肝豆状核变性致门静脉高压症的外科处理

目的:探讨脾切除、贲门周围血管离断术治疗肝豆状核变性所致门静脉高压症的疗效。方法:总结分析我院脾切除、贲门周围血管离断术治疗肝豆状核变性致门静脉高压症患者5例资料,并对其进行随访,评价该手术疗效。结果:3例于术后3~5年因肝性脑病死亡,余2例坚持驱铜治疗,生命质量显著提高,无再发上消化道出血。结论:肝功处于Child-pugh A或B级,合并有显著门静脉高压症表现和(或)上消化道出血,且无肝移植治疗条件的肝豆状核变性患者,脾切除、贲门周围血管离断术是有效的对症处理,是药物驱铜治疗的重要辅助手段。

Surgical Treatment of Portal Hypertension Caused by Hepatolenticular Degeneration

Objective:To investigate and analysis the therapeutic effect of splenectomy and portal-systemic venous devascularization in patients with portal hypertension caused by hepatolenticular degeneration (HLD).Methods:The clinical data of five patients with HLD treated with splenectomy and portal-systemic venous devascularization in our hospital were analyzed.Meanwhile,all patients were followed up to evaluate the effect of the treatment.Results:Three cases died from hepatic encephalopathy three to five years after operation.Other two cases persist in the depriving copper treatment.Their life quality obviously elevated and upper digestive tract bleeding didn't occur again.Conclusions:In patients with hepatolenticular degeneration whose liver function being at Child-pugh A or B grade,complicated with obvious portal hypertension and/or upper digestive tract bleeding and having no condition for liver transplantation,splenectomy and portal-systemic venous devascularization may be considered as an effective symptomatic treatment and important adjuvant treatment means of deprive copper treatment.