OCULAR FUNDUS IMAGES BY SCANNING LASER OPHTHALMOSCOPY IN A PATIENT WITH ENHANCED S-CONE SYNDROME

Abstract

To present ocular fundus images in a patient with enhanced S-cone syndrome by scanning laser ophthalmoscopy. A 34-year-old Japanese woman whose parents were consanguineous showed mismatched electroretinographic responses to photopically balanced single-flash stimuli, with a larger signal to blue light than to red light. The central macula lacked a foveal reflex, and the surface was dull. Yellowish flecks and retinal pigment epithelium atrophy were evident in a ring at and around the vascular arcades. Faint black pigmentation was deposited in the mid peripheral retina. The ocular fundus of the patient was observed by scanning laser ophthalmoscopy with the use of an argon blue laser (wavelength, 488 nm), a helium-neon laser (633 nm), and an infrared laser (780 nm). The argon blue laser showed numerous black spots of pigment, which were observed as faint pigmentation by conventional ophthalmoscopy. The spots were more enhanced with the argon blue laser than with the helium-neon laser. The white spots, which corresponded to the yellowish flecks in a ring at and around the vascular arcades, were more enhanced with the helium-neon laser than with the argon blue or infrared laser. Hypopigmentation of the retinal pigment epithelium was best shown with the infrared laser. An abnormality of the retinal structure in enhanced S-cone syndrome may exist in the inner and outer retinal levels, in at least some patients.