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Abstract：
BACKGROUND: Caroli\'s disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli\'s syndrome, there is additionally an associated congenital hepatic fibrosis.
METHODS: With institutional review board approval, we identified all patients with Caroli\'s disease and syndrome.
RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli\'s disease (n = 6) and Caroli\'s syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli\'s syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months).
CONCLUSIONS: Caroli\'s disease and syndrome have a varied presentation. Most individuals with Caroli\'s disease may be adequately treated by resection, but transplantation is required for Caroli\'s syndrome patients due to the associated hepatic fibrosis.
METHODS: With institutional review board approval, we identified all patients with Caroli\'s disease and syndrome.
RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli\'s disease (n = 6) and Caroli\'s syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli\'s syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months).
CONCLUSIONS: Caroli\'s disease and syndrome have a varied presentation. Most individuals with Caroli\'s disease may be adequately treated by resection, but transplantation is required for Caroli\'s syndrome patients due to the associated hepatic fibrosis.
摘要：
背景：Caroli病是一种罕见的先天性疾病，其特征是肝内导管的非阻塞性扩张。在Caroli综合征中,此外，还有相关的先天性肝纤维化。
方法：经机构审查委员会批准，我们确定了所有患有Caroli病和综合征的患者。
结果：确定了9名患者，七个男性和两个女性，平均年龄为40岁。最终的病理诊断包括Caroli病（n=6）和Caroli综合征（n=3）。患者表现为肝功能紊乱，胆管炎,胆管癌,腹痛,肝硬化,或者是偶然被诊断出来的.4例患者接受了切除术，2例接受了肝移植。切除组的，两名患者随后接受移植治疗，其中一名患者因吻合口狭窄导致复发性胆管炎，另一名患者因终末期肝病。所有Caroli综合征患者均接受肝移植。3例患者在诊断为胆管癌复发后26.2、7.8和3个月随访期间死亡，肝功能衰竭,和转移性胆管癌,分别。6名患者存活，自就诊以来的中位随访时间为60个月（范围=10-134个月）。
结论：Caroli的疾病和综合征有不同的表现。大多数患有Caroli病的人可以通过切除得到充分的治疗，但Caroli综合征患者需要移植，因为相关的肝纤维化。
方法：经机构审查委员会批准，我们确定了所有患有Caroli病和综合征的患者。
结果：确定了9名患者，七个男性和两个女性，平均年龄为40岁。最终的病理诊断包括Caroli病（n=6）和Caroli综合征（n=3）。患者表现为肝功能紊乱，胆管炎,胆管癌,腹痛,肝硬化,或者是偶然被诊断出来的.4例患者接受了切除术，2例接受了肝移植。切除组的，两名患者随后接受移植治疗，其中一名患者因吻合口狭窄导致复发性胆管炎，另一名患者因终末期肝病。所有Caroli综合征患者均接受肝移植。3例患者在诊断为胆管癌复发后26.2、7.8和3个月随访期间死亡，肝功能衰竭,和转移性胆管癌,分别。6名患者存活，自就诊以来的中位随访时间为60个月（范围=10-134个月）。
结论：Caroli的疾病和综合征有不同的表现。大多数患有Caroli病的人可以通过切除得到充分的治疗，但Caroli综合征患者需要移植，因为相关的肝纤维化。
