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Show ! oumal of CIi" ical Ne"",- ophthalmology 11( 4): 259- 261. 1991. Steroid- Induced Visual Recovery in Fibrous Dysplasia Jorge G. Arroyo, M. D., Simmons Lessell, M. D., and William W. Montgomery, M. D. ( i) 1991 Raven Press, Ltd., New York Fibrous dysplasia is a rare disorder of bone that may be associated with acute loss of vision. Surgical intervention is generally advocated and sometimes restores vision in these patients. We report a case of a patient with unilateral optic neuropathy from fibrous dysplasia who suffered two acute episodes of visual impairment. The first episode of vision loss was reversed by surgery and the second by corticosteroid treatment. A trial of corticosteroid th~ rapy may be beneficial in cases of acute vision loss due to fibrous dysplasia. Key Words: Fibrous dysplasia- Optic neuropathyCorticosteroids. From the Departments of Ophthalmology and Otolaryngology, Harvard Medical School, and the Massachusetts Eye and Ear Infirmary, Boston, Massachusetts: U. S. A. . Address correspondence and repnnt requests to Dr. Simmons Lessell at 243 Charles Street, Boston, MA 02114, U. S. A. 259 Fibrous dysplasia is a rare disorder of unknown etiology in which bone is replaced by proliferating fibrous connective tissue. Derome et a1. ( 1) reported that visual impairment was present in 18 of 43 patients with fibrous dysplasia of the skull base; 8 of these patients experienced bilateral visual disturbances. A variety of mechanisms have been postulated to explain the visual loss in fibrous dysplasia. These include compression of the optic nerve by a narrowed optic canal, a sinus mucocele, or a cyst; a hemorrhage into an adjacent area of dysplastic bone; or optic nerve vaso- occlusion. Prompt surgical intervention is generally advocated when an optic neuropathy develops in a patient with fibrous dysplasia and sometimes effects dramatic improvement. However, surgery is not always effective. We have observed the case of a young woman with a unilateral optic neuropathy from fibrous dysplasia who suffered two acute episodes of visual impairment, the first of which was reversed by surgery and the second by corticosteroid treatment. CASE REPORT Since early adolescence the patient had been aware of a facial asymmetry ( Fig. 1), but fibrous dysplasia of the sphenoid, ethmoid, and frontal bones was not recognized until age 22 ( February 1989) when a computerized tomographic scan of the head was obtained to investigate bifrontal and bimalar pain ( Fig. 2). Shortly thereafter, during the first trimester of pregnancy, vision became blurred in the right eye. There was no pain initially, but as vision declined over the next 6 days, she noticed slight pain behind the right eye on shifting her gaze. She was then referred for neuro- ophthalmic 260 J. G. ARROYO ET AL. FIG. 1. Photograph of patient showing depression and lateral displacement of right orbit from fibrous dysplasia. evaluation. Her eye and neurologic histories had been unremarkable. Her best- correctable visual acuity was 20/ 200 right eye with profound dyschromatopsia, a large dense central scotoma, and a relative afferent pupillary defect. The right globe was depressed and three millimeters proptotic. The left eye was normal in all particulars, and both fundi were normal. Vision failed over the next day to 2/ 200 and a decompression via the ethmoid and sphenoid sinuses were performed. The excised tissue showed FIG. 2. Computerized tomography showing orbital bone lesion of fibrous dysplasia involving the right orbit. / qql the histopathological findings typical of fibrous dysplasia. No corticosteroids were administered. Vision steadily improved thereafter. She could see 20/ 70 within 36 hours and 20/ 15 after 5 days by which time her color vision was normal! One month after surgery, vision was 20/ 13 right eye, no visual field defect could be demonstrated, and the right optic disc was only questionably atrophic. She delivered a healthy baby 7 months later and then was well until age 23 Gune 1990), fifteen months after the original episode of vision loss, when the right eye became " a little hazy." Two days later, right periorbital and hemicranial pain unaffected by touching or moving the eye supervened. Visual acuity in the right eye was 20/ 15, she missed most of the Ishihara color test plates and there was a right relative afferent pupillary defect. The Goldmann visual field was constricted to the Be stimulus and she could not see the I2e stimulus. Computerized tomographic scanning of the head and orbits showed no changes since her surgery. Oral prednisone therapy was initiated immediately at dosages of 20 mg three times per day and vision improved within days. She was maintained on this dose for 10 days, and was placed on 30 mg per day for 7 additional days. She recovered completely during the 9 weeks the prednisone was being tapered and has remained well during the 9 months since the prednisone was discontinued. COMMENT Some patients with fibrous dysplasia of the skull lose vision acutely, presumably from a retrobulbar optic neuropathy. The constellation of findingsloss of visual acuity and visual field, pain, relative afferent pupillary defect, and normal fundus- is identical to that in patients with demyelinative or idiopathic optic neuritis, with which the acute optic neuropathy of fibrous dysplasia might easily be confused. The report by Derome et al. ( 1) includes 5 cases in which vision was lost over 1- 15 days. Gass ( 2), Melen et al. ( 3), and Weisman et a1. ( 4) each reported similar cases. Three of the patients were originally diagnosed as having retrobulbar optic neuritis. Although a sinus mucocele is occasionally identified, in cases such as our own the pathogenesis remains uncertain even after radiological investigations and surgical exploration. The proper management of such cases is problematic, but. our favorable experience, albeit in only one patient with mild impairment of visual function, suggests that a trial of oral corticosteroid therapy is STEROID- INDUCED VISUAL RECOVERY 261 warranted before surgically decompressing the optic nerve. If vision is restored and retained after the drug is discontinued, surgery may be avoided. REFERENCES 1. Derome PJ, Visot A, Akerman M. Ocular symptoms in fibrous dysplasia of the skull. In: Smith JL, ed. Neuro-ophthalmology now! New York: Field, Rich & Associates, 1986: 125- 49. 2. Gass 10M. Orbital and ocular involvement in fibrous dysplasia. South Med J 1965; 58: 32~ 9. 3. Melen 0, Weinberg PE, Kim KS, Hemmali M, Siqueira EB. Fibrous dysplasia of bone with acute visual loss. Ann OphthalmoI1980; 12: 73~ 9. 4. Weisman JS, Hepler RS, Vinters HV. Reversible visual loss caused by fibrous dysplasia. Am J Ophthalmol 1990; 110: 24+- 9. JClin Neuro- ophthlltmol, Vat. 11, No. 4, 1991 |
