Barros J L, Polo J R, Sanabia J, Garcia-Sabrido J L, Gomez-Lorenzo F J
Surgery. 1979 May;85(5):589-92.
A case of congenital cystic dilatation of the intrahepatic bile ducts (Caroli's disease) followed for more than 7 years is reported. This patient presented also with congenital hepatic fibrosis, gallstones, and biliary hypersecretion of more than 3,000 ml in 24 hours. An analysis of the literature relating to Caroli's disease disclosed 46 well-documented cases of both hepatic histopathology and biliary tree studies. Six cases (13%) were found to be isolated forms of intrahepatic cystic dilatations; 16 (34.7%) were associated with congenital hepatic fibrosis; 10 (21.7%) presented with either a choledochal cyst or nonobstructive extrahepatic biliary tree dilatation; and in 14 cases (30%) the three anomalies were found together in the same patient. After these findings, we think that congenital hepatic fibrosis, congenital cystic dilatations of the intrahepatic bile ducts (Caroli's disease), choledochal cyst and other nonobstructive dilatations of the extrahepatic biliary tree are possibly the same congenital disease with different levels of involvement.
报告了一例肝内胆管先天性囊性扩张（卡罗利病）随访7年以上的病例。该患者还伴有先天性肝纤维化、胆结石以及24小时胆汁分泌超过3000毫升。对与卡罗利病相关的文献分析发现了46例有充分肝脏组织病理学和胆管树研究记录的病例。其中6例（13%）为孤立性肝内囊性扩张；16例（34.7%）与先天性肝纤维化相关；10例（21.7%）伴有胆总管囊肿或非梗阻性肝外胆管扩张；14例（30%）在同一患者中发现三种异常同时存在。基于这些发现，我们认为先天性肝纤维化、肝内胆管先天性囊性扩张（卡罗利病）、胆总管囊肿以及肝外胆管的其他非梗阻性扩张可能是同一先天性疾病的不同累及程度。
