Background/purpose: Congenital diaphragmatic hernia (CDH) is a malformation requiring neonatal surgical repair with in-hospital survival rates above 90%. We examined the long-term functional impact of CDH repair on a cross-sectional cohort of survivors.
Methods: A cohort of 53 CDH families participated in this study. Functional impact was evaluated with parent report of the Functional Status IIR and the Child Health Ratings Inventories General Health Module. Parents also provided a clinical severity score, the child's medical history, and family demographic information. The primary outcome was the effect of medical morbidity on the Functional Status IIR total score.
Results: Congenital diaphragmatic hernia survivors had a median age of 8 years; 50% were in third grade or above. Sixty-six percent had major medical issues at hospital discharge, whereas 48% had current clinical problems. Functional Status IIR total score was strongly correlated with child's clinical severity (r = -0.65; P < .0001) and was lower among children with ongoing medical morbidity, denoting worse functioning (P = .01). Child Health Ratings Inventories General Health Module scores followed a similar pattern.
Conclusions: A subset of long-term CDH survivors continues to have ongoing clinical problems a median of 8 years after surgery, translating to lower functional status. Affected children and their families may benefit from prospective identification and ongoing interventions.