Desmet V J
Laboratory of Histochemistry and Cytochemistry, Universitair Ziekenhuis Sint Rafaël, Leuven, Belgium.
Mayo Clin Proc. 1998 Jan;73(1):80-9. doi: 10.4065/73.1.80.
Intrahepatic bile ducts (IHBDs) develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein and thus form the "ductal plates." The ductal plates are remodeled into mature tubular ducts. Lack of remodeling results in the persistence of periportal epithelial sleeves or "ductal plate malformation" (DPM). A proposal is that virtually all congenital diseases of IHBDs represent examples of DPM. Some early, severe types of extrahepatic bile duct atresia are characterized by DPM, a suggestion of a prenatal beginning of the disease. Several congenital diseases are characterized by dilatation of segments of IHBDs and variable degrees of fibrosis. Such "fibrocystic diseases" represent DPM at different levels of the biliary tree. Autosomal recessive polycystic kidney disease represents DPM of interlobular bile ducts, associated with tubular dilatation of collecting renal tubules. Congenital hepatic fibrosis may derive from the same type of liver lesion, through a superimposed destructive type of cholangiopathy associated with scarring fibrosis. Caroli's disease represents DPM of the larger IHBDs, whereas Caroli's syndrome combines the lesions of Caroli's disease and congenital hepatic fibrosis. von Meyenburg complexes represent DPM of smaller interlobular ducts; their dilatation gives rise to the liver cysts in autosomal dominant polycystic kidney disease. Finally, DPM is a component of the tissue abnormalities in so-called mesenchymal hamartoma.
肝内胆管（IHBDs）由与门静脉间充质接触的双潜能肝祖细胞发育而来，从而形成“胆管板”。胆管板会重塑为成熟的管状胆管。重塑失败会导致门静脉周围上皮袖套持续存在或出现“胆管板畸形”（DPM）。有一种观点认为，几乎所有肝内胆管的先天性疾病都是胆管板畸形的例子。一些早期、严重类型的肝外胆管闭锁的特征是胆管板畸形，这表明该疾病始于产前。几种先天性疾病的特征是肝内胆管节段扩张和不同程度的纤维化。这种“纤维囊性疾病”代表了胆管树不同水平的胆管板畸形。常染色体隐性多囊肾病代表小叶间胆管的胆管板畸形，与集合肾小管的管状扩张有关。先天性肝纤维化可能源于同一类型的肝脏病变，是由一种叠加的伴有瘢痕纤维化的破坏性胆管病导致的。卡罗利病代表较大肝内胆管的胆管板畸形，而卡罗利综合征则合并了卡罗利病和先天性肝纤维化的病变。冯·迈恩伯格复合体代表较小的小叶间胆管的胆管板畸形；它们的扩张在常染色体显性多囊肾病中导致肝囊肿。最后，胆管板畸形是所谓间叶性错构瘤组织异常的一个组成部分。
