What is Retinoschisis?

Juvenile retinoschisis, RS, is a genetic disease of the retina. The retina is the thin layer of nerve tissue which lines the inside of the back of the eye. The retina converts pictures into nerve signals that are formed into a visual image by the brain. Damage to the retina will impair vision.

External View of Eye

RS affects two primary aspects of vision. First, central vision is frequently impaired. Visual acuity in RS typically ranges from 20/30 to 20/200. The acuity loss in retinoschisis is caused by the formation of tiny cysts in the retina. These cysts often form a "spoke-wheel" pattern which frequently is quite subtle and is detected only by a trained clinician. Since the nerve tissue is damaged by these cysts, the visual acuity cannot be improved with glasses. Second, peripheral vision can be lost due to the splitting of the inner layer of nerve cells from the outer layer of cells. “Schisis” is derived from a Latin word meaning "splitting" and gives the name to this disease.

Axial (top) view of eye

Retinal detachments can occur if the anchoring of the outer layer of the retina to the eye wall is impaired. RS patients are more susceptible to retinal detachments and regular examinations with an ophthalmologist are important. When detected early, retinal detachments can be treated surgically (see information about retinal detachment). However, the more typical feature of splitting or schisis of the retina cannot be corrected by medication or surgery.

RS affects primarily boys and young men. It is estimated that retinoschisis affects one in 5,000 to 25,000 individuals, primarily young males. Affected males are usually identified in grade school, but occasionally are identified as young infants. This disease affects families all over the world.

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