X-linked juvenile retinoschisis

What is X-Linked Juvenile Retinoschisis?

X-linked juvenile retinoschisis is an inherited eye disorder that makes the inner layer of the retina split in a spoke-wheeled pattern. It usually occurs in both eyes at once. This disease affects more males than females.

Boys with the condition may have symptoms in early childhood, but are often not diagnosed until they have their first visual screening before starting school. They usually have vision of 20/60 to 20/120, with tiny lesions, splits, or cysts visible on their retinas. Fewer than 10% of people with the condition experience full retinal detachment or bleeding inside the eye, both of which can cause blindness.

As boys with X-linked juvenile retinoschisis go through their teens, their vision may slowly worsen, but it typically stabilizes in their twenties. When they reach their forties and fifities, their vision may start to deteriorate again. Many people with the condition eventually have vision of 20/200 or worse, making them legally blind.

How common is X-Linked Juvenile Retinoschisis?

X-linked juvenile retinoschisis is estimated to affect 1 in every 5,000 to 25,000 people. Due to its pattern of inheritance, the disease primarily affects males.

How is X-Linked Juvenile Retinoschisis treated?

In general, treatment focuses on monitoring the progress of the disease and helping affected individuals learn to cope with poor vision. For example, children can use large-text books and high-contrast reading materials. Adults can purchase special magnifiers, clocks, and adaptive software to help them at home and at work.

Because X-linked juvenile retinoschisis affects only the inner layers of the retina, surgery is rarely effective in treating the disease. However surgery may help with complete retinal detachment.

Low-vision specialists such as optometrists can help both children and adults make the most of the vision they have. Some people with the condition can get restricted driver's licenses if they wear special telescopic lenses behind the wheel. (Please note that these lenses are not legal in all states.)

Children under the age of 10 should see a pediatric ophthalmologist or retina surgeon every year. Older children and adults need less frequent monitoring.

People with X-linked juvenile retinoschisis should avoid high contact sports and other activities that might cause a hard blow to the head. This minimizes the risk of retinal detachment or bleeding in the eye.

What is the prognosis for a person with X-Linked Juvenile Retinoschisis?

X-linked juvenile retinoschisis does not affect lifespan, but does cause progressive vision problems which can result in legal blindness after middle age.