1/20. Coats-like lesions in Usher syndrome type II.BACKGROUND: An unusual case of Usher syndrome type II associated with bilateral Coats-like exudative retinopathy is described. methods: A 14-year-old boy with congenital sensorineural deafness and normal vestibular functions presented with a recent history of night blindness. He was followed for 3 years with fundus photography, intravenous fluorescein angiography, electroretinography and audiometric testings. His parents refused any form of treatment. RESULTS: Fundoscopy showed bilateral retinitis pigmentosa and a single focus of subretinal exudation and overlying telangiectatic retinal vessels inferotemporal to the vascular arcade in the right eye. He had bilateral mild macular edema. A year later, a similar lesion developed inferotemporally in the left fundus. electroretinography responses, particularly the rod-mediated signals, were significantly reduced. Audiometric studies documented hearing loss in high frequencies. His visual acuity declined from 20/40 to 20/80 RE and from 20/80 to 20/100 LE during follow-up. No new lesions developed. CONCLUSIONS: Coats-type exudative lesions may develop in patients with Usher syndrome type II. Although left untreated, only a minimal increase in exudation occurred over 3 years.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/20. Midperipheral mottling pigmentation with familial choroidal osteoma.PURPOSE: To describe a rare presentation of familial choroidal osteoma in two siblings. methods: The clinical findings in two siblings over 4 years' follow-up. RESULTS: Two brothers (15 and 12 years old) had bilateral choroidal osteomas. Both had bilateral peripapillary yellowish-white lesions and midperipheral mottling pigment appearance, which are not seen in sporadic cases. Extensive midperipheral area with mottling pigment appearance was noted by fluorescein angiography (FA) as scattered multiple hyperfluorescent dots. The yellowish-white lesions showed diffuse hyperfluorescence with FA and hypofluorescence with indocyanine green angiography (ICG). ICG also revealed irregular hyperfluorescent areas within the tumor, indicating abnormal choroidal vessels on the tumor. In the left eye of the younger brother, the subretinal fibrosis due to choroidal neovascularization superior to the macula extended down toward the foveal region over 2 years, resulting in visual deterioration. CONCLUSION: The midperipheral mottling pigment appearance of familial choroidal osteoma cases is unique and different from most sporadic cases, suggesting that familial choroidal osteoma might have separate etiologic or modified factors.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/20. Bilateral pigmented retinopathy following measles: long-term follow-up and possible association with keratoconus.A case of pigmentary retinopathy following measles is presented with follow-up of 24 years. A 29-year-old man complained of almost complete blindness during measles infection at the age of 5 (1966). ophthalmoscopy revealed retinal edema with pallor of the optic disc and in a few months pigment alterations gave the fundus a salt-and-pepper appearance. In 1974 and 1985 a pigmentary retinopathy was present with optic atrophy and typical bone corpuscle deposits, and marked impairment of visual function. In 1990 (i.e. 24 years after the onset of symptoms) the fundus shows pigmentary degeneration with optic atrophy and narrowed vessels, ERG is extinguished and visual fields and dark adaptation cannot be recorded owing to amaurosis. The patient also present bilateral keratoconus. The possible association between these two entities, which to our knowledge has not been previously reported, is discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/20. retinitis pigmentosa and retinal neovascularization.Four patients with retinitis pigmentosa and either disc or peripheral retinal neovascularization with recurrent vitreous hemorrhage are described. One patient with peripheral retinal neovascularization also had rubeosis and neovascular glaucoma. The effects of relative hyperoxia on the retinal microcirculation in retinitis pigmentosa as well as intraocular inflammation may account for such changes. Laser photocoagulation appears effective in preventing vitreous hemorrhage in these patients, but systemic administration of corticosteroids did not cause the new vessels to regress.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/20. retinitis pigmentosa: immunohistochemical and biochemical studies of the retina.Several immunohistochemical and biochemical characteristics of retinas from two patients with retinitis pigmentosa (RP) were examined. Histochemically, interphotoreceptor retinoid-binding protein (IRBP) was found in the interphotoreceptor space in small areas of preserved peripheral retina, although enzyme-linked immunosorbent assay suggested that little IRBP was present. antibodies to glial fibrillary acidic protein and factor viii-related antigen corroborated retinal gliosis and retinal vessel attenuation, respectively, in the RP cases. Fibronectin showed similar staining in normal and affected tissue.