Contributor: Gordon K. Klintworth
Congenital glaucoma (developmental glaucoma, infantile glaucoma) refers to a variety of disorders in morphogenesis in which increased intraocular pressure and glaucoma follow an obstruction to the drainage of aqueous humor by developmental anomalies. Infantile glaucoma may be primary [glaucoma - primary infantile] or secondary [glaucoma - secondary infantile]. The condition is often evident at birth (40%) (congenital glaucoma), but more often only during the first year of life (80%)(infantile glaucoma). Excessive tearing, photophobia and blepharospasm occur and often herald infantile glaucoma. Both eyes are usually involved and corneal cloudiness may result. It is most prominent centrally but may involve the entire cornea. Developmental anomalies are often limited to the anterior chamber angle, but a variety of other ocular malformations may also be present. The intraocular pressure may not become elevated until early infancy or childhood and when it occurs at < 3 years of age the globe enarges and  buphthalmos may eventually develop. As the cornea stretches and enlarges, Descemet membrane and the corneal endothelium often rupture causing stromal edema of the corneal epithelium and corneal stroma [edema - cornea] and corneal clouding. Control of intraocular pressure may clear the cornea, but endothelial damage and Haab striae from breaks in Descemet membrane can persist and cause recurrent corneal clouding. Common symptoms are photophobia and epiphora.