Published online Apr 6, 2019. doi: 10.12998/wjcc.v7.i7.903
Peer-review started: December 19, 2018
First decision: January 6, 2019
Revised: January 24, 2019
Accepted: February 26, 2019
Article in press: February 26, 2019
Published online: April 6, 2019
Processing time: 108 Days and 1.8 Hours
Congenital extrahepatic portosystemic shunt, also known as Abernethy deformation, is a rare malformation caused by dysplasia in the portal vein system. There are few reports of liver transplantation as a treatment for Abernethy deformation, and our report is the first case in China. This is the second reported case with congenital extrahepatic portosystemic shunt combined with focal nodular hyperplasia and hepatopulmonary treated with liver transplantation.
The patient was a 14-year-old girl, diagnosed preoperatively as type Ib Abernethy deformation, intrahepatic multiple space-occupying lesion, and hepatopulmonary syndrome. The patient recovered well after undergoing classic orthotopic liver transplantation. Liver function, pulmonary function, and portal vein computed tomography angiography imaging were reexamined 20 mo postoperatively, and no abnormality was observed.
Liver transplantation is an effective treatment for type I Abernethy deformation combined with focal nodular hyperplasia and hepatopulmonary syndrome.
Core tip: Congenital extrahepatic portosystemic shunt is a rare congenital abnormal vascular anastomosis between the portal vein and vena cava. This report discusses clinical, angiographic diagnostic and histopathological features in one patient with congenital extrahepatic portosystemic shunt, as well as its surgical strategy. Liver transplantation appears to be a feasible treatment option for patients with congenital extrahepatic portosystemic shunt because it achieves optimal treatment effectiveness.