先天性胆管囊性扩张症的临床X线分析

Clinical and Radiological Analysis of Congenital Cystic Dilatation of the Bile Duct

经手术证实的34例先天性胆管囊性扩张症的临床和直接胆道造影所见,按胆管受累部位分肝外型、肝内型和肝内外型。归纳各型X线诊断和鉴别要点。肝外及肝内外型均显示有特征性的胆胰管连接异常,胆总管远端狭窄。肝内型多呈肝内二级以下分支节段性囊、柱或串珠样扩张,多无远侧胆管狭窄和胆胰管连接异常。本病癌变率高(本纽14.7％),临床症状缺少特征性。采用B超为首选,结合经内镜逆行胆胰管造影(ERCP)和经皮肝穿刺胆管造影(PTC),基本均可在术前确诊。

The clinical and cholangiographic findings of 34 cases of congenital cystic dilatation of the bile duct(all verified by surgery) were studied. The cases were classified as three types according to the involved sites: extrahepatic, intrahepatic, intra- and extrahepatic. The points of diagnosis and differential diagnosis were discussed. In types 1 and 3 the cases usually showed anomalous junction of pancreatic and biliary ducts, stenosis at the end of the common bile duct. In type 2,disa-tal branches of secondary intrahepatic duct were usually involved with segmental cystic, columnar or bead-like dilatation without distal stenosis. The rate of malignant changes was high (14.7%). The clinical manifestations showed little specific features. Diagnosis could be made prior to operation by US combined with ERCP and PTC.