Cases reported "Scleritis"

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11/203. Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis.

    PURPOSE: The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. CASE REPORT/methods: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. RESULTS: Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. The deafness had been due to inner ear failure, and the patient died of a cardiac failure. CONCLUSIONS: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications. ( info)

12/203. Choroidopathy of systemic lupus erythematosus.

    PURPOSE: To describe the ocular and systemic manifestations associated with systemic lupus erythematosus (SLE) choroidopathy. methods: Three new cases of choroidopathy in patients with active SLE were described. Twenty-five published cases of lupus choroidopathy were summarized. RESULTS: There have been 28 cases of lupus choroidopathy (47 involved eyes) that have been reported in the English literature since 1968, including the three current cases. Only two of the patients were male. The choroidopathy was bilateral in 19 patients (68%). All 28 patients (100%) had active systemic vascular disease at the onset of their choroidopathy; 18 (64%) had nephropathy and 10 (36%) had central nervous system (CNS) lupus vasculitis. All but one of the patients had a known diagnosis of SLE at the onset of choroidopathy. 30 of the 47 involved eyes had presenting visual acuity of 20/40 or better; 14 eyes showed improvement in visual acuity with therapy. 23 patients (82%) had resolution of their choroidopathy when their systemic disease was brought under control. Despite treatment, 4 of the 28 patients (14%) died from complications of SLE. CONCLUSIONS: Although less known than retinopathy, lupus choroidopathy may be more common than generally appreciated. It usually serves as a sensitive indicator of lupus activity. The presence of SLE choroidopathy is generally indicative of coexistent (although sometimes occult) nephropathy, CNS vasculitis, and other SLE visceral lesions. immunomodulation of the systemic disease can lead to improvement and resolution of the systemic vasculitis as well as the choroidopathy. ( info)

13/203. Relapsing polychondritis.

    BACKGROUND: Relapsing polychondritis (RPC) is a rare, chronic, and potentially fatal multisystemic inflammatory disorder targeting cartilaginous structures. This disorder is frequently associated with rheumatoid arthritis, systemic vasculitis, connective tissue diseases, and/or hematologic disorders. RPC afflicts patients with recurrent and often progressive episodes of inflammation with the potential for destruction of the affected structures. tissues involved include the ears, joints, nose, larynx, trachea, eyes, heart valves, kidneys,and skin. Ocular manifestations commonly include episcleritis, scleritis, conjunctivitis, iridocyclitis, chorioretinitis, and proptosis. Lid edema, orbital inflammation, muscle palsies, and corneal melting may also occur. CASE REPORT: An 83-year-old man previously diagnosed with RPC presented to our clinic with acute unilateral chemosis, conjunctivitis, lid edema, proptosis, and extraocular muscle restriction. After orbital cellulitis was ruled out, further evaluation revealed posterior scleritis with choroidal detachment OS. A course of oral indomethacin and topical antibiotic-steroid combination drops was implemented in the treatment of the ocular manifestations. The quick positive response to the anti-inflammatory agents confirmed the diagnosis of ocular complications secondary to RPC. DISCUSSION: The presenting ocular signs and symptoms of RPC often resemble other commonly encountered ocular conditions. It is important for the eye care practitioner to be familiar with the ocular manifestations of RPC because the eyes are sometimes the initial site of involvement and may be a marker of severity. early diagnosis and intervention may significantly improve the patient's outcome. This case report with literature review will hopefully bring to light features of this disease which will help the eye care practitioner in the diagnosis and management of this condition. ( info)

14/203. mycobacterium chelonae conjunctivitis and scleritis following vitrectomy.

    The atypical, or nontuberculous, mycobacteria are opportunistic pathogens that usually cause infection following accidental trauma or surgery. These organisms are ubiquitous in nature but have been found with increasing frequency in other environments that include medical offices and surgical suites. Management of atypical mycobacterial ocular infections can be difficult because in vitro antibiotic activity does not always correlate with in vivo efficacy and because normal immune defenses against mycobacteria may work too slowly to prevent irreversible damage to infected ocular tissues. This report describes a patient who developed a severe ocular infection due to mycobacterium chelonae after vitrectomy. Despite eradication of the infection, the eye became blind and painful. Arch Ophthalmol. 2000;118:1125-1128 ( info)

15/203. The tyranny of the anecdote: Waldenstrom's macroglobulinemia and scleritis.

