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CONDITIONS

 

   

JUVENILE RETINOSCHISIS

Juvenile retinoschisis is an inherited disease diagnosed in childhood that causes progressive loss of central and peripheral vision (side, upper and lower) due to degeneration of the retina.

Clinical Desription

Juvenile retinoschisis (also known as X-linked retinoschisis) occurs almost exclusively in males. Although the condition begins at birth, symptoms do not typically become apparent until after the age of 10. About half of all patients diagnosed with juvenile retinoschisis first experience a decline in vision. Other early symptoms of the disease include an inability of both eyes to focus on an object (strabismus) and roving, involuntary eye movements (nystagmus).

The splitting of the retina into two layers causes vision loss associated with juvenile retinoschisis. This retinal splitting most notably affects the macula, the central portion of the retina responsible for fine visual detail and colour perception. On examination, the fovea (the centre of the macula) has spoke-like streaks. The spaces created by the separated layers are often filled with blisters and ruptured blood vessels that can leak blood into the vitreous body (the transparent, colourless mass of jelly-like material filling the centre of the eye). The presence of blood in the vitreous body causes further visual impairment. The vitreous body degenerates and may eventually separate from the retina. The entire retina may also separate from underlying tissue layers causing retinal detachments.

The extent and rate of vision loss vary greatly among individuals with juvenile retinoschisis. Central vision is almost always affected. Peripheral vision loss occurs in about half of all cases. Some affected individuals retain useful vision well into adulthood; with others experience a rapid decline during childhood.

Related Diseases

Juvenile retinoschisis can resemble other retinal degenerative diseases such as retinitis pigmentosa (RP), Goldman-Favre viteoretinal dystrophy. Wagner's vitreoretinal dystrophy, and Stickler's syndrome. A thorough eye examination, including diagnostic tests measuring retinal function and visual field, combined with an accurate documentation of family history, can distinguish between these disease.

Inheritance

Juvenile retinoschisis is genetically passed through families by the X-linked pattern of inheritance.

Sometimes, however, when carrier females are examined, the retina shows minor signs of the disease.

Click here for Inheritance Pattern.

Treatment

At this time, there is no treatment for juvenile retinoschisis. However, in some cases, surgery can repair retinal detachments. Ongoing scientific research is directed at identifying the gene that causes juvenile retinoschisis as the first step in developing means of treatment and prevention.

Individuals with juvenile retinoschisis may benefit from the use of low-vision aids, including electronic, computer-based and optical aids, as well as orientation and mobility training.

 

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