Author(s): Alemu Kerie Tesfaw*, Paul S Bernstein
Purpose: To describe the clinical and optical coherence tomography findings of Adult-Onset Foveo-Macular Vitelliform Dystrophy (AFVD) mimicking age related macular degeneration.
Method: A retrospective study design was employed which reviewed clinical and optical coherence tomography images of patients with adult-onset foveo-macular vitelliform dystrophy from September 2019 to March 2021.
Result: There were 12 (9 male and 3 female) study subjects with a mean age of 62.75 years. The presenting visual acuity ranged from 20/100 to 20/20. Of 19 eyes, 10 eyes showed vitelliform lesions; 5 eyes had pseudo-hypopyons, and 4 eyes were in the vitelliruptive stage. OCT showed disruption of the IS/OS interface in 8/19 eyes (3 eyes with stage I, 1 in stage II, and 4 eyes with stage III disease). Optically clear (non-reflective) sub retinal lesions were present in OCT images from 6 eyes (5 in pseudo-hypo yon and 1 in vitelliruptive stages). Four of five eyes in the pseudo-hypo yon stage had intact IS/OS photoreceptor interfaces.
Conclusion: An optically clear space is a distinct feature of adult-onset fovea-macular vitelliform dystrophy that is different from the pattern of fluid in endovascular AMD that may have a protective role against the disruption of the photoreceptor interface. It is important to be aware of the stages of AFVD and to differentiate them from endovascular AMD, as AFVD does not require treatment with injections of anti-vascular endothelial growth factor.
