Gillet M, Favre S, Fontolliet C, Halkic N, Mantion G, Heyd B
Service de chirurgie, centre hospitalier universitaire Vaudois, Lausanne, Suisse.
Chirurgie. 1999 Feb;124(1):13-8; discussion 18-9. doi: 10.1016/s0001-4001(99)80037-3.
Caroli's disease is the dilatation of the segmental intrahepatic bile ducts. It usually affects the entire liver but can occasionally involve only one lobe, commonly the left. This study included 12 cases of unilobular disease, nine localised in the left lobe and three in the right lobe, which were all treated by liver resection.
These 12 patients underwent surgery between 1974 and 1997. There were six men and six women (mean age: 51 years). The initial presentation and diagnosis were reported. The mean interval between the first symptoms and diagnosis was 12.5 years. Eight of the 12 patients had undergone 22 surgical or endoscopic procedures prior to liver resection. In the present series a preoperative ultrasonogram or CT scan established the diagnosis in all cases. Six patients did not have stones in the gallbladder.
Surgical treatment consisted in seven left lobectomies, two left hepatectomies and three right hepatectomies (Couinaud's classification). A intrahilar cholangiojejunostomy was performed in five cases. Pathological examination showed cystic dilatation of the intrahepatic segmental and subsegmental bile ducts, measuring from a few millimetres to 4 cm, which contained calculi. Two cases were associated with congenital hepatic fibrosis. An intrahepatic focus of ectopic pancreatic tissue was seen in one case. There were no cases with cholangiocarcinoma. One patient developed a biliary fistula which required reoperation. All patients had an uneventful long term postoperative course except for one patient who died of colon carcinoma 3 years postoperatively.
When associated with other malformations, most notably congenital hepatic fibrosis, this commonly diffuse disease is called Caroli's syndrome. The unilobar form, most usually involving the left lobe of the liver, is called Caroli's disease. Both monolobar and diffuse types are often characterised by recurrent bouts of cholangitis and, in over half of the cases, by common bile duct stones without gallbladder stones.
卡罗里病是节段性肝内胆管扩张。它通常累及整个肝脏，但偶尔也可能仅累及一个肝叶，常见于左叶。本研究纳入了12例单叶病变患者，其中9例局限于左叶，3例局限于右叶，均接受了肝切除术治疗。
这12例患者于1974年至1997年间接受手术。其中男性6例，女性6例（平均年龄：51岁）。报告了初始表现和诊断情况。首次症状出现至诊断的平均间隔时间为12.5年。12例患者中有8例在肝切除术前接受了22次手术或内镜检查。在本系列研究中，所有病例术前均通过超声检查或CT扫描确诊。6例患者胆囊内无结石。
手术治疗包括7例左半肝切除术、2例左肝切除术和3例右肝切除术（根据Couinaud分类法）。5例患者进行了肝门部胆管空肠吻合术。病理检查显示肝内节段性和亚节段性胆管呈囊性扩张，直径从几毫米到4厘米不等，内有结石。2例患者合并先天性肝纤维化。1例患者可见肝内异位胰腺组织灶。无胆管癌病例。1例患者发生胆瘘，需要再次手术。除1例患者术后3年死于结肠癌外，所有患者术后长期恢复顺利。
当与其他畸形相关时，最显著的是先天性肝纤维化，这种通常弥漫性的疾病被称为卡罗里综合征。单叶型，最常累及肝左叶，被称为卡罗里病。单叶型和弥漫型通常都以反复胆管炎发作为特征，超过半数的病例伴有胆总管结石而无胆囊结石。
