J Korean Ophthalmol Soc > Volume 53(7); 2012 > Article
Journal of the Korean Ophthalmological Society 2012;53(7):1041-1045.
DOI: https://doi.org/10.3341/jkos.2012.53.7.1041    Published online July 15, 2012.
Specific Spectral Domain Optical Coherence Tomographic Findings of Oguchi Disease.
Joo Hyun, Tae Kwann Park, Jee Ho Chang, Young Hoon Ohn
Department of Ophthalmology, Soonchunhyang University College of Medicine, Bucheon, Korea. genophilus@hanmail.net
오구치병에서 관찰된 특징적 빛간섭단층촬영 소견 1예
현 주⋅박태관⋅장지호⋅온영훈
Department of Ophthalmology, Soonchunhyang University College of Medicine, Bucheon, Korea
Abstract
PURPOSE
To report specific spectral domain OCT findings of Oguchi disease diagnosed with fundoscopic examination and electrophysiological study. CASE SUMMARY: A 14-year-old patient visited our clinic with a complaint of night blindness for ten years. Fundoscopic examination showed a golden-yellow fundus reflex. After three hours of dark adaptation, the fundus color returned to normal (Mizuo-Nakamura phenomenon). In full-field ERG, rod b-wave was not detectable. The a-wave amplitude in maximal combined response increased after three hours of dark adaptation, although the b-wave amplitude was similar to the amplitude before dark adaptation, demonstrating a negative waveform. In the spectral domain OCT images of the perifoveal area, no gap between the retinal pigment epithelium and the inner segment/outer segment (IS/OS) junction was detected before prolonged dark adaptation, and a highly reflective band was shown. However, the gap appeared after three hours of dark adaptation, and two highly reflective bands were detected in the OCT images. CONCLUSIONS: The characteristic OCT finding in addition to the specific fundoscopic finding and full-field ERG results may be useful to diagnose Oguchi disease.
Key Words: Dark adaptation;Electroretinography;Mizuo-Nakamura phenomenon;Oguchi disease;Optical coherence tomography


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