Cases reported "Refractive Errors"

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1/7. Voluntary nystagmus associated with accommodation spasms.

    BACKGROUND: Voluntary nystagmus has been recognized as a pendular, rapid, conjugate, primarily horizontal, benign eye movement initiated and maintained by voluntary effort. CASE: A 10-year-old Japanese girl presented with voluntary nystagmus associated with accommodation spasms. Her chief complaints, intermittent blurred vision, headache, and soreness of the eyes, were thought to be related to the voluntary nystagmus and accommodation spasms. FINDINGS: The waveform of the nystagmus appeared pendular, the frequency was 13-15 Hz, and the amplitude was 3-5 degrees. Scanning laser ophthalmoscopic video images clearly demonstrated vertical and torsional components in addition to the horizontal eye movements. Her refraction was unstable, varying between -0.5 diopters (D) and -5.5 D, and the recording of the accommodometer increased to -12.0 D when nystagmus was initiated. CONCLUSIONS: This may be a unique form of voluntary nystagmus that consists of horizontal, vertical, and rotational components associated with accommodation spasms. observation of this patient continues, without any further treatment or examination.
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2/7. Bilateral papillomacular retinal folds and posterior microphthalmus: new features of a recently established disease.

    Clinical findings of a 2.5-year-old girl presenting with barely detectable horizontal nystagmus and high hypermetropia are described. Despite the normal appearing anterior segments, the child had posterior microphthalmus and bilateral papillomacular retinal folds, conforming to a recently described, rare congenital disease. The patient also had significant posterior pole excyclorotation and avascular zones at the extreme temporal periphery without ridge formation or neovascularization. These findings were not reported previously. Other remarkable features include mildly depressed photopic and scotopic electroretinogram amplitudes and a short axial length of the vitreous cavity compared to age-matched normals, measured by ultrasonography. The present case adds new elements to this relatively rare ocular developmental abnormality.
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3/7. Idiopathic macular hypoplasia: a report of four cases and refinement of the phenotype of so-called ateliotic macula.

    PURPOSE: To refine the phenotype of idiopathic macular hypoplasia, also referred to as ateliotic macula, by describing a series of cases with this diagnosis. methods: A review of the clinical characteristics of four patients as documented in medical records with regard to refractive error, visual acuity, anterior segment examination, retinal findings, and ancillary tests such as electroretinography (ERG). RESULTS: All patients had oval circumscribed or diffuse areas in the posterior pole where the retina appeared not to have developed normally; the fovea was involved in three patients with reduced visual acuity, and one patient had parafoveal lesions with preserved visual acuity. There were three males and one females. patients' age ranged from 4 to 16 years. Errors of refraction ranged from severe myopia to hypermetropia and mild astigmatism. The anterior segment was normal in all patients. Three patients had strabismus and two had nystagmus. ERG was normal in the one patient in whom it was performed. One patient was mosaic for trisomy of chromosome 9. CONCLUSIONS: The term idiopathic macular hypoplasia can be applied to a spectrum of abnormalities in which a localized area of the posterior pole has a primordial or underdeveloped appearance. Lesions involving the fovea result in poor acuity. Generalized retinal dysfunction is absent. At least one of the genes involved in macular development may be located on chromosome 9.
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4/7. Normalization of binocular VERs after early onset visual deprivation in man.

    Visually evoked responses (VERs) were elicited by a reversing checkerboard target from a patient who suffered early onset bilateral deprivation of form vision as a result of a high refractive error, large amplitude strabismus, and congenital nystagmus. Monocular and binocular steady-state VERs were abnormal in amplitude and wave form even when a correction for the patient's large refractive error (compound hyperopic astigmatism) was worn. Although monocular VERs could not be normalized, a normal wave form and amplitude were restored to the binocular VER by the addition of horizontal prisms to the patient's ophthalmic prescription. The initially degraded binocular VER gradually acquired a normal morphology and amplitude as the magnitude of compensating base-out prisms in the patient's habitual ophthalmic prescription was systematically increased. The relationship between the binocular VER amplitude and the correcting prisms derived by the method described in this paper was subsequently used to arrive at a practical clinical solution for the patient's unusual and debilitating visual symptoms. This electrophysiological evaluation of binocular function at the cortical level proved to be a very useful diagnostic procedure with prognostic value; standard clinic procedures were ineffective in elaborating the patient's sensory and oculomotor disorders. The theoretical and practical implications of managing patients with a history of early onset visual deprivation are discussed.
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5/7. Binocular versus monocular acuity in a patient with latent nystagmus.

    This is a case of latent nystagmus with a significant improvement in acuity with both eyes open rather than with either eye alone. If this patient's visual acuity was tested only O.D. alone and O.S. alone, the best visual acuity obtainable would have been 20/60. This might have had serious implications for A.F.'s work since a minimum visual acuity of 20/40 is needed to keep a driver's license in new york State. Since binocular acuity was 20/20, I assured him that driving is safe.
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6/7. Centrally tinted contact lenses. A useful visual aid for patients with achromatopsia.

    Achromatopsia (rod monochromacy) is a congenital color-vision defect of autosomal recessive inheritance due to severely abnormal or totally absent cone function. The disease is characterized by markedly reduced visual acuity, nystagmus, and, often, ametropia. Even under normal daylight conditions, these patients are extremely handicapped by glare because of a lack of rod inhibition by the abnormal or deficient cones. light-absorbing glasses (absorption > 90%) can ameliorate this visual impairment to a certain extent but are sometimes not accepted by the patient since they are felt to disfigure the face. Especially during the first few years of school, this can lead to psychological problems. A special contact lens (Hydroflex, Wohlk Company, Kiel) with a centrally tinted area (absorption 80%) that is slightly greater in diameter than the pupil under daylight conditions can correct ametropia and reduce light exposure and dazzle in a cosmetically much better way. Our first experience with this kind of visual aid in a 9-year-old girl suffering from incomplete achromatopsia is presented.
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7/7. Multifaceted treatment of congenital nystagmus: a report of 6 cases.

    patients with congenital nystagmus (CN) often have associated conditions (e.g., strabismus, high astigmatism, and binocular inefficiency) that also impair visual acuity. Unfortunately, individually used techniques directed at reducing nystagmus have generally produced only slight increases in Snellen acuity. We present case reports of six patients with nystagmus seen in our clinic (University of Houston, College of optometry) to illustrate the use of problem-specific management to improve visual acuity and/or binocular function. The results suggest that a comprehensive management strategy should include treatment of all associated conditions as well as therapy to improve the characteristics of the nystagmus wave form.
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