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ENT manifestations of Fraser syndrome

Published online by Cambridge University Press:  29 June 2007

G. R. Ford ,
R. M. Irving ,
N. S. Jones  and
C. M. Bailey
G. R. Ford*
Affiliation:
London
R. M. Irving
Affiliation:
London
N. S. Jones
Affiliation:
London
C. M. Bailey
Affiliation:
London
*
Mr G. R. Ford, F.R.C.S., ENT Department, Cambridge Military Hospital, Aldershot, Hants Gu11 2AN.
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Abstract

Fraser Syndrome is a rare autosomal recessive disorder (Gupta and Saxena, 1962; Smith, 1982). The most consistent feature is cryptophthalmos (hidden eye), but frequently abnormalities of the ears (meatal stenosis, dysplastic pinna), nose (hypoplastic notched nares, choanal stenosis or atresia), and larynx (glottic web, subglottic stenosis), as well as numerous other anomalies are encountered. We present four cases that have been treated at the Hospital for Sick Children in the last ten years, and describe the various ENT anomalies characteristic of this syndrome.

Keywords

Genetic/familialCryptophthalmosFraser Syndrome
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 106 , Issue 1 , January 1992 , pp. 1 - 4
Copyright
Copyright © JLO (1984) Limited 1992

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