Peters' congenital glaucoma results from a genetic developmental disorder occurring at the end of the third week of uterine life when the mesencephalic neural crests migrate. Trabecular dysgenesis causes glaucoma, which is generally bilateral. Descemet's membrane and the endothelium at the center of the cornea fail to develop, leading to opacities in the central cornea with iridocorneal synechia that stretch from the edge of the irides to the central opacity. The anterior chamber is narrow and may adhere to the lens. Cataract can be associated. Other possible signs include dysmorphic facies and hypospadias. The disease is transmitted as an autosomal recessive trait. Along with the treatment of glaucoma and cataract, total keratoplasty may sometimes be necessary because of the affected cornea.
Last update: July 2005