Cases reported "Uveitis"

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1/33. Scleral ectasia as a complication of deep sclerectomy.

    Nonpenetrating deep sclerectomy is a filtration surgical technique to treat glaucoma. A 12-year-old girl presented with chronic arthritis complicated with glaucoma secondary to a chronic uveitis. A sclerectomy without a collagen implant was performed for uncontrollable glaucoma with deterioration in visual function. Three weeks later, the patient had a rise in intraocular pressure and a scleral ectasia on the sclerectomy. The eye showed an area of scleral ectasia in the bleb as well as iris adhesion. Partial resection of the bleb after iris detachment led to poor anatomic and IOP results. The indications for deep sclerectomy must be carefully considered, especially in patients at a young age with this type of glaucoma.
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keywords = iris
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2/33. Uveitis masquerade syndromes.

    BACKGROUND: Incorrect diagnosis of the uveitic masquerade syndromes (UMS) may have severe consequences. In this study, the frequency, clinical manifestations, and informative diagnostic tests for UMS are described. DESIGN: Retrospective observational case series. PARTICIPANTS: Forty patients with UMS were identified in a cohort of 828 consecutive patients with uveitis. The mean follow-up was 4.5 years. methods: A review of clinical, laboratory, photographic, and angiographic records was performed. MAIN OUTCOME MEASURES: Clinical features, associated systemic diseases, diagnostic procedures and their role in the diagnostic process, and systemic and visual outcomes. RESULTS: Nineteen patients had intraocular malignancy (48% of all with UMS; 2.3% of all with uveitis), mainly intraocular lymphoma (n = 13) and leukemia (n = 3). The ophthalmologist was the first to recognize malignant disease in 11 of 19 patients (58%). Of 21 patients with nonmalignant UMS, 10 had an ocular vascular disease and 5 a hereditary ocular disorder. The patients with malignant UMS were older than those with nonmalignant UMS (average age, 50 vs 44 years, P: < 0.05). During follow-up, 9 of 19 patients with malignant UMS died. The most informative diagnostic procedure for malignant UMS was intraocular fluid analysis; for nonmalignant UMS, fluorescein angiography. The cytologic analysis of intraocular fluids yielded the best results for diagnosing intraocular malignancies (7 of 11 patients, 64%); the association of negative cytologic results with the recent administration of systemic corticosteroids was noted. immunophenotyping of the aqueous confirmed the diagnosis of hematologic malignancy for 3 of 5 patients examined. panuveitis was the most frequent manifestation of malignant UMS. Intraocular lymphomas presented with isolated vitreitis (n = 6), chorioretinal lesions (n = 5) and iris infiltration (n = 2). Clinical presentation of nonmalignant UMS was diverse but consisted mainly of abnormalities of the retinal vasculature. CONCLUSIONS: UMS was diagnosed in 5% of the patients with uveitis at a tertiary center. Despite the variety of underlying disorders and different clinical presentations, a high frequency of malignant and vascular diseases was found. awareness of the clinical manifestations of UMS and application of the correct diagnostic procedures should promote timely diagnosis and treatment, which are essential not only for visual acuity but also for the life of the patient.
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3/33. Latent intracellular Epstein-Barr Virus dna demonstrated in ocular posttransplant lymphoproliferative disorder mimicking granulomatous uveitis with iris nodules in a child.

    A case of intraocular posttransplant lymphoproliferative disorder (PTLD) is described in a 9-year-old female who underwent orthotopic liver transplantation at the age of 18 months. The prevalence of ophthalmic involvement in PTLD can be expected to rise with the increasing number of major organ transplantations, as well as improved survivorship. Children are particularly at risk for this posttransplant complication because they are usually seronegative for the Epstein-Barr virus (EBV) prior to transplant. Accurate diagnostic classification of PTLD to include confirmation of EBV infection carries significant therapeutic and prognostic implications.
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keywords = iris
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4/33. Hypopyon uveitis and iris nodules in non-Hodgkin's lymphoma: ocular relapse during systemic remission.

