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      Optic atrophy, necrotizing anterior scleritis and keratitis presenting in association with Streptococcal Toxic Shock Syndrome: a case report

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          Abstract

          Introduction

          We report a case of optic atrophy, necrotizing anterior scleritis and keratitis presenting in a patient with Streptococcal Toxic Shock Syndrome.

          Case presentation

          A 43-year-old woman developed streptococcal toxic shock syndrome secondary to septic arthritis of her right ankle. Streptococcus pyogenes (b-haemolyticus Group A) was isolated from blood cultures and joint aspirate. She was referred for ophthalmology review as her right eye became injected and the pupil had become unresponsive to light whilst she was in the Intensive Therapy Unit (ITU). The iris appeared atrophic and was mid-dilated with no direct or consensual response to light. Three zones of sub-epithelial opacification where noted in the cornea. There where extensive posterior synechiae. Indirect ophthalmoscopy showed a pale right disc. The vision was reduced to hand movements (HM). A diagnosis of optic atrophy was made secondary to post-streptococcal uveitis. She subsequently developed a necrotizing anterior scleritis.

          Conclusion

          This case illustrates a previously unreported association of optic atrophy, necrotizing anterior scleritis and keratitis in a patient with post-streptococcal uveitis. This patient had developed Streptococcal Toxic Shock Syndrome secondary to septic arthritis. We recommend increased awareness of the potential risks of these patients developing severe ocular involvement.

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          Most cited references6

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          Poststreptococcal syndrome uveitis: a descriptive case series and literature review.

          To describe the clinical features in a series of patients with poststreptococcal uveitis and to review literature on the pathophysiology and management. Retrospective and descriptive case series. Ten consecutive cases of poststreptococcal syndrome uveitis diagnosed between 1996 and 2003. Review of patient case notes. Age, laterality, clinical features, and anti-streptococcal lysin O titers. Ten consecutive cases of poststreptococcal syndrome uveitis were identified. All our cases had bilateral nongranulomatous inflammation and raised anti-streptococcal lysin O titers. Collating data from previous reports and this series showed that 96% of the patients were below 40 years of age, and 87.5% had evidence of previous streptococcal infection. One third of the patients had posterior segment involvement. In our patients, this was in the form of vitritis, focal retinitis, optic disc swelling, and multifocal choroiditis. Poststreptococcal syndrome uveitis should be considered in the etiology of acute bilateral nongranulomatous uveitis in children and young patients.
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            Implication of pneumolysin as a virulence factor in Streptococcus pneumoniae endophthalmitis.

            To determine if pneumolysin, a multifunctional cytotoxin produced by Streptococcus pneumoniae, may be a virulence determinant in the pathogenesis of pneumococcal endophthalmitis. Lewis rats (n = 20) were injected intravitreally with purified recombinant pneumolysin at the following doses; 3.9 hemolytic units (HU), 39 HU, 390 HU, 3.9 x 10(3) HU, and 3.9 x 10(4) HU. After 24 hours, eyes were examined clinically and enucleated for histopathologic examination to elucidate the dose-response relationship. To determine the temporal progression of the disease model, a second group of rats (n = 8) were injected intravitreally with 390 HU of pneumolysin. At 6 and 48 hours, eyes were examined clinically and enucleated for histopathology. Eyes injected with pneumolysin demonstrated increasing anterior and posterior segment inflammation in response to increasing doses of administered toxin. The onset of inflammation and tissue damage occurred rapidly, and was maximal at 24 to 48 hours. The clinical and histopathologic changes observed mimicked those of S. pneumoniae endophthalmitis. Histopathologic analysis demonstrated rapid onset of iridocyclitis and vitritis with polymorphonuclear leukocyte influx, inner retinal necrosis, and retinal detachment. Retinal pigment epithelial necrosis and choroiditis were noted at the highest doses administered. Inflamed eyes were shown to be sterile. Pneumolysin injected intravitreally induces many of the clinical and histopathologic features of pneumococcal endophthalmitis, and may play an important role in the inflammation and tissue damage that occurs in pneumococcal endophthalmitis.
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              Post-streptococcal uveitis.

              To describe the clinical features of post-streptococcal uveitis (PSU) and examine management strategies in the treatment of this under-recognized condition. Patients were identified from the world literature using the Pubmed search engine. We examined two new cases of post-streptococcal intermediate uveitis. The epidemiology, immune mechanisms, clinical features, investigations, treatments and visual outcomes were examined and recorded. We reviewed 11 patients including our own two cases. There was a statistically significant seasonal difference in antistreptolysin-O titres (ASOT), and age-related ASOT was identified. Of the 11 patients, eight (72.7%) had anterior uveitis, two (18.2%) had intermediate uveitis and one (9.1%) had panuveitis. Their ages ranged from 5 to 56 years (mean 17 years). The majority of cases had significantly elevated ASOT; most patients were treated with topical steroids and oral antibiotics and four cases underwent adenotonsillectomy. The visual prognosis was good in most cases. Uveitis may be the sole presenting clinical feature, or it may occur in combination with other features of post-streptococcal infection. Ophthalmologists should be aware of the clinical features of PSU and maintain a high level of suspicion, particularly in childhood uveitis.
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                Author and article information

                Journal
                J Med Case Reports
                Journal of Medical Case Reports
                BioMed Central
                1752-1947
                2008
                29 February 2008
                : 2
                : 69
                Affiliations
                [1 ]Department of Ophthalmology, Mid Essex NHS Trust, Court Road, Chelmsford, Essex CM1 7ET, UK
                Article
                1752-1947-2-69
                10.1186/1752-1947-2-69
                2277429
                18312659
                37ba8bc9-fcb3-40b8-943d-158c2773c2b5
                Copyright © 2008 Papageorgiou et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 14 May 2007
                : 29 February 2008
                Categories
                Case Report

                Medicine
                Medicine

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