Benhidjeb T, Müller J M, Gellert K, Zanow J, Rudolph B
klinik und Poliklinik f[r Chirurgie, Universitätsklinikums Charité der Humboldt-Universität Berlin.
Chirurg. 1996 Mar;67(3):238-43.
17 patients with a Caroli's syndrome are reviewed with emphasis on clinical features and late results (follow-up: 2-10 years). The group consists of 9 women and 8 men ranging in age from 17 to 80 years (mean of 42.6). 14 patients had a diffuse form, 5 of whom had periportal fibrosis with secondary biliary cirrhosis, which in 3 cases resulted in a portal hypertension with hepatic failure. In only 3 cases the dilatation was limited to the left lobe of the liver. 9 of the patients had previously undergone surgery in the form of cholecystectomy and choledocholithotomy elsewhere. The disease was complicated by lithiasis (14 intrahepatic, 9 extrahepatic). Curative treatment was only possible in the 3 patients with unilobar Caroli's syndrome (partial liver resection). In 5 patients we performed a cholecystectomy and choledocholithotomy combined in 2 cases with a transduodenal sphincterotomy. There was no operative death. Endoscopic treatment consisted in removal of stones and decompression of the biliary tree by sphincterotomy in 9 cases and endoscopic retrograde biliary drainage (endoprosthesis) in 5 cases. Excepting the 3 curative operated patients who are asymptomatic respectively 2, 4 and 5 years after surgery, the remaining cases had repeated bouts of acute pains accompanied by recurrent episodes of cholangitis. A late mortality of 23.5% (4 patients) is proof of the poor prognosis of this disease.
对17例卡罗里氏综合征患者进行了回顾性研究，重点关注临床特征和远期结果（随访时间：2至10年）。该组患者包括9名女性和8名男性，年龄在17岁至80岁之间（平均42.6岁）。14例患者为弥漫型，其中5例伴有门静脉周围纤维化及继发性胆汁性肝硬化，3例导致门静脉高压伴肝功能衰竭。仅3例扩张局限于肝左叶。9例患者此前在其他地方接受过胆囊切除术和胆总管切开取石术。该疾病并发结石（14例肝内结石，9例肝外结石）。仅3例单叶型卡罗里氏综合征患者可行根治性治疗（部分肝切除术）。5例患者接受了胆囊切除术和胆总管切开取石术，其中2例联合十二指肠括约肌切开术。无手术死亡病例。内镜治疗包括9例通过括约肌切开术取出结石和解除胆道梗阻，5例进行内镜逆行胆道引流（放置内支架）。除3例根治性手术患者术后分别在2年、4年和5年无症状外，其余病例均反复出现急性疼痛伴胆管炎复发。23.5%的远期死亡率（4例患者）证明了该疾病预后不良。