    We report the case history of a patient with peripheral non-Hodgkin's lymphoma in systemic remission, who presented with a pseudohypopyon and iris nodules. iris biopsy confirmed the presence of intraocular tumour, which subsequently responded to orbital radiation. Ocular remission, however, was soon followed by a systemic relapse, suggesting that the eye may act as a sanctuary site for tumour cells during chemotherapy, and that cells may metastasize from the eye back to the periphery during systemic remission.
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ranking = 2.5
keywords = iris
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5/33. iris and anterior chamber involvement in acute lymphoblastic leukemia.

    The authors describe a 6-year-old child, with a history of acute lymphoblastic leukemia, who presented with leukemic infiltration of the anterior segment of one eye mimicking anterior uveitis. After tissue diagnosis, administration of radiation therapy to the affected eye resulted in dramatic improvement of the condition. Involvement of the iris and anterior chamber is a recognized but unusual manifestation of leukemia. In the absence of systemic relapse, local radiation therapy without chemotherapy is highly effective, although the prognosis in these patients may be guarded.
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keywords = iris
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6/33. Ocular manifestations in Blau syndrome associated with a CARD15/Nod2 mutation.

    PURPOSE: To report cases of Blau syndrome with a CARD15/Nod2 mutation. DESIGN: Observational and interventional case report. PARTICIPANTS: A 10-year-old Japanese boy (proband) was seen with secondary angle-closure glaucoma (iris bombe), uveitis, skin rashes, and camptodactyly. His sister had posterior synechia and camptodactyly. She had iritis in both eyes during the follow-up period. Both eyes of the father were phthisical because of granulomatous uveitis and secondary glaucoma. The father also had camptodactyly. methods: Surgery was performed to release the iris bombe. Ocular inflammation was treated by topical and systemic steroids. biopsy specimens from the skin rash and from the iris (from iridectomy) were obtained from the proband. Genetic analyses were performed on the proband, his sister, and their mother for a CARD15/Nod2 mutation. MAIN OUTCOME MEASURES: Clinical features, pathologic findings of the skin and iris specimens, and genetic analysis of the CARD15/Nod2 gene. RESULTS: phacoemulsification, intraocular lens implantation, and peripheral iridectomy released the iris bombe. The biopsy specimen from the skin rash showed noncaseating, granulomatous infiltration with epithelioid cells and lymphocytes. The iridectomy specimen showed nonspecific inflammation. Systemic and topical steroid therapy partly reduced the ocular inflammation. Genetic analyses showed that the proband and his sister had an R334W mutation in the CARD15/Nod2 gene, but their mother was of the wild type. CONCLUSIONS: Blau syndrome should be considered in the differential diagnosis of childhood uveitis. Genetic analysis of the CARD15/Nod2 gene is helpful in the diagnosis.
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ranking = 2.5
keywords = iris
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7/33. EBV-induced polymorphic lymphoproliferative disorder of the iris after heart transplantation.

    BACKGROUND: Posttransplantation lymphoproliferative disorder (PTLD) of the iris is a rare entity with only ten cases having been published as yet. Its clinical aspect is typical. Therapy is multimodal and affords an interdisciplinary approach. methods: A 7-year-old boy developed a lymphoproliferative mass of the iris with uveitis 4 years after heart transplantation and immunosuppression. A progressive, flesh-colored thickening of the iris with secondary angle closure glaucoma necessitated a diagnostic and therapeutic iridectomy. Morphological investigation of the iris specimen disclosed a polymorphic posttransplantation lymphoproliferative disorder (PTLD) and the presence of Epstein-Barr virus (EBV) within the tissue. The EBV load in peripheral blood monocytes was massively elevated, thus indicating a chronic EBV infection. After conservative treatment and radiation therapy, the iris mass quickly resolved. There was no evidence of systemic PTLD. CONCLUSIONS: PTLD is a well-known, EBV-induced entity that rarely affects the eye. EBV is principally detectable in specimens of iris PTLD. If conservative, antiviral treatment fails, the iris lesions can be treated by local radiation therapy with very good success. In the near future, patients with PTLD of the eye may benefit from immunologic treatment with ex vivo generation of virus-specific t-lymphocytes.
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ranking = 5.5
keywords = iris
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8/33. Choroidal circulatory disturbance in ocular sarcoidosis without the appearance of retinal lesions or loss of visual function.

