Pathology, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany.
⁵ Faculty of Medicine, Medical Center, Institute for Pathology, Ruhr-University Bochum, Bochum, Germany.
⁶ Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany.
⁷ Department of Congenital Heart Defects and Pediatric Cardiology, Faculty of Medicine, University Heart Center Freiburg-Bad Krozingen, University of Freiburg, Freiburg im Breisgau, Germany.

Abstract

Congenital extrahepatic portosystemic shunts (CEPS), previously also described as Abernethy malformations, are rare malformations in which the extrahepatic portal system directly communicates with the vena cava inferior, thereby bypassing the liver. A hypoplastic portal vein (PV) exists in most cases. CEPS have been associated with the development of liver nodules, ranging from mostly focal nodular hyperplasia (FNH) to hepatic adenoma (HA) and even hepatocellular carcinoma (HCC). Tumor development in CEPS may be due to changes in perfusion pressures, oxygen supply or endocrine imbalances. It is important to rule out CEPS in children with liver tumors, because resection could impede future shunt occlusion procedures, and benign masses may regress after shunt occlusion. Here, we review the case of a 9-years-old male with CEPS and hepatic nuclear Factor 1-alpha (HNF-1-alpha) inactivated HA to raise awareness of this condition and review histopathological changes in the liver of CEPS.

Keywords: Abernethy malformation; CEPS; child; congenital extrahepatic portosystemic shunt; hepatic adenoma.

Publication types

Case Reports