Congenital diaphragmatic hernia: a narrative review of controversies in neonatal management

Abstract

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.

Keywords: Hernias; congenital; diaphragmatic; medicine; surgery; treatment.

Figure 1

CDHSG staging system. This figure was published in Journal of Pediatric Surgery, Vol 48, Lally KP, Lasky RE, Lally PA et al., Standardized reporting for congenital diaphragmatic hernia--an international consensus, pages 2408-2415, Copyright Elsevier (2013) (2). Right to reproduce obtained by Elsevier.