1/58. 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.- - - - - - - - - - ranking = 1keywords = detachment, retinal detachment (Clic here for more details about this article) |
2/58. Focal retinal pigment epithelial dysplasia associated with fundus flavimaculatus.BACKGROUND: One or more focal dysplastic lesions of the retinal pigment epithelium (RPE) occurred in 15 eyes of 10 patients with fundus flavimaculatus. methods: review of patient records including an attempt to obtain follow-up information concerning a history of previous ocular trauma. RESULTS: Mild antecedent ocular trauma occurred to the eye with a dysplastic lesion in two patients. Dysplastic lesions were most frequently solitary and located temporal to the macula. Subretinal neovascularization accompanied two of the dysplastic lesions. The lesions were multifocal and present bilaterally in two patients. CONCLUSIONS: In fundus flavimaculatus, progressive lipofuscin storage is responsible for engorgement and hypertrophy of the RPE. Dysplastic lesions of the RPE probably result from reactive hyperplasia and fibrous metaplasia of RPE cells in response to acute disruption of fragile, hypertrophied RPE cells that may be enormously enlarged in the area of yellow flecks. This disruption may occur in response to trauma, focal inflammation, or other localized stimuli. patients with fundus flavimaculatus should be cautioned concerning the possible role of trauma in causing dysplastic changes in the RPE and visual loss.- - - - - - - - - - ranking = 3.0903673021418keywords = retinal pigment, pigment (Clic here for more details about this article) |
3/58. 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.- - - - - - - - - - ranking = 0.66938585863225keywords = retinal pigment, pigment (Clic here for more details about this article) |
4/58. 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.- - - - - - - - - - ranking = 3.7236230521217keywords = detachment, retinal detachment (Clic here for more details about this article) |
5/58. Idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy.PURPOSE: To report a patient who had concomitant idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy. METHOD: Case report. A 46-year-old black woman presented with sudden onset of "blurring" in the left eye. Her ocular history was unremarkable, and her medical history was relevant for sickle cell disease with yearly episodes of sickle cell crises. RESULTS: Both eyes had evidence of idiopathic polypoidal choroidal vasculopathy and peripheral retinal neovascularization secondary to sickle cell disease. The left eye had hemorrhagic retinal pigment epithelial detachments secondary to idiopathic polypoidal choroidal vasculopathy. CONCLUSION: We present this case as an example of concomitant sickle cell retinopathy and idiopathic polypoidal choroidal vasculopathy. We are unaware of previous reports with these findings.- - - - - - - - - - ranking = 41.523777631822keywords = pigment epithelial detachment, epithelial detachment, detachment, retinal pigment, pigment (Clic here for more details about this article) |
6/58. Familial spastic paraplegia and maculopathy with juxtafoveolar retinal telangiectasis and subretinal neovascularization.PURPOSE: To describe a previously unreported condition involving familial spastic paraplegia and a peculiar type of maculopathy. methods: Two pairs of siblings were studied. All four cases underwent serial clinical examinations, fundus photography, and fluorescein angiography. Two siblings had extensive investigations. RESULTS: Characteristics of the four cases include spastic paraplegia diagnosed in the first decade of life and visual loss at about age 9 due to a maculopathy with an abnormal vascular complex. In the early stages, parafoveal dilatation of the capillary network was noted. The later stages were characterized by cystic macular degeneration, and seven of eight eyes developed fibrovascular scars with retinochoroidal anastomoses, pigment migration, and atrophic changes. In two siblings, electro-oculographic findings were subnormal, whereas results of electroretinography, magnetic resonance imaging of the brain and spinal cord, and metabolic and karyotype studies were normal. These siblings were an Indonesian girl and boy; the other siblings were white males. There was no consanguinity of the parents and family history was unremarkable. CONCLUSIONS: This study suggests that the two pairs of siblings have an identical familial and probably recessive disorder with neurodegenerative changes that have caused paraplegia and a peculiar maculopathy associated with anomalous retinal vascular complexes, retinochoroidal anastomoses, and subretinal neovascularization.- - - - - - - - - - ranking = 0.051312398203883keywords = pigment (Clic here for more details about this article) |
7/58. 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.- - - - - - - - - - ranking = 0.61807346042836keywords = retinal pigment, pigment (Clic here for more details about this article) |
8/58. Central serous retinopathy complicating systemic lupus erythematosus: a case series.Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with widespread manifestations including the eye. Central serous retinopathy (CSR) has been associated as a complicating event in SLE, although it is uncommon. We present a case series of four female Chinese SLE patients who developed CSR during the course of their systemic disease. All four presented clinically with typical CSR. Angiographic findings did not show evidence of choroidal ischaemia or delayed choroidal filling. Resolution of the serous retinal detachment occurred in all four patients. Recovery of vision was seen in three patients. The clinical outcome was similar to that occurring in the usual male population. Central serous retinopathy as a manifestation of SLE may be caused by various factors. These include SLE-associated choroidopathy, systemic hypertension, renal disease, retinal pigment epithelial dysfunction and glucocorticoid therapy.- - - - - - - - - - ranking = 1.6180734604284keywords = detachment, retinal pigment, retinal detachment, pigment (Clic here for more details about this article) |
9/58. retinitis pigmentosa associated with peripheral sea fan neovascularization.PURPOSE: To describe a case with retinitis pigmentosa associated with sea fan type retinal neovascularization. methods: Complete ocular examination including fluorescein angiography was performed in a 9-year-old girl. RESULTS: Ophthalmoscopically, in addition to arteriolar narrowing and bone corpuscular pigmentation of both retinae, a vascular lesion with surrounding intraretinal exudation was noted in the upper equatorial region of the right eye. On fluorescein angiography, the lesion stained in the form of a sea fan neovascularization. CONCLUSION: Sea fan type of neovascularization can be seen in association with retinitis pigmentosa. fluorescein angiography is important in identifying the exact nature of such a lesion.- - - - - - - - - - ranking = 0.35918678742718keywords = pigment (Clic here for more details about this article) |
10/58. 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.- - - - - - - - - - ranking = 2.2565619910194keywords = detachment, retinal detachment, pigment (Clic here for more details about this article) |
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