Congenital Diaphragmatic Hernia

Abstract

Despite advances in neonatal and surgical care, the management of congenital diaphragmatic hernia (CDH) remains challenging with no deinitive standard treatment guidelines. Several centers report mortality rates as low as 20%, but if extracorporeal membrane oxygenation (ECMO) support is required, the mortality rate rises to 50%. The disease severity is related to the degree of pulmonary hypoplasia and pulmonary hypertension that occurs with CDH. Both conditions decrease the infant's ability to ventilate and oxygenate adequately at delivery. These physiologic conditions that impair gas exchange are the important determinants of morbidity and mortality in CDH infants. Presently, delivery of infants with CDH is recommended close to term gestation. The focus of care includes gentle ventilation, hemodynamic monitoring, and treatment of pulmonary hypertension followed by surgery for the defect. Extracorporeal membrane oxygenation (ECMO) is considered after failure of conventional medical management for infants ≥ 34 weeks' gestation or with weight >2 kg and no associated major lethal anomalies. This chapter discusses long-term follow-up recommendations for survivors, which should involve a multidisciplinary approach, as there are many surgical and nonsurgical consequences to the disease process. Clinical strategies that address these multifaceted aspects of care, from prenatal to long-term follow-up, may further reduce the high mortality rate for these infants.