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Total laryngotracheal hypoplasia in a case of G syndrome

Published online by Cambridge University Press:  29 June 2007

J. G. Buckley ,
A. E. Hinton ,
G. Penter  and
P. A. Fardon
J. G. Buckley*
Affiliation:
E.N.T. Department Selly Oak Hospital
A. E. Hinton
Affiliation:
E.N.T. Department Selly Oak Hospital
G. Penter
Affiliation:
Radiology Department, Birmingham Children's Hospital.
P. A. Fardon
Affiliation:
Clinical Genetics Department, Birmingham Maternity Hospital.
*
ENT Department, St. Thomas' Hospital, London SE1 7EH.
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Abstract

We report a case of congenital hypoplasia of the larynx and trachea in the presence of an essentially normal cartilaginous structure. To knowledge this abnormality is not recognized in the literature. Previously reported hypoplasitc laryngeal anomalies have all shown anatomical defects ranging form clefts to atresia (Smith and Bain 1965, Gatti et al.,1987). Similarly total congenital tracheal stenosis is accompanied by abnormalities of the cartilaginous structure, usually complete tracheal rings. These types of anomaly typically present either at, or soon after, birth and are associated with other congenital abnormalities. In this particular case the largngotracheal hypoplasia occurred in the context of a hereditary condition of multiple congenital abnormalities known as G syndrome (also known as Opitz-Frias syndrome or the Opitz-G syndrome).

Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 102 , Issue 11 , November 1988 , pp. 1056 - 1059
Copyright
Copyright © JLO (1984) Limited 1988

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