Hostname: page-component-78c5997874-s2hrs Total loading time: 0 Render date: 2024-11-19T11:46:05.498Z Has data issue: false hasContentIssue false
  • English
  • Français

Double epiglottis in Weyer's acrofacial dysostosis

Published online by Cambridge University Press:  29 June 2007

F. J. Wittig ,
S. A. Hickey  and
M. Kumar
F. J. Wittig
Affiliation:
Department of Otorhinolarngology, Torbay Hospital, Torquay, U.K.
S. A. Hickey*
Affiliation:
Department of Otorhinolarngology, Torbay Hospital, Torquay, U.K.
M. Kumar
Affiliation:
Department of Otorhinolarngology, Torbay Hospital, Torquay, U.K.
*
Address for correspondence: Mr. S. A. Hickey, Torbay Hospital, Lawes Bridge, TorquayTQ2 7AA. Fax: 01803 65 4898
Get access Rights & Permissions [Opens in a new window]

Abstract

While evaluating a 61-year-old patient for stridor we incidentally detected a double epiglottis. The patient was also diagnosed of having Weyer's acrofacial dysostosis which is characterized by hexadactyly affecting all four extremities, small and deeply set nails, dental deformities with small, conical teeth and mandibular hypoplasia. The double epiglottis was not the cause for the stridor. Because of the covert symptomatology of double epiglottis it is suggested that the association with Weyer's syndrome is common. Embryological evidence and a review of the literature on laryngeal abnormalities is discussed.

Keywords

EpiglottisCongenital defects
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 112 , Issue 10 , October 1998 , pp. 976 - 978
Copyright
Copyright © JLO (1984) Limited 1998

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Biesecker, L. G., Graham, J. M. (1996) Pallister-Hall syndrome. Journal of Medical Genetics 33: 585–589.CrossRefGoogle ScholarPubMed
Curry, C. J., Hall, B. D. (1979) Polydactyly, conical teeth, nail dysplasia and short limbs: a new autosomal dominant malformation syndrome. Birth Defects: Originat Articles Series. 15 (5B): 253–263.Google ScholarPubMed
Goldenberg, J. D., Holinger, L. D., Bressler, F. J., Hutchinson, L. R. (1996) Bifid epiglottis. Annals of Ototogy, Rhinology and Laryngology 105 (2): 155–157.CrossRefGoogle ScholarPubMed
Graham, J. M., Brown, F. E., Saunders, R. L., Hinkle, A. J., Frank, J. E., Harris, M. S., Klein, R. Z. (1985) Bifid epiglottis, hand anomalies and congenital hypopituitarism. Lancet 2 (8452): 443.CrossRefGoogle ScholarPubMed
Manning, K. P. (1977) The larynx in the cri du chat syndrome. Journal of Laryngology and Ototogy 91 (10): 887–892.CrossRefGoogle ScholarPubMed
McClay, J. E., Wiatrak, B., Proud, V. K. (1997) Bifid epiglottis and polydactyly: A new genetic syndrome. Otolaryngology – Head and Neck Surgery 116: 129–133.Google ScholarPubMed
O'Brien, F. C., Ginsberg, B. (1994) Cockayne syndrome: a case report. AANA Journal 62 (4): 346–348.Google ScholarPubMed
Roubicek, M., Spranger, J. (1984) Weyers acrodental dysostosis in a family. Clinical Genetics 26 (6): 587–590.CrossRefGoogle ScholarPubMed
Shapiro, S. D., Jorgenson, R. J., Salinas, C. F. (1984) Brief clinical report: Curry Hall syndrome. American Journal of Medical Genetics 17 (3): 579–583.CrossRefGoogle ScholarPubMed
Smith, R. J., Catlin, F. I. (1984) Congenital anomalies of the larynx. American Journal of Diseases of Children 138 (1): 35–39.Google ScholarPubMed
Spranger, S., Tariverdian, G. (1995) Symptomatic heterozygosity in the Ellis-van Creveld syndrome? Clinical Genetics 47 (4): 217–220.CrossRefGoogle ScholarPubMed