    A 49-year-old male developed a bilateral scleritis that eventually involved the optic nerve and was associated with cotton wool spots. Investigation of a markedly elevated erythrocyte sedimentation rate revealed a diagnosis of Waldenstrom's macroglobulinemia. Although it was plausible to believe that the two rare diseases were causally related, the scleritis resolved while the gammopathy progressed. ( info)

16/203. Long-term follow-up of giant nodular posterior scleritis simulating choroidal melanoma.

    A 41-year-old asymptomatic woman was referred for enucleation of a 7. 5-mm-thick intraocular tumor suspected to be choroidal melanoma. The clinical findings combined with imaging studies suggested instead a diagnosis of giant nodular posterior scleritis. A scleral biopsy was performed to confirm the diagnosis. After 12 years of observation, the lesion has remained stable and visual acuity has been preserved. Nodular posterior scleritis can present with no symptoms of pain, redness, or visual disturbance and can remain quiet for many years. It must be clinically differentiated from choroidal melanoma. Arch Ophthalmol. 2000;118:1290-1292 ( info)

17/203. Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.

    OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation. ( info)

18/203. Congenital porphyria with necrotizing scleritis in a 9-year-old child.

    Porphyria is a rare metabolic disorder that is characterized by the accumulation of photosensitive, toxic intermediates of the heme metabolic pathway in various organs of the body including the skin, eye and neural tissue. Porphyria as a potential cause for bilateral necrotizing scleritis in children is very infrequently emphasized in literature, probably due to the relatively rare occurrence and lack of well-documented cases. A case of a 9-year-old child with congenital porphyria who had developed necrotizing scleral ulceration in both eyes in addition to severe cutaneous hypersensitivity and facial disfigurement is herein presented. ( info)

19/203. Bilateral choroidal infarction in a patient with antiphospholipid syndrome: a case report.

    PURPOSE: To report a case of bilateral choroidal infarction occurring as a complication of primary antiphospholipid syndrome. methods: The case notes of the patient were reviewed. RESULTS: A 25-year-old man with primary antiphospholipid syndrome and a previous history of deep vein thrombosis and pulmonary embolism, developed episcleritis and bilateral choroidal infarction, with deterioration of vision to 6/12 OD and counting fingers at 5 feet OS. Anticoagulation therapy with warfarin was administered and the patient's vision gradually improved to 6/6 OD and 6/9 OS. CONCLUSION: Primary antiphospholipid syndrome is associated with thrombotic phenomena, which may affect the ocular arterial and venous vasculature. Therefore, a detailed ophthalmological examination is warranted in those patients who present with ocular symptoms and deteriorating vision. Retinal fluorescein angiography is valuable in confirming the diagnosis when in doubt, and in determining the extent of vessel occlusion. ( info)

20/203. Pachymeningitis with pseudo-Foster Kennedy syndrome.

    PURPOSE: To report a case of pachymeningitis with pseudo-Foster Kennedy syndrome in a patient who was positive for perinuclear antineutrophil cytoplasmic antibody. methods: Case report. A 44-year-old man was examined for headache and diplopia. RESULTS: Left eye showed limitation of abduction. Ocular fundus, computed tomography (CT), and magnetic resonance imaging (MRI) of the head and orbits were normal. The diplopia subsided spontaneously. Six months later, he noticed sudden visual loss in the left eye. The left eye showed optic disk atrophy and episcleritis, and the right eye showed papilloedema. Computed tomography and MRI exhibited thickened dura mater. serum perinuclear antineutrophil cytoplasmic antibody level was highly increased. CONCLUSION: Pachymeningitis with ocular involvement sometimes requires repeated CT or MRI for diagnosis. This disorder may be caused by microvasculitis. ( info)
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