vitalwiki

Reticular dystrophy of the retinal pigment epithelium

ORPHA:99002· ICD-10 H35.5

Definition

A rare, patterned dystrophy of the retinal pigment epithelium, of progressive course, characterized by the presence of a bilateral hyperpigmented reticular pattern resembling a fishnet with knots, resulting in a slowly progressive loss of vision that often only becomes apparent in old age. This disorder is sometimes associated with scleral staphyloma, choroidal neovascularization, convergent strabismus, spherophakia with myopia and luxated lenses, and partial atrophy of the iris.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive, Unknown
Age of onset
Adult