肝门胆管切开整形、肝门空肠吻合术治疗Ⅰ型胆道闭锁

HILAR CHOLANGIOPLASTY-PORTOENTEROSTOMY FOR TYPE Ⅰ BILIARY ATRESIA

目的 了解肝门胆管切开整形、肝门空肠吻合术治疗 型胆道闭锁的疗效。方法 1992～ 2 0 0 1年本院对 4例 型胆道闭锁患儿行肝门胆管切开整形、肝门空肠吻合术 ,对临床资料 (病史、体征、肝脏功能、B超、术中胆道造影、组织学检查等 )进行回顾性分析 ,并对患儿的症状、体征、肝脏功能及肝胆系统 B超进行长期随访。结果 4例患儿的临床表现均为阻塞性黄疸 ,B超示胆囊及胆管囊肿均小于同龄胆总管囊肿组 ,术中胆道造影确立诊断并发现肝门部肝管和左右肝管狭窄 ,肝脏组织学改变与 型胆道闭锁者相似。术后患儿全部成活 ,无近期并发症发生 ,黄疸于术后 3 0～ 74 d完全消退。术后远期随访患儿生长发育正常 ,无黄疸 ,肝功能恢复正常。结论 型胆道闭锁常伴有肝管及肝内胆管异常 ,肝门胆管切开整形、肝门空肠吻合术治疗该畸形可取得满意的疗效。

Objective To evaluate the results of hilar cholangioplasty-portoenterostomy for the infants with type Ⅰ biliary atresia.Methods Four infants with type Ⅰ biliary atresia were underwent modified portoenterostomy from 1992 to 2001.All patients were diagnosised through symptom,sign,hepatic function,B ultrasound,intraoperative cholangiogrphy,and histopathological examination.All of them were followed up from 3 to 96 months.The symptom,sign,hepatic function and B ultrasound were recorded during the term of follow-up.Results Obstructive jaundice was the common manifestation of these patients.The length and width of the gallbladder and the cyst of extrahepatic bile duct in type Ⅰ biliary atresia group were smaller than that in infantile choledochal cyst group.The right diagnosis and segmental stenosis of the hilar and intrahepatic bilewas made by the intraoperative cholangiography.The histopathological patterns of the liver in type Ⅰ biliary atresia were as the same as that in type Ⅲ one either through light microscopy or transmission electron microscopy.All patients were survived,and the jaundice was completely dismissed during 30 to 74 days after operation.The normal physical development can be obtained and the hepatic function can be recovered to normal during the term of follow up.Conclusion The stenosis of the hilar and intrahepatic bile duct is the common manifestation of type Ⅰ biliary atresia,and the procedure of hilar cholangioplasty-portoenterostomy is the rational choice to correct this malformation.