AI Article Synopsis
- Crouzon syndrome, which affects 1 in 25,000-31,000 newborns, is rarely linked with optic canal stenosis, a condition that can affect vision.
- A 6-year-old boy with Crouzon syndrome underwent multiple surgeries but experienced worsening vision, leading to the discovery of optic canal stenosis via MRI and confirmed by 3D CT.
- After surgical decompression of the optic canals, the patient's vision improved, suggesting that addressing optic canal stenosis can be beneficial even after prolonged symptoms.
Article Abstract
Background: Incidence of Crouzon syndrome is 1 per 25.000-31.000 newborns. This syndrome is extremely rarely accompanied by optic canal stenosis.
Objective: To present a patient with Crouzon syndrome and optic canal stenosis, to discuss the management of such patients considering own and literature data.
Material And Methods: A 6-year-old boy presented with Crouzon syndrome (verified by molecular genetic research, i.e. FGFR2 gene mutation). The patient underwent 3 surgeries for craniosynostosis and hydrocephalus. Nevertheless, visual acuity progressively decreased despite patent ventriculoperitoneal shunt. Examination revealed severe decrease in visual functions with optic disc congestion under secondary atrophy. MRI data on subarachnoid CSF accumulation over both optic nerves potentially indicated optic canal stenosis. This assumption was confirmed by 3D CT.
Results: The patient underwent decompression of both optic canals with subsequent improvement of visual functions.
Conclusion: Vision decrease following Crouzon syndrome may be due to optic canal stenosis. Decompression may be effective, even in long-term course of disease, and improve visual functions.
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| http://dx.doi.org/10.17116/neiro202488041100 | DOI Listing |
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