产前诊断的先天性胆管扩张症手术时机探讨

Therapeutic strategy for prenatally diagnosed congenital biliary dilatation

目的对产前诊断的先天性胆管扩张症的手术时机进行探讨。方法回顾我院2006年至2010年收治的产前诊断10例先天性胆管扩张症患儿，入院手术年龄27d至2岁，9例接受腹腔镜胆总管囊肿切除术，肝管空肠Roux-Y吻合术，1例接受开腹手术。观察其临床表现、超声和实验室指标、术中情况、术后恢复情况等。结果产前囊肿均随孕周逐渐增大。产后5例患儿曾经出现黄疸，4例曾经出现白便。4例超声提示囊肿增大明显，6例ALT升高。术中造影见全部发生胆总管远端梗阻，其中I型3例。Ⅳ型7例，3例可见胰胆合流异常。5例患儿术中及术后病理可见肝硬化、肝脏损伤表现。全部10例患儿术后恢复好。结论产前诊断的先天性胆管扩张症可能是由于胆总管远端梗阻，造成胆管扩张形成。产前诊断先天性胆管扩张症的患儿，应密切观察，发现黄疸白便表现、ALT和AST升高明显、超声提示囊肿短期增大明显应尽快手术治疗。如无上述表现，可定期复查，但也应尽早手术根治。

Objective To present the therapeutic strategy for prenatally diagnosed congenital biliary dilatation （CBD）. Methods From 2006 to 2010, 10 patients with prenatally diagnosed CBD were treated at this center. Their age ranged from 27 days to 2 years old. Among the 10 patients, 9 underwent the laparoscopic total cyst excision with Roux-en-Y hepatojejunostomy, 1 was performed open surgery. The clinical features, ultrasonography, laboratory tests and postoperative complications were retrospectively analyzed. Results Results The ultrasonograohy revealed the biliary cysts＇ size increased with the gestational ages. After birth, 5 of the 10 patients had jaundice, 4 had acholic stools, 4 patients＇ cysts increased in size, and 6 had elevated transaminases. Intraoperative cholangiography was performed, and distal common bile duct stenosis was found on all patients. Three of them were type I of CBD, 7 of them were type IV, and the other 3 had pancreatieobiliary malunion. Liver cirrho- sis was found in 5 of the 10 patients. All patients recovered from the surgery. Conclusions Distal common bile duct obstruction is the leading etiology of the prenatally diagnosed CBD. Surgical intervention is recommended for the patients with jaundice, acholic stool, elevated transaminase or cysts obviously increasing in size.