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Axenfeld's syndrome

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A syndrome characterised by posterior corneal embryotoxon, prominent Schwalbe's line and iris adhesion to the Schwalbe’s line. Other features may be hypertelorism (wide spacing of the eyes), hypoplasia of the malar bones, congenital absence of some teeth and mental retardation. When associated with tooth anomalies, the disorder is known as Rieger's syndrome. The eponym of Axenfeld is also used as synonym for Hagedoom's mesostromal digenesis. Axenfeld and Rieger syndromes are suspected as being expressions of the same gene. Aetiology unknown. Inheritance is autosomal dominant.

See also Rieger's syndrome, under Herwigh Rieger, Austrian ophthalmologist, 1898-1986.


  • T. Axenfeld:
    Embryotoxon cornea posterius.
    Berichte der Deutschen ophthalmologischen Gesellschaft, 1920, 42: 301.
  • H. Rieger:
    Über Subconjunctivitis epibulbous metastatica bei Parotitis epidemica.
    Archiv für Ophthalmologie, Berlin, 1935, 133: 505-507.
  • A Hagedoorn:
    Congenital anomalies of the anterior segment of the eye.
    Archives of Ophthalmology, Chicago, 1937, 17: 223-227.
  • J. G. Montes, J. C. G. Montes:
    Syndrome de Rieger, anomalie de Axenfeld con glaucoma juvenil familiar.
    Archivos de la Sociedad oftalmologica hispano americana, 1967, 27: 93-99.

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