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Diagnostic value of neuro-ophthalmological signs in cases of Chiari I malformation

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Abstract

Introduction

Our purpose was to evaluate the diagnostic value of measuring diameters of optic nerve sheath (ONSD), presence/absence of papilledema, tortuosity of the optic nerve, flattening of the posterior sclera, and intraocular protrusion of the prelaminar optic nerve for intracranial pressure assessment in cases of Chiari I malformation.

Methods

In a retrospective study, MRI data of 37 consecutive pediatric patients with Chiari malformation and data of 400 patients without intracranial pathology were compared and analyzed. ONSDs were measured at the point where the ophthalmic artery crosses the optic nerve (anatomical landmark). The correlation analysis was performed with clinical findings, gender, age, papilledema, and other neuro-ophthalmological findings.

Results

ONSD was enlarged in 38 % of cases of Chiari malformation. The enlargement was bilateral, no correlation with age or gender was found (p = 0.67 and p = 0.76, respectively). The presence of papilledema was detected in 19 % of cases presenting less valuable diagnostic sign if compared with ONSD. The tortuosity of the optic nerve was found in 22 % of cases, but in three patients, it was unilateral. All patients with enlarged ONSD and other neuro-ophthalmological signs present were treated surgically, while most of the patients without these signs (20/23) were treated conservatively.

Conclusion

In majority of pediatric cases of Chiari malformation, the ONSD is not enlarged and other neuro-ophthalmological signs are not present. Detecting the enlarged ONSD and other neuro-ophthalmological signs in cases of Chiari malformation may indicate the elevated intracranial pressure and necessity for urgent surgical intervention.

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Acknowledgments

The authors would like to thank most sincerely Evelina Shevtsov, MD, Department of Neurology, who assisted us in our research.

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Correspondence to Michael Vaiman.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Conflict of interest

Michael Vaiman, Tal Sigal, Itzhak Kimiagar, Zina Evy Almer, and Inessa Bekerman declare that they have no conflict of interest.

Informed consent

The institutional review board of our institute approved this retrospective study, and the requirement to obtain informed consent was waived.

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Bekerman, I., Sigal, T., Kimiagar, I. et al. Diagnostic value of neuro-ophthalmological signs in cases of Chiari I malformation. Childs Nerv Syst 32, 2423–2428 (2016). https://doi.org/10.1007/s00381-016-3270-4

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  • DOI: https://doi.org/10.1007/s00381-016-3270-4

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