    BACKGROUND: Birdshot chorioretinopathy, acute posterior multifocal placoid pigment epitheliopathy, and retinal pigment epithelial detachment have been reported as rare manifestations associated with sarcoidosis, suggesting that ocular sarcoidosis may affect the choroidal circulation. We report a case of ocular sarcoidosis representing a choroidal circulatory disturbance without the appearance of retinal lesions or loss of retinal function. CASE: A 20-year-old woman was referred with blurred vision in the left eye. Inflammatory change in the anterior segment of the eye was noted with multiple nodules on the iris. OBSERVATIONS: Hematological examination revealed elevated lysozyme levels. Bilateral hilar lymphadenopathy was noted on chest X-ray. Specimens obtained by transbronchial lung biopsy revealed granuloma with Langhans giant cells, which led to the diagnosis of sarcoidosis. The eye was treated with topical steroid. The symptoms and the inflammatory change in the anterior segment disappeared within 10 days. However, despite the normal appearance of the ocular fundus, fluorescein angiography revealed multiple puncta of hyperfluorescence. In indocyanine green angiography, a filling delay was noted in the area corresponding to the punctate lesions. Static visual field testing and multifocal electroretinography showed no significant changes. At the last visit, 15 months after the left eye became asymptomatic, the choroidal lesions had disappeared with no residual alteration of the funduscopic appearance or visual function. CONCLUSIONS: This case indicates that choroidal circulatory disturbance can underlie ocular sarcoidosis even in the absence of funduscopically detectable lesions and loss of visual function.
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ranking = 0.5
keywords = iris
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9/33. Ischemic retinopathy and uveitis in a patient with tetralogy of fallot.

    PURPOSE: To describe ischemic retinopathy in a patient with tetralogy of fallot. DESIGN: Interventional case report. TESTING: Clinical and imaging evaluation. MAIN OUTCOME MEASURES: Clinical, imaging, and laboratory findings in a patient with tetralogy of fallot. RESULTS: A 20-year-old female patient with tetralogy of fallot had progressive visual loss of 3 weeks duration. Bilateral examination revealed dilated, tortuous, conjunctival vessels; prominent anterior chamber reaction; iris neovascularization; posterior synechia; retinal vascular tortuosity in both eyes; and inferior exudative retinal detachment. fluorescein angiography revealed delayed retinal and choroidal filling. The working diagnosis was ischemic retinopathy with uveitis. The patient was treated for 6 months with a high-dose oral corticosteroid combined with a topical corticosteroid, a topical mydriatic, and panretinal photocoagulation. Conjunctival, vascular congestion subsided with a decrease in anterior chamber reaction. The inferior, exudative retinal detachment resolved, and vision was restored. CONCLUSIONS: Retinal ischemic syndrome combined with uveitis can develop in patients with tetralogy of fallot. Treatment can restore vision in such patients.
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keywords = iris
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10/33. Anterior segment ischemia in chronic herpes simplex keratouveitis.

    Six patients with chronic herpes simplex keratouveitis developed a rapidly progressive ocular inflammation unresponsive to corticosteroid and antiviral therapy. Severe secondary glaucoma unresponsive to maximum medical therapy ensued and was treated by cyclocryotherapy in five patients. Ocular ischemia with secondary corneal and scleral calcification subsequently developed in all six patients. All had also received prolonged topical therapy with topical antiviral agents, corticosteroids, beta adrenergic blockers and epinephrine compounds. Three eyes eventually required enucleation for the relief of pain; one stabilized, and two others became phthisical. One of the phthisical eyes developed a secondary fungal endophthalmitis. Histopathologic examination of the three enucleated globes revealed extensive corneal, scleral and conjunctival calcification, secondary angle closure, iris and ciliary body necrosis, focal choroiditis, retinal necrosis and atrophy. The syndrome recognized in these patients appears to be a rare but devastating complication of herpes simplex keratouveitis, possibly exacerbated by the application of cyclocryotherapy and other factors.
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