关键词:胆道闭锁;胆管炎;诊断
【摘要 】 目的 总结10年来118例胆道闭锁诊断治疗的经验。方法 通过观察患儿大便、肝脏体检、B超和ECT可迅速早期确诊。采用的基本术式为肝门空肠吻合术,行Kasai术Ⅰ式(肝门空肠Roux-Y吻合)10例,Kasai术Ⅱ式(肝门胆囊吻合术)3例,Suruga术式(肝门空肠吻合,空肠皮肤造瘘术)60例,KasaiⅠ式加矩形防反流瓣35例,单纯肝活检9例,肝移植1例。结果 105例术后有胆汁排出,最多500ml/天,平均100~200ml/天,亦有少于20ml/天。再次行肝门空肠吻合术19例,10例术后重新有胆汁排出。存活47例(47/108, 43.5%),近3年随着诊断治疗方法不断改进,存活率已上升至63.8%(23/36),最长10年,最短7个月,无黄疸生存42人。结论 提高疗效的关键是早期诊断,争取40~60天内手术,术中正确解剖肝门,术后积极防治胆管炎。黄疸消退快,肝功能恢复好的患儿,其体格和智力发育可达到正常儿童水平。
Biliary Atresia: 10-yearexperience
LI Guisheng, LIU Juncheng, ZHOULi, et al.
Department of Pediatric Surgery, First Affiliated Hospital, Sun Yat-sen University ofMedical Sciences, Guangzhou 510080
【 Abstract 】 Objective To review the experience ofmanaging 118 children with biliary atresia over the last 10 years. Methods An early diagnosis was confirmedby jaundiced infants with clay-colored feces and enlarged, firm liver, ultrasonography, and hepatic scintigraphy. Thebasic operative procedure was hepticojejunostomy, including Kasai Ⅰ procedure (hepaticojejunostomy, Roux-Yanastomosis) in 10 cases, Kasai Ⅱ procedure (hepaticocystostomy) in 3, Suruga Ⅱ procedure (hepaticojejunostomy with a jejunalfistula) in 60, Kasai Ⅰ procedure plus antireflux valves 35, liver biopsy 9 and livertransplantation 1. Results Bile drainage was recorded in 105 patients with mean volume of100-200mls/day (range=20 to 500mls/day). 19 patients underwent re-hepaticojejunostomy, and10 of them had bile excretion post-operatively. The overall survival rate was 43%(47/108). The survival rate over the last three years improved to 63% (23/36). Theduration of survival ranged from 7 month to 10 years. Forty-two patients werejaundice-free. Conclusions Early diagnosis and operation preferably within 40-60 days,accurate dissection of porta hepatis and active treatment of cholangitis may improve theprognosis. Those children with normal liver function tests enjoy normal development.
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