Congenital glaucoma unresponsive to conventional therapy: a clinicopathological case presentation.

A clinicopathological case report of a black male with congenital glaucoma is presented. The child died at age three months of the Sudden Infant Death Syndrome. The clinical course was characterized by persistent corneal clouding and elevated intraocular pressure in spite of vigorous medical and surgical therapy. Histopathological findings included corneal enlargement, marked hypoplasia of the canal of Schlemm, poorly developed scleral spur, insertion of the ciliary musculature directly into the trabecular meshwork anterior to the scleral spur, and anterior displacement of the iris root. There was also anomalous dispersion of the sphincter muscle of the iris from the pupillary margin almost to the iris root, and attachment of some ciliary processes to the posterior surface of the iris. A poor clinical response to conventional glaucoma therapy, including trabeculotomy, goniotomy, trabeculectomy and medical therapy, supports the belief that the marked hypoplasia of Schlemm's canal was a major factor contributing to the elevated intraocular pressure. Furthermore, the multiplicity of abnormalities of anterior chamber angle structures suggests that some cases of congenital glaucoma may result from an early and extensive disorder in the formation of anterior segment tissues.