Vitreoretinal Dystrophies

Vitreoretinal Dystrophies

Juvenile Retinoschisis

Juvenile retinoschisis is an inherited X-linked recessive disorder that affectsonly males. A retinal schisis at the macula sometimes referred to clinically as a “spoke phenomenon” usually develops between the ages of 20 and 30. This isassociated with a significant loss of visual acuity. A peripheral retinal schisis is also present in about half of these cases. This splitting of the retina is pre-sumably due to traction of the vitreous body. This splitting occurs in the nervefiber layer in contrast to typical senile retinoschisis, in which splitting occurs in the outer plexiform layer.

Wagner’s Disease

This disorder is also inherited (autosomal dominant) and involves central liquefaction of the vitreous body. This “visual void” in the vitreous chamber and fibrillary condensation of the vitreous stroma associated with a cataract characterize vitreoretinal degeneration in Wagner’s disease.