Congenital Diaphragmatic Hernia: NICU and Perioperative Management

Congenital diaphragmatic hernia, commonly on the left side, is a major birth anomaly that results in lung hypoplasia (often bilateral) and hypoxic respiratory failure. Fetal MRI aids in estimating prognosis which aids multidisciplinary discussion with the family to guide postnatal management as well as crucial for ongoing trials of fetal tracheal occlusion therapy. Most babies need immediate tertiary level intensive care and a multi-disciplinary team approach for optimizing outcomes. Advances in postnatal care include gentle ventilation, ameliorating pulmonary hypertension, optimizing hemodynamics and parenteral nutrition support. Extracorporeal life support is often used as a bridge for sicker babies. Surgical repair via laparoscopic or open approach is undertaken after initial stabilization and needs experienced surgical and anesthetic care especially for those with associated cardiac pathology. Optimizing analgesia and sedation during pre-/intra- and post-op care is essential especially for infants needing prolonged intubation. Complex subspecialty follow-up as well as neurodevelopmental monitoring aids in improving quality of life after hospital discharge.