Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair.

BACKGROUND/PURPOSE: Poor prognosis (approximately 50% survival rate and significant morbidity) traditionally has been associated with congenital diaphragmatic hernia (CDH). The authors reviewed a single institution experience and challenged conventional wisdom in the context of a care strategy based on permissive hypercapnea/spontaneous respiration/elective repair.

METHODS: From August 1992 through February 2000, all infants with CDH and (1) respiratory distress requiring mechanical ventilation, (2) in-born or (3) transferred preoperatively within hours of birth are reported. All respiratory care strategy used permissive hypercapnea/spontaneous respiration and combined with elective repair. Arterial blood gas values and concomitant ventilator support were recorded. Outcome markers were (1) need for extracorporeal membrane oxygenation ECMO, (2) discharge to home, (3) supplemental oxygen need at discharge, and (4) influence of non-ECMO ancillary therapies (surfactant, nitric oxide, high-frequency oscillatory ventilation).

RESULTS: One hundred twenty consecutive infants were reviewed. Overall survival rate was 75.8%, but, excluding 18 of 120 not treated (6 lethal anomalies, 10 overwhelming pulmonary hypoplasia, 3 prerepair ECMO-related neurocomplications), 84.4% survived to discharge. A total of 67/120 were inborn. Non-ECMO ancillary treatments had no impact on survival rate. ECMO was used in 13.3%. Surgery was transabdominal; prosthetics were used in 7%. Tube thoracostomy was rare. Every inborn patient (n = 11) requiring a chest tube for pneumothorax died. Respiratory support before surgery was peak inspiratory pressure (PIP), 22, FIO(2),.43 with PaO(2), 66 torr; PaCO(2), 41 torr; and pH, 7.32. The survivors discharged on oxygen (n = 2) died at 4 and 7 months.

CONCLUSIONS: The majority of infants with life-threatening CDH treated with permissive hypercapnea/spontaneous respiration/elective surgery survive to discharge with minimal pulmonary morbidity.