Background/purpose: The high mortality rate in congenital diaphragmatic hernia (CDH) has been ascribed to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). One of the principal treatment strategies has been the use of hyperventilation to reverse ductal shunting, but the wisdom of this approach is being questioned because of parenchymal lung injury from high inflation pressures. The authors hypothesize that the use of hyperventilation to reverse or prevent ductal shunting would result in ventilator-induced lung injury, which would be evident on postmortem examination. A retrospective review of clinical and autopsy information was conducted.
Methods: Clinical and autopsy information gathered for a previously published series of 223 infants with CDH presenting in the first 24 hours of life was reviewed. Autopsy and clinical data were analyzed from 68 of 101 nonsurvivors who died with severe hypoxemia.
Results: Sixty-two of 68 cases (91%) had evidence of diffuse alveolar damage and hyaline membrane formation, which was more evident in the ipsilateral lung. Forty-four (65%) infants had pneumothoraces, and 4 infants had interstitial fibrosis. Pulmonary hemorrhage was seen in 35 cases (50 maximum peak inspiratory pressure [mean +/- SD] was 40.4+/-7.9 cm H2O and lowest modified ventilatory index [respiratory rate x peak airway pressure] was 2323+/-836). The degree of pulmonary hypoplasia was evaluated by lung weight with the ratio of the observed combined lung weight to the expected lung weight based on birth weight and gestational age. The ratio based on birth weight was 57%+/-25%, and the ratio based on gestational age was 60%+/-26%. Twenty-one infants (35%) had nonpulmonary anomalies. The most significant was a 10% incidence of congenital heart disease. Apart from this, lethal nonpulmonary anomalies were rare.
Conclusion: These results suggest that lung injury secondary to mechanical ventilation plays an important role in the mortality rate of patients with CDH, which may become increasingly significant when there is underlying pulmonary hypoplasia.