Abstract

The clinical course of patients with congenital diaphragmatic hernia (CDH) is often complicated by lung hypoplasia and pulmonary hypertension. The implementation of gentle forms of ventilation and extracorporal membrane oxygenation (ECMO) led to improved survival rates, but patients with CDH are still at risk of developing pulmonary sequelae including chronic lung disease (CLD).

We performed a retrospective analysis of all neonates with CDH treated at our institution between 2002 and 2007. CLD was defined as the need for supplemental oxygen or mechanical ventilation for at least 28 days after birth, and its severity was graded according to the respiratory support after 56 day postnatal age or discharge.

9.3% of all 259 neonates with CDH presented with severe concomitant disease or ongoing asphyxia, did not receive maximum therapy and died within the first days of life.

123 of the remaining 235 patients did not need ECMO therapy. All 123 patients survived and 44 developed CLD (35.8%).

112 patients met ECMO criteria. 69 patients survived (61%) and 61 patients developed CLD (54%).

Overall survival was 81.7%. CLD was evident in 44.7% of all patients.

Patients in the ECMO-group were significantly longer on mechanical ventilation than patients who did not require ECMO-therapy (median 27.9 d vs. 13.6 d).

Children with CDH have a significant risk of developing chronic lung disease, which is increased in children who received ECMO therapy. Follow-up studies with focus on lung function tests and strategies to prevent pulmonary infections are needed to evaluate the long term outcome for these children.