Modern management of congenital diaphragmatic hernia

V O L U M E 7 I S S U E 3 2 0 1 1 infant Congenital diaphragmatic hernia (DH) is a life-threatening malformation (incidence 1 in 2,000-5,000 live births). The diaphragmatic defect (80% are leftsided) is of varying size through which abdominal viscera including liver, spleen, stomach and intestine can herniate into the chest from about the tenth week of gestation. Lung growth and development are impaired through limitation of space, reduced fetal breathing movements and loss of fetal lung liquid, leading to variable degrees of pulmonary hypoplasia. The combination of pulmonary hypoplasia and pulmonary hypertension (PHT) is the main determinant of postnatal survival. Over the last two decades there has been an improvement in survival, predominantly through advances in neonatal care with ventilation strategies to avoid lung injury, and targeted therapy for pulmonary hypertension. The following article outlines the strategies used in Glasgow for the management of infants born with DH at the severe end of the clinical spectrum. Presentation and indicators of severity