目的　探讨不同年龄先天性胆管扩张症的特征、病因及防治措施。方法　对 1988年 1月～ 1998年 12月收治的 2 13例先天性胆管扩张症按年龄分 3组 ,将分型、临床表现、治疗及预后等相关因素综合分析。结果　①新生儿、婴儿 17例 ,囊肿型 16例 ( 94 .12 % ) ,临床以黄疸 ( 11例 )、肿物( 6例 )为主要症状 ,15例手术治疗 ,13例 ( 86.67% )治愈 ,2例 ( 13 .3 3 % )死亡。②幼儿、儿童组 163例 ,均以间歇性腹痛就诊 ,囊肿型 74例 ( 4 5 .4 0 % ) ,梭型 89例 ( 5 4 .60 % )。 76例行影像学检查 ,62例( 81.5 8% )伴胰胆管合流异常。 161例手术治疗 ,治愈 160例 ( 99.3 8% ) ,死亡 1例 ( 0 .62 % )。③成人组 3 3例 ,囊肿型及混合型占多数 ,因反复感染并发症日益增多 ,3 3例中 2 4例 ( 72 .73 % )合并胆管结石等并发症 ,癌变 2例。手术治疗 3 3例 ,治愈 3 0例 ( 90 .91% ) ,死亡 3例 ( 9.0 9% )。结论　本病应根据不同年龄、临床表现 ,采用现代的影像技术检查 ,做到早期诊断、早期行根治术 Objective To investigate the characteristics, etiology and prevention and treatment of congenital bile duct dilatation at different ages. Methods A total of 213 cases of congenital bile duct dilatation admitted to our hospital from January 1988 to December 1998 were divided into three groups according to their ages. The typing, clinical manifestation, treatment and prognosis were analyzed comprehensively. Results ①New infants and infants in 17 cases and cyst type in 16 cases (94.12%) were clinically treated with jaundice (11 cases) and tumor (6 cases) as main symptoms. Fifteen cases were treated by surgery and 13 cases (86.67%) were cured , 2 cases (13.33%) died. ② There were 163 infants and children with intermittent abdominal pain. Among them, 74 cases were cyst (45.5%) and 89 cases were shuttle (54.6%). 76 routine imaging examination, 62 cases (81.5%) with pancreaticobiliary duct anomalies. 161 cases were treated surgically, 160 cases (99.3%) were cured and 1 case died (0.62%). (3) In the adult group, 33 cases were cystic and mixed, accounting for the increasing number of complications due to repeated infections. There were 24 cases (72.73%) of 32 cases complicated with complications such as bile duct stones and 2 cases of canceration. Surgical treatment of 33 cases, cured 30 cases (90.91%), 3 patients died (9.0 9%). Conclusion The disease should be based on different age, clinical manifestations, the use of modern imaging techniques, early diagnosis, early radical mastectomy