Isolated bilateral posterior scleritis after eye trauma Marco A. Ramirez-Ortiz, MD, MPH and Alejandro Vasquez-Resendis, MD
Posterior scleritis is an unusual disease that may cause serious ocular inflammation and potentially blinding consequences. To date, there are few cases of isolated posterior scleritis in children described in the literature. We report an unusual case of recurrent bilateral posterior scleritis after direct blunt trauma to the postequatorial aspect of the eye successfully controlled with long-term steroids and immunosuppressive therapy.
Case Report
disease. Physical examination revealed an otherwise healthy girl. Chest x-ray showed normal lungs. CT imaging of the orbits revealed thickening of the posterior sclera of the right eye (Figure 2). B-scan echography measured the macular choroidal thickening as 4.5 mm in the right eye. Posterior scleritis was diagnosed and indomethacin 25 mg three times daily by mouth was initiated. After 5 days of therapy, the patient had relief of pain, improvement of the visual acuity with normalization of ocular motility,
A previously healthy 8-year-old girl came to Hospital Infantil de Mexico, Mexico City, complaining of a rapidly progressive decrease in the visual acuity of her right eye. Her previous history was unremarkable, except that 1 week earlier the patient had accidentally introduced the semicircular metallic headband of a tiara under her upper right eyelid, deep through the upper conjunctival fornix. She experienced acute pain that persisted for several days despite analgesic medication. There was minimal bleeding and no eyelid swelling. Five days later the patient was evaluated at a local hospital for blurred visual acuity and persistent eye discomfort. Two ophthalmologists diagnosed retinal detachment secondary to intraocular tumor and proposed immediate enucleation. The parents took their daughter to our hospital for a second opinion. Ocular examination revealed a visual acuity of hand motion on the right and 20/20 on the left. Anterior segment evaluation of the right eye showed mild temporal conjunctival hyperemia. Ocular motility displayed Vpattern exotropia (40⌬) with no limitation of gaze. Dilated fundus examination on the right showed a circumscribed subretinal mass beneath the macula with no vitreous cells associated (Figure 1A). Examination of the left eye was normal with 20/20 visual acuity. Fluorescein angiography of the right eye showed multiple pinpoint spots of dye leakage with late staining of the nerve head. Serologic evaluation (antinuclear antibody, rheumatoid factor, erythrocyte sedimentation rate, complete blood cell count, and Venereal Disease Research Laboratory tests) were within normal range. There was no history of fever, anorexia, upper and lower respiratory symptoms, or previous rheumatologic
Author Affiliations: Hospital Infantil de Mexico, Pediatric Ophthalmology and Strabismus, Mexico City, D.F., Mexico Submitted June 1, 2006. Revision accepted September 28, 2006. Published online January 26, 2007. Reprint requests: Marco A. Ramirez-Ortiz, MD, MPH, Hospital Infantil de Mexico, Pediatric Ophthalmology and Strabismus, Dr. Marquez No. 162, Col. Doctores, 06720 Mexico City, D.F., Mexico (email:
[email protected]). J AAPOS 2007;11:284-285. Copyright © 2007 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2007/$35.00 ⫹ 0 doi:10.1016/j.jaapos.2006.09.019
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FIG 1. (A) Posterior scleritis of the right eye manifested as a circumscribed subretinal mass at the posterior pole with some scattered yellowishwhite lesions on its surface and blurred disk. (B) Three months after indomethacin treatment was prescribed, the left eye spontaneously developed posterior scleritis manifested as serous retinal detachment confined to posterior pole with disk edema.
