1/23. retinal degeneration associated with ectopia lentis.Two brothers had retinal degeneration, lens subluxation, and myopia since early life. There was no evidence of marfan syndrome, homocystinuria, or other systemic disease. They had nystagmus, myopia, inferior dislocation of the lens, and posterior subcapsular opacities in both eyes. Fundus examination showed attenuated retinal vessels, macular atrophy with occasional pigment accumulation as clumps, and perivascular sleeves. electroretinography revealed decreased photopic and scotopic responses. The visual fields were constricted. We believe this to be the first report of retinal degeneration with bilateral lens subluxation in a family. It appears to be inherited in an autosomal recessive fashion.- - - - - - - - - - ranking = 1keywords = myopia (Clic here for more details about this article) |
2/23. Unilateral macular retinoschisis with stellate foveal appearance in two females with myopia.Stellate foveal retinoschisis is considered the hallmark of X-linked retinoschisis. We describe the finding of unilateral retinoschisis with stellate foveal appearance in 2 females with myopia who had no evidence of familial disease. Optical coherence tomography (OCT) and fluorescein angiography were obtained in both cases. Neither patient had a family history of low vision, night blindness, or retinal detachment. visual acuity in the affected eyes was 20/50 and 20/25, respectively. Both fellow eyes had normal fundi, except for mild myopic changes. With OCT, widespread retinal splitting was detected at the posterior pole in the affected eyes, whereas the fellow eyes were normal. In one case, OCT showed that foveal retinoschisis represented the macular involvement of a flat inferior retinoschisis, although this was not clinically apparent. Macular retinoschisis with stellate foveal appearance may rarely be associated with pathologic myopia. OCT was useful to establish the true extension of these macular changes.- - - - - - - - - - ranking = 3keywords = myopia (Clic here for more details about this article) |
3/23. A child with muscle-eye-brain disease. Ophthalmological and neurological characteristics.PURPOSE: To describe a child with Muscle-eye-brain disease (MEB), one of three types of congenital muscular dystrophy associated with ocular abnormalities. methods: Case report. RESULTS: The child showed severe visual impairment due to progressive myopia and retinal degeneration, a pachygyria-type of migration disorder of the brain with a nodular cortical surface, i.e. cobblestone cortex, as well as muscular weakness and severe mental retardation. CONCLUSION: Ophthalmological assessments are important to help to diagnose and follow children with congenital muscular dystrophy.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
4/23. An Italian family affected by autosomal dominant microcephaly with chorioretinal degeneration.PURPOSE: We studied an Italian family affected by the autosomal dominant form of microcephaly and chorioretinal degeneration that was characterized by various degrees of clinical expression. methods: An ophthalmologic examination, including visual acuity, visual field testing, an electroretinogram, and fundus photography, and a neurologic examination, including neurodevelopmental status and neuroimaging studies, were performed for all subjects. Skeletal radiography, chromosome studies, and serologic investigations were also performed. RESULTS: In this family, only two of the six affected members had an association of microcephaly, myopia, and chorioretinal degeneration. The other family members showed microcephaly, slight mental retardation, and short stature, but not chorioretinopathy. CONCLUSIONS: The significant finding in members from this dominant pedigree of microcephaly was the association of short stature and high myopia, heretofore seen only in families with recessive microcephaly. These findings could be useful for genetic counseling in the apparently isolated forms of microcephaly with chorioretinopathy.- - - - - - - - - - ranking = 1keywords = myopia (Clic here for more details about this article) |
5/23. Pseudoinflammatory macular dystrophy.A family with pseudoinflammatory macular dystrophy (PMD) is presented. This dominantly inherited macular dystrophy has its onset in the 3rd to 5th decades with the earliest manifestation being a macular subretinal neovascular network. Visual function tests (ERG, EOG, visual fields, retinal sensitivity) in the early and late stages indicates this is local or geographic disease. This dystrophy should be differentiated from other hereditary causes for subretinal neovascularization (angioid streaks, vitelliform dystrophy, dominant drusen of Bruch's membrane, optic nerve drusen and myopia). It is suggested that treatment be directed at early obliteration of the subretinal neovascularization with intense photocoagulation since the outcome in virtually all cases of untreated PMD is legal blindness.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
