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

Q4 Medicine

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko Pub Date : 2024-01-01 DOI:10.17116/neiro202488041100

I V Bolotnikova, A S Shapovalov, T B Bazarkhandaeva, V P Ivanov, V A Khachatryan, D A Gulyaev, V V Brzhesky, A V Kim

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引用次数: 0

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.

查看原文本刊更多论文

[克鲁宗综合征视神经管狭窄：病例报告和文献综述]。

背景：克鲁宗综合征的发病率为每 2.5 万至 3.1 万名新生儿中 1 例。该综合征极少伴有视神经管狭窄：介绍一名患有克鲁宗综合征和视神经管狭窄的患者，并根据患者自身情况和文献资料讨论此类患者的治疗方法：一名 6 岁男孩患有克鲁宗综合征（经分子遗传学研究证实，即 FGFR2 基因突变）。患者曾因颅骨发育不良和脑积水接受过 3 次手术。尽管如此，尽管脑室腹腔分流管通畅，患者的视力仍逐渐下降。检查发现，患者视功能严重下降，视盘充血，继发性萎缩。核磁共振成像数据显示，蛛网膜下腔CSF积聚在双侧视神经上，这可能表明视神经管狭窄。三维 CT 证实了这一推测：结果：患者接受了双侧视神经管减压术，随后视功能得到改善：结论：克鲁宗综合征后视力下降可能是视神经管狭窄所致。结论：克鲁宗综合征后视力下降可能是由于视神经管狭窄所致，减压术即使在长期病程中也可能有效，并能改善视功能。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko Medicine-Neurology (clinical)

CiteScore

0.70

自引率

0.00%

发文量

期刊介绍： Scientific and practical peer-reviewed journal. This publication covers the theoretical, practical and organizational problems of modern neurosurgery, the latest advances in the treatment of various diseases of the central and peripheral nervous system. Founded in 1937. English version of the journal translates from Russian version since #1/2013.