Department of Gastroenterology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui Province, China.
J Int Med Res. 2020 Feb;48(2):300060519877993. doi: 10.1177/0300060519877993. Epub 2019 Oct 8.
Caroli's disease (CD) is a very rare congenital disorder that is characterized by non-obstructive, segmental and cystic dilatation of intrahepatic ducts. Most patients with CD are asymptomatic, but some patients may suffer from hepatic fibrosis, liver cirrhosis or/and portal hypertension. In complex CD, cystic dilatations of the intrahepatic bile ducts can be present with congenital hepatic fibrosis, liver cirrhosis, portal hypertension, oesophageal varices and autosomal recessive polycystic kidney disease; a condition known as Caroli's syndrome. This report describes the case of a 28-year-old woman that had gastro-oesophageal varices that were caused by hepatic fibrosis and portal hypertension as part of Caroli's syndrome. The patient underwent successful treatment with endoscopic injection sclerotherapy with lauromacrogol and endoscopic variceal obturation using tissue adhesive. There were no immediate complications and the patient remained free of complications at 1-year follow-up. There are no current reports in the published literature describing Caroli's syndrome induced by gastro-oesophageal varices that were treated by a combination of endoscopic injection sclerotherapy and endoscopic variceal obturation. Endoscopic therapy was an effective technique for the treatment of gastro-oesophageal varices in a patient with Caroli's syndrome awaiting a liver transplant.
先天性肝内胆管囊状扩张症（Caroli 病）是一种非常罕见的先天性疾病，其特征为肝内胆管非梗阻性、节段性和囊状扩张。大多数 Caroli 病患者无症状，但部分患者可能患有肝纤维化、肝硬化或/和门静脉高压。在复杂的 Caroli 病中，肝内胆管的囊性扩张可与先天性肝纤维化、肝硬化、门静脉高压、食管静脉曲张和常染色体隐性多囊肾病并存，这种情况被称为 Caroli 综合征。本报告描述了一例 28 岁女性病例，其因肝纤维化和门静脉高压导致的食管胃静脉曲张是 Caroli 综合征的一部分。该患者成功接受了内镜下聚桂醇注射硬化治疗和组织胶内镜下曲张静脉闭塞术。无即刻并发症，患者在 1 年随访时无并发症。目前在已发表的文献中没有描述因食管胃静脉曲张而导致的 Caroli 综合征，且这些静脉曲张采用内镜下注射硬化治疗和内镜下曲张静脉闭塞术联合治疗的病例报告。对于等待肝移植的 Caroli 综合征患者，内镜治疗是治疗食管胃静脉曲张的有效方法。
