Marchal G J, Desmet V J, Proesmans W C, Moerman P L, Van Roost W W, Van Holsbeeck M T, Baert A L
Radiology. 1986 Feb;158(2):507-11. doi: 10.1148/radiology.158.2.3510448.
Three infants with nonobstructive bile duct dilatation (Caroli disease) are described. The bile duct pathology was associated with autosomal recessive polycystic kidney disease in two patients and with severe destruction of the renal parenchyma of unknown origin in the other. Sonograms of the liver showed, besides bile duct dilatations, intraluminal bulbar protrusions, bridge formation across dilated lumina, and portal radicles partially or completely surrounded by dilated bile ducts. Liver biopsy was performed in two patients, and in one patient, the biopsy sample confirmed the findings noted on sonograms. These findings support the hypothesis that the normal embryogenesis of intrahepatic bile ducts is arrested in the pathogenesis of this disease.
本文描述了3例非梗阻性胆管扩张（卡罗利病）的婴儿。其中2例患者的胆管病理改变与常染色体隐性多囊肾病相关，另1例患者的胆管病理改变与不明原因的严重肾实质破坏相关。肝脏超声检查显示，除胆管扩张外，还有管腔内的球状突起、横跨扩张管腔的桥形成，以及部分或完全被扩张胆管包围的门静脉分支。对2例患者进行了肝活检，其中1例患者的活检样本证实了超声检查所见。这些发现支持了以下假说：在该疾病的发病机制中，肝内胆管的正常胚胎发育过程受阻。
