先天性胆管扩张症合并肝内胆管扩张及复杂胆道畸形的诊断与治疗对策

目的研究先天性胆管扩张症合并肝内胆管扩张及复杂胆道畸形的特殊检查方法及其临床意义，探讨在标准术式即囊肿切除胆道重建的基础上对肝内胆管扩张及可能存在的复杂胆道畸形处理的方法。方法对于合并肝内胆管扩张患儿，以术中胆管造影、胆道镜观察等仔细进行术中检查，了解肝总管、胆囊管、左右肝管、肝内胆管扩张及狭窄情况，以及胰胆管共同通道的病理变化，同时仔细检查有无复杂胆道畸形的存在并予以手术处理。结果全组57例，33．3％合并肝内胆管扩张，其中10例为肝内胆管囊样扩张，其汇入肝总管的开口呈瓣膜状、隔膜状或细短管状狭窄。9例为自肝门向肝内胆管起始端逐渐变细的锥形扩张，无明显狭窄。2例合并迷走胆管，3例为左右肝管分别汇入扩张的胆总管囊肿。结论对合并肝内胆管扩张者进行相应的特殊检查具有重要的临床意义，可以指导在标准根治术，即囊肿切除胆道重建的基础上对近端肝内胆管狭窄矫治、可能存在的迷走胆管、复杂胆道畸形及远端胰胆管共同通道病变的处理。

Diagnosis and treatment of choledochal cyst with intrahepatic bile duct dilatation and complicated bile duct malformation

Objective To review imaging studies of choledochal cysts. Methods Fifty-seven patients with choledocal cysts between the age of 15 days to 14 years managed in our hospital in the past 10 years (1994 to 2004) were reviewed. The biliary pathologies were studied with, cholangiography and choledochoscopy and compared with the operative findings. Results (33.3% )of 57 cases had intrahepatic bile duct dilatation, 10 of them showed cystic malformation and stenosis. Stenosis includes an intraluminal membrane or septum. Nine cases showed funnel-shaped stenosis tapering from porta hepatic to distal end of bile duct. Two cases were accompanied with variant bile duct. In 3 cases, right and left hepatic ducts flow to choledochal cyst respectively.Conclusions The image examinations for choledocal cysts provides a helpful guidance for the surgical procedure.