Phugat Shivani, Choudhury Prativa, Jain Vishesh, Dhua Anjan Kumar, Yadav Devendra Kumar, Divya Gali, Agarwala Sandeep, Goel Prabudh
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
J Indian Assoc Pediatr Surg. 2025 May-Jun;30(3):277-289. doi: 10.4103/jiaps.jiaps_204_24. Epub 2025 Mar 3.
The type VI choledochal cyst of the cystic duct (CC-CD) is not only a taxonomical conundrum but also a matter of research in terms of its etiopathogenesis and optimal therapeutic options. The role of anomalous pancreaticobiliary junction (PBJ) and pancreatic juice regurgitation in the etiopathogenesis of CC-CD and its association with biliary malignancies has not been elucidated yet.
To evaluate the role of anomalous PBJ in the etiopathogenesis of CC-CD and generate a scientific opinion through a systematic review of the literature.
A literature review (PUBMED, PUBMED Central, PMC, Google Scholar, Google search engine) following the PRISMA guidelines revealed 32 articles of interest. Search terms included choledochal cyst, choledochal, cyst, type VI, type 6, cystic duct dilatation, dilated cystic duct and cystic anomaly. Two independent reviewers extracted the data; the third reviewer had been involved in case of discrepancy.
The study-cohort comprised of 45 patients. Roughly, 30% and 60% of the patients were diagnosed in the first and first-two decades of life, respectively. Sex ratio was skewed in favor of females (M:F = 1:1.4). Although males presented earlier than females, females were twice as likely to be diagnosed in the first decade of life. Anomalous PBJ was found in only one-third of these patients and was more common in females. Patients with anomalous PBJ presented early with three-fourth presenting in the first two decades of life. Anomalous PBJ was twice as prevalent in patients with concomitant involvement of other parts of the biliary tract (Group II) as compared to those with isolated CC-CD (Group I). Besides, patients in Group II presented early when compared to Group I, within the group females presented earlier. No significant association of biliary malignancies with anomalous PBJ was seen; the incidence of biliary malignancies was higher in those with a normal PBJ.
Observations from the study-cohort fail to establish a strong association of anomalous pancreaticobiliary junction in the etiopathogenesis of CC-CD or the development of biliary malignancies in these patients. It is likely that there are additional or alternative mechanisms that are important in the context of CC-CD.
胆囊管的VI型胆总管囊肿（CC-CD）不仅是一个分类难题，而且在其发病机制和最佳治疗选择方面也是一个研究课题。异常胰胆管连接（PBJ）和胰液反流在CC-CD发病机制中的作用及其与胆道恶性肿瘤的关联尚未阐明。
评估异常PBJ在CC-CD发病机制中的作用，并通过对文献的系统回顾得出科学观点。
按照PRISMA指南进行的文献综述（PUBMED、PUBMED Central、PMC、谷歌学术、谷歌搜索引擎）共筛选出32篇相关文章。检索词包括胆总管囊肿、胆总管、囊肿、VI型、6型、胆囊管扩张、扩张的胆囊管和胆囊异常。两名独立的审阅者提取数据；如有分歧，则由第三名审阅者参与。
研究队列包括45名患者。大致上，分别有30%和60%的患者在生命的第一个十年和前两个十年被诊断出来。性别比例偏向女性（男：女 = 1：1.4）。虽然男性比女性发病更早，但女性在生命的第一个十年被诊断出的可能性是男性的两倍。在这些患者中，仅三分之一发现有异常PBJ，且在女性中更为常见。有异常PBJ的患者发病较早，四分之三在生命的前两个十年发病。与孤立性CC-CD患者（I组）相比，伴有胆道其他部位受累的患者（II组）中异常PBJ的发生率是前者的两倍。此外，II组患者比I组发病早，在II组中女性发病更早。未发现异常PBJ与胆道恶性肿瘤有显著关联；PBJ正常的患者中胆道恶性肿瘤的发生率更高。
研究队列的观察结果未能证实异常胰胆管连接与CC-CD发病机制或这些患者中胆道恶性肿瘤的发生有强烈关联。在CC-CD的背景下，可能存在其他重要的机制或替代机制。