6/23. Combined surgical procedure for the prevention of blindness caused by progressive high myopia.A combined prophylactic procedure consisting of the strengthening of the sclera by fascia lata implants and the laser photocoagulation of peripheral retinal degenerations has been developed for the prevention of blindness caused by progressive high myopia. More than 7 years of experience gained with over 400 patients showed that none of the treated eyes developed retinal detachment or other severe complications of high myopia. Moreover, the degree of myopia decreased by a mean value of 3.0 D in 90%, and the visual acuity improved by a mean value of 0.15 in 70% of the patients treated. The authors suggest that their combined prophylactic procedure should be tried out and introduced to general ophthalmological practice.- - - - - - - - - - ranking = 3.5keywords = myopia (Clic here for more details about this article) |
7/23. Vitreoretinal degeneration in spondyloepiphyseal dysplasia congenita.Eighteen patients had ocular changes associated with spondyloepiphyseal dysplasia congenita, a rare cause of congenital dwarfism with normal mental development. Seven patients had nonprogressive myopia of 5.00 or more diopters. Vitreoretinal degeneration was encountered in six patients with high myopia, and vitreous syneresis was present in all patients. Corrected visual acuity was 20/50 or better in all patients. retinal detachment was not encountered, although reports in the nonophthalmic literature claim up to 50% retinal detachment rate and poor visual prognosis in these patients.- - - - - - - - - - ranking = 1keywords = myopia (Clic here for more details about this article) |
8/23. Autosomal recessive vitreoretinopathy and encephaloceles.We conducted a ten-year follow-up of an unusual pedigree with an autosomal recessive vitreoretinal degeneration, severe myopia, and congenital encephalocele. All five affected members (four girls and one boy) also had early, recurrent bilateral detachments. color vision testing disclosed an acquired tritan dyschromatopsia and electroretinography showed subnormal photopic and scotopic amplitudes, delayed b-wave implicit times and 30-Hz flicker-phase relations, and absent scotopic b-wave oscillations.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
9/23. Autosomal dominant vitreoretinochoroidopathy.Autosomal dominant vitreoretinochoroidopathy is a newly described fundus dystrophy characterized by abnormal chorioretinal hypopigmentation and hyperpigmentation, usually lying between the vortex veins and the ora serrata for 360 degrees. In this zone, there are a discrete posterior boundary, preretinal punctate white opacities, retinal arteriolar narrowing and occlusion, and, in some cases, choroidal atrophy. Most affected family members have diffuse retinal vascular incompetence, cystoid macular edema, and presenile cataracts. The vitreous is characterized by fibrillar condensation and a moderate number of cells. Electroretinograms are normal in younger affected individuals and are only moderately abnormal in older ones. Preretinal neovascularization, present in the posterior pole, is progressive in the proband. There are no identifiable systemic or skeletal abnormalities, high myopia, optically empty vitreous, lattice degeneration, areas of white-without-pressure, retinal breaks, or retinal detachment; thus, previously described vitreoretinopathies can be excluded from diagnostic consideration. Progression of this diagnostic seems to be extremely slow in most family members.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
10/23. Pseudoinflammatory fundus dystrophy with autosomal recessive inheritance.A family in southwest finland with bilateral hemorrhagic degeneration of the retina and choroid was followed up for more than 16 years. The maculas showed subretinal hemorrhages, glial cicatrization of the outer retinal layers, and profound choroidal atrophy, particularly in the advanced stages of the disease. fluorescein angiography demonstrated leakage through the pigment layer in the retinal tissue. The age of onset varied from the second to the fourth decade. The clinical pattern was similar to Sorsby's pseudoinflammatory dominant fundus dystrophy, except that the disorder appeared earlier in this Finnish family, the members of which show secondary dyschromatopsia, many deep hyaloid bodies in the retina, disturbed dark adaptation (1 to 4 log units), subnormal light-peak/dark-trough ratios, progressive myopia, and a mode of inheritance which is probably autosomal recessive. The affected parents are consanguineous in many ways and each of their eight children is affected.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
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