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Abstract：
Caroli\'s disease is a very rare congenital malformation, currently included in cystic diseases of the biliary tract, and is characterized by ectasia and dilatation of the intrahepatic bile ducts. Two clinical entities can be distinguished, Caroli\'s disease in which congenital hepatic impairment is limited to cystic dilatation and Caroli\'s syndrome in which congenital hepatic fibrosis coexists. We present two cases of atypical presentations of Caroli\'s disease. Case one was a 76-year-old man who was referred to our hospital for chronic non-remitting epigastric pain prior to diagnosis. Magnetic resonance cholangiopancreatography (MRCP) was performed, which revealed findings consistent with Caroli\'s disease. Laboratory investigation disclosed a raised α-fetoprotein. Left hepatectomy was performed due to suspected cholangiocarcinoma. Morphological findings were compatible with Caroli\'s disease and no evidence of malignancy was found. Case two was a 47-year-old man who presented with chronic epigastric pain and generalized abdominal discomfort. MRCP revealed findings compatible with Caroli\'s disease. The patient was discharged with ursodeoxycholic acid treatment and was later admitted twice due to inaugural episodes of cholangitis that were medically managed. Bisegmentectomies II and III were performed for suspected neoplasia after a gradual rise in α-fetoprotein and CA19-9 values were noted during follow-up. The surgical specimen confirmed Caroli\'s disease and there was no evidence of malignancy. Postoperative periods for both patients were favorable, and they remain asymptomatic and well to date.
摘要：
Caroli病是一种非常罕见的先天性畸形,目前包括在胆道囊性疾病中，表现为肝内胆管扩张和扩张。可以区分两个临床实体，先天性肝损伤仅限于囊性扩张的Caroli病和先天性肝纤维化并存的Caroli综合征。我们介绍了2例不典型的Caroli病。病例1是一名76岁的男子，他在诊断前因慢性非缓解性上腹痛而被转诊到我们医院。磁共振胰胆管造影（MRCP），这揭示了与Caroli病一致的发现。实验室调查显示，甲胎蛋白升高。由于怀疑胆管癌，进行了左肝切除术。形态学发现与Caroli病一致，没有发现恶性肿瘤的证据。病例2是一名47岁的男子，他患有慢性上腹痛和全身腹部不适。MRCP揭示了与Caroli病相符的发现。该患者接受熊去氧胆酸治疗后出院，后来因首次发作的胆管炎发作而入院两次。在随访期间发现甲胎蛋白和CA19-9值逐渐升高后，对可疑的瘤形成进行了双切除切除术II和III。手术标本证实为Caroli病，没有恶性肿瘤的证据。两个患者的术后时期都是有利的，到目前为止，他们仍然无症状。
