Vitelliform macular dystrophy

Abstract EN

A twenty-one-year-old generally healthy gentleman presented to the ophthalmology clinic with progressive reduction of vision over 3 years and family history of Best’s Disease.

Best’s disease is a rare autosomal dominant congenital vitelliform macular dystrophy.

Patients usually present with deterioration of central vision in the second decade of life and gradually worsening over the years.

The disease is untreatable and low visual aids are used.

Genetic and clinical counseling is accessible to affected individuals along with their asymptomatic relatives.