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1/47. Histopathological findings in proliferative membrane from a patient with sarcoid uveitis.

    BACKGROUND: Sarcoid uveitis is occasionally accompanied by proliferative changes, such as retinal neovascularization and vitreous hemorrhage. Steroid administration, retinal photocoagulation, and vitrectomy may be indicated in such proliferative cases. CASE: A 19-year-old woman presented with proliferative sarcoid uveitis accompanied by recurrent vitreous hemorrhage. OBSERVATIONS: At the initial examination, bilateral vitreous opacity, retinal exudates, mild vitreous hemorrhage, retinal vasculitis, and neovascularization of the retina and optic disc were observed. Although prednisolone was administered and panretinal photocoagulation was performed several times, recurrent vitreous hemorrhage continued. Since the vitreous hemorrhage was not absorbed, pars plana vitrectomy and lensectomy were performed. After surgery, neovascularization and intraocular inflammation decreased, and the corrected visual acuity in the right eye improved to 20/50. Histopathologic analysis of the proliferative membrane removed during surgery revealed substantial neovascularization and numerous neutrophils in the vessels. CONCLUSIONS: Based on these findings, an inflammatory reaction as well as retinal ischemia were thought to be involved in the proliferative changes in this patient.
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2/47. Vitreoretinal findings similar to retinopathy of prematurity in infants with compound heterozygous protein s deficiency.

    OBJECTIVE: To present previously undescribed vitreoretinal findings similar to severe retinopathy of prematurity (ROP) in two siblings (daughter and son) with a thrombophilic disorder, compound heterozygous protein S (PS) deficiency. DESIGN: family genotype study and literature review. PARTICIPANTS: Two unrelated heterozygous PS-deficient parents and their two children with compound heterozygous PS deficiency were studied. The gestational age and birth weight of the daughter were 40 weeks and 3200 g, respectively, and those of the son were 34 weeks and 2150 g, respectively. Three other neonates with homozygous or compound heterozygous PS deficiency and ophthalmologic findings were identified in the literature. INTERVENTION: The daughter underwent lensectomy-vitrectomy at 48 weeks adjusted age bilaterally. The son underwent therapy developed for severe ROP: laser therapy of the peripheral avascular retina at 39 weeks adjusted age, and bilateral lensectomy-vitrectomy with membrane peel of intravitreous proliferation from the optic disc at 42 weeks adjusted age. MAIN OUTCOME MEASURES: The main clinical outcome measures were retinal appearance and functional vision. Genotypes of the family members were determined. RESULTS: One of the four eyes retained functional vision. A normal-appearing posterior retina, normal scotopic and photopic flash electroretinograms, and a normal flash visual-evoked response were documented from the left eye of the son at 62 weeks adjusted age. The other three eyes had inoperable retinal detachments and no functional vision. The mother had type I PS deficiency and the father had type II PS deficiency. Compound heterozygous PS deficiency was confirmed in both children. CONCLUSION: In both children, normal vasculogenesis was interrupted. At 39 weeks adjusted age, the retinal examination of the son revealed extraretinal fibrovascular proliferation at the optic disc (reactivation of the hyaloid system) and in the peripheral retina (interruption of inner retinal vascularization). patients with homozygous or compound heterozygous PS deficiency may present as infants with severe ROP. The authors' experience suggests that appropriately timed surgical procedures, which are efficacious for ROP, can preserve vision in infants with thrombophilic disorders.
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3/47. radiation-associated choroidal neovasculopathy, exudative detachment and neovascular glaucoma. A case report.

    radiotherapy remains a controversial type of therapy for subfoveal neovascularization. Recently a peculiar pattern of neovascular growth of the irradiated choroidal neovascular membrane has been described. This evolution may be associated with extensive exudative reaction. In one of our patients with this complication, the disease progressed to a total exudative retinal detachment and neovascular glaucoma.
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4/47. choroideremia associated with subretinal neovascular membrane.

