Case #74: Pentosan Polysulfate Maculopathy: Another Differential Diagnosis for Night Blindness – Page 18 of 23

1. Macular hyperpigmented spots with yellow-orange deposits and patchy RPE atrophy on fundus photography

2. Densely packed clusters of hyper and hypoAF areas within the posterior pole on FAF

3. Focal thickening of the RPE with hyperreflectance on NIR

4. Peripapillary hypoAF halo

5. Maximum size of the hyperAF spots of 2 venule widths.

6. Absence of typical drusen

 


All 6 criteria met

Severe: Well-demarcated lesions with associated hypoAF atrophy with RPE and outer retinal atrophy, which involves the central fovea