Retinoschisis- A genetic eye disease

Retinoschisis is a disease of the nerve tissue in the eye. It is a splitting of the retinal layers derived from the inner layer of the optic cup. A rare inherited or acquired condition where the retina separates into two layers resulting in progressive vision loss. Retinoschisis is a genetic eye disease that affects the vision of men who inherit the disease from their mothers. The juvenile type occurs before 10 years of age, and macular involvement is frequent. The senile type occurs from age 20 on, commonly after age 40, and there is usually no significant visual loss. Holes in the two layers are common and are a sign of progression. The inner layer contains blood vessels and sometimes has small whitish flakes on it, which are called 'snowflakes'.


Types of Retinoschisis:

Juvenile retinoschisis- A genetic disease of the eye characterized by retinoschisis (splitting of the retina) symmetrically involving the macula, the area of the retina responsible for central vision, in some cases as early as three months of age. Fundus examination shows areas of schisis (splitting of the nerve fiber layer of the retina) in the macula, sometimes giving the impression of a spoke wheel pattern. X-linked juvenile retinoschisis occurs almost exclusively in males.


Senile retinoschisis- In senile retinoschisis, the problem occurs with old age and rarely causes severe vision impairment. Very few affected individuals go blind from retinoschisis. It can occur in males or females. This condition is not senile or age related, as it has been reported in patients in the third decade.

Symptoms:

• Macula anomalies

• Splitting of the retina

• Decreased vision

• Loss of peripheral vision

• Progressive vision impairment

• Hemeralopia

• Squinting
• How Retinoschisis Affect Me?

In the early stages of the senile form you are unlikely to experience symtoms but as the condition progresses you may notice visual loss, 'seeing' floating objects in the eye, or 'seeing' flashes of lights in the eye. The juvenile form usually has some degree of visual loss which children may notice as difficulty in resding at school.


Treatments:

Generally there is no treatment for the condition but surgery may be needed if complications develop. The Center for Retinal and Macular Degeneration at the University of Michigan Kellogg Eye Center is one of the only centers in the country performing genetic research. Gene therapy can prevent retinoschisis, and therapy apparently demonstrates that even if disease has begun, there is a healing that takes place. Watchful monitoring- if there is no symptoms, no treatment will be needed.

However, depending on the severity of disease, ongoing screening with fundoscopy mayl be required annually or biannually.Surgery is considered if there is progression of the disease, e.g. retinal detachment. Vitamin A will probably not help in retinoschisis but may have benefits for other genetic retinal diseases, particularly in cases of retinitis pigmentosa where the retinal nerve cells are slowly dying.