Abstract
Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm, occurring in approximately 1 in 3,000 live births with a high mortality rate of between 30 and 50%. This narrative review provides an update on antenatal and postnatal management strategies and current outcomes emphasizing where further research is required. Fetal endotracheal obstruction (FETO) has been demonstrated in a large RCT to improve survival in the most severe CDH and is now adopted into clinical practice. Other antenatal interventions such as those aimed at reducing pulmonary hypertension require further study. The results of respiratory function monitoring in the delivery suite have changed practice with avoidance of routinely administering neuromuscular blocking agents, as well as providing predictors of poor outcomes. The proportion of CDH infants benefiting from spontaneously breathing from birth remains to be robustly determined. The delicate lungs of CDH infants require gentile ventilation avoiding volutrauma, conventional ventilation with volume targeting may be the most appropriate option. Although neurally-adjusted ventilatory assist (NAVA) looks promising, it needs further testing. Extracorporeal membrane oxygenation (ECMO) should be offered for infants with severe CDH with a realistic likelihood of a positive outcome; further research is required to identify accurate predictors. Pulmonary vasodilators are commonly used in CDH infants, but supportive evidence is limited. Inhaled nitric oxide (iNO) should be trialled only in the absence of left ventricular systolic dysfunction and stopped if there are no echocardiographic findings of improvement within 24 hours. Milrinone should be used to treat cardiac dysfunction if associated with PH and Sildenafil should be considered in refractory PH or as an adjunct when weaning iNO, but for all such pulmonary vasodilators the evidence needs strengthening. CDH survivors can suffer long term morbidity including chronic lung disease, exertional dyspnea, gastroesophageal reflux disease (GERD), and scoliosis. Well-structured long-term follow-up programs are required to identify morbidities.
Keywords
Pulmonary hypoplasia, Pulmonary hypertension, FETO, Mechanical ventilation, ECMO, Pulmonary vasodilators, Inhaled nitric oxide