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

被引：4

作者：

Xiang, Wen ^{[1
]}

Wang, Hao ^{[1
]}

Si, Zhong-Zhou ^{[1
]}

Chen, Guang-Shun ^{[1
]}

Wang, Guan-Wu ^{[1
]}

Li, Ting ^{[1
]}

机构：

[1] Cent S Univ, Xiang Ya Hosp 2, OrganTransplantat Ctr, 139 Middle Renmin Rd, Changsha 410011, Hunan, Peoples R China

来源：

WORLD JOURNAL OF CLINICAL CASES | 2019年 / 7卷 / 07期

关键词：

Congenital extrahepatic portosystemic shunt; Abernethy deformation; Liver transplantation; Case report; PORTAL-VEIN; ABSENCE;

D O I：

10.12998/wjcc.v7.i7.903

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

BACKGROUND 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. CASE SUMMARY 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. CONCLUSION Liver transplantation is an effective treatment for type I Abernethy deformation combined with focal nodular hyperplasia and hepatopulmonary syndrome.

引用

页码：903 / 907

页数：5

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