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What is the prevalence of Best Vitelliform Macular Dystrophy?

How many people does Best Vitelliform Macular Dystrophy affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Best Vitelliform Macular Dystrophy

Best Vitelliform Macular Dystrophy (BVMD) is a rare inherited eye disorder that affects the macula, the central part of the retina responsible for sharp central vision. BVMD typically manifests in childhood or adolescence and progresses slowly over time. While exact prevalence rates are not well-established, it is estimated to affect around 1 in 10,000 to 1 in 50,000 individuals worldwide. BVMD can cause vision loss and impair daily activities such as reading and recognizing faces. Early diagnosis and regular monitoring are crucial for managing the condition and preserving vision.



Best Vitelliform Macular Dystrophy (BVMD), also known as Best disease, is a rare inherited eye disorder that affects the macula, the central part of the retina responsible for sharp central vision. BVMD is characterized by the accumulation of lipofuscin, a fatty yellow pigment, in the macula, leading to progressive vision loss.


The prevalence of BVMD is estimated to be around 1 in 10,000 to 1 in 50,000 individuals worldwide. It is considered a rare disease, but the exact prevalence may vary among different populations. BVMD typically manifests in childhood or adolescence, although it can appear later in life. The inheritance pattern of BVMD is autosomal dominant, meaning that an affected individual has a 50% chance of passing the condition on to each of their children.


While BVMD is a rare condition, it is important to raise awareness about it to facilitate early diagnosis and appropriate management. Regular eye examinations and genetic testing can aid in the identification of BVMD, allowing for timely interventions and support for affected individuals and their families.


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