Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: A case report

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 seco...

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Published inWorld journal of clinical cases Vol. 7; no. 7; pp. 903 - 907
Main Authors Xiang, Wen, Wang, Hao, Si, Zhong-Zhou, Chen, Guang-Shun, Wang, Guan-Wu, Li, Ting
Format Journal Article
LanguageEnglish
Published United States Baishideng Publishing Group Inc 06.04.2019
Subjects
Case Report
Case report
Abernethy deformation
Congenital extrahepatic portosystemic shunt
Liver transplantation
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Summary: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.
Bibliography:Corresponding author: Ting Li, MD, PhD, Associate Research Scientist, Attending Doctor, Surgeon, Organ Transplantation Center, The Second Xiang-Ya Hospital, Central South University, No. 139 Middle Renmin Road, Changsha 410011, Hunan Province, China. liting001@csu.edu.cn
Author contributions: All authors contributed to the acquisition of data and the writing and revision of the manuscript.
Telephone: +86-731-85295108 Fax: +86-731-85295808
Supported by the Natural Science Foundation of Hunan Province, No. 2016JJ3165 and No. 2015JJ2169; and the Fundamental Research Funds for the Central Universities of Central South University, No. 2017zzts902.
ISSN:2307-8960
2307-8960
DOI:10.12998/wjcc.v7.i7.903