Kassahun Woubet Tefera, Kahn Thomas, Wittekind Christian, Mössner Joachim, Caca Karel, Hauss Johann, Lamesch Peter
Department of Surgery II, Diagnostic Radiology, University of Leipzig, Leipzig, Germany.
Surgery. 2005 Nov;138(5):888-98. doi: 10.1016/j.surg.2005.05.002.
The aim of this study was to review and discuss our observations on 33 patients who underwent surgical treatment for Caroli's disease (CD), focusing on diagnosis, current surgical management, and long-term outcome.
Between May 1993 and June 2004, 642 liver resections and 286 liver transplantations in 252 patients were performed in our department of surgery. Thirty-three patients were referred to our center for diagnostic and therapeutic management of CD. Prior surgical interventions for hepatobiliary disorders, current diagnostic and surgical procedures, procedure-specific complications, duration of hospital stay, duration of follow-up, outpatient information, and long-term outcome were reviewed.
Fifteen male and 18 female patients were treated in this study. Initial symptoms and signs of the disease noted in our patients included right upper quadrant pain, fever, and jaundice. In 2 of the 33 patients, we noted clinical evidence of cirrhosis followed by histologic confirmation. One patient suffered from variceal bleeding. In 26 patients, diagnoses were established by a combined endoscopic retrograde cholangiopancreatography, ultrasonography, and computed tomographic studies. The disease was localized in 25 and diffuse in 8 patients. Liver resection was carried out in 29 patients. Partial hepatectomies were performed in 27 of these 29 at our institution. Two female patients with the diffuse disease underwent orthotopic liver transplantation. Thirteen of the 31 patients who underwent surgery at our institution had an uneventful postoperative course. Fourteen patients had minor postoperative complications and responded well to medical management. Four patients had major complications that required further surgical treatment. Two patients died of complications related to postoperative hemorrhage and sepsis. Two patients with intrahepatic cholangiocarcinoma died because of primary tumor progress. One patient with cholangiocarcinoma died 1 year after a successful left hepatectomy because of metastatic disease recurrence. The long-term results of the 26 surviving patients were assessed during a mean follow-up of 3.7 years (range, 1-11 years). All 26 patients remained free of biliary symptoms or complications. In 25 patients, surgery including liver transplantation was curative.
Partial hepatectomy for localized CD is potentially curative. In patients with diffuse CD, liver transplantation provides gratifying long-term results.
本研究旨在回顾并讨论我们对33例接受卡罗利病（CD）手术治疗患者的观察结果，重点关注诊断、当前的手术治疗方法及长期预后。
1993年5月至2004年6月期间，我们外科共对252例患者实施了642例肝脏切除术和286例肝移植术。33例患者因CD的诊断和治疗被转诊至我们中心。回顾了先前针对肝胆疾病的手术干预、当前的诊断和手术操作、特定手术并发症、住院时间、随访时间、门诊信息及长期预后。
本研究共治疗了15例男性和18例女性患者。我们的患者中疾病的初始症状和体征包括右上腹疼痛、发热和黄疸。33例患者中有2例，我们发现有肝硬化的临床证据，随后经组织学证实。1例患者发生静脉曲张出血。26例患者通过内镜逆行胰胆管造影、超声检查和计算机断层扫描联合检查得以确诊。25例患者疾病为局限性，8例为弥漫性。29例患者接受了肝切除术。在我们机构，这29例中有27例实施了部分肝切除术。2例患有弥漫性疾病的女性患者接受了原位肝移植。在我们机构接受手术的31例患者中，13例术后恢复顺利。14例患者有轻微术后并发症，经药物治疗反应良好。4例患者有严重并发症，需要进一步手术治疗。2例患者死于与术后出血和脓毒症相关的并发症。2例肝内胆管癌患者因原发肿瘤进展死亡。1例胆管癌患者在成功实施左肝切除术后1年因转移性疾病复发死亡。在平均3.7年（范围1 - 11年）的随访期间，对26例存活患者的长期结果进行了评估。所有26例患者均无胆道症状或并发症。25例患者包括肝移植在内的手术治疗是治愈性的。
局限性CD行部分肝切除术可能治愈。对于弥漫性CD患者，肝移植可提供令人满意的长期结果。
