Management of congenital diaphragmatic hernia.

PURPOSE OF REVIEW: To evaluate the impact of recent research on the management of congenital diaphragmatic hernia in the light of new theories on embryological development, earlier antenatal diagnosis, fetal and postnatal interventions together with advances in perinatal intensive care.

RECENT FINDINGS: The year 2007 provided in excess of 200 publications that address various aspects of congenital diaphragmatic hernia. The genetic basis and the causes of pulmonary hypoplasia at the molecular level are slowly being unravelled. Fetal MRI of lung volume, lung-head ratio, liver position and size of diaphragmatic defect have all been evaluated as early predictors of outcome and with a view to prenatal counselling. The impact of fetal interventions such as fetal endoluminal tracheal occlusion, the mode of delivery, the surgical techniques and agents for treating pulmonary hypertension were evaluated. The influence of associated anomalies and therapeutic interventions on the outcome and quality of life of survivors continue to be appraised.

SUMMARY: Deferred surgery after stabilization with gentle ventilation and reversal of pulmonary hypertension remain the cornerstones of management. Optimal presurgery and postsurgery ventilatory settings remain unproven. Continued improvement in neonatal intensive care raises the bar against which any intervention such as fetal endoluminal tracheal occlusion and extracorporeal membrane oxygenation will be judged.