Congenital diaphragmatic hernia (CDH) in the newborn poses challenges to the multi-disciplinary teams involved in its management. Mortality remains significantly high, despite growing understanding and treatment options. Early intubation of antenatally diagnosed cases is crucial in preventing deterioration and persistent pulmonary hypertension. Early recognition of cases not diagnosed on antenatal scan, with appreciation of differential diagnosis, requires an index of suspicion and imaging. Increasing options and modalities are available, with only modest, if any, survival advantage. Permissive hypercapnea and minimal ventilation have made the most significant impact on survival in modern era. High-frequency oscillatory ventilation (HFOV), inhaled nitric oxide (iNO), treatment of pulmonary hypertension, and ECMO are used in a somewhat stepwise manner for stabilisation. Delayed surgery has become established later in management plan. The impact of individual therapies (e.g. HFOV, iNO, ECMO) on outcome is difficult to ascertain. Little level 1 or 2 evidence exists. Randomised studies and reviews on the role of ECMO have not yet proven any long-term survival benefit. One pilot randomised study of thoracoscopic repair suggests increased acidosis; intraoperative blood gases and CO2 levels should be closely monitored. Monitoring tissue oxygenation should be considered. There is no evidence to suggest the best patch material.
Keywords: Congenital diaphragmatic hernia; Hypercapnea; Hypoxia; Pulmonary hypertension; Surgery; Thoracoscopy.