Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas, USA.
J Surg Res. 2012 Oct;177(2):365-72. doi: 10.1016/j.jss.2012.04.022. Epub 2012 Apr 29.
Caroli disease (CD) is characterized by dilation of the intrahepatic biliary tree, which may result in malignancy. Treatments include management of symptoms and hepatic resection to decrease disease burden. In patients with CD not amenable to these treatments, orthotopic liver transplantation (OLT) has been used. This study examines if OLT is a reasonable treatment for patients with CD.
The United Network of Organ Sharing/Organ Procurement and Transplantation Network database between September 30, 1987 and March 31, 2011 was queried. Cases without patient or allograft survival time or without a diagnosis were excluded from analysis. Patients with CD were compared to patients with primary biliary cirrhosis (PBC), secondary biliary cirrhosis (BC), primary sclerosing cholangitis (PSC), and all indications for OLT. Survival analysis was performed by log-rank test and Kaplan-Meier.
One hundred forty patients with CD were compared to 4797 patients with PBC, 489 patients with secondary BC, 6033 patients with PSC, and 92,210 patients post-OLT. Patient and allograft survivals of CD patients at 1, 3, 5, and 10 y are, respectively, 88.5%, 83.4%, 80.9%, and 77.8%; and 81.2%, 74.8%, 70.6%, and 67.9%. CD patients have significantly improved patient and allograft survivals after OLT compared to patients with secondary BC (P = 0.003, P = 0.015) and all other patients undergoing OLT (P = 0.003, P = 0.026). There is a trend towards long-term improved patient and allograft survival in transplanted patients with CD compared to patients with PBC and PSC.
These results suggest that OLT should be considered an effective treatment modality for patients with CD resulting in excellent long-term outcomes.
Caroli 病（CD）的特征是肝内胆管扩张，可能导致恶性肿瘤。治疗包括症状管理和肝切除术以减轻疾病负担。对于这些治疗方法不可行的 CD 患者，肝移植（OLT）已被使用。本研究探讨 OLT 是否是 CD 患者的合理治疗方法。
检索 1987 年 9 月 30 日至 2011 年 3 月 31 日期间 United Network of Organ Sharing/Organ Procurement and Transplantation Network 数据库。排除无患者或移植物生存时间或无诊断的病例。将 CD 患者与原发性胆汁性胆管炎（PBC）、继发性胆汁性胆管炎（BC）、原发性硬化性胆管炎（PSC）和所有 OLT 适应证患者进行比较。通过对数秩检验和 Kaplan-Meier 进行生存分析。
将 140 例 CD 患者与 4797 例 PBC 患者、489 例继发性 BC 患者、6033 例 PSC 患者和 92210 例 OLT 后患者进行比较。CD 患者的患者和移植物 1、3、5 和 10 年生存率分别为 88.5%、83.4%、80.9%和 77.8%；和 81.2%、74.8%、70.6%和 67.9%。与继发性 BC 患者（P = 0.003，P = 0.015）和所有其他接受 OLT 的患者（P = 0.003，P = 0.026）相比，OLT 后 CD 患者的患者和移植物生存率显著提高。与 PBC 和 PSC 患者相比，接受移植的 CD 患者的患者和移植物长期生存率有改善趋势。
这些结果表明，OLT 应被视为治疗 CD 患者的有效治疗方法，可获得极好的长期结果。