    PURPOSE: To report two Japanese patients with choroideremia, one male and one heterozygous female, who developed subretinal neovascular membrane and/or subretinal fibrosis in the intermediate stage of the disease, and, in addition, to describe marked clinical manifestation in a heterozygous carrier female. METHOD: Two patients were examined by slip-lamp biomicroscopy, ophthalmoscopy and other ophthalmoloscopic examinations. RESULTS: Two cases showed moderately advanced ophthalmoscopic and functional abnormalitities compatible with choroideremia, and in addition, subretinal lesion in the fovea and/or midperiphery. CONCLUSION: The intermediate stage of choroideremia may occasionally be complicated with choroidal neovascular membrane in the fovea, the midperiphery, or both, which resolves spontaneously and results in subretinal fibrous scarring. The occurrence of this complication in the fovea leads to episodic central visual loss, while midperipheral lesion may remain unrecognized.
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5/47. Malattia leventinese presenting with subretinal neovascular membrane and hemorrhage.

    PURPOSE: To report a case of malattia leventinese involving subretinal hemorrhage. methods: Case report. RESULTS: Two weeks after initial presentation, the visual acuity of this 34-year-old man decreased to LE: 20/100. Funduscopic evaluation revealed a subretinal hemorrhage involving the center of the foveal in the left eye that was interpreted as secondary to a neovascular membrane on fluorescein angiography. The patient did well after the removal of the submacular material by pars plana vitrectomy. CONCLUSION: patients with malattia leventinese may occasionally present with submacular hemorrhage. Prompt diagnosis and intervention may enhance the patient's chance for visual improvement.
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6/47. Juxtapapillary subretinal pigment epithelial polypoid pseudocysts associated with unilateral tilted optic disc: case report with clinicopathologic correlation.

    OBJECTIVE: To describe the unique preoperative appearance, successful postoperative clinical course, and histopathologic features of a cluster of progressively enlarging pseudocysts that arose at the temporal margin of a unilateral tilted optic disc. STUDY DESIGN: Case report. methods: Clinical observation, color fundus photography, fluorescein angiography, and optical coherence tomography, as well as routine histologic and immunohistochemical studies of tissue removed by subretinal surgery. RESULTS: Subretinal surgical excision of the lesions resulted in retinal reattachment with improved postoperative visual acuity. Histologic examination disclosed a cluster of fluid-filled polypoid pseudocysts lined by small vessels of choroidal origin lying beneath the basement membrane of the overlying retinal pigment epithelium (RPE). CONCLUSIONS: We postulate that buds of small vessels of choroidal origin grew through or around the edge of Bruch's membrane at the temporal margin of the tilted optic disc and then passed under the juxtapapillary RPE. Ensuing leakage of proteinaceous fluid from these vessels eventuated in formation of a cluster of polypoid pseudocysts and subsequent localized papillomacular retinal separation with visual loss. The lesions were amenable to subretinal surgical removal with restoration of visual acuity.
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7/47. Photodynamic therapy of a subretinal neovascular membrane in type 2A idiopathic juxtafoveolar retinal telangiectasis.

    PURPOSE: To report the outcome of a patient with a subretinal neovascular membrane secondary to idiopathic juxtafoveolar retinal telangiectasis treated with photodynamic female therapy. methods: Interventional case report. A 43-year-old female with type 2A idiopathic juxtafoveolar retinal telangiectasis presented with a 2-week history of decreased vision and a subretinal neovascular membrane in the left eye. RESULTS: Visual improvement from 20/70-2 to 20/50-2 in the left eye was observed after two photodynamic therapy treatments of a juxtafoveal subretinal neovascular membrane with verteporfin, and was maintained through 7 months of follow-up. fluorescein angiography showed no leakage specific to the subretinal neovascular membrane, but leakage from the juxtafoveal telangiectasis continued. CONCLUSION: A substantial improvement was seen in this patient after treatment of the juxtafoveal subretinal neovascular membrane by photodynamic therapy. Given the poor prognosis of these lesions, photodynamic therapy may therefore be a useful treatment for patients with subretinal neovascular membranes associated with idiopathic juxtafoveolar retinal telangiectasis.
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8/47. Peripapillary subretinal neovascularization in sarcoidosis: remission and exacerbation during oral corticosteroid therapy.

