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Published on: September 8, 2023
Ocular biometric features of pediatric patients with fibroblast growth factor receptor-related syndromic
Byung Joo Lee1, Kihwang Lee2, Seung Ah Chung3
1Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Korea.
Insights
Children with fibroblast growth factor receptor (FGFR)-related craniosynostosis have distinct ocular biometric features, including longer axial length and lower corneal/lens power, compared to other groups. These parameters did not correlate with age, suggesting abnormal refractive growth in this population.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Background:
- Ametropia is a frequent ophthalmic issue in craniosynostosis.
- Fibroblast growth factor receptor (FGFR) gene mutations are linked to syndromic craniosynostosis.
- Understanding ocular development in these conditions is crucial for patient management.
Purpose of the Study:
- To compare refractive error and ocular biometric parameters in children with FGFR-related syndromic craniosynostosis versus non-syndromic craniosynostosis and controls.
- To investigate the relationship between age and ocular biometric parameters in these groups.
Main Methods:
- Retrospective analysis of 36 eyes (18 patients) with FGFR-related syndromic craniosynostosis.
- Comparison with 76 eyes (38 patients) with non-syndromic craniosynostosis and 114 eyes (57) of intermittent exotropes.
- Ocular biometric measurements included axial length, corneal power (keratometry), and lens power.
Main Results:
- No significant differences in mean age or refractive error between groups.
- Syndromic craniosynostosis patients exhibited significantly longer axial length, lower corneal power, and lower lens power (p < 0.01).
- Age-related correlations for axial length, keratometry, and lens power were observed in non-syndromic and control groups, but not in the FGFR-syndromic group.
Conclusions:
- Ocular biometric parameters in FGFR-related syndromic craniosynostosis differ significantly from non-syndromic craniosynostosis and controls.
- The lack of age-related changes in ocular parameters suggests abnormal refractive development in FGFR-related craniosynostosis.
- These findings highlight unique ocular growth patterns in FGFR-related craniosynostosis.
Abstract:
Ametropia is reported as a common ophthalmic manifestation in craniosynostosis. We retrospectively compared childhood refractive error and ocular biometric features of fibroblast growth factor receptor (FGFR)-related syndromic craniosynostosis patients with those of non-syndromic craniosynostosis and control subjects. Thirty-six eyes (18 patients) with FGFR-related syndromic craniosynostosis, 76 eyes (38 patients) with non-syndromic craniosynostosis, and 114 eyes (57 patients) of intermittent exotropes were included in the analysis. Mean age at examination was 7.82 ± 2.51 (range, 4-16) years and mean spherical equivalent was -0.09 ± 1.46 Diopter. Mean age and refractive error were not different between groups, but syndromic craniosynostosis patients had significantly longer axial length, lower corneal power, and lower lens power than other groups (p < 0.01, p < 0.01, and p < 0.01, respectively). Axial length was positively correlated and keratometry and lens power were negatively correlated with age in non-syndromic craniosynostosis and controls, while these correlations between age and ocular biometric parameters were not present in the FGFR-related syndromic craniosynostosis. In conclusion, ocular biometric parameters in FGFR-related syndromic craniosynostosis differed from those of non-syndromic craniosynostosis and age-matched controls, and did not show the relations with age, suggesting this cohort may have abnormal refractive growth.
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