Calinescu Ana M, Wildhaber Barbara E
Swiss Pediatric Liver Center, Geneva University Hospitals, Geneva, Switzerland; Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, Geneva, Switzerland.
Swiss Pediatric Liver Center, Geneva University Hospitals, Geneva, Switzerland; Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, Geneva, Switzerland.
Semin Pediatr Surg. 2024 Dec;33(6):151471. doi: 10.1016/j.sempedsurg.2025.151471. Epub 2025 Jan 17.
Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR). In the literature many different decision algorithms exist for BA-associated cholangitis underlining the clear need for standardizing diagnostic criteria. As an illustration, in 2015 the Swiss center introduced structured guidelines to diagnose BA-associated cholangitis. Upon retrospective analysis of SBAR data (2010-2022) we noted significant differences in disease management before and after implementation: The number of multiple diagnosed cholangitis episodes was reduced and we observed a reduced transplantation rate. Building on this, the Biliary Atresia and Related Diseases consensus has developed key diagnostic and management guidelines with objective criteria that have yet to undergo prospective validation. Indeed, prospective multicenter assessment is clearly needed to enhance acceptance and improve outcomes. Further, rigorous evaluation of prophylactic and therapeutic interventions, including antibiotics and immunomodulatory treatments, seems essential, with randomized trials required to establish their effectiveness and explore microbiota modulation in cholangitis management. The BA-community should strive to establish and adopt a widely accepted protocol for the diagnosis, management and prevention of BA-associated cholangitis. The implementation of such protocols is expected to significantly improve clinical outcomes for these patients.
胆管炎，定义为胆管的炎症，是胆道闭锁（BA）患者行肝门空肠吻合术后最常见的并发症。本综述旨在全面综合当前关于诊断和管理BA相关性胆管炎的知识，同时找出现有文献中的差距。我们进行了一项范围界定文献综述，以收集全球对肝门空肠吻合术后胆管炎的定义、评估和管理的见解，并通过瑞士胆道闭锁登记处（SBAR）的数据进行说明。文献中存在许多针对BA相关性胆管炎的不同决策算法，这突出表明明确需要标准化诊断标准。例如，2015年瑞士中心引入了诊断BA相关性胆管炎的结构化指南。通过对SBAR数据（2010 - 2022年）的回顾性分析，我们注意到实施前后疾病管理存在显著差异：多次诊断的胆管炎发作次数减少，且我们观察到移植率降低。在此基础上，胆道闭锁及相关疾病共识制定了具有客观标准的关键诊断和管理指南，但这些标准尚未经过前瞻性验证。事实上，显然需要进行前瞻性多中心评估以提高接受度并改善结果。此外，对包括抗生素和免疫调节治疗在内的预防性和治疗性干预措施进行严格评估似乎至关重要，需要进行随机试验以确定其有效性并探索胆管炎管理中的微生物群调节。BA领域应努力建立并采用一种被广泛接受的诊断、管理和预防BA相关性胆管炎的方案。预计实施此类方案将显著改善这些患者的临床结局。
