Treatment and outcome of congenital diaphragmatic hernia

Background and purpose: Congenital diaphragmatic hernia (CDH) is a challeng ing condition and is associated with a high mortality rate; optimal therapy remains unclear. This retrospective study describes the clinical character istics of treatment and outcome in 48 infants with CDH. Methods: Twenty-eight mate (58%) and 20 female (42%) infants with CDH were treated from 1987 through 1998. The goals of the ventilator strategy were p ermissive hypercapnea (PaCO2 less than or equal to 55 mm Hg) and avoidance of hyperventilation. Infants were initially ventilated with an intermittent mandatory rate of 40 to 60 per minute, peak inspiratory pressure of 20 to 25 cm H2O, and positive end-expiratory pressure of 5 cm H2O. High-frequency positive hypercapnea (PaCO2 > 60 mm Hg) occurred. Most infants underwent r epair after 3 days of age and only four infants underwent early repair with in 2-1 hours of birth. A prophylactic chest tube was placed in the ipsilate ral hemithorax postoperatively in all patients treated before 1996. The sev erity of respiratory distress was estimated by alveolar-arterial oxygen dif ference, oxygenation index, and alveolar-arterial ratio. Results: Forty-six patients presented with Bochdalek CDH, and two with Morg angni CDH. Antenatal diagnosis was made in 10 cases. Respiratory distress w as the major manifestation and usually occurred immediately after birth. Si s cases were diagnosed several months after birth and presented mainly with gastrointestinal symptoms. Eleven patients died before surgery and 37 pati ents underwent surgical repair. Two infants died postoperatively because of congestive heart failure and tension pneumothorax, respectively. The overa ll mortality rate was 27%. The major causes of mortality were severe respir atory failure, persistent pulmonary hypertension, pneumothorax, and associa ted anomalies. Conclusion: Nearly 75% of patients in this series survived. This suggests t hat noninvasive respiratory care combined with delayed surgery may be an ac ceptable strategy for the treatment of CDW, and can be used in most medical institutions without equipment for extracorporeal membrane oxygenation the rapy.