Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Department of Radiology, John Hopkins University, Baltimore, MD, USA.
HPB (Oxford). 2023 Jan;25(1):14-25. doi: 10.1016/j.hpb.2022.09.010. Epub 2022 Oct 5.
Choledochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. We review the pathophysiology, diagnosis, and management of CCs.
MEDLINE/PubMed and Web of Science databases were queried for "choledochal cyst", "bile duct cyst", "choledochocele", and "Caroli disease". Data were synthesized and systematically reviewed.
Classified according to the Todani Classification, CCs are generally believed to arise secondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis, liver abscess, liver cirrhosis and malignant transformation (3-7.5%). Radiological and endoscopic imaging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper management. Management is generally guided by cyst classification with complete cyst excision necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall survival after choledochal cyst excision is 95.5%.
Most CCs should undergo surgical intervention to mitigate the risk of cyst related complications such as cholangitis and malignant transformation.
胆总管囊肿（CCs）是一种罕见的肝内外胆管囊性扩张。我们回顾了 CCs 的病理生理学、诊断和治疗方法。
在 MEDLINE/PubMed 和 Web of Science 数据库中检索了“choledochal cyst”、“bile duct cyst”、“choledochocele”和“Caroli disease”。对数据进行了综合和系统的回顾。
根据 Todani 分类，CCs 通常被认为是由于胰胆管异常联合导致胰酶反流到胆道系统而引起的。CCs 的并发症包括腹痛、黄疸、胆石症、胆囊炎、胰腺炎、肝脓肿、肝硬化和恶性转化（3-7.5%）。影像学和内镜检查是 CC 诊断的基石，全面描绘囊肿解剖结构对于正确管理至关重要。治疗一般根据囊肿分类进行指导，对于具有高恶性转化潜能的 CCs（如 I 型和 IV 型）需要进行完全囊肿切除。胆总管囊肿切除术后 5 年总生存率为 95.5%。
大多数 CCs 应进行手术干预，以降低与囊肿相关的并发症（如胆管炎和恶性转化）的风险。
Zotero 插件