Friedmacher Florian, Ford Kathryn E, Davenport Mark
Department of Pediatric Surgery, King's College Hospital, Denmark Hill, London, SE5 9RS, UK.
Department of Pediatric Surgery, The Royal London Hospital, London, UK.
Pediatr Surg Int. 2019 Mar;35(3):273-282. doi: 10.1007/s00383-018-4392-4. Epub 2018 Nov 7.
Choledochal malformations (CMs) represent a spectrum of relatively rare and complex congenital anomalies, characterized by abnormal dilatation of the biliary tract in the absence of any acute obstruction. Today, almost 20% of CMs can be detected in-utero using maternal ultrasonography. Formal scientometric analysis was used to identify where modern CM research is taking place and perhaps where our attention should be directed in the future. Thus, this article offers a comprehensive review of recent scientific advances relating to CMs including the current understanding of etiology and classification, whilst also discussing key controversies such as risk of malignant transformation and the role of newer modalities of surgical treatment. Although laparoscopic excision of CMs and biliary reconstruction is nowadays feasible and safe, care should be taken before dispensing with standard open techniques, which have minimal complication rates and proven long-term benefit.
胆管畸形（CMs）是一系列相对罕见且复杂的先天性异常，其特征是在没有任何急性梗阻的情况下胆道出现异常扩张。如今，近20%的胆管畸形可在孕期通过母体超声检查检测出来。本文采用正式的科学计量分析方法来确定现代胆管畸形研究的开展领域，以及未来我们可能应关注的方向。因此，本文全面综述了与胆管畸形相关的近期科学进展，包括目前对病因和分类的认识，同时也讨论了诸如恶性转化风险和新型手术治疗方式的作用等关键争议问题。尽管如今腹腔镜下切除胆管畸形并进行胆道重建是可行且安全的，但在摒弃标准开放手术技术之前应谨慎，因为标准开放手术技术的并发症发生率极低且已证实具有长期益处。
