Rieger Syndrome: A genetically determined syndrome of the
anterior chamber of the eyes and the teeth, combining features of the
Axenfeld syndrome with oligodontia. An opaque ring at the margin of the
cornea, iris adhesion to the Schwalbe ring, hypoplasia of the anterior
stroma of the iris, and tooth anomalies. Other features include hypoplasia
of the malar bones, broad and flat nose, prognathism, and hypertelorism.
Myotonic dystrophy, mental retardation, brachydactyly, clinodactyly,
arachnodactyly, and polydactyly have been reported. Autosomal dominant
inheritance but sporadic cases are also seen, suggesting a recessive
pattern. When occurring without the dental and skeletal defects, it is
called the SHORT syndrome.