Infants with congenital diaphragmatic hernia (CDH) are at increased risk of neuroimaging abnormalities and neurodevelopmental impairment. There is no standardized neuroimaging scoring system in this patient population. Our objective was to develop a novel Neonatal Brain Injury Score (NBIS) and evaluate its association with neurodevelopmental outcomes.

We conducted a retrospective observational study using data from a quaternary single-center clinical registry. Patients with CDH born between 2013 and 2021 without a chromosomal abnormality were included. The NBIS was developed to capture neuroimaging findings seen in critically ill infants, such as patients with CDH. Neurodevelopment was assessed with the Bayley Scales of Infant and Todder Development, 3rd or 4th editions (Bayley) at a mean age of 20-21 months. Relationships between the NBIS and Bayley scores were assessed at latest follow-up visit using linear regression models and longitudinal follow-up visits using linear mixed models. Models were adjusted for age at brain magnetic resonance imaging, treatment with extracorporeal membrane oxygenation, and CDH severity.

Two hundred twenty-three infants met eligibility criteria. The majority (69%) had an abnormal NBIS (median 2 [0, 5]; range 0-29 out of 100). A higher NBIS was associated with lower Bayley scores at the latest follow-up visit and longitudinally over time.

We developed a novel NBIS that was significantly associated with neurodevelopmental outcomes in the CDH population.