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Greig's syndrome II

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Hereditary/familial ocular hypertelorism. Presents as abnormal increase in distance between the eyes due to development arrest of the greater wings of the sphenoid bone. The vertex is high and there is a low forehead. It is sometimes associated with osteogenesis imperfecta, Sprengel deformity, and some other anomalies of the skull. Mental deficiency occasionally associated. Etiology unknown; sporadic or hereditary (autosomal dominant or X-linked). The familial form of the condition is inherited as an autosomal dominant. 1926. ?

First described by Greig in 1924. He introduced the descriptive term «hypertelorism» to designate the wide separation of the eyes. Associated malformations and mental impairment may be present. Some cases occur spontaneously, but there is a familial tendency, cases having been observed in three successive generations. It is no longer regarded as a distinct entity, but as a malformation that may occur in a variety of different syndrome.


  • D. M. Greig:
    Hypertelorism. A hitherto undifferentiated congenital cranio-facial deformity.
    Edinburgh Medical Journal, 1924, 31: 560-593.

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