Abstract
Methods: :
The presented patient is a 31-year-old man with prolonged dark adaptation from his childhood. He noticed visual disturbance of his right eye 3 months before and referred to us. His both ocular fundus showed golden-brown shiny color during ophthalmoscopical examination. Full-field electroretinogram (ERG) showed normal photopic and flicker responses while scotopic ERG was totally flat.Goldmann’s perimeter was normal. He was diagnosed as Oguchi disease. His right eye had serous retinal detachment. Fluorescence angiography (FAG) showed a leak point of dye at subfoveal area Although he was diagnosed as central serous chorioretinopathy, we decided to observe the lesion without photocoagulation because of subfoveal dye leakage. His best-corrected visual acuity (BCVA) was 20/30 in his right eye and 20/20 in left eye. Serous retinal detachment spontaneously resolved over 3 months and his BCVA recovered to 20/20.
Results: :
SD-OCT showed intact IS/OS line (junction between photoreceptor inner and outer segment) at the macular area with 2 discs diameter while IS/OS line was not detected as another reflective line apart from retinal pigment epithelium peripheral to the macula in his left eye and right eye after resolution of CSC. The outer nuclear layer was also attenuated periphery to the macula.
Conclusions: :
In Oguchi disease, cone cells were normal in SD-OCT and ERG. However in rod cells, photoreceptor outer segment appeared to be degenerative. Attenuation of outer nuclear layer suggested decreased a large number of rod cells.
Keywords: retina • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)