PMID: 9176000Apr 1, 1997Paper

Craniofrontonasal dysplasia

British Journal of Plastic Surgery
D J OrrM D Poole

Abstract

A series of 10 patients with craniofrontonasal dysplasia presenting to the Oxford Craniofacial Unit since 1983 is presented. In addition to the well-described combination of coronal synostosis and frontonasal dysplasia, 9 patients had very characteristic dry, curly or frizzy hair. All the patients were female. Recognition of the syndrome is important for genetic counselling, although the precise mode of genetic transmission is unclear with females predominating and males being less severely affected. Surgical correction was in two stages: early frontal advancement followed by correction of hypertelorism when the child became aware of the deformity. Four patients had their craniosynostosis treated in the Oxford Craniofacial Unit. Three patients had previously had frontal remodelling elsewhere. Nine patients had surgery for hypertelorism. The preferred technique for hypertelorism correction was facial bipartition. Following hypertelorism correction, the excess skin was allowed to redrape and subsequently dealt with by medial canthoplasties, thus avoiding a midline scar. Careful attention to the primary frontal advancement procedure is important to avoid complications following difficult dissection of the frontal bone flap at the ...Continue Reading

References

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Citations

May 10, 2002·Prenatal Diagnosis·Pasquale MartinelliDario Paladini
Nov 28, 2013·European Journal of Human Genetics : EJHG·M E P van den ElzenI M J Mathijssen
Apr 28, 2006·European Journal of Human Genetics : EJHG·Pradeep C VasudevanAndrew O M Wilkie
May 29, 2004·Proceedings of the National Academy of Sciences of the United States of America·Stephen R F TwiggAndrew O M Wilkie
Sep 16, 2010·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Bradley A HubbardArshad R Muzaffar
Mar 12, 2002·The Journal of Craniofacial Surgery·John B Mulliken
Apr 30, 2019·Plastic and Reconstructive Surgery·Rafael DenadaiCassio Eduardo Raposo-Amaral
May 21, 2019·The Journal of Craniofacial Surgery·Ahmed Mohamed MedraKhalid Nasser Fadle
May 7, 2020·The Journal of Craniofacial Surgery·Sophie DupréSarah Kilcoyne
Dec 20, 2007·Plastic and Reconstructive Surgery·Henry K KawamotoJames P Bradley
Oct 12, 2017·Revista do Colégio Brasileiro de Cirurgiões·Rafael DenadaiCassio Eduardo Raposo-Amaral
Feb 27, 2018·The Journal of Craniofacial Surgery·Rafael DenadaiCassio Eduardo Raposo-Amaral
Jan 6, 2004·The Journal of Craniofacial Surgery·Jolanda M E OkkerseJosien A Heineman-de