Retinoschisis is a retinal disease affecting the macula, causing impaired vision. The hereditary juvenile form primarily affects boys due to a genetic mutation on the X chromosome. Age-related retinoschisis affects both men and women and can occur as early as the third decade of life. Symptoms include vision loss, floaters, and photopsia. Medical treatment cannot prevent deterioration, but surgery can reduce the risk of retinal detachment. Children with the juvenile form are advised to avoid head impact activities.
Retinoschisis is an eye disease that affects the retinal cells in a part of the eye called the macula. The macula is located at the back of the eye and is the central point where visual signals are received and focused. Retinoschisis can occur as an inherited disease in children and the elderly, in which it mainly results from aging. This condition rarely causes blindness, but often results in impaired vision.
When it occurs as a hereditary juvenile disorder, retinoschisis primarily affects boys. This is because the genetic mutation that causes the disorder is found on the X chromosome. Males have one X chromosome and females two. Therefore, a mutation of the X chromosome often affects only males, because females still have a normal copy of the gene, and this masks the effects of the mutation.
In age-related retinoschisis, the disease is not caused by a genetic mutation. Instead, it is the result of damage to the retina that occurs as a result of aging. For this reason, women are just as likely as men to have age-related retinoschisis. Despite its name, this type of retinal disease does not only occur in the elderly, as it can develop as early as the third decade of life.
Retinoschisis can affect both peripheral and central vision. Someone with the juvenile form of the condition is more likely to experience central vision impairment that is mild at first but can gradually get worse. A young adult with this disorder is likely to have a visual acuity of about 20/70, but vision impairment as severe as 20/200, defined as legal blindness, is possible.
In both juvenile and age-related disease, common symptoms include vision loss, floaters, and photopsia, or flashes of light. These symptoms occur because the retina gradually divides into two or more layers. In the case of adult-onset disease, this is simply the result of age-related deterioration of eye tissues. When the juvenile form is involved, the cause is a defect in a protein that helps retinal cells stick to each other. Deterioration of the retina can also lead to complications: there is a risk of holes developing in the retina and the risk of retinal detachment.
Medical treatment cannot prevent deterioration of the retinal layers, but surgery can help reduce the risk of retinal detachment. Glasses or contact lenses can improve your vision, but they can’t prevent further vision loss. In the case of children with the juvenile form of the disease, many doctors recommend limiting or eliminating activities that pose a risk of impact to the head. Children are usually closely monitored for signs of vision deterioration so that surgery can be performed promptly if needed.
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