Radiographic Severity of Metopic Craniosynostosis Correlates with Long-Term Neurocognitive Outcomes.

Background: Reports of neurodevelopmental delays in adolescents with metopic craniosynostosis have ranged from 15 to 61 percent. Previously, event-related potentials have correlated preoperative radiographic severity with language deficiencies in infancy. This study sought to characterize neurocognitive testing at cranial maturity and correlate outcomes to preoperative radiographic severity.

Methods: Patients diagnosed with metopic craniosynostosis who underwent surgical correction in infancy completed a neurodevelopmental battery evaluating age-normalized intelligence quotient, academic achievement, and visuomotor integration. Data were stratified by preoperative endocranial bifrontal angle (moderate, >124 degrees; severe, <124 degrees). Multiple variable regression was used to control measured intelligence and achievement for age at surgery, age at testing, parental education, and income. Significance was set at p < 0.05.

Results: Twenty patients completed neurodevelopmental testing. Mean intelligence quotient was 111.7 ± 13 and academic achievement was similar to national averages (word reading, 53.4 percent; reading comprehension, 53.4 percent; reading composite, 53.5 percent; spelling, 44 percent; and math, 52.9 percent). Radiographic measurements revealed 36 percent of patients with moderate phenotype and 64 percent with severe. Patients with severe phenotypes had lower intelligence quotient measures and scored more poorly in every academic measure tested. Word reading (113 versus 95; p = 0.035) and reading composite (109 versus 98; p = 0.014) reached significance.

Conclusions: Overall, cranial mature patients with metopic craniosynostosis had above average intelligence quotient and academic achievement near the national mean. Long-term neurocognitive function was correlated to preoperative radiographic severity in metopic craniosynostosis, with more severe cases performing worse.

Clinical Question/level Of Evidence: Risk, II.