Journal List > Korean J Pediatr Gastroenterol Nutr > v.11(1) > 1110177

Korean J Pediatr Gastroenterol Nutr. 2008 Mar;11(1):56-59. Korean.
Published online Mar 31, 2008.  https://doi.org/10.5223/kjpgn.2008.11.1.56
Copyright © 2008 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
A Case of Congenital Extra Hepatic Portocaval Shunt (Abernethy Malformation Type 2) with a very Large Liver Mass and an Atrial Septal Defect
Hae Jeong Lee, Jee Hyun Lee, June Huh, I Seok Kang, Heung Jae Lee, Yeon Lim Suh,* So Young Yoo, and Yon Ho Choe
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
*Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Abstract

Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.

Keywords: Abernethy malformation; Focal nodular hyperplasia; Hepatic encephalopathy