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 Журнал «Вопросы нейрохирургии» имени Н.Н. Бурденко (Burdenko's Journal of Neurosurgery)

Использование инфографики авторами научных работ

Close metadata

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

Brzheskiy V.V.

Saint 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

Close metadata

References:

Gray TL, Casey T, Selva D, Anderson PJ, David DJ. Ophthalmic sequelae of Crouzon syndrome. Ophthalmology. 2005;112(6):1129-1134. https://doi.org/10.1016/j.ophtha.2004.12.037
Kirman CN, Tran B, Sanger C, Railean S, Glazier SS, David LR. Difficulties of delayed treatment of craniosynostosis in a patient with Crouzon, increased intracranial pressure, and papilledema. The Journal of Craniofacial Surgery. 2011;22(4):14091412. https://doi.org/10.1097/SCS.0b013e31821cc50c
Simonin A, Maduri R, Viaroli E, Levivier M, Daniel RT, Messerer M. Correlation between Papilledema and Intracranial Hypertension in Crouzon Syndrome: A Case Report and Review of the Literature. Pediatric Neurosurgery. 2019;54(4):223-227. https://doi.org/10.1159/000500767
Pal US, Gupta C, Chellappa AA. Crouzon syndrome with primary optic nerve atrophy and normal brain functions: A case report. Journal of Oral Biology and Craniofacial Research. 2012;2(2):116-118. https://doi.org/10.1016/j.jobcr.2012.03.011
Hariri F, Farhana NA, Abdullah NA, Ibrahim N, Ramli NM, Mohd Abdullah AA, May CM, Khaliddin N. Optic canal characteristics in pediatric syndromic craniosynostosis. Journal of Cranio-Maxillo-Facial Surgery. 2021;49(12):1175-1181. https://doi.org/10.1016/j.jcms.2021.06.017
Koltunov DE, Bel’chenko VA. Harakteristika skeletnyh deformacij u pacientov s sindromami Aperta, Kruzona, Pfajffera. Voprosy prakticheskoj pediatrii. 2012;7(6):57-62. (In Russ.).
Amlang CJ, Nouh A, Anderson DE, Biller J. Neurological complications in Pfeiffer syndrome. Journal of the Neurological Sciences. 2015;357(2015):e193. https://doi.org/10.1016/j.jns.2015.08.669
Fries PD, Katowitz JA. Congenital craniofacial anomalies of ophthalmic importance. Survey of Ophthalmology. 1990;35(2):87-119. https://doi.org/10.1016/0039-6257(90)90067-6
Liasis A, Nischal KK, Walters B, Thompson D, Hardy S, Towell A, Dunaway D, Jones B, Evans R, Hayward R. Monitoring visual function in children with syndromic craniosynostosis: a comparison of 3 methods. Archives of ophthalmology. 2006;124(8):111926. https://doi.org/10.1001/archopht.124.8.1119
Hariri F, Farhana NA, Abdullah NA, Ibrahim N, Ramli NM, Mohd Abdullah AA, May CM, Khaliddin N. Optic canal characteristics in pediatric syndromic craniosynostosis. Journal of Cranio-Maxillofacial Surgery. 2021;49(12):1175-1181. https://doi.org/10.1016/j.jcms.2021.06.017
Salyer KE. A passion for excellence. Journal of Craniofacial Surgery. 2009;20(suppl 2):1632-1634. https://doi.org/10.1097/SCS.0b013e3181b1c0d5
Kadyshev VV, Ginter EK, Kucev SI, Oganezova ZhG, Zinchenko RA. Epidemiology of hereditary diseases of the organ of vision in populations of the Russian Federation. RMJ. Clinical ophthalmology. 2022;22(2):69-79. (In Russ.). https://doi.org/10.32364/2311-7729-2022-22-2-69-79
Koltunov DE. Crouzon syndrome: etiology and clinical manifestations. Questions of practical pediatrics. 2011;6(5):49-52. (In Russ.).
Kurenkova NV, Zol’nikova IV, Klitvina GA, Demenkova ON. Visual evoked cortical potentials in children with craniosynostosis. Description of cases and analysis of literature data. Russian pediatric ophthalmology. 2017;12(3):170-176. (In Russ.).
Verma R, Gupta M, Chaudhari TS. Neurogenic vision loss: Causes and outcome. An experience from a tertiary center in Northern India. Journal of Neurosciences in Rural Practice. 2014;5(4):340-348. https://doi.org/10.4103/0976-3147.139971
Zhang X, Lee Y, Olson D, Fleischman D. Evaluation of optic canal anatomy and symmetry using CT. BMJ Open Ophthalmology. 2019;4(1):e000302. https://doi.org/10.1136/bmjophth-2019-000302
Fearon JA, Barrientos S, Ditthakasem K, Herbert M. Optic Nerve Atrophy in Syndromic Craniosynostosis. Plastic and reconstructive surgery. 2022;150(2):381e-386e. https://doi.org/10.1097/PRS.0000000000009367
Ivanov VP, Kim AV, Hachatrjan VP. 3D-pechat’ v kraniofacial’noj hirurgii. Opyt FGBU «NMIC im. V.A. Almazova». Nejrohirurgija i nevrologija detskogo vozrasta. 2018;3:28-39. (In Russ.).
Bulters DO, Shenouda E, Evans BT, Mathad N, Lang DA. Visual recovery following optic nerve decompression for chronic compressive neuropathy. Acta Neurochirurgica. 2009;151(4):325-334. https://doi.org/10.1007/s00701-009-0192-x
Di Somma A, Andaluz N, Gogela SL, Cavallo LM, Keller JT, Prats-Galino A, Cappabianca P. Surgical Freedom Evaluation During Optic Nerve Decompression: Laboratory Investigation. World Neurosurgery. 2017;101:227-235. https://doi.org/10.1016/j.wneu.2017.01.117
Hart CK, Theodosopoulos PV, Zimmer LA. Anatomy of the optic canal: a computed tomography study of endoscopic nerve decompression. Annals of Otology, Rhinology & Laryngology. 2009;118(12):83944. https://doi.org/10.1177/000348940911801203
Bartels MC, Vaandrager JM, de Jong TH, Simonsz HJ. Visual loss in syndromic craniosynostosis with papilledema but without other symptoms of intracranial hypertension. Journal of Craniofacial Surgery. 2004;15(6):1019-1022; discussion 1023-1024. https://doi.org/10.1097/00001665-200411000-00026
Barkatullah AF, Leishangthem L, Moss HE. MRI findings as markers of idiopathic intracranial hypertension. Current Opinion in Neurology. 2021;34(1):75-83. https://doi.org/10.1097/WCO.0000000000000885
Bidot S, Clough L, Saindane AM, Newman NJ, Biousse V, Bruce BB. The Optic Canal Size Is Associated With the Severity of Papilledema and Poor Visual Function in Idiopathic Intracranial Hypertension. Journal of Neuroophthalmology. 2016;36(2):120-125. https://doi.org/10.1097/WNO.0000000000000318
Sleep TJ, Hodgkins PR, Honeybul S, Neil-Dwyer G, Lang D, Evans B. Visual function following neurosurgical optic nerve decompression for compressive optic neuropathy. Eye. 2003;17(5):571-478. https://doi.org/10.1038/sj.eye.6700439