成人先天性胆管扩张症68例临床研究

目的 探讨先天性胆管扩张症的临床特点,了解胆总管扩张的分型及其并发症的发生.方法 回顾性研究2000年1月至2009年12月在天津南开医院确诊的成人先天性胆管扩张病例68例,采用Alonso分类法分型,比较各型的临床表现、合并疾病、病情进展.结果 共收集先天性胆管扩张病例68例.其中Ⅰ型占47.1%,Ⅳa型占35.3%,Ⅳb型17.6%,未见Ⅱ、Ⅲ、Ⅴ型.67.6%患者合并胆囊炎,70.6%患者合并胆道结石,胆管狭窄占26.5%,胆囊萎缩占17.6%,胰腺炎占8.8%,胆汁性肝硬变占5.9%,脂肪肝占11.8%,胆囊息肉占17.6%,脾轻度肿大14.7%,肝囊肿8.8%,肾囊肿2.9%.癌变占23.5%.结论 先天性胆管扩张症Ⅰ型最多见,其次为Ⅳa型,未见Ⅱ、Ⅲ、Ⅴ型.常见并发症为慢性胆囊炎、胆道结石,约1/4发生癌变.

Abstract：

Objective To investigate the clinical characteristics of congenital cholangiectasis,the classification of choledochectasia and its complications. Methods Sixty-eight patients with congenital cholangiectasis admitted to Nankai hospital of Tianjin from Jan. 2000 to Dec. 2009 were retrospectively studied. The patients were classified using Todani method. The clinical presentation,complications and development were compared among patients with different types. Results In 68patients, type Ⅰ , Ⅳa and Ⅳb were accounted for 47. 1%, 35.3%, 17.6%,respectively. Wheraes type Ⅱ , Ⅲ and Ⅴ were not found. The complications included cholecystitis (67.6%) and bile duct stone (70. 6%). Biliary stricture and gallbladder atrophy were accounted for 26.5% and 17.6%,respectively. Pancreatitis was found in 8.8% patients, biliary cirrhosis in 5.9% patients,carcinogenesis in 23.5 % patients, fatty liver steatosis in 11.8 % patients, cholecystic polyps in 17.6 %patients, slight spleen enlargement in 14.7% patients, hepatic cyst in 8. 8% patients, renal cyst in 2.9% patients. Conclusions Tyep Ⅰ is commonly found in congenital cholangiectasis, and the next is the type of Ⅳa, but no type of Ⅱ , Ⅲ or Ⅴ is found. The common complications are cholecystitis and bile duct stone. Carcinogenesis is found in 25 % patients.

The clinical study on 68 patients with congenital cholaniectasis

Objective To investigate the clinical characteristics of congenital cholangiectasis,the classification of choledochectasia and its complications. Methods Sixty-eight patients with congenital cholangiectasis admitted to Nankai hospital of Tianjin from Jan. 2000 to Dec. 2009 were retrospectively studied. The patients were classified using Todani method. The clinical presentation,complications and development were compared among patients with different types. Results In 68patients, type Ⅰ , Ⅳa and Ⅳb were accounted for 47. 1%, 35.3%, 17.6%,respectively. Wheraes type Ⅱ , Ⅲ and Ⅴ were not found. The complications included cholecystitis (67.6%) and bile duct stone (70. 6%). Biliary stricture and gallbladder atrophy were accounted for 26.5% and 17.6%,respectively. Pancreatitis was found in 8.8% patients, biliary cirrhosis in 5.9% patients,carcinogenesis in 23.5 % patients, fatty liver steatosis in 11.8 % patients, cholecystic polyps in 17.6 %patients, slight spleen enlargement in 14.7% patients, hepatic cyst in 8. 8% patients, renal cyst in 2.9% patients. Conclusions Tyep Ⅰ is commonly found in congenital cholangiectasis, and the next is the type of Ⅳa, but no type of Ⅱ , Ⅲ or Ⅴ is found. The common complications are cholecystitis and bile duct stone. Carcinogenesis is found in 25 % patients.