Background:
Elevated intracranial pressure (ICP) in sagittal craniosynostosis has a wide spectrum of reported incidence, and patterns are not well understood across infancy and childhood. Characterizing the natural history of ICP in this population may clarify risks for neurocognitive delay and inform treatment decisions.
Methods:
Infants and children with sagittal craniosynostosis and unaffected control subjects were prospectively evaluated with spectral-domain optical coherence tomography from 2014 to 2021. Elevated ICP was determined based on previously validated algorithms using retinal optical coherence tomography parameters.
Results:
Seventy-two patients with isolated sagittal craniosynostosis and 25 control subjects were evaluated. Overall, 31.9% (n = 23) of patients with sagittal craniosynostosis had evidence of ICP greater than or equal to 15 mmHg, and 27.8% (n = 20) of patients had ICP greater than or equal to 20 mmHg. Children with sagittal craniosynostosis younger than 6 months were more likely to have normal ICP (88.6% <15 mmHg; 91.4% <20 mmHg) than those aged between 6 and 12 months (54.5%, P = 0.013; 54.5%, P = 0.005) than those older than 12 months (46.2%, P < 0.001; 53.8%, P = 0.001). ICP was directly correlated with severity of scaphocephaly (P = 0.009). No unaffected control subjects at any age exhibited retinal thickening suggestive of elevated ICP.
Conclusion:
Elevated ICP is rare in isolated sagittal craniosynostosis younger than 6 months, but it becomes significantly more common after 6 months of age, and may correlate with severity of scaphocephaly.
CLINICAL QUESTION/LEVEL OF EVIDENCE:
Risk, II.