    BACKGROUND: In sarcoidosis, peripapillary subretinal neovascularization is rare. The role of corticosteroid therapy for subretinal neovascularization is controversial. CASE: A 38-year-old female patient weighing 38 kg with histologically diagnosed sarcoidosis presented with peripapillary subretinal neovascularization, retinal phlebitis, a hyperemic disc, and snowball vitreous opacities in the left eye. observation: Oral betamethasone therapy at an initial dose of 3 mg/day reduced the size of subretinal neovascular membrane, and the membrane became fibrous. Despite the total initial 140 mg of betamethasone given over 2.5 months and the additional total 700 mg of prednisolone given over the next 2 months, the subretinal neovascularization recurred. Six months after the first recurrence, a second recurrence developed during the tapering-off period of oral corticosteroid therapy. At the second recurrence, the oral corticosteroid therapy was ineffective in reducing the size of the neovascular membrane. CONCLUSION: In our patient, oral corticosteroids temporarily suppressed peripapillary subretinal neovascularization but failed to prevent extension of neovascular membrane to the fovea because of recurrent sarcoidosis. Over time, oral corticosteroids appear to lose their effectiveness for treating repeated recurrence of peripapillary subretinal neovascularization associated with sarcoidosis.
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9/47. Optical coherence tomography in choroidal neovascular membrane associated with Best's vitelliform dystrophy.

    A 29-year-old black male with Best's dystrophy presented an elevated choroidal neovascular membrane in the right eye that was diagnosed and followed with fluorescein-indocyanine green angiography and optical coherence tomography. The subretinal neovascularization was successfully treated with argon laser photocoagulation. One month later, the visual acuity improved and an optical coherence tomography confirmed regression of the serous macular detachment. The final clinical picture remained stable for 12 months of follow-up.
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10/47. Massive vascular endothelium growth factor (VEGF) expression in Eales' disease.

    BACKGROUND: Eales' disease is an idiopathic retinal vasculitic and vaso-occlusive process complicated by extensive retinal neovascularisation and vitreous hemorrhages. The great propensity to produce retinal neovessels is one of the particular aspects of the disease that deserves to be further investigated. We report a case of Eales' disease having evolved over more than three decades, with a typical clinical presentation in one eye, while the other eye had to be enucleated because of a terminal neovascular glaucoma, thus allowing pathological examination. methods: The functional right eye was treated by vitrectomy, cerclage, cryocoagulation and endolaser. The non-functional phthitic left eye was enucleated and submitted for histopathological and immunohistochemical examination using antibodies against vascular endothelial growth factor, T-cells, B-cells and Muller cells. RESULTS: Evolution was favourable in the operated right eye, following management of the inflammatory reaction. The histopathological examination of the left eye revealed an occlusion of the anterior chamber angle by rubeosis iridis, tractional retinal detachments, pre-, intra- and sub-retinal neovascular membranes, and vitrous hemorrhages. Diffuse positive anti-VEGF immunostaining was found at the level of the retinal neovascular membranes. The retina exhibited prominent Muller cell immunostaining, indicating extensive gliosis, and predominantly B cell infiltrates were found in the eye. CONCLUSION: The present study indicates a close relationship between the prominent neovascular proliferation in Eales' disease and the intense expression of VEGF. The increased expression of VEGF, when compared to other conditions inducing neovascularisation, might explain the severity of neovascular growth and the propensity of repeated vitrous hemorrhages in Eales' disease.
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