对２０例小儿先天性肝外胆道畸形的肝脏活俭组织进行了光镜和电镜观察，肝汇管区扩大，纤维细胞及胶原纤维增生。小胆管增多，腔内有大小及密度不同的胆汁块，肝细胞之间的毛细胆管扩张，腔内亦有大小及密皮不同的胆汁块，肝血多内皮细胞普遍极度肿胀甚至破裂。枯否氏细胞增生增大，胞质内有大量的溶酶体和胆汁块。部分肝细胳肿胀，其间有坏死灶，部分肝细胞呈浓缩状。多数肝组织中有多核巨肝细胞存在。肝细胞胞质中有胆汁淤积，膜性细胞器减少，其中滑面内质网消失或坏死。结果提示，小儿肝外胆道畸形有类似的肝脏病变，但胆道完全闭塞者病变重。 Liver biopsy tissues of 20 patients with congenital extrahepatic biliary deformities were observed under light microscope and electron microscope. Hepatic portal area was enlarged and fibroblasts and collagen fibers proliferated. Small bile duct increases, the cavity size and density of different bile chondrocytes, hepatocytes between the bile duct dilatation, the size and density of the cavity also have different bile blocks, liver and blood endothelial cells were extremely extreme swelling or even rupture. Kupffer cells increased proliferation of cytoplasm with a large number of lysosomes and bile blocks. Part of the liver swelling, during which necrosis, some of the liver cells were concentrated. Most of the liver tissue in the presence of multi-nuclear giant hepatocytes. Liver cell cytoplasm in cholestasis, membranous organelles decreased, in which slip surface endoplasmic reticulum disappeared or necrosis. The results suggest that children with extrahepatic biliary deformities have similar liver lesions, but the biliary completely obstructive disease weight.