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1/46. 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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ranking = 1
keywords = optic
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2/46. 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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ranking = 2
keywords = optic
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3/46. Histopathologic study of presumed parafoveal telangiectasis.

    PURPOSE: To report the postmortem histopathologic features that closely resemble the clinical features of parafoveal telangiectasis. methods: light and electron microscopy. RESULTS: Histopathologic features included macular edema; telangiectatic vessels; retinal, subretinal, and superficial retinal neovascularization; retinal pigment epithelial hyperplasia around neovascular aggregates; retinal-choroidal vascular anastomosis; and superficial pigmented cells with lipofuscin. CONCLUSION: The postmortem histopathologic findings in a 36-year-old woman with down syndrome and other systemic conditions correlate with features noted in previous reports of presumed parafoveal telangiectasis.
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ranking = 0.015553081868001
keywords = edema
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4/46. retinal vasculitis occurring with common variable immunodeficiency syndrome.

    PURPOSE: To report severe retinal vasculitis causing decreased vision in three patients with the common variable immunodeficiency syndrome. METHOD: Case report. Three patients with common variable immunodeficiency syndrome developed decreased vision secondary to retinal vasculitis. fluorescein angiography was performed in all three patients. Peribulbar injections were given in one patient, and two patients were treated with oral steroids and cyclosporin. RESULTS: All three patients were young and had classic common variable immunodeficiency syndrome. Bilateral retinal vasculitis and diffuse retinal edema were present in all three patients, and two patients had retinal neovascularization in the absence of ischemia. No evidence of intraocular infection was present, and none was detected systematically. visual acuity decreased in five of the six eyes and was responsive to treatment in only one patient (both eyes). CONCLUSION: retinal vasculitis may be another autoimmune manifestation of common variable immunodeficiency syndrome.
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ranking = 0.015553081868001
keywords = edema
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5/46. Posterior segment neovascularization associated with acute ophthalmic artery obstruction.

    PURPOSE: To report previously unrecorded ocular neovascularization associated with acute ophthalmic artery obstruction (AOAO) that presents clinical manifestations of acute concomitant obstruction of the retinal and posterior ciliary circulations. methods: This report documents the clinical, fluorescein angiographic, and histopathologic findings in two patients with AOAO followed by posterior segment neovascularization. RESULTS: Ophthalmoscopic findings showed whitening of the posterior pole, arterial attenuation, and a pale optic disk. Serial fluorescein angiograms showed a nearly total shutdown of choroidal and retinal perfusion, degeneration of the retinal pigment epithelium and choriocapillaris, and eventual development of a huge neovascular frond at the posterior pole. Histopathologic examination of the enucleated eyeball showed inner retinal necrosis caused by central retinal artery obstruction, degeneration of the outer retina with choriocapillaris obstruction caused by impairment of choroidal circulation, and a thick preretinal neovascular frond at the posterior pole. CONCLUSIONS: These results suggest that AOAO can induce ocular neovascularization, which to the authors' knowledge has not yet been reported.
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ranking = 4.6388018970555
keywords = optic disk, optic, disk
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6/46. Laser photocoagulation in preproliferative retinopathy of incontinentia pigmenti.

    incontinentia pigmenti is a rare, X-linked, dominant disorder in which affected female infants develop characteristic abnormalities of the skin, central nervous system, hair, teeth, and eyes. Ocular abnormalities occur in about 35% of patients and consist of proliferative vitreoretinopathy, retinal detachment, strabismus, cataract, microphthalmia, optic nerve atrophy, and iris hypoplasia. Retinal vascular abnormalities, ranging from peripheral retinal avascularity to neovascular and fibrous proliferation with traction retinal detachment, are the primary cause of severe visual dysfunction in patients. Therapeutic intervention with laser photocoagulation and cryotherapy for the proliferative vitreoretinopathy of incontinentia pigmenti has met with variable success. We report a case in which laser photocoagulation was used at the onset of retinopathy with subsequent resolution of the vasculopathy.
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ranking = 1
keywords = optic
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7/46. 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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ranking = 7
keywords = optic
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8/46. 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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ranking = 2
keywords = optic
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9/46. Retinal manifestations of ophthalmic artery hypoperfusion.

    ophthalmic artery hypoperfusion is a relatively uncommon clinical entity. This study illustrates the posterior segment findings of ophthalmic artery hypoperfusion in a series of nine patients. Colour photographs and relevant fluorescein angiograms highlighting the findings are shown. The retinal manifestations of ophthalmic artery hypoperfusion in this series of patients include midperipheral haemorrhages, dilated retinal veins, optic disk collaterals, optic disk neo-vascularization, cotton wool spots, grey intraretinal lesions, fundus pallor, optic disk swelling and choroidal infarcts. Recognition of the ophthalmic changes in this condition may lead to detection of carotid artery disease, the surgical and medical treatment of which has important bearing on patient management.
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ranking = 13.916405691166
keywords = optic disk, optic, disk
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10/46. Increased diode laser uptake in inner retinal layers after indocyanine green staining of the internal limiting membrane.

    Intraoperative use of indocyanine green to stain the internal limiting membrane assists in its visualization and removal. This article describes increased uptake of diode laser energy by the superficial layers of the retina after indocyanine green staining of the internal limiting membrane in a patient with diabetic macular edema and a taut, attached posterior hyaloid. The patient was undergoing pars plana vitrectomy, removal of the posterior hyaloid, and indocyanine green-assisted peeling of the internal limiting membrane. In addition, panretinal diode laser endophotocoagulation was performed after discovering retinal neovascularization. In areas that were more intensely stained with indocyanine green, the clinical appearance and optical coherence tomography scans demonstrated markedly increased laser energy uptake in the superficial layers of the retina. This case indicates near-infrared or infrared laser procedures performed in areas of indocyanine green-stained internal limiting membrane may necessitate adjustment of laser power and technique.
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ranking = 1.015553081868
keywords = optic, edema
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