Peripheral Cone Dystrophy: Expanded Clinical Spectrum, Multimodal and Ultrawide-Field Imaging, and Genomic Analysis

Figure 4

Two hundred degree ultrawide-field fundus autofluorescence with 488 nm laser excitation demonstrates macular changes as described in Figure 2 but reveals additional peripheral autofluorescence abnormalities in all patients. (a, b) Right and left fundi of case 1 had grouped nummular hypoautofluorescent areas anterior to the equator from cobblestone degeneration. A hypoautofluorescent tongue with hyperautofluorescent borders extended posteriorly towards the optic nerve in both eyes. Areas of funduscopically visible black pigment clumping appeared densely hypoautofluorescent. (c, d) Hypoautofluorescence from prior laser treatment of lattice degeneration in the right eye was the only evident peripheral autofluorescence abnormality seen in case 2. (e, f) A bifocal area of hyperautofluorescence with central hypoautofluorescence to the nasal portion was observed in the right and left fundi of case 3. In the right eye, the lesions were contiguous and the area of hypoautofluorescence was larger.

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