Pseudovitelliform macular degeneration.

Forty-two patients with pseudovitelliform macular degeneration (VMD) were studied. The macular lesions were yellow in color, appeared as a slight elevation at the level of the retinal pigment epithelium (RPE), were usually 1/4 to 1/2 disc diameter in size and often showed a round or oval shape. Progression occurred over many years, resulting in a circumscribed atrophic area in the RPE. The lesions in VMD were morphologically similar to those seen in Best's vitelliform dystrophy, but the electrooculogram was normal or slightly subnormal in all cases. The average Lp/Dt ratio in 71 eyes tested was 2.16. There were 31 women and 11 men with a median age of 51 years at initial presentation. Family studies did not reveal other affected members. Long-term follow-up (five years or longer) in ten cases showed that useful vision is retained in at least one eye.