Pulmonary surfactant dysfunction in congenital diaphragmatic hernia: experimental and clinical findings

Experimental and clinical findings indicate immaturity of pulmonary surfactant in congenital diaphragmatic hernia (CDH). Lung histology has shown a decreased amount of lamellar bodies. A low lecithin/sphingomyelin ratio in the amniotic fluid, and decreased concentrations of surfactant protein A and disaturated phosphatidylcholine in the pulmonary tissue and the amniotic fluid have been reported. Furthermore, low compliance and high surface tension have also been found. Evidence of clinical and experimental findings of structural, biochemical and functional pulmonary immaturity in CDH is reviewed. Prenatal administration of corticosteroids to accelerate fetal pulmonary maturation, and the use of early surfactant therapy, should be further evaluated in the clinical management of CDH.