胆道闭锁10年诊治经验

　　【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-year experience

　　【Abstract】　Objective　To review the experience of managing 118 children with biliary atresia over the last 10 years.Methods　An early diagnosis was confirmed by jaundiced infants with clay-colored feces and enlarged,　firm liver, ultrasonography, and hepatic scintigraphy. The basic operative procedure was hepticojejunostomy, including Kasai Ⅰ procedure (hepaticojejunostomy, Roux-Y anastomosis) in 10 cases, Kasai Ⅱ procedure (hepaticocystostomy) in 3, Suruga Ⅱ procedure (hepaticojejunostomy with a jejunal fistula) in 60, Kasai Ⅰ procedure plus antireflux valves 35, liver biopsy 9 and liver transplantation 1.Results　Bile drainage was recorded in 105 patients with mean volume of 100-20 0mls/day (range=20 to 500mls/day). 19 patients underwent re-hepaticojejunostomy, and 10 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). The duration of survival ranged from 7 month to 10 years. Forty-two patients were jaundice-free.Conclusions　Early diagnosis and operation preferably within 40-60 days, accurate dissection of porta hepatis and active treatment of cholangitis may improve the prognosis. Those children with normal liver function tests enjoy normal development.

　　【Key words】　Biliary atresia　　Cholangitis　　Diagnosis

　　胆道闭锁（biliaryatresia,BA）是新生儿期阻塞性黄疸的常见病因之一，黄疸原因不明，与病毒感染有一定关系［1］。其发病率在存活的新生儿中约为1∶8　000～1∶14　000，治疗效果不佳，我国只有个案成功的报道［2］，其

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