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/20. Autosomal recessive pericentral pigmentary retinopathy.A brother and sister, born to consanguineous parents, had pigmentary retinopathy in a pericentral distribution. The retinopathy was noted in infancy when the siblings were examined for strabismus. The optic disks, maculae, and retinal vessels were normal. There was mild reduction in amplitude of both scotopic and photopic electroretinographic responses. Both patients had moderate hyperopic astigmatism and esotropia. The fundus and visual acuity remained unchanged over periods of nine and 13 years in the brother and sister, respectively. Results of ocular examinations on the father, mother, and an older sister were normal. These findings support an autosomal recessive mode of inheritance in this family with pericentral pigmentary retinopathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/20. Pigmentary retinal degeneration and Coats' disease: a case study.We report a 22-year-old Hispanic man with familial congenital nystagmus, iris transillumination, and pigmentary retinal anomalies with subretinal fatty exudates similar to those seen in Coats' disease. Direct treatment of the telangiectatic vessels with cryotherapy caused a subretinal hemorrhage. We therefore recommend surrounding the area of abnormal vessels (posterior to vessels and between vessels and ora serrata) with cryotherapy to reverse the formation of the vascular anomalies.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
8/20. Snowflake degeneration: an independent entity or a variant of retinitis pigmentosa?We examined eight members of three generations of a family with a history of hereditary retinal degenerative disease. Four affected members have multiple whitish retinal dots resembling snowflakes. Other positive ocular findings in the four members with retinal flakes include myopia, cataract, fibrillary vitreous degeneration with occasional strands, attenuated retinal arterioles, sheathed retinal vessels, occlusion of small retinal vessels, white with and without pressure, annular macular pigment defects, and occasional spicules of pigment. Goldmann perimetry showed irregular annular scotomas in three patients. Electroretinographic response was extinguished in one and subnormal in three. Electro-oculography showed the light rise-to-dark trough ratio to be decreased in the affected patients. Based on results of vitreoretinal biomicroscopy, Goldmann perimetry, and visual electrodiagnostic testing, we suspect that snowflake degeneration may be just a variant of retinitis pigmentosa.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
9/20. Extensive subretinal pigment epithelial deposit in two brothers suffering from dominant retinitis pigmentosa. A histopathological study.The eyes of two brothers with retinitis pigmentosa were removed after death and examined by a variety of techniques, including conventional histology, fluorescence microscopy and both scanning and transmission electron microscopy. Their condition was considered to be of an autosomal dominant type but with some atypical clinical features. The outstanding histological feature in both pairs of eyes was a predominantly acellular deposit of amorphous material situated between the retinal pigment epithelium and Bruch's membrane. This material extended from the disc to beyond the ora serrata. In some regions of the retinae of both brothers, there was a cellular infiltrate into the deposit and this included multinucleate cells. In one brother the deposit was lined externally by a fibrovascular membrane in some few locations. All retinae were degenerate, but all showed preservation of abnormally short and sparse photoreceptor cells in both the peripheral and macular areas. There was only patchy loss of the choriocapillaris, which could have been age-dependent rather than disease-dependent, and the remaining choroidal vessels were patent in all cases. The widespread distribution of the deposit is unusual and suggests that it arises from disordered metabolism of the retinal pigment epithelium. We could not determine whether it was a primary disease process or if it arose as a secondary phenomenon.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/20. Retinal changes in myotonic dystrophy: a clinicomorphological study.This report appears to be the first ultrastructural study of the maculopathy and peripheral pigmentary retinopathy in myotonic dystrophy. Nine eyes from five patients observed during life are described. The findings were similar in all eyes, the retinal pigment epithelium in the macular region containing an accumulation of lipofuscin in large hyperpigmented cells. Pigment-laden profiles found in the subpigment epithelial space or subretinal space were interpreted as an attempt to discharge the pigment. Stress fibres of actin microfilaments were thrown into prominence by the irregularity of the pigment epithelium. In the periphery migration of retinal pigment cells into the retina occasionally resulted in the formation of bone corpuscles around occluded vessels, as occurs in retinitis pigmentosa; but more often the clumps were coarser and surrounded basement membrane material. Central and peripheral epiretinal membranes were also observed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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