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Abstract：
BACKGROUND: Caroli\'s disease (CD) management is still controversial.
OBJECTIVE: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD.
METHODS: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed.
RESULTS: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70.8%) patients were female, with average age of 48.7 years old (20-71), and 95.5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8.3%) and cholangiocarcinoma in one (4.2%).
CONCLUSIONS: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.
OBJECTIVE: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD.
METHODS: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed.
RESULTS: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70.8%) patients were female, with average age of 48.7 years old (20-71), and 95.5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8.3%) and cholangiocarcinoma in one (4.2%).
CONCLUSIONS: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.
摘要：
背景：Caroli的疾病（CD）管理仍然存在争议。
目的：本研究的目的是报告最常见的临床特征，治疗方案,以及CD手术治疗后获得的结果。
方法：进行自愿调查。人口统计,临床,外科,并对病理变量进行了分析。
结果：六个中心包括1991年至2009年接受手术治疗的24名患者。17名（70.8%）患者为女性，平均年龄48.7岁（20-71岁），95.5%有症状。75％的患者有左半侧受累。外科手术包括9次左外侧切除术，八个左肝切除术,对于患有半乳病的人来说，有四个右肝切除术，而对于双侧疾病的患者，进行了一次右肝切除术和两次Roux-en-Y肝空肠吻合术。平均住院时间为7天。围手术期并发症（25%），3例患者出现轻微并发症（1-2型），而主要并发症发生在3例患者(3a型)。没有死亡报告。经过166个月的中位随访，所有病人都活着，没有症状。CD诊断通过组织学证实。两名患者（8.3％）存在先天性肝纤维化，其中一名患者存在胆管癌（4.2％）。
结论：阿根廷的CD多见于左半侧受累的女性。手术切除是单侧疾病的最佳治疗选择，提供长期生存无症状和并发症。在选定的无实质累及的双侧疾病病例中，应建议肝空肠吻合术。然而,密切随访是强制性的,因为患者可能会有进展,应进行移植.
目的：本研究的目的是报告最常见的临床特征，治疗方案,以及CD手术治疗后获得的结果。
方法：进行自愿调查。人口统计,临床,外科,并对病理变量进行了分析。
结果：六个中心包括1991年至2009年接受手术治疗的24名患者。17名（70.8%）患者为女性，平均年龄48.7岁（20-71岁），95.5%有症状。75％的患者有左半侧受累。外科手术包括9次左外侧切除术，八个左肝切除术,对于患有半乳病的人来说，有四个右肝切除术，而对于双侧疾病的患者，进行了一次右肝切除术和两次Roux-en-Y肝空肠吻合术。平均住院时间为7天。围手术期并发症（25%），3例患者出现轻微并发症（1-2型），而主要并发症发生在3例患者(3a型)。没有死亡报告。经过166个月的中位随访，所有病人都活着，没有症状。CD诊断通过组织学证实。两名患者（8.3％）存在先天性肝纤维化，其中一名患者存在胆管癌（4.2％）。
结论：阿根廷的CD多见于左半侧受累的女性。手术切除是单侧疾病的最佳治疗选择，提供长期生存无症状和并发症。在选定的无实质累及的双侧疾病病例中，应建议肝空肠吻合术。然而,密切随访是强制性的,因为患者可能会有进展,应进行移植.
