Current therapy of infants with congenital diaphragmatic hernia

Abstract

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a significant mortality rate. Despite widespread prenatal diagnosis, few parameters have been well defined to aid in prediction of outcome of these infants. Antenatal maternal steroid administration and foetal surgery are not proven interventions. Postnatal treatment has changed over the last 10 years, with avoidance of hyperventilation and ventilator-induced lung injury resulting in improved survival. Therapies such as inhaled nitric oxide, exogenous surfactant administration and extracorporeal membrane oxygenation (ECMO) have undergone limited study, but show no clear benefit in this population. With improved outcome, principally due to avoidance of barotrauma, greater opportunity exists for long-term evaluation of survivors. To date, continuing problems with pulmonary function, nutrition and growth, effects of right ventricular hypertension and developmental issues have been identified. Through co-ordinated, multidisciplinary evaluation of CDH survivors, improved long-term outcome for these challenging patients can be attained.