S8A-13 SESSION 8A: METOPIC SYNOSTOSIS OPHTHALMOLOGIC FINDINGS IN CHILDREN WITH METOPIC SUTURE ABNORMALITIES

Abstract

Introduction: Ophthalmic abnormalities in children with syndromic craniosynostosis have been reported, and referral to an ophthalmologist is recommended. We have previously reported children with isolated metopic craniosynostosis demonstrate an increased rate of amblyopia and its risk factors. It is known that there is a wide spectrum of metopic suture abnormalities ranging from ridge to severe trigonocephaly, and that metopic suture abnormalities can be seen with deformational plagiocephaly (DP) and/or brachycephaly (B). It is not clear if milder forms of metopic abnormalities need to be referred to an ophthalmologist. The aim of this study is to report the incidence of ophthalmic abnormalities in children with isolated metopic abnormalities ranging from those with mild trigonocephaly to severe and those in conjunction with DP and/or B. Methods: An IRB–approved, retrospective review was performed on 189 children under 2 years of age diagnosed with isolated metopic abnormalities from 2007–2017. 110 children diagnosed with metopic ridge to mild/moderate trigonocephaly and DP/B were evaluated by ophthalmology and included in the study. Patients were stratified into the following categories: DP (n=28), B (n=10), DP/B (n=39), mild/moderate trigonocephaly (n=16), or severe trigonocephaly (n=17). Records were reviewed for diagnoses of amblyopia, strabismus, and refractive errors. Patients with other suture involvement, syndromic diagnoses, and primary ocular disorders were excluded. Results: Of the 110 children, 66 (60%) had no abnormal ophthalmologic findings, 13 (11.8%) had strabismus, 13 (11.8%) had amblyopia, 28 (25.5%) had astigmatism, 14 (12.7%) had hyperopia, 5 (4.5%) had myopia, and 8 (7.3%) had anisometropia. The incidence of amblyopia in the general pediatric population is 1.5% to 1.8% (Multi-ethnic Pediatric Eye Disease, 2008; McKean-Cowdin, 2013); strabismus is 2.4% to 3.6% (McKean-Cowdin, 2013); and rates of 1.2% to 4% for myopia, 13.5% to 25.7% for hyperopia, and 6.3% to 8.3% for astigmatism (Wen et al., 2013). Conclusion: In our population, children with isolated metopic abnormalities, regardless of their associated head shape, demonstrate an increased rate of amblyopia and its risk factors, with the exception of hyperopia, than the general pediatric population. The increased rate of visual abnormalities places this population at higher risk for improper visual development. Those with isolated metopic abnormalities including minor trigonocephaly or deformational head shapes are often not referred to ophthalmology, leaving a large group with undiagnosed visual issues. Thus, prompt referral to an ophthalmologist could benefit this population, as early treatment leads to improved outcomes.