ABSTRACT
The congenital glaucomas are a broad group of entities defined as glaucoma associated with a developmental anomaly that is present at birth. Primary congenital glaucoma is characterized by an isolated trabeculodysgenesis in the absence of visible iris or corneal abnormalities. Glaucoma may result from angle closure secondary to anterior segment crowding. Lowe’s syndrome is a crosslinked recessive condition with findings of aminoaciduria and mental retardation in affected males. Characteristic ocular findings are cataracts and open-angle glaucoma in infancy. The glaucoma may be due to a primary angle dysgenesis, synechial angle closure from neurofibroma tissue on the iris, or from direct infiltration of the angle by iris neurofibroma tissue. Epiphora, blepharospasm, and photophobia in the infant or young child comprise the classical triad of congenital glaucoma. The most common forms of congenital glaucomas are characterized by genetic heterogeneicity.
