Vergnes P, Lamireau T, Pontailler J R, Colombani J F, Bondonny J M
Service de Chirurgie Pédiatrique, Hôpital des Enfants, Bordeaux.
Chir Pediatr. 1990;31(2):73-8.
The authors report the case of an 3 months old caucasian boy which had an extrahepatic biliary atresia associate with a congenital biliary cyst and an anomalous pancreaticobiliary junction. The patency between gallbladder and duodenum permits the operative opacification of this long common pancreaticobiliary channel. The congenital biliary cyst was located on the upper part of the common bile duct and was obstructed by biliary concretions. Unfortunately, no patency between intrahepatic bile ducts and this cyst was found. Because this long common pancreaticobiliary channel may cause biliary dilatation or biliary cancer, we performed an hepatic portoenterostomy rather an hepatic portocholecystostomy. A few weeks later, the bile flow was restored and the jaundice decreased. With a follow up of 18 months, this boy is currently anicteric, despite the hepatic fibrosis found on the operative biopsy. The association extrahepatic biliary atresia-long common pancreaticobiliary channel is uncommon and only 20 cases were reported. In our case, the pancreatic juice reflux into the biliary tract might have caused the dilatation of the common bile duct and the biliary atresia by chronic obstructive cholangiopathy.
作者报告了一例3个月大的白种男孩病例,其患有肝外胆道闭锁,合并先天性胆管囊肿和胰胆管异常连接。胆囊与十二指肠之间的通畅性使得这条长的共同胰胆管通道能够在手术中显影。先天性胆管囊肿位于胆总管上部,被胆石阻塞。不幸的是,未发现肝内胆管与该囊肿之间有通畅性。由于这条长的共同胰胆管通道可能导致胆管扩张或胆管癌,我们进行了肝门肠吻合术而非肝门胆囊吻合术。几周后,胆汁流动恢复,黄疸减轻。经过18个月的随访,尽管手术活检发现有肝纤维化,但该男孩目前无黄疸。肝外胆道闭锁-长的共同胰胆管通道这种关联并不常见,仅报道过20例。在我们的病例中,胰液反流至胆道可能通过慢性阻塞性胆管病导致胆总管扩张和胆道闭锁。