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先天性胆管扩张症(congenital biliary dilatation,CBD)高发于亚洲人群,普遍认为其与胆总管壁薄弱和远端胆总管梗阻有关。胆总管壁远端梗阻的成因尚不清楚,比较流行的为胰胆管合流异常学说。产前诊断的年龄越小,扩张越明显,肝损害越重,生后要越早进行手术矫治。目前CBD被简化分为两型:胆总管远端狭窄型和非狭窄型。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CBD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。

Study advance of congenital biliary dilatation in children

Congenital biliary dilatation 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. The pathogenetic implications of pancreaticobiliary maljunction (PBM) is a popular hypothesis. If growth of the epithelium in the common bile duct fails to keep up with this anomalous elongation during embryonic, the duct may become attenuated, resulting in stenosis and weakness of the wall, and eventually dilatation. In the 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 abnormblity in the intrahepatic bile duct, pancreatic duct and common channel.