Knoop M, Keck H, Langrehr J M, Peter F J, Ferslev B, Neuhaus P
Chirurgische Klinik und Poliklinik, Universitätsklinikum Rudolf Virchow, Freie Universität Berlin.
Chirurg. 1994 Oct;65(10):861-6.
Caroli's disease is characterized by uni- or bilobar cystic enlargement of the intrahepatic biliary tract. Clinical symptoms include recurrent cholangitis, right upper quadrant abdominal pain and jaundice. The diagnosis is usually established preoperatively based on abdominal ultrasound, computertomography and ERCP. Eight patients suffering from unilobar Caroli's disease underwent liver resection during a period of six years. The postoperative course was uneventful, and after a median follow-up of 39.6 months in one case only a temporary jaundice occurred. Liver resection is the method of choice for Caroli's syndrome when feasible. Apart from the curative intention liver resection represents also a prophylactic method since the risk of malignant transformation to cholangiocellular carcinoma in the cystic walls after draining procedures is considerably high. For diffuse spread of Caroli's disease in the liver orthotopic liver transplantation has emerged as therapeutic option.
卡罗里病的特征是肝内胆管单叶或双叶囊性扩张。临床症状包括复发性胆管炎、右上腹腹痛和黄疸。诊断通常在术前根据腹部超声、计算机断层扫描和内镜逆行胰胆管造影术来确立。在六年期间，八名患有单叶卡罗里病的患者接受了肝切除术。术后过程平稳，在一例患者中位随访39.6个月后，仅出现了短暂性黄疸。在可行的情况下，肝切除术是卡罗里综合征的首选治疗方法。除了具有治愈目的外，肝切除术也是一种预防方法，因为引流术后囊肿壁发生胆管细胞癌恶性转化的风险相当高。对于卡罗里病在肝脏中的弥漫性扩散，原位肝移植已成为一种治疗选择。
