How to cite this article: Lees MM, Kangesu L, Hall P, Hennekam RCM. 2007. Two siblings with an unusual nasal malformation: Further instances of craniorhiny? Am J Med Genet Part A 143A:3290–3294.
Version of Record online: 26 OCT 2007
Copyright © 2007 Wiley-Liss, Inc.
American Journal of Medical Genetics Part A
Special Issue: M. Michael Cohen Jr. Festschrift
Volume 143A, Issue 24, pages 3290–3294, 15 December 2007
How to Cite
Lees, M. M., Kangesu, L., Hall, P. and Hennekam, R. C.M. (2007), Two siblings with an unusual nasal malformation: Further instances of craniorhiny?. Am. J. Med. Genet., 143A: 3290–3294. doi: 10.1002/ajmg.a.32026
Dedicated to M. Michael Cohen Jr. for his friendship and sharing his knowledge about “noses nobody knows.”
- Issue online: 28 NOV 2007
- Version of Record online: 26 OCT 2007
- Manuscript Accepted: 4 JUL 2007
- Manuscript Received: 2 MAY 2007
- bifid nose;
- nasal dermoid;
- autosomal recessive
We report a brother and sister born to consanguineous parents. The siblings have hypertelorism, bifid nose, upturned nares, histologically proven intranasal dermoid, and soft-tissue swellings of the philtrum. One sibling also has a midline cleft lip and the other has narrowing of the posterior choanae. We suggest that they have craniorhiny, despite the absence of an abnormal skull shape. The differential diagnosis is discussed. © 2007 Wiley-Liss, Inc.