Affected individuals often have an unusual
dark-gray or chocolate-brown eye color as a result of the iris epithelium
showing through the hypoplastic iris stroma. All patients are at risk for
developing juvenile glaucoma, which is often diagnosed in the second decade
of life, although it can begin at any age. The glaucoma is often resistant
to medical therapy and, in the absence of surgical intervention, blindness
may occur. Nonocular features, such as maxillary hypoplasia, dental
anomalies (microdontia, hypodontia), umbilical hernia, and hypospadias, may
present as part of the syndrome.