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Rieger's syndrome

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A genetically determined syndrome of malformations of the anterior chamber of the eye and the teeth, combining features of the Axenfeld syndrome with oligodontia. Eye anomalies consist of posterior embryotoxon (an opaque ring at the margin of the cornea), a prominent Schwalbe ring, iris adhesion to the Schwalbe line, hypoplasia of the anterior stroma of the iris, and occasional glaucoma. Tooth anomalies include anondontia vera and microdontia.

Other features can be hypoplasia of the malar bones, broad flat nasal root, prominent supraorbital ridges, relative prognathism, and mild telecanthus with or without hypertelorism. There may also be other defects such as short stature, myotonic dystrophy, mental deficiency, brachydactyly, clinodactyly, arachnodactyly, and polydactyly. Inheritance is autosomal dominant but sporadic cases are seen and this suggests a recessive pattern also. When occurring without dental and skeletal defects, it is called the SHORT syndrome. The Axenfeld and Rieger syndromes are suspected of being expressions of the same gene.

We thank Hilde Nordgarden for correcting an error on our original entry.

See also Axenfeld's syndrome, under Karl Theodor Paul Polykarpus Axenfeld, German ophthalmologist, 1867-1930.


  • H. Rieger:
    Dysgenesis mesodermalis corneae et iridis.
    Zeitschrift für Augenheilkunde, 1935, 86: 333. Beiträge zur Kenntnis seltener Missbildungen der Iris. II. Über Hypoplasie des Irisvorderblattes mit Verlagerung und Entrundung der Pupille.
    Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie, 1935, 133: 602-635. Erbfragen in der Augenheilkunde.
    Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie, 1941, 143: 277-299.
  • T. Axenfeld:
    Embriotoxon corneal posterius.
    Berichte der Deutschen ophthalmologischen Gesellschaft, 1920, 42: 301.
  • Robert James Gorlin (born 1923), et al:
    Rieger anomaly and growth retardation (the S-H-O-R-T syndrome).
    Birth Defects Original Article Series, New York, 1975, 11(2): 46-48.
  • J. A. Sensenbrenner, et al:
    A low birthweight syndrome, Rieger syndrome.
    Birth Defects Original Article Series, New York, 1975, 11(2): 423-426.

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