活体供肝肝移植治疗一名患有心力衰竭儿童的先天性门静脉缺失。

Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure.

作者信息

Sumida Wataru, Kaneko Kenitiro, Ogura Yukio, Tainaka Takahisa, Ono Yasuyuki, Seo Takahiko, Kiuchi Tetsuya, Ando Hisami

机构信息

Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya 466-8560, Japan.

出版信息

J Pediatr Surg. 2006 Nov;41(11):e9-e12. doi: 10.1016/j.jpedsurg.2006.07.014.

DOI:10.1016/j.jpedsurg.2006.07.014

PMID:17101345

Abstract

Congenital absence of the portal vein (CAPV) requires liver transplantation when encephalopathy develops. However, transplantation has technical difficulties because no collateral circulation exists except for the portosystemic shunt. Ligating the shunt will cause disastrous mesenteric venous congestion. We report a 19-month-old female infant with CAPV, who had portosystemic encephalopathy and cardiac failure, and underwent living donor liver transplantation with a partial clamp technique using a vein graft. This is the first case of successful liver transplantation for CAPV with cardiac failure.

摘要

先天性门静脉缺如（CAPV）在发生肝性脑病时需要进行肝移植。然而，由于除了门体分流外不存在侧支循环，移植存在技术困难。结扎分流会导致灾难性的肠系膜静脉充血。我们报告了一名19个月大的患有CAPV的女婴，她患有门体性脑病和心力衰竭，并采用静脉移植的部分钳夹技术接受了活体供肝移植。这是首例成功为伴有心力衰竭的CAPV患者进行肝移植的病例。