Abstract

Introduction: Little has been described in the literature regarding ophthalmic abnormalities in non-syndromic midline craniosynostosis. The few studies on non-syndromic trigonocephaly and ocular abnormalities have shown that non-syndromic trigonocephaly is associated with a greater risk of abnormal visual functioning in young children, up to the age of 8 years. However, visual functioning in children after that age, when optic development is complete, hasn’t been studied yet. The purpose of this study is therefore to assess the prevalence of visual abnormalities in children with non-syndromic trigonocephaly between the age of 8 to 18 years. Methods: A retrospective, cross-sectional study of patients between the age of 8 and 18 years, with non-syndromic trigonocephaly was conducted. Ophthalmologic records were assessed for the following parameters: refractive errors, astigmatism, strabismus, and a history of amblyopia. Refractive error was determined using autorefraction or skiascopy after use of cycloplegia. Myopia was classified as spherical equivalent (SE) < -0.50 dioptre (D), emmetropia as SE between -0.50 D and +0.50 D, mild hyperopia as SE between +0.50 D and +2.00 D, moderate hyperopia as SE between +2.00 D and +6.00 D, and high hyperopia as SE > +6.00 D. Astigmatism was defined as > 1.00 D. Strabismus was subdivided in manifest and latent strabismus. Presence of strabismus and amblyopia was determined using the cover test, binocular sensory testing, and visual acuity. Results: We included 83 patients with non-syndromic trigonocephaly, with a mean age of 10.5 years (SD 2.0). Thirty-three of these 83 patients had abnormal visual functioning (39.7%). Of all 83 patients, 23 had hyperopia (27.7%), 7 had myopia (8.4%), 19 had astigmatism (22.9%), 7 had amblyopia (8.3%), and 12 had strabismus (14.3%). Manifest strabismus was present in 7 patients (8.3%), while latent strabismus was present in 5 patients (6.0%). Conclusion: Prevalence of hyperopia, astigmatism, amblyopia, and strabismus was higher in patients with non-syndromic trigonocephaly, compared to the prevalence found in the healthy paediatric population. Therefore, long-term surveillance of visual functioning of paediatric patients with non-syndromic trigonocephaly by an ophthalmologist is recommended.

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