Abnormalities of the central iris plane may progress during life, whereas irregularities of the peripheral iris or anterior chamber do not tend to progress after birth. Glaucoma is a major consideration in these patients. The extent of iris defects and iridocorneal strands do not correlate with presence or severity of glaucoma. On the other hand, high insertion of peripheral iris into trabecular meshwork is often more pronounced in eyes with glaucoma. Extraocular Features Facial anomalies that may be seen are maxillary hypoplasia, protrusion of lower lip broad and flat nose, hypertelorism, and telecanthus.
Dental anomalies can occur including anodontia, oligodontia, microdontia, or hypodontia.
Histopathology Most of the work has been done by Shields and colleagues. In some areas, the strand is composed of both the iris tissue and the membrane. Iris peripheral to iridocorneal adhesions usually inserts into posterior aspects of trabecular meshwork. There is a reduced number of openings in each trabecular layer, especially near Schlemm's canal. Examination by transmission electron microscopy has revealed incomplete development of trabecular meshwork.