Antenatal and postnatal lung and vascular anatomic and functional studies in congenital diaphragmatic hernia: implications for clinical management.

Congenital diaphragmatic hernia is characterized by fetal and neonatal lung hypoplasia as well as vascular hypoplasia. Antenatal imaging studies have been performed that attempt to quantify the degree of hypoplasia and its impact on infant prognosis. Prenatal and perinatal growth of the lung and vasculature are interdependent and their continued coordinated growth is critical for survival after birth in this patient population. Lung protection strategies appear to improve survival in newborns with diaphragmatic hernia, but a subset of infants remain who demonstrate sufficiently severe lung hypoplasia that we are unable to provide support long-term after birth. Fetal intervention is a strategy designed to enhance fetal lung growth towards improving survival in this most severely affected group, though other therapies to enhance postnatal lung and vascular growth should be concurrently investigated. However, any of these interventions will require careful selection of those infants at risk for poor outcome and thorough follow up, since long-term morbidity is significant in children with diaphragmatic hernia.