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FIG 2. Computed axial tomographic scan showing thickening of the posterior sclera of the right eye (arrow).
and complete resolution of the subretinal mass. Antiinflammatory treatment was continued for 90 days. Three months later, the patient returned, complaining of decreased visual acuity in her left eye. Visual acuity was 20/20 in the right eye and 20/100 in the left eye. The left pupil was oval and a relative afferent pupillary defect was detected. Dilated fundus examination showed serous retinal detachment with disk edema in her left eye (Figure 1B). Because of the bilateral nature of the disease, we prescribed prednisone in a dose of 1.5 mg/kg daily and azathioprine at 0.8 mg/kg daily. After 2 months prednisone was tapered to 5 mg weekly, whereas azathioprine was increased to 2 mg/kg daily. Antinuclear antibody test and cytoplasmic staining antineutrophil cytoplasmic antibodies titers were negative. One month after beginning immunosuppressive therapy the patient recovered normal visual acuity in her left eye with complete resolution of the serous retinal detachment. Prednisone was prescribed every other day and then discontinued after 6 months. Maintenance of azathioprine was continued for 1 year after resolution of disease signs, and then it was gradually tapered to a dose of 0.33 mg/kg daily with close laboratory controls for leukopenic and hepatotoxic side effects. After 6 years of follow-up, the patient has remained free of systemic and ocular disease, with 20/20 visual acuity in each eye. Laboratory tests, such as complete blood cell count, antinuclear antibody, rheumatoid factor, erythrocyte sedimentation rate, and liver test have also remained within the normal limits.
Discussion Posterior scleritis is an uncommon inflammatory ocular disorder in children. In adults it is often associated with systemic conditions such as rheumatoid arthritis and other collagen-vascular and infectious diseases.1,2 In children, the underlying etiology is seldom identified.3,4 Current knowl-
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edge supports an autoimmune etiology of scleral inflammation that may be initiated by the release of scleral tissue components after infection, injury, or surgical trauma.5 These components may start an immunological reaction against scleral antigens that could affect one or both eyes. Few articles have reported details on clinical features and progression of posterior scleritis in children. The youngest patient with confirmed posterior scleritis reported in the medical literature is an 8-year-old boy.1 Eye manifestations ranged from eyelid swelling and abnormalities in eye movements with no retinal manifestations to circumscribed subretinal masses and serous retinal detachment. A recent review article by Okhravi and colleagues mentions cases of surgical and nonsurgical eye trauma leading to infection and then anterior scleritis in adults.6 Nonsteroidal antiinflammatory drugs have been prescribed with good results in controlling mild posterior scleritis with normal optic nerve.7 However this disease may show severe inflammatory progress in some pediatric patients and scleral disease can be extended to the fellow eye if insufficient antiinflammatory treatment is indicated. Woon and colleagues reported permanent visual acuity loss secondary to the optic nerve atrophy despite immunosuppression in two children with disk swelling.3 This case underlines the importance of steroidal and immunosuppressive treatment when the optic nerve is affected. Even when the inflammatory scleral process may rapidly disappear with appropriate treatment, we also suggest continuing immunosuppressive therapy for one additional year to obtain a longer regulatory effect by antigen-specific inhibition. Although direct eye trauma was closely related to the beginning of scleritis in this patient, there is a possibility that the trauma was coincidentally associated with the disease. Because of its infrequency, posterior scleritis can present a diagnostic challenge to ophthalmologists. It is important to recognize posterior scleritis clinically and differentiate it from retinoblastoma and other simulating conditions to avoid unnecessary enucleations.8 References 1. Benson WE. Posterior scleritis. Surv Ophthalmol 1988;32:297-316. 2. Foster CS, Sainz de la Maza M. The sclera. New York: SpringerVerlag; 1994. p. 112-23. 3. Woon WH, Stanford MR, Graham EM. Severe idiopathic posterior scleritis in children. Eye 1995;9:570-4. 4. Wald KJ, Spaide R, Patalano VJ, Sugin S, Yannuzzi LA. Posterior scleritis in children. Am J Ophthalmol 1992;113:281-6. 5. Watson PG, Young RD. Scleral structure, organization and disease: A review. Exp Eye Res 2004;78:609-29. 6. Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol 2005;50:351-63. 7. Singh G, Guthoff R, Foster CS. Observations of long-term follow-up of posterior scleritis. Am J Ophthalmol 1986;101:570-5. 8. Calthorpe CM, Watson PG, McCartney CE. Posterior scleritis: A clinical and histological survey. Eye 1988;2:267-77.
