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 |
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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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