Vitreoretinal Dystrophies
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.
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.
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