中国实用外科杂志 ›› 2010, Vol. 30 ›› Issue (05): 348-353.

• 专题笔谈 • 上一篇    下一篇

胰胆合流异常与先天性胆总管囊肿病因的关系及治疗原则

李    龙,张金山   

  1. 首都儿科研究所外科,北京100020
  • 出版日期:2010-05-01 发布日期:2010-05-06

  • Online:2010-05-01 Published:2010-05-06

摘要:

先天性胆总管囊肿(choledochal cyst,CC)是一种以先天性胆道系统扩张为特征的病理表现,高发于亚洲人群,其病因目前尚不完全清楚。普遍认为与胆总管壁薄弱和远端胆总管梗阻有关。目前关于CC胆总管壁薄弱和远端胆总管梗阻的病因,尚不清楚,比较流行的为胰胆合流异常学说,即胆总管和胰管汇合于十二指肠壁外,形成较长的共同管,使胰液和胆汁在共同管内混合,Oddi括约肌在共同管的远端形成的高压带会导致胰液和胆汁的混合液体向胆总管和胰管内双向反流,导致胆管管壁损伤,使其薄弱而发生扩张。关于胰胆合流异常的病因,目前倾向于胚胎早期肝憩室远端异位导致胰胆共同管变长和胆总管拉长薄弱。在治疗方面,无论患儿的年龄大小或者症状的轻重,囊肿彻底切除肝管空肠Roux?en?Y吻合术是根治CC的标准术式。术前及术中详细的了解患儿整个胰胆道系统的改变情况,同时处理肝内胆管和胰管及共同管的异常病变,是减少手术后并发症的关键。腹腔镜微创根治术是成熟的治疗方法。

关键词: 胰胆合流异常, 先天性胆总管囊肿

Abstract:

Etiology relationship and principles of treatment of pancreatic and biliary anomalous junction and choledochal cyst        LI Long, ZHANG Jin-shan. Pediatrics Surgery of Capital Institute, Beijing 100020, China
Corresponding author: LI Long, E-mail:zjs851@163.com
Abstract    Choledochal cyst (CC) is a pathologic condition characterized by varying degrees of congenital dilatation of the biliary system. Most of the reported cases come from Asia and its etiology remains unclear. Some people suggested that CC is a result of weakness of the wall and the distal obstruction of the common bile duct. But its underlying precise mechanisms remain unclear. The pathogenetic implications of pancreaticobiliary maljunction (PBM) is a popular hypothesis: In PBM, the common channel can be so long that the junction of the pancreatic and bile ducts is located outside of the duodenal wall; in such cases, sphincter action does not functionally affect the junction. As the hydropressure within the pancreatic duct is usually greater than in the bile duct, pancreatic juice frequently refluxes into the biliary duct (pancreatobiliary reflux) in PBM, resulting in the incidence of lesion in the wall of the common bile duct and dilatation of the common bile duct. Currently, most scholars tend to believe the ectopic hepatic diverticulum may cause the primitive common bile duct and common channel to become stretched, producing a long common bile duct and a long common channel. If growth of the epithelium in the common bile duct fails to keep up with the anomalous elongation during embryonic, the duct may become attenuated, resulting in stenosis and weakness of the wall, and eventually dilatation. In treatment, the cyst excision with Roux-en-Y hepatoenterostomy is the standard surgical method for choledochal cyst. To reduce the post-operative complications, doctors need to learn about the changes of the biliary system, deal with the abnormality in the intrahepatic bile duct, pancreatic duct and common channel. Laparoscopic surgery has become a successful method in treatment of choledochal cyst.

Key words: pancreaticobiliary anomalous junction, choledochal cyst