Attar Mohammad A, Donn Steven M
Department of Pediatrics and Communicable Diseases, Division of Neonatal-Perinatal Medicine, C.S. Mott Children's Hospital, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.
Department of Pediatrics and Communicable Diseases, Division of Neonatal-Perinatal Medicine, C.S. Mott Children's Hospital, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.
Semin Fetal Neonatal Med. 2017 Aug;22(4):234-239. doi: 10.1016/j.siny.2017.03.005. Epub 2017 Mar 27.
Congenital chylothorax (CC) results from multiple lymphatic vessel anomalies or thoracic cavity defects and may accompany other congenital anomalies. Fetal chylothorax may increase the risk of death and complications from pleural space lymphatic fluid accumulation, which compromises lung development, pulmonary, and cardiovascular function and from complications arising from the loss of drained lymphatic contents. Prenatal interventions might improve survival in severe cases of fetal chylothorax. The neonatal treatment strategy is generally supportive with interventions that include thoracostomy drainage and attempts to decrease chyle flow using a stepwise approach that begins with the least invasive means. Evidence-based treatment choices are lacking and are much needed. Most cases of CC resolve with time even without specific lymphatic system studies to identify the exact pathology. Expertise in performing lymphatic studies is not universally available. Data on both efficacy and safety of the various therapeutic options are needed to determine the best approach to the treatment of CC.
先天性乳糜胸（CC）由多种淋巴管异常或胸腔缺陷引起，可能伴有其他先天性异常。胎儿乳糜胸可能会增加因胸腔内淋巴液积聚导致死亡和并发症的风险，这会损害肺发育、肺部和心血管功能，以及因引流淋巴内容物丢失而引发的并发症。产前干预可能会提高重症胎儿乳糜胸的存活率。新生儿治疗策略通常是支持性的，干预措施包括胸腔造口引流，并尝试采用从侵入性最小的方法开始的逐步方法来减少乳糜流量。缺乏基于证据的治疗选择，这非常需要。即使没有进行特定的淋巴系统研究以确定确切病理，大多数CC病例也会随着时间的推移而自行缓解。进行淋巴研究的专业知识并非普遍可得。需要各种治疗选择的疗效和安全性数据，以确定治疗CC的最佳方法。
