Volume 87, Issue 5 pp. 599-600

Optic neuropathy in McCune-Albright syndrome: an indication for aggressive treatment

G Bocca

G Bocca

Departments of Paediatrics, University Hospital Nijmegen St Radboud, The Netherlands

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J de Vries

J de Vries

Neurosurgery, University Hospital Nijmegen St Radboud, The Netherlands

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JRM Cruysberg

JRM Cruysberg

Ophthalmology, University Hospital Nijmegen St Radboud, The Netherlands

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GHJ Boers

GHJ Boers

and Internal Medicine, University Hospital Nijmegen St Radboud, The Netherlands

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LAH Monnens

Corresponding Author

LAH Monnens

Departments of Paediatrics, University Hospital Nijmegen St Radboud, The Netherlands

LAH Monnens, Department of Paediatrics, University Hospital Nijmegen St Radboud, PO Box 9101, 6500 HB Nijmegen, The NetherlandsSearch for more papers by this author
First published: 02 January 2007
Citations: 16

Abstract

McCune-Albright syndrome consists of the triad polyostotic fibrous dysplasia, café-au-lait spots and autonomous hyperfunction of one or more endocrine systems. The most frequent neurological complication of craniofacial fibrous dysplasia is visual loss. We describe a 17-y-old boy with McCune-Albright syndrome and acute loss of vision in the left eye caused by encroachment of the left optic nerve by fibrous dysplastic lesions. Neurosurgical intervention improved left eye vision. Aggressive intervention is indicated in cases of acute visual loss in patients with craniofacial fibrous dysplasia. This is supported by a review of other reported cases.

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