Yang Michelle J, Russell Katie W, Yoder Bradley A, Fenton Stephen J
Division of Neonatology, University of Utah School of Medicine, Salt Lake City, UT, USA.
Division of Pediatric Surgery, University of Utah School of Medicine, Salt Lake City, UT, USA.
Transl Pediatr. 2021 May;10(5):1432-1447. doi: 10.21037/tp-20-142.
The consequences of most hernias can be immediately corrected by surgical repair. However, this isn't always the case for children born with a congenital diaphragmatic hernia. The derangements in physiology encountered immediately after birth result from pulmonary hypoplasia and hypertension caused by herniation of abdominal contents into the chest early in lung development. This degree of physiologic compromise can vary from mild to severe. Postnatal management of these children remains controversial. Although heavily studied, multi-institutional randomized controlled trials are lacking to help determine what constitutes best practice. Additionally, the results of the many studies currently within the literature that have investigated differing aspect of care (i.e., inhaled nitric oxide, ventilator type, timing of repair, role of extracorporeal membrane oxygenation, etc.) are difficult to interpret due to the small numbers investigated, the varying degree of physiologic compromise, and the contrasting care that exists between institutions. The aim of this paper is to review areas of controversy in the care of these complex kids, mainly: the use of fraction of inspired oxygen, surfactant therapy, gentle ventilation, mode of ventilation, medical management of pulmonary hypertension (inhaled nitric oxide, sildenafil, milrinone, bosentan, prostaglandins), the utilization of extracorporeal membrane oxygenation, and the timing of surgical repair.
大多数疝气的后果可通过手术修复立即得到纠正。然而,对于患有先天性膈疝的患儿来说,情况并非总是如此。出生后立即出现的生理紊乱是由于在肺发育早期腹腔内容物疝入胸腔导致的肺发育不全和高血压所致。这种生理功能受损的程度可轻可重。这些患儿的产后管理仍存在争议。尽管进行了大量研究,但缺乏多机构随机对照试验来帮助确定最佳治疗方案。此外,目前文献中的许多研究调查了不同方面的护理(即吸入一氧化氮、呼吸机类型、修复时机、体外膜肺氧合的作用等),由于研究对象数量少、生理功能受损程度不同以及各机构之间存在的护理差异,其结果难以解释。本文的目的是回顾这些复杂患儿护理中的争议领域,主要包括:吸入氧分数的使用、表面活性剂治疗、轻柔通气、通气模式、肺动脉高压的药物治疗(吸入一氧化氮、西地那非、米力农、波生坦、前列腺素)、体外膜肺氧合的应用以及手术修复的时机。