The diagnosis of vitelliform macular dystrophy can be made from fundus examination alone when the classical egg-yolk lesion is intact. After the lesion has scrambled, however, other information is needed to be certain of the diagnosis. The electrooculogram (EOG) has been reported abnormal in vitelliform macular dystrophy and has been called "an indispensable diagnostic element." This paper, how...

PURPOSE To report an interesting case of concomitant bilateral Terrien's marginal degeneration-like corneal ectasia and posterior polymorphous corneal dystrophy in a young man with quiescent rheumatoid arthritis. CASE REPORT A 24-year-old man with history of rheumatoid arthritis presented with bilateral decreased vision since four years ago. Slit lamp examination revealed bilateral circumfere...

OBJECTIVE To describe the ophthalmic and genetic findings of a large kindred (UM:H389) with autosomal dominant hemorrhagic macular dystrophy. METHODS The disease state of family members was documented by dilated fundus examination, electroretinography, color vision tests, fluorescein angiography, measurement of visual fields, biomicroscopy, gonioscopy, and intraocular pressure measurement. Li...

purpose: to report bilateral terrien’s marginal degeneration, circumferential peripheral corneal ectasia and posterior polymorphous dystrophy in a patient with rheumatoid arthritis. case report: a 24-year-old male patient, who was known case of rheumatoid arthritis from 8 years before, presented with decreased vision since four years ago. comprehensive ophthalmic eye exami-nation revealed bilat...

We studied 34 family members at risk of having dominant progressive foveal dystrophy of Lefler, Wadsworth, and Sidbury (also called North Carolina macular dystrophy) and found 17 to be affected. Fifteen of these affected subjects were observed over at least a 10-year period for evidence of progressive macular degeneration. Only one subject showed objective evidence of progressive deterioration ...

PURPOSE To describe a bilateral, central, oval corneal opacity not resembling any known corneal degeneration or dystrophy. METHODS Ophthalmic examination, corneal topography, and pachymetry. RESULTS A 30-year-old woman presented complaining of blurred vision. Biomicroscopic examination revealed bilateral, centrally located, oval, diffuse opacification of the anterior corneal stroma. Corneal...

PURPOSE To describe a case of keratoconus and Fuchs' corneal endothelial dystrophy in the left eye with no corneal disease in the right eye. METHODS A 64-year-old woman presented with visual impairment in her left eye; computer-assisted topographic analysis and specular microscopy were performed in both eyes and left cornea was histopathologically examined. RESULTS Keratoconus was diagnosed...

PURPOSE To investigate the clinical and genetic features of Korean patients with corneal dystrophies associated with mutations in the human transforming growth factor-β-induced (TGFBI) gene. METHODS In this study, 387 subjects (71 families and 89 individuals - 268 patients having TGFBI corneal dystrophies and 119 normal relatives) were assessed. All subjects underwent a complete ophthalmologi...

PURPOSE To describe a case of severe corneal granular dystrophy with clinicopathologic and molecular genetic findings. METHODS The DNAs of a 53-year-old male patient suffering from corneal granular dystrophy and nonaffected family members were analyzed by molecular genetic methods. Clinical features, and histopathologic and immunohistochemical findings from the penetrating keratoplasty specim...

PURPOSE In scanning laser polarimetry with variable corneal compensation (SLP-VCC), the macula is used as an intraocular polarimeter to calculate and neutralize corneal birefringence based on an intact Henle's layer. The purpose of this investigation was to validate this strategy in eyes with macular structural disease. METHODS A nerve fiber analyzer was modified to enable the measurement of ...