Fteriche Fadhel, Makni Amine, Ksantini Rachid, Rebai Wael, Bedioui Haikal, Nouira Kaies, Daghfous Amine, Ayadi Sofiane, Chebbi Faouzi, Jouini Mohamed, Ammous A, Montassar Kacem Jameleddine, Ben Safta Zouheir
Service de chirurgie A, Hopital La Rabta Tunis, Tunisie.
Tunis Med. 2012 Aug-Sep;90(8-9):630-5.
Caroli's disease is a congenital dilatation of the intrahepatic biliary duct.
To analyse and discuss diagnostic and therapeutics difficulties through 16 patients with Caroli's disease.
Between January 1990 and September 2010, 16 patients underwent surgical procedure for Caroli's disease. Data recorded for each patient included clinical symptoms, biologic findings, previous biliary procedures, and the presenting symptoms. The distribution of the biliary lesions, the surgical procedure and the postoperative outcomes and follow up were detailed.
The mean age was 55 years. The mean interval between the first symptoms and diagnosis was 27 months. Five of 16 patients had undergone 12 surgical or endoscopic procedures prior to liver resection. Before the definitive diagnosis, 9 patients presented 15 episodes of acute cholangitis. The diagnosis was established preoperatively in 13 cases, 5 among them underwent previous biliary surgical procedures. The diagnosis was documented peroperatively in 2 cases and postoperatively in 1 case. The distribution of the biliary lesions was monolobar in 13 and bilobar in 3 patients. 13 patients underwent liver resection, in two cases we perfomed biliojejunostomy and the last one had endoscopic sphincterotomy. There was no mortality and the overall postoperative morbidity is about 43%. The follow-up shows that 12 patients still alive with a mean follow up for 53 months, from whom only one patient have intrahépepatic lithiasis.
The clinical course of Caroli's disease is often complicated by recurrent episodes of angiocholitis and requires iterative surgery. The hepatectomy witch prevented septic complications and degenerescence is possible only in a restricted number of patients.
卡罗里病是肝内胆管的先天性扩张。
通过分析16例卡罗里病患者来探讨诊断和治疗的难点。
1990年1月至2010年9月期间，16例卡罗里病患者接受了外科手术。记录的每位患者的数据包括临床症状、生物学检查结果、既往胆道手术史及当前症状。详细记录了胆道病变的分布、手术过程、术后结果及随访情况。
平均年龄为55岁。首次症状出现至确诊的平均间隔时间为27个月。16例患者中有5例在肝切除术前接受了12次外科或内镜手术。在明确诊断之前，9例患者出现了15次急性胆管炎发作。13例术前确诊，其中5例曾接受过胆道外科手术。2例术中确诊，1例术后确诊。13例患者的胆道病变为单叶分布，3例为双叶分布。13例患者接受了肝切除术，2例行胆肠吻合术，最后1例行内镜括约肌切开术。无死亡病例，术后总体发病率约为43%。随访显示，12例患者存活，平均随访53个月，其中仅1例有肝内结石。
卡罗里病的临床病程常因反复发生胆管炎而复杂化，需要反复手术。只有少数患者可行肝切除术以预防感染并发症和病情恶化。
