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小儿外科门体分流术和雷克斯旁路术:单中心经验。

Surgical portosystemic shunts and the Rex bypass in children: a single-centre experience.

机构信息

Recanati Miller Transplant Institute, Mount Sinai School of Medicine, New York, NY 10029, USA.

出版信息

HPB (Oxford). 2009 May;11(3):252-7. doi: 10.1111/j.1477-2574.2009.00047.x.

Abstract

OBJECTIVES

This study aimed to illustrate the indications for, and types and outcomes of surgical portosystemic shunt (PSS) and/or Rex bypass in a single centre.

METHODS

Data were collected from children with a PSS and/or Rex bypass between 1992 and 2006 at Mount Sinai Medical Center, New York.

RESULTS

Median age at surgery was 10.7 years (range 0.3-22.0 years). Indications included: (i) refractory gastrointestinal bleeding in portal hypertension associated with (a) compensated cirrhosis (n= 12), (b) portal vein thrombosis (n= 10), (c) hepatoportal sclerosis (n= 3); (ii) refractory ascites secondary to Budd-Chiari syndrome (n= 3), and (iii) familial hypercholesterolaemia (n= 4). There were 20 distal splenorenal, four portacaval, three Rex bypass, two mesocaval, two mesoatrial and one proximal splenorenal shunts. At the last follow-up (median 2.9 years, range 0.1-14.1 years), one shunt (Rex bypass) was thrombosed. Two patients had died and two had required a liver transplant. These had a patent shunt at last imaging prior to death or transplant.

CONCLUSIONS

Portosystemic shunts and Rex bypass have been used to manage portal hypertension with excellent outcomes. In selected children with compensated liver disease, PSS may act as a bridge to liver transplantation or represent an attractive alternative.

摘要

目的

本研究旨在展示单一中心行手术门体分流(PSS)和/或Rex 旁路术的适应证、类型和结局。

方法

数据收集自 1992 年至 2006 年在纽约西奈山医疗中心行 PSS 和/或 Rex 旁路术的儿童。

结果

手术时的中位年龄为 10.7 岁(范围 0.3-22.0 岁)。适应证包括:(i)门静脉高压相关难治性胃肠道出血,伴(a)代偿性肝硬化(n=12)、(b)门静脉血栓形成(n=10)、(c)肝门静脉性硬化(n=3);(ii)Budd-Chiari 综合征继发难治性腹水(n=3);(iii)家族性高胆固醇血症(n=4)。手术类型包括 20 例远端脾肾分流术、4 例门腔分流术、3 例 Rex 旁路术、2 例肠系膜腔静脉分流术、2 例间腔静脉心房分流术和 1 例近端脾肾分流术。末次随访(中位时间 2.9 年,范围 0.1-14.1 年)时,1 例分流术(Rex 旁路)发生血栓形成。2 例患者死亡,2 例需要肝移植。这 2 例患者在死亡或移植前的最后影像学检查时分流术仍然通畅。

结论

门体分流和 Rex 旁路术已用于治疗门静脉高压,疗效良好。在选择的代偿性肝病儿童中,PSS 可作为肝移植的桥梁,或者是一种有吸引力的替代方法。

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