Bloomfield Grace C, Nigam Aradhya, Calvo Inochi Gonzalez, Dorris C Scott, Fishbein Thomas M, Radkani Pejman, Winslow Emily R
Georgetown University School of Medicine, Washington, District of Columbia, United States.
Department of Surgery, Medstar Georgetown University Hospital, Washington, District of Columbia, United States.
J Gastrointest Surg. 2024 Jan;28(1):77-87. doi: 10.1016/j.gassur.2023.11.007.
The approach to patients with choledochal cysts (CCs) remains varied and subject to institutional practices. Owing to the rarity of the disease, the optimal treatment remains poorly defined, particularly in the adult population. This study aimed to review the literature on adult patients with CCs to evaluate trends of diagnosis and management in Western countries.
A literature search of 3 electronic databases was performed on adult patients diagnosed with CCs in Western institutions. A review of published literature was completed with comprehensive screening by 2 independent reviewers. Studies were analyzed, and data on surgical approach, malignancies, and follow-up were collected. Findings are presented in concordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines.
Of the 3488 articles retrieved, 21 studies evaluated Western adults with CCs for a combined population of 1337 patients. The most common Todani subtypes included types I (64%) and IV (22%). Symptoms at presentation included abdominal pain and jaundice, although many were asymptomatic. Ultrasound was used most frequently for diagnosis, followed by computed tomography and endoscopic cholangiopancreatography. The combined malignancy rate was 10.9%, with cholangiocarcinoma being the most prevalent. Complete extrahepatic cyst resection was standard for type I and IV CCs. Among malignancies, 18.5% and 16.4% were observed in patients with prior resection and internal drainage, respectively.
A significant proportion of patients who undergo resection of CC disease harbor malignancy. Cancer risk seems reduced but not eliminated with complete resection, which remains the standard treatment. Additional studies are needed to standardize guidelines for the diagnosis and postoperative care of patients in Western countries.
胆管囊肿（CCs）患者的治疗方法仍然多样，且因机构而异。由于该疾病罕见，最佳治疗方案仍不明确，尤其是在成人患者中。本研究旨在回顾有关成年CCs患者的文献，以评估西方国家的诊断和管理趋势。
对3个电子数据库进行文献检索，纳入在西方机构诊断为CCs的成年患者。由2名独立审阅者进行全面筛选，完成对已发表文献的综述。分析研究并收集有关手术方式、恶性肿瘤和随访的数据。研究结果按照系统评价和Meta分析的首选报告项目（PRISMA）指南呈现。
在检索到的3488篇文章中，21项研究评估了西方成年CCs患者，共1337例。最常见的Todani亚型包括I型（64%）和IV型（22%）。就诊时的症状包括腹痛和黄疸，尽管许多患者无症状。超声最常用于诊断，其次是计算机断层扫描和内镜逆行胰胆管造影。恶性肿瘤合并发生率为10.9%，其中胆管癌最为常见。I型和IV型CCs的标准治疗是完全肝外囊肿切除术。在恶性肿瘤患者中，先前接受过切除术和内引流术的患者分别占18.5%和16.4%。
接受CC疾病切除术的患者中有很大一部分患有恶性肿瘤。完全切除似乎能降低但不能消除癌症风险，完全切除仍是标准治疗方法。需要进一步研究以规范西方国家患者的诊断和术后护理指南。
