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Optic neuropathy in McCune-Albright syndrome: an indication for aggressive treatment |
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| Authors: | G Bocca J de Vries JRM Cruysberg GHJ Boers LAH Monnens |
| Institution: | Departments of Paediatrics, University Hospital Nijmegen St Radboud, The Netherlands;Neurosurgery, University Hospital Nijmegen St Radboud, The Netherlands;Ophthalmology, University Hospital Nijmegen St Radboud, The Netherlands;and Internal Medicine, University Hospital Nijmegen St Radboud, The Netherlands |
| 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. |
| Keywords: | Craniofacial surgery fibrous dysplasia optic nerve visual loss |