오구치병에서 관찰된 특징적 빛간섭단층촬영 소견 1예

• 영문명: Specific Spectral Domain Optical Coherence Tomographic Findings of Oguchi Disease
• 발행기관: 대한안과학회
• 저자명: 현주 박태관 장지호 온영훈,Joo Hyun, MD, Tae Kwann Park, MD, PhD, Jee Ho Chang, MD, PhD, Young-Hoon Ohn, MD, PhD
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume53,number7, 141~145쪽, 전체 5쪽
• 주제분류: 의약학 > 기타의약학
• 파일형태: PDF
• 발행일자: 2012.07.15

영문 초록

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. J Korean Ophthalmol Soc 2012;53(7):1041-1045

키워드

Dark adaptation, Electroretinography, Mizuo-Nakamura phenomenon, Oguchi disease, Optical coherence tomography