Severe Congenital Diaphragmatic Hernia (CDH): A critical analysis of eight years' experience

Objectives: 1) To define the best outcome of severe Congenital Diaphragmatic Hernia (CDH); 2) to critically evaluate deaths in order to identify possible criteria of exclusion from ECMO; and 3) to identify CDHs which could benefit from ECMO. Materials and Methods: 63 severe CDHs, 35 (55.6%) survivors and 28 (44.4%) nonsurvivors, subdivided into 2 groups according to age at death: Group I dying at 12 ≤ 24 hours, and Group II dying at > 24 hours after birth. The three groups were compared on the basis of prenatal diagnosis, polyhydramnios, gestational age, birth weight, pneumothorax, best values of postductal PaCO₂ and PaO₂, clinical and echocardiographic signs of persistent pulmonary hypertension, and severity of pulmonary hypoplasia (i.e., body weight to bilateral lung weight ratio at autopsy). Results: PaCO₂, PaO₂ and degree of pulmonary hypoplasia were significantly worse in Group I compared to Group II and to survivors. PaCO₂ and PaO₂ in Group II did not differ significantly from those of survivors. Conclusions: In severe CDH it is possible: 1) to achieve a survival rate of 56% without ECMO; 2) to identify a group of patients (Group 1 = 27%) with severe pulmonary hypoplasia who would probably die even with ECMO; and 3) to identify a group of patients (Group II = 17%) who might benefit from ECMO treatment.