What Is Juvenile Macular Dystrophy and How Does It Affect You?

Juvenile macular dystrophy (also known as juvenile macular degeneration) is a category of hereditary eye diseases that mostly affect children and young adults. Age-related macular degeneration is not the same as juvenile macular dystrophy (AMD). While AMD is a normal part of the aging process, juvenile macular dystrophy is an inherited disorder.

The macula is a small section of the retina, which lines the back of the eye and is light-sensitive tissue. The macula is the part of your eye that provides you with central vision and helps you to see fine details clearly. Even though the macula is just a small part of the retina, it is much more information-oriented than the rest of the retina (called the peripheral retina). The macula is responsible for threading needles, reading small print, and recognizing street signs. Your peripheral retina will tell you if anyone is standing on one side of you. It accomplishes this by enabling you to view their general shape.

The most common form of macular dystrophy in children is Stargardt disease. The following are examples of other forms of juvenile macular dystrophy:

● Juvenile Retinoschisis
● Best’s disease (also called Best’s vitelliform retinal dystrophy)

All of these diseases are uncommon and cause vision loss in the central vision.

What Are the Signs and Symptoms of Juvenile Macular Degeneration?

what is juvenile macular dystrophy

Juvenile macular dystrophy manifests itself in a variety of ways. All of them wreak havoc on your core vision. It is possible that your central vision becomes fuzzy, blurred, or has dark areas. Color perception can be impaired in the later stages, but side vision is normally unaffected. The first signs of the disease appear in infancy or adolescence. These signs and symptoms do not always affect each eye in the same way.

Some people with juvenile macular dystrophy can maintain useful vision well into adulthood. Others experience a faster progression of the disease. For several decades, people with Best’s disease have an almost normal vision. Many people might be completely unaware that they even have it. Stargardt disease, on the other hand, often results in a vision of 20/200. This is what it means to be legally blind.

Children with juvenile retinoschisis may also exhibit the following symptoms:

● Nystagmus (involuntary eye movement)
● Strabismus (misaligned eyes)

Who Is at Risk?

what is juvenile macular dystrophy

Macular dystrophy in children is caused by a genetic mutation. This indicates that the disease is passed on from parent to infant. Different forms of illness have different patterns of inheritance.

Stargardt syndrome, for example, is commonly recessive. For the disease to manifest, a child must inherit it from both parents. Parents are the most common disease “carriers.” This means they don’t have the disease but will pass it on to their children invisibly.

It is a dominant gene that causes Best’s disease. To acquire the disorder, a child only needs to inherit the gene from one parent. An infant born to an infected parent and an unaffected spouse has a 50% (5 out of 10) risk of contracting the disease.

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