成人先天性胆管囊肿的治疗现状及进展

Treatment status and progress in congenital choledochal cyst in adults

胆管囊肿是一种罕见的先天性胆管囊性扩张病变,可单独累及肝外胆管、肝内胆管或两者都累及。成人患者常伴有腹痛、黄疸等临床表现,而腹部包块较为少见。由于胆管囊肿癌变的风险随年龄增长,故现均提倡完全切除囊肿以降低癌变风险。根据胆管囊肿的类型,其手术方案包括完整切除囊肿及Roux-en-Y胆肠吻合,累及肝段的肝切除术。肝移植成为治疗Todani V型囊肿(Caroli病)的重要选项。Todani III型胆管囊肿可通过ERCP行内镜下切除。胆管囊肿囊括了几种不同的临床病变,故内镜切除、肝部分切除和肝移植在其治疗中均占有一定地位并相互补充。微创技术在胆管囊肿治疗中的应用也在逐渐展开。故胆管囊肿患者应在多学科团队充分进行临床评估后针对性地制定个体化的治疗方案。

Choledochal cyst is a rare congenital cystic dilation of biliary tract. It may involve extrahepatic or intrahepatic or both locations simultaneously. Adult patients often complain abdominal pain ,jaundice and/or other clinical manifestations, but abdomi- nal mass is relatively rare. Choledochal cyst should be completely reseeted due to an increase in the risk of malignant transformation with age. According to the types of cyst, surgical procedures include the Roux-en-Y hepaticojejunostomy and variable types of hepatic resection. Liver transplantation has become an important option for the treatment of Todani type V cysts （ Caroli disease）. The mainstay of the treatment for Todani type III bile duct cysts is resection via endoscopic retrograde cholangiopanereatography. Since choledochal cyst comprises quite different clinical pathological entities, endoscopic therapy, hepatic resection, and liver transplantation all have their place and are mutual complementation. Minimally invasive techniques are also gradually adopted in the treatment of bile duct cysts. Each patient should receive individualized treatment after all the clinical findings have been considered fully by an interdisciplinary team.