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Optic Nerve Atrophy in Syndromic Craniosynostosis.
Jeffrey A Fearon1, Stephan Barrientos1, Kanlaya Ditthakasem1
1From The Craniofacial Center; Slocum-Dickson Medical Group; and the Department of Clinical Research, Medical City Dallas Hospital.
Plastic and Reconstructive Surgery
|June 7, 2022
Summary
Children with syndromic craniosynostosis, particularly Apert, Crouzon, and Pfeiffer syndromes, have a significant risk of optic nerve atrophy. Chiari malformations are a key predictor, necessitating closer ophthalmologic monitoring.
Area of Science:
- Ophthalmology
- Pediatric Neurosurgery
- Medical Genetics
Background:
- Syndromic craniosynostosis often leads to visual impairments in children.
- Elevated intracranial pressure is hypothesized to be a primary driver of vision loss.
- This review investigates the prevalence and predictors of optic nerve atrophy in syndromic craniosynostosis.
Purpose of the Study:
- To determine the prevalence of optic nerve atrophy in children with syndromic craniosynostosis.
- To identify potential predictive factors for the development of optic nerve atrophy.
- To inform strategies for preventing vision loss in this patient population.
Main Methods:
- Retrospective chart review of patients with syndromic craniosynostosis.
- Analysis of ophthalmologic records for 253 patients.
- Statistical analysis to identify correlations between clinical factors and optic nerve atrophy.
Main Results:
- Optic nerve atrophy prevalence varied by syndrome: Apert (7.8%), Crouzon (27.9%), Pfeiffer (23.1%).
- No atrophy was observed in Saethre-Chotzen or Muenke syndromes.
- Chiari malformation significantly correlated with optic nerve atrophy (OR, 3.544; p = 0.002).
Conclusions:
- Apert, Crouzon, and Pfeiffer syndromes show substantial optic nerve atrophy rates.
- Chiari malformations are the sole significant predictor identified.
- Increased ophthalmologic monitoring and imaging are recommended to prevent visual loss.
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