Two Cases of Occult Macular Dystrophy in a Family.
10.3341/jkos.2008.49.5.858
- Author:
Joo Hoon KIM
1
;
Kyu Hyung PARK
Author Information
1. Department of Ophthalmology, Seoul National University College of Medicine Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea. jiani4@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Multifocal electroretinogram;
Occult macular dystrophy
- MeSH:
Central Nervous System;
Fluorescein Angiography;
Humans;
Macular Degeneration;
Optic Nerve Diseases;
Tomography, Optical Coherence;
Vision Disorders;
Vision, Ocular;
Visual Acuity;
Visual Field Tests;
Visual Fields
- From:Journal of the Korean Ophthalmological Society
2008;49(5):858-864
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
