Optic canal stenosis in Crouzon syndrome: a case report and literature review

Bolotnikova IV, Shapovalov AS, Bazarkhandaeva TB, et al. . Optic canal stenosis in Crouzon syndrome: a case report and literature review. Burdenko's Journal of Neurosurgery. 2024;88(4):100‑106. (In Russ., In Engl.)
https://doi.org/10.17116/neiro202488041100

Подписка 2025-2 (Burdenko's Journal of Neurosurgery)

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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.

Keywords:

Crouzon syndrome

craniosynostosis

optic canal stenosis

secondary optic disc atrophy

optic disc congestion

children

Authors:

Bolotnikova I.V.

Almazov National Medical Research Center

ORCID: 0009-0002-4798-007X

Shapovalov A.S.

Almazov National Medical Research Center

ORCID: 0000-0002-2357-1653

Bazarkhandaeva T.B.

Almazov National Medical Research Center

ORCID: 0000-0002-1632-3213

Ivanov V.P.

Almazov National Medical Research Center

ORCID: 0000-0002-1009-4077

Khachatryan V.A.

Almazov National Medical Research Center

ORCID: 0000-0002-1635-6621

Gulyaev D.A.

Almazov National Medical Research Center

ORCID: 0000-0002-5509-5612

Brzhesky V.V.

St. Petersburg State Pediatric Medical University

ORCID: 0000-0001-7361-0270

Kim A.V.

Almazov National Medical Research Center

ORCID: 0000-0002-6219-7270

Received:

14.12.2023

Accepted:

31.05.2024

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