Synonyms Rieger anomaly Rieger malformation dysgenesis mesodermalis corneae et iridis dysgenesis mesostromalis anterior dysplasia marginalis posterior goniodysgenesis iridocorneal mesodermal dysgenesis iridodental dysplasia iridogoniodysgenesis with somatic anomalies mesodermal dysgenesis of anterior segment Summary Malformations of the anterior chamber of the eye and teeth with abnormalities involving the craniofacial structures, myotonic dystrophy, umbilical anomalies, and other variable defects.
Psychomotor retardation occurs in some cases.
Anterior displacement and thickening of Schwalbe's line, adhesion of the iris to Schwalbe's line, hypoplasia of the anterior stroma of the iris, vertical slip pupil, dyscoria, secondary aniridia, microcornea or megalocornea, corneal opacity, strabismus, coloboma, and glaucoma. Broad nasal root. Transmitted as an autosomal dominant trait.