Caroli病是一种先天性的肝内胆管囊性扩张症。外科治疗的目的包括清除病灶，防止胆管癌变；通畅胆汁引流，缓解症状。肝切除是目前最常用的外科治疗方式，对局限性的Caroli 病可达到根治的效果，病人术后胆管炎等症状可长期缓解。而对于少部分弥漫型病人，也可选择性清除肝内主要的扩张病灶，并进行密切的随访,如病人进一步进展，肝移植常成为治疗的选择。对于多数弥漫型病人，肝移植是最终的治疗选择，可获得满意的长期效果。

Liver resection for Caroli's disease        DOU Ke-feng, YANG Zhao-xu. Department of Hepatopancreatobiliary Surgery,Xijing Hospital, the Fourth Military Medical University, Xi’an 710032, China
Corresponding author: DOU Ke-feng, E-mail：doukef@fmmu.edu.cn
Abstract    Caroli's disease (CD) is defined as a congenital cystic dilatation of the intrahepatic bile ducts. The goals of surgical management include removing the cystic dilated bile duct, avoiding cancer formation, reconstructing bile flow and easing the symptoms. Partial hepatectomy is one of the most common adopted surgical options for CD. For localized CD, it is potentially curative，providing long-term survival free of symptoms and complications. In selected cases of diffuse disease，surgical resection to remove major dilated intrahepatic bile duct cyst is also proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated. In most patients with diffuse CD, liver transplantation is the ultimate option，provides gratifying long-term results.