Mannai S, Kraïem T, Gharbi L, Haoues N, Mestiri H, Khalfallah M-T
Service de Chirurgie Générale, Hôpital Mongi-Slim, 1004 Tunis, Tunisie.
Ann Chir. 2006 Jul-Aug;131(6-7):369-74. doi: 10.1016/j.anchir.2006.03.008. Epub 2006 Mar 30.
Congenital cystic dilatation of bile ducts is a rare condition. We report a retrospective study about 18 patients having congenital bile duct cysts. According to Todani's classification, 11 cases were type I and 7 were type V. Six patients from the first group had a pancreatobiliary maljunction. A total resection of the cyst was conducted in the type I cysts. Anatomopathologic examination showed an adenocarcinoma of a common bile duct cyst. In one case, a cancer of the gall bladder associated to a common bile duct cyst in another case. Three patients with segmental dilatation of intrahepatic bile ducts (type V) underwent liver resection. Four patients had a diffuse form, one of them was treated by percutaneous drainage, and in the other cases a hepatojejunostomy was performed. Postoperative course was complicated with acute cholangitis in these four cases. Percutaneous drainage and antibiotics allowed a positive outcome in most of the cases. In one case, secondary biliary cirrhosis occurred as a long-term complication. Congenital cystic dilatation of bile ducts is considered to be a precancer state. Enterocystic anastomosis is proscribed and the resection has to be as complete as possible.
先天性胆管囊性扩张是一种罕见疾病。我们报告了一项针对18例先天性胆管囊肿患者的回顾性研究。根据托达尼分类法，11例为I型，7例为V型。第一组中有6例存在胰胆管合流异常。I型囊肿均进行了囊肿全切术。病理检查显示胆总管囊肿腺癌1例。1例为胆囊癌合并胆总管囊肿。3例肝内胆管节段性扩张（V型）患者接受了肝切除术。4例为弥漫型，其中1例接受经皮引流治疗，其他病例均行了肝空肠吻合术。这4例患者术后均并发急性胆管炎。经皮引流及使用抗生素后，多数病例预后良好。1例出现继发性胆汁性肝硬化这一远期并发症。先天性胆管囊性扩张被认为是一种癌前状态。禁忌行肠囊肿吻合术，必须尽可能完整切除。
