Schoenbart Vision Care:
Long Islands Leading Juvenile Macular Degeneration Specialist
Stargardt's, Best Disease, Juvenile Retinoschisis
Our Optometrists specialize in providing the highest quality care for patients with Juvenile Macular Degeneration such as Stargardts, Best Disease, and Juvenile Retinoschisis. Unlike age related macular degeneration, these diseases are not a function of age but are genetically inherited. Similar to AMD they effect the macula which is responsible for central vision. The effects are particularly difficult to cope with as they diminish the independence of the patient by reducing their ability to read, see faces, and drive.
Stargardt’s is the most common form of juvenile macular degeneration, in almost all cases it eventually leads to vision of 20/200 or legal blindness. The disease is the result of the deterioration of photo-receptor cells in the macula, leading to impaired central vision.
The patient may have normal vision for many years before any serious changes to their vision. The progress of vision loss is extremely devastating as the progress is rapid. Once vision reaches 20/40 it can reach the status of legal blindness within as short as a few months.
This disease afflicts 1/10,000 Americans, and although the disease usually presents itself in mid teens, serious vision loss that is noticeable may not occur until the mid 30’s.
The disease is a result of a mutated gene passed down from both parents. If a child receives the gene from both parents they have a 25% probability of developing the disease.
In clinical trials close to 50% of those studied had visual acuity of 20/200 to 20/400 by the time they reached 50 years old. Eventually almost everyone with Stargardt’s will have visual acuity in the category of legally blind.
How is it diagnosed?
The disease is diagnosed by your eye doctor noticing atypical fatty deposits on and surrounding your macula. These deposits are small dots that look yellow or white in color.
Best Disease is the second most prevalent form of juvenile macular degeneration after Stargardt’s disease. Unlike Stargardt’s, it can be noticed in a low vision eye exam in a patient as young as 3 years old.
It is detected when your eye doctor sees a fluid filled sac beneath the macula. The vision loss occurs when this cyst ruptures and spreads throughout the macula.
In many cases vision loss will only occur years after diagnosis. As the disease progresses you may have vision loss in one eye and not the other, in some cases the disease causes distortion of central vision. The disease is passed on by a parent who has the disease, if neither parent has best disease it can not be transmitted.
Commonly found in young males, vision loss begins in the early teens and after the initial vision loss vision, vision remains stable until the patient reaches their 50’s. In many cases vision will remain relatively normal until later in life.
The disease causes the retina to break apart into two, with blisters and blood vessels forming in between each layer of the retina. Over time blood leakage causes the fluid inside your eye called the vitreous body to disconnect with the retina or alternatively the retina itself may detach.
In around 50% of cases there will be loss of side vision as well as central vision. There is also severe vision loss which is common, although usually less than Stargardt’s. Common vision loss is 20/60 to 20/120.
Children with this disease may show signs of misaligned eyes or involuntary eye movements (strabismus or nystagmus).
The disease is past down in the X chromosome. Boys who receive the gene from their mother have a 50% chance of getting the disease.