Subretinal Lesions Point to Best’s Disease

Photojournalism
Ophthalmology Times
November 15, 1996 

This 10-year-old boy was referred by his optometrist for evaluation of a macular abnormality. The patient’s vision was 20/25 OU and he had no visual complaints. Medical history was essentially negative and family history revealed a history of Best’s disease in his father and paternal grandmother.

 The anterior segment examination was within normal limits. Dilated examination revealed normal optic nerves and typical Best’s vitelliform lesions in the macular area of both eyes. The lesions are oval, subretinal, yellow, and slightly larger in the right eye.

 Discussion

Patients with Best’s disease, or vitelliform dystrophy, may experience no visual problems until their third or fourth decade.

Best’s disease is usually a bilateral abnormality. The structure is described as being egg-shaped or round, slightly elevated, and yellow. Vitelloform dystrophy is typically seen in patients who are between 3 and 15 years old. Vision is usually only minimally affected, although it can gradually decrease with age. Studies have shown that roughly half of the children of affected individuals are affected as well.

Since EOG can be abnormal even in carriers, electrophysiologic testing of the patient’s siblings was recommended.