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M Goisis, F Biglioli, P Mortini, M Guareschi, R Brusati; Optic Nerve Compression in Craniofacial Fibrous Dysplasia: Early Diagnosis and Treatment . Invest. Ophthalmol. Vis. Sci. 2002;43(13):3055.
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Purpose: Craniofacial fibrous dysplasia manifests clinically as masses of bone with abnormal growth. These masses may originate within bone adjacent to the optic canal and may produce compression of the optic nerve, leading initially to loss of color vision, then to restricted visiual fields, decreased visual acuity, and even blindness. We present a case series, describing the efficacy of early diagnosis and treatment in patients with radiologic or clinical evidence of involvement of the optic canal. Methods: Ten young patients (mean age 9 years ranging between 8 to 19 years) with craniofacial fibrous dysplasia involving the sphenoid bone were studied with a every three months measurement of visual acuity, visual fields and color perception. The patients were also instructed to rapidly report any changes in their vision, including color loss and rapid loss of vision. CT scans were done every year. The mean period of follow-up was 2 years and 7 months (range 14 to 47 months). Results: Three patients underwent decompression of the optic canal. The surgical indication in one of these patients was a relatively rapid progressive loss of vision (over a period of four months) from 10/10 to 8/10. The two others patients had TC evidence of progressive bilateral optic canal reduction without clinical signs. In the first patient, vision improved from 8/10 to 10/10 whithin six months of the intervention. No loss of color vision, no visual fields restriction or decreased visual acuity were observed in the two patients who underwent profilactic nerve decompression and in the seven patients who did not underwent decompression. Conclusion: Craniofacial fibrous dysplasia is a progressive desease, and early diagnosis of optic nerve involvement is an indication for surgery. According with Chen et Al (1), we think that the need of early decompression is evidenced by the speed of visual deterioration, in order to avoid a permanent visual loss. Chen YR, Breidahal A, Chang CN. Optic nerve decompression in fibrous dysplasia: indications, efficacy and safety. Plast Reconstr Surg 1997: 99, 22-33
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