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.

Scientific Reports
|March 18, 2021
PubMed

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.

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