Rafat Neysan, Schaible Thomas
Department of Neonatology, University Children's Hospital Mannheim, University of Heidelberg, Mannheim, Germany.
Front Pediatr. 2019 Aug 8;7:336. doi: 10.3389/fped.2019.00336. eCollection 2019.
Congenital diaphragmatic hernia (CDH) is characterized by failure of diaphragmatic development with lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). If conventional treatment with gentle ventilation and optimized vasoactive medication fails, extracorporeal membrane oxygenation (ECMO) may be considered. The benefits of ECMO in CDH are still controversial, since there are only few randomized trials demonstrating the advantages of this therapeutic option. At present, there is no precise prenatal and/or early postnatal prognostication parameter to predict reversibility of PPHN in CDH patients. Indications for initiating ECMO include either respiratory or circulatory parameters, which are also undergoing continuous refinement. Centers with higher case numbers and the availability of ECMO published promising survival rates, but data on long-term results, including morbidity and quality of life, are rare. Survival might be influenced by the timing of ECMO initiation and the timing of surgical repair. In this regard a trend toward early initiation of ECMO and early surgery on ECMO exists. The results concerning the cannulation modes are similar and a consensus on time limit for ECMO runs does not exist. The use of ECMO in CDH will continue to be evaluated, and prospective randomized trials and registry network are necessary to help answering the addressed questions of patient selection and management.
先天性膈疝(CDH)的特征是膈肌发育不全,并伴有肺发育不全和新生儿持续性肺动脉高压(PPHN)。如果采用温和通气和优化血管活性药物的传统治疗方法失败,则可考虑使用体外膜肺氧合(ECMO)。ECMO在CDH治疗中的益处仍存在争议,因为仅有少数随机试验证明了这种治疗选择的优势。目前,尚无精确的产前和/或产后早期预后参数来预测CDH患者PPHN的可逆性。启动ECMO的指征包括呼吸或循环参数,这些参数也在不断完善。病例数较多且具备ECMO设备的中心公布了可观的生存率,但关于长期结果的数据,包括发病率和生活质量,却很少见。生存率可能受ECMO启动时间和手术修复时间的影响。在这方面,存在着尽早启动ECMO和尽早在ECMO支持下进行手术的趋势。关于插管模式的结果相似,并且对于ECMO治疗的时间限制也没有共识。CDH患者使用ECMO仍将继续接受评估,需要开展前瞻性随机试验和注册网络,以帮助解答有关患者选择和管理的问题。