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Show Journal of Clinical Nturo- ophthalmology 11( 1): 4~, 1991 Unilateral Enophthalmos Systemic Scleroderma In © 1991 Raven Press, Ltd., New York Pinar A. Kirkali, M. D., Tulay Kansu, M. D., and Ali Sefik Sanae, M. D. Atrophy of orbital fat is a reported complication of localized scleroderma but not of systemic scleroderma. Here we present a case of systemic scleroderma with unilateral enophthalmos. Orbital fat atrophy was the presumed cause on computerized tomography. Key Words: Scleroderma, enophthalmos, orbit, orbital fat atrophy. From the Neuro- ophthalmology Unit, Hacet. tepe University, Institute of Neurological Sciences and Psychiatry ( P. A. K, T. K), and the Department of Ophthalmology, Hacettepe Uru-versity School of Medicine, Ankara, Turkey. . Address correspondence and reprint requests to Dr. PInar A. KirkaIi, at Hacettepe University Hospitals and ClIrucs, Department of Neurology, Sihhiye, Ankara 06100, Turkey. 43 Orbital complications are rarely seen in scleroderma. We recently saw a patient with systemic scleroderma with unilateral enophthalmos in Hacettepe University Hospitals, Ankara, Turkey. CASE REPORT A 58- year- old woman presented with vascular type headaches for 6 months. She also complained of palpitations, dyspnea, and dysphagia for the previous 3 years. She had cyanosis in the extremities, as well as claudication. She denied any loss of weight. Her neuro- ophthalmic examination revealed 3 mm of enophthalmos in the left eye with Hertel exophthalmometer measurements. Lagophthalmos on downgaze was also noted in the left eye. Schirmer's test revealed 7 mrn/ 5 min in both eyes. Her eye examination was otherwise unremarkable. On general examination she had alopecia, thin lips, and tight skin ( Fig. 1). Electromyography showed chronic denervation in extensor digitorum brevis muscle. A skin- muscle biopsy revealed vasculitis. On echocardiography, the aortic valve was seen to be slightly calcified. On esophagosgraphy peristalsis was diminished, and the passage was somewhat slow. Orbital computerized tomography scans showed less fat in the left orbit than in the right and marked enophthalmos ( Fig. 2). The patient received no specific treatment for her eye disorders. When she was seen 6 months later, her eyelid and ocular motility findings were unchanged. DISCUSSION Scleroderma is a chronic connective tissue and vascular disease of unknown etiology, with either local ( linear) or systemic sclerosis and atrophy. 44 P. A. KIRKALl ET AL. FIG. 1. General appearance of the face: thin, slim lips, tight skin, and left enophthalmos. Systemic scleroderma is characterized by diffuse involvement of the skin and many internal organs. Ophthalmological complications of systemic scleroderma have been reported as rare and nonspecific ( 1,2). Enophthalmos related to scleroderma has been reported, but orbital fat atrophy is a rare complication which has been reported only in linear scleroderma with cicatrization of the lid and medial rectus muscle ( 3,4). Atrophy of the orbital fat I Clin Neuro- ophthalmol, Vol. 11, No. 1, 1991 FIG. 2. Orbital CT scans showing less hypodensity of fat in the retrobulbar region of the left orbit than in the right. is a basic mechanism of enophthalmos ( 3). To our knowledge, orbital fat atrophy as the sole manifestation of orbital involvement in systemic scleroderma has not been reported previously. Acknowledgment: The authors wish to thank Jack Rootman, M. D. for his critical review of the manuscript. REFERENCES 1. West, RH, Barnett, AJ. Ocular involvement in scleroderma. Br I Ophthalmol 1979; 63: 845- 7. 2. Horan, EC. Ophthalmic manifestations of progressive systemic sclerosis. Br I Ophthalmol 1969; 53: 388-- 92. 3. Cline, RA, Rootman, J. Enophthalmos: a clinical review. Ophthalmology 1984; 91 : 229- 37. 4. Rootman, J. Pathophysiologic patterns of orbital disease. In: Rootman J, ed. Diseases of the orbit. Philadelphia: Lippincott, 1988: 51--{' 7. |
