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What is Retinoschisis?

Retinoschisis is a rare eye condition where the retina, the light-sensitive layer at the back of the eye, splits into two. This can lead to vision problems or even detachment. It's often inherited and can affect both children and adults. Intrigued by how this condition impacts vision and what treatments are available? Keep reading to uncover the full picture.
Emma Lloyd
Emma Lloyd

Retinoschisis is an eye disease that affects 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 at which visual signals are received and focused. Retinoschisis can occur as a hereditary disorder in children and in the elderly, in whom it primarily results from aging. This condition rarely causes blindness, but it does often result in impaired vision.

When it occurs as a juvenile hereditary disease, retinoschisis mainly affects male children. This is because the genetic mutation that causes the disorder is located on the X chromosome. Males have one X chromosome, and females have two. Therefore, a mutation of the X chromosome often affects males only, because females still have a normal copy of the gene, and this masks the effects of the mutation.

The genetic mutation that causes retinoschisis is located on the X chromosome.
The genetic mutation that causes retinoschisis is located on the X chromosome.

In age-related retinoschisis, the disease is not caused by a genetic mutation. Instead, it is the result of retinal damage that occurs because of aging. For this reason, women are as likely as men to have age-related retinoschisis. Despite its name, this type of retinal disease does not solely occur in the elderly, because it can develop as early as the third decade of life.

Age-related retinoschisis can cause elderly people to experience changes in vision.
Age-related retinoschisis can cause elderly people to experience changes in vision.

Retinoschisis can affect both peripheral and central vision. Someone with the juvenile form of the condition is more likely to experience a deficiency in central vision that is mild at onset but can gradually worsen. A young adult with this disorder is likely to have visual acuity of approximately 20/70, but visual impairment as severe as 20/200, defined as legal blindness, is possible.

Retinoschisis can occur as a hereditary disorder in children and in the elderly.
Retinoschisis can occur as a hereditary disorder in children and in the elderly.

In both the juvenile and the age-related disease, common symptoms include loss of vision, floaters and photopsia, or flashes of light. These symptoms occur because the retina gradually splits into two or more layers. In the case of adult-onset disease, this simply is 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 adhere to one another. Retinal deterioration also can lead to complications: There is a risk of holes developing in the retina and a risk of retinal detachment.

Retinoschisis can affect both peripheral and central vision.
Retinoschisis can affect both peripheral and central vision.

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 vision, but cannot prevent further vision loss. In the case of children with the juvenile form of the disease, many doctors recommend that activities that carry a risk of head impact should be limited or eliminated. Children usually are closely monitored for signs of vision deterioration so that surgery can be carried out promptly, if it is needed.

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    • The genetic mutation that causes retinoschisis is located on the X chromosome.
      By: Giovanni Cancemi
      The genetic mutation that causes retinoschisis is located on the X chromosome.
    • Age-related retinoschisis can cause elderly people to experience changes in vision.
      By: fred goldstein
      Age-related retinoschisis can cause elderly people to experience changes in vision.
    • Retinoschisis can occur as a hereditary disorder in children and in the elderly.
      By: Jaren Wicklund
      Retinoschisis can occur as a hereditary disorder in children and in the elderly.
    • Retinoschisis can affect both peripheral and central vision.
      By: iko
      Retinoschisis can affect both peripheral and central vision.
    • Retinoschisis affects the retinal cells in the macula part of the eye, often times in elderly patients.
      By: jamstockfoto
      Retinoschisis affects the retinal cells in the macula part of the eye, often times in elderly patients.