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KMID : 0360220080490050858
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Journal of the Korean Ophthalmological Society
2008 Volume.49 No. 5 p.858 ~ p.864
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Two Cases of Occult Macular Dystrophy in a Family
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Kim Joo-Hun
Park Kyu-Hyung
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Abstract
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Purpose: We report two familial cases of occult macular dystrophy (OMD) presenting with a progressive decrease in central vision.
Case summary:Both patients exhibited a normal ophthalmologic examination including slit lamp biomicroscope, fundus examination, fluorescein angiography, and full-field electroretinogram. However, there were central visual field defects on a Humphrey static visual field test (C 24-2) and abnormal multifocal electroretinogram (mfERG) findings affecting the central portion of the test field. Foveal thinning was also observed by optical coherence tomography images in 1 case. These findings are consistent with the clinical characteristics of occult macular dystrophy, and close observation was recommended.
Conclusions: OMD is a disease characterized by a reduction in central visual acuity without visible fundus abnormalities and full field ERG. It may be misdiagnosed as optic nerve disease, a central nervous system problem, non-organic visual disorder or malingering; therefore, mfERG is essential for the diagnosis of this rare type of macular dystrophy.
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KEYWORD
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Multifocal electroretinogram, Occult macular dystrophy
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