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成人至小儿活体肝移植治疗门静脉海绵样变性。

Adult to pediatric living donor liver transplantation for portal cavernoma.

机构信息

Department of Hepatobiliary Surgery, Children's Hospital, Chongqing Medical University, Chongqing, China.

出版信息

Hepatol Res. 2009 Sep;39(9):888-97. doi: 10.1111/j.1872-034X.2009.00526.x. Epub 2009 May 7.

Abstract

AIM

Portal cavernoma (PC) is an important cause of non-cirrhotic portal hypertension with severe complications, such as variceal hemorrhage in pediatric patients. With the development of new surgical techniques, living donor liver transplantation (LDLT) has recently been recognized as a viable but challenging treatment option for PC. The purpose of the present study was to summarize the efficacy of LDLT in PC patients and to carry out a follow-up study of pediatric recipients.

METHODS

The primary indication for LDLT in our research was PC with severe variceal bleeding and liver function decompensation. Three patients were diagnosed with PC following evaluation with computed tomography angiography and abdominal color Doppler ultrasonography (CDU).

RESULTS

Various surgical techniques, including jump bypass grafting for portal vein anastomosis, were carried out according to the range and degree of cavernous transformation within the splenic vein and superior mesenteric vein. Postoperative CDU confirmed the early integrity of the portal vein (PV) in each patient. PV rethrombosis occurred in one patient 7 days after LDLT, despite anticoagulation therapy with coumadin. Two of the three patients had no further episodes of variceal hemorrhage during the 2-year follow-up period.

CONCLUSIONS

The present study is the first report of the successful use of LDLT to treat pediatric PC patients. We conclude that LDLT is effective for the majority of pediatric patients with PC.

摘要

目的

门静脉海绵样变性(PC)是导致非肝硬化性门静脉高压的重要原因,可导致儿童患者出现严重并发症,如静脉曲张出血。随着新的手术技术的发展,活体肝移植(LDLT)最近已被认为是 PC 的一种可行但具有挑战性的治疗选择。本研究的目的是总结 LDLT 在 PC 患者中的疗效,并对儿科受者进行随访研究。

方法

本研究中 LDLT 的主要适应证是 PC 伴严重静脉曲张出血和肝功能失代偿。3 例患者经 CT 血管造影和腹部彩色多普勒超声(CDU)评估后诊断为 PC。

结果

根据脾静脉和肠系膜上静脉内海绵窦转化的范围和程度,采用各种手术技术,包括门静脉吻合的跳跃旁路移植术。术后 CDU 证实每位患者的门静脉(PV)早期完整。尽管使用华法林进行抗凝治疗,但 1 例患者在 LDLT 后 7 天发生 PV 再血栓形成。3 例患者中有 2 例在 2 年随访期间无再发静脉曲张出血。

结论

本研究首次报告了 LDLT 成功治疗儿科 PC 患者。我们得出结论,LDLT 对大多数儿科 PC 患者有效。

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