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Optic Nerve Head Morphological Variation in Craniosynostosis: A Cohort Study
Sohaib R Rufai1, Mervyn G Thomas2, Oliver R Marmoy3
1From the Clinical and Academic Department of Ophthalmology (S.R.R., O.R.M., V.P., D.A.T., R.H. H., S.G., R.B.), Great Ormond Street Hospital for Children NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health, London, UK; Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health (S.R.R., G.J., D.J.D., R.H., N.u.O.J.), London, UK; The University of Leicester Ulverscroft Eye Unit, Leicester Royal Infirmary (S.R.R., M.G.T., I.G., F.A.P.), Leicester, UK.
Objective:
To evaluate optic nerve head (ONH) morphology in children with craniosynostosis versus healthy controls.
Design:
Single-center, prospective cohort study.
Methods:
Handheld optical coherence tomography (OCT) was performed in 110 eyes of 58 children (aged 0-13 years) with craniosynostosis. Inclusion criteria were as follows: normal intracranial pressure on invasive overnight monitoring, or clinically stable intracranial pressure. The latter was defined as stable VA within 1 logMAR line and no papilledema on fundoscopy for at least 4 months following OCT, and normal/stable visual evoked potentials. Control data for 218 eyes of 218 children were obtained from a published normative dataset. The main outcome measures were disc width, cup width, rim width, and retinal nerve layer thickness (nasal and temporal). Outcome measures were compared using three-way linear mixed model regression analysis (fibroblast growth factor receptor [FGFR] 1/2-associated craniosynostosis, non-FGFR 1/2-associated craniosynostosis, and controls).
Results:
Out of 63 eligible children with craniosynostosis, handheld OCT imaging was successful in 110 eyes of 58 children (92%). Of these, 22 (38%) were female. Median subject age at OCT examination was 53 months (range: 2-157; IQR: 39-73). Twelve children (21%) had FGFR1/2-associated syndromes (Crouzon, n = 6; Apert, n = 4; Pfeiffer, n = 2). Control data were available for 218 eyes of 218 healthy children. 122 controls (56%) were female. Median control age at OCT examination was 20 months (range: 0-163; IQR: 6-59). When comparing ONH morphology in craniosynostosis (n = 58) versus controls (n = 218), disc width was 6% greater (P = .001), temporal cup width was 13% smaller (P = .027), rim width was 16% greater (P < .001) and temporal retinal nerve fiber layer was 11% smaller (P = .027). When comparing FGFR1/2-associated syndromes (Crouzon, Apert, and Pfeiffer syndromes, n = 12) to the rest of the craniosynostosis group (n = 46), disc width was 10% smaller (P = .014) and temporal cup width was 38% smaller (P = .044).
Conclusions:
This cohort demonstrated morphological differences of the ONH in craniosynostosis, most markedly in Crouzon, Apert, and Pfeiffer syndromes. These findings could help improve ophthalmological monitoring and surgical decision-making in children with craniosynostosis. Further work on longitudinal ONH changes in syndromic and nonsyndromic craniosynostosis would be valuable.
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