先天性胆管扩张症56例临床分析

Clinical analysis of 56 cases of children with congenital biliary dilatation

目的探讨先天性胆扩张症(CBD)的诊断和治疗。方法对56例14岁以下CBD患儿一般资料、诊断依据、扩张胆管类型和术式进行回顾性分析。结果本组患儿发病年龄在3岁以下者占46.4%;男女比为1∶2.5;具有"三联症"者约占21.4%,具有"四大症状"者约占12.5%;经B超、磁共振胰胆管成像(MRCP)、CT等检查,诊断符合率分别为92.9%、96.4%和81.8%,全部患儿行手术治疗,除1例因术后并发胆汁性腹膜炎死亡,其余55例痊愈。结论 B超、MRCP是诊断CBD的常规检查手段,小儿重症胆总管囊肿先行B超引导下囊肿穿刺置管外引流术是一种较好的临时措施,扩张胆管切除+肝总管空肠Roux-en-Y吻合术是治疗CBD的标准术式。

Objective To discuss the diagnosis and treatment of children with congenital biliary dilatation（CBD）Methods From January 2000 to July 2010,data from 56 samples of children with CBD were used for this study.General status,diagnosis,types of CBD and style of surgery were retrospectively analyzed.Results 46.4% of the sampled patients have a outbreak age under 3 years old.The ratio of gender（male：female） was 1∶ 2.5.21.4% of the children had the three correlated symptoms which are abdominal pain、abdominal mass and jaundice.While 12.5% of the children had fever besides the three correlated symptoms.The children were examined by B-ultrasonic（B-us）,MRCP,CT and the confirmation rates were 92.9%、96.4% and 81.8 % respectively.All cases were cured by surgical operation except that there was one case dead from bile peritonitis.Conclusion B-us examination,MRCP and CT is the routine examination to diagnose CBD.The puncture and drainage to treat acute CBD under the guidance of B-type ultrasonography was proved to be a better temporary emergency technique before radical operation.Cystectomy and biliary reconstruction are both effective to treat this disease.