Ulrich Frank, Pratschke Johann, Pascher Andreas, Neumann Ulf P, Lopez-Hänninen Enrique, Jonas Sven, Neuhaus Peter
Department of General, Visceral and Transplantation Surgery, Charité, Campus Virchow Clinical Centre, Berlin, Germany.
Ann Surg. 2008 Feb;247(2):357-64. doi: 10.1097/SLA.0b013e31815cca88.
To assess the preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS).
The clinical course of monolobar or diffuse CD or CS is often characterized by multiple conservative treatment attempts and interventions with recurrent episodes of cholangitis and a serious reduction in quality of life. The role and effectiveness of surgical treatment is still not well defined.
Between June 1989 and December 2002, we treated 44 consecutive patients with CD or CS who had failure of conservative treatment before and were referred for surgical intervention. Demographic and clinical data, operative procedures and related morbidity, course of symptoms, and long-term follow-up were reviewed. Four patients with palliative resection for cholangiocarcinoma and incidental diagnosis of CD were excluded from the analysis.
Twenty-two women and 18 men had a median period of 26.5 months from onset of symptoms to surgical therapy. Their median age at therapy was 49 years and 80% of the patients had monolobar disease with a left-right ratio of 2.6 to 1. Thirty-three (82.5%) patients underwent liver resection, while 4 (10%) patients received OLT for diffuse disease. Biliodigestive anastomosis alone was performed in 3 (7.5%) patients with contraindications to OLT. Patients (37.5%) had minor postoperative complications, which were treated conservatively, while 2 (5%) transplanted patients had a reoperation due to intraperitoneal bleeding. After a median follow-up of 86.5 months, we observed a favorable patient and graft survival. Three deaths during follow-up were not related to treatment or disease complications. Follow-up of disease-related symptoms, biliary complications, and antibiotic treatment revealed a significant improvement.
Our data show that liver resection for monolobar CD or CS and OLT for diffuse manifestations can achieve excellent long-term patient survival with marked symptom relief. Because of life-threatening long-term complications such as biliary sepsis and development of cholangiocarcinoma, timely indication for surgical treatment is crucial.
评估肝切除或原位肝移植（OLT）治疗卡罗里病（CD）或卡罗里综合征（CS）的术前疾病特征、术后并发症发生率、患者生存率及症状病程。
单叶或弥漫性CD或CS的临床病程通常表现为多次保守治疗尝试及干预，伴有复发性胆管炎发作，生活质量严重下降。手术治疗的作用和效果仍未明确界定。
1989年6月至2002年12月，我们连续治疗了44例CD或CS患者，这些患者此前保守治疗失败并被转诊接受手术干预。回顾了人口统计学和临床数据、手术操作及相关发病率、症状病程和长期随访情况。4例因胆管癌姑息性切除并偶然诊断为CD的患者被排除在分析之外。
22名女性和18名男性从症状出现到手术治疗的中位时间为26.5个月。他们接受治疗时的中位年龄为49岁，80%的患者为单叶疾病，左右叶比例为2.6比1。33例（82.5%）患者接受了肝切除，4例（10%）弥漫性疾病患者接受了OLT。3例（7.5%）有OLT禁忌证的患者仅进行了胆肠吻合术。患者中有（37.5%）发生轻微术后并发症，经保守治疗，而2例（5%）移植患者因腹腔内出血接受了再次手术。中位随访86.5个月后，我们观察到患者和移植物生存率良好。随访期间的3例死亡与治疗或疾病并发症无关。对疾病相关症状、胆道并发症和抗生素治疗的随访显示有显著改善。
我们的数据表明，单叶CD或CS的肝切除及弥漫性表现的OLT可实现患者长期良好生存，症状明显缓解。由于存在如胆源性败血症和胆管癌发生等危及生命的长期并发症，及时进行手术治疗的指征至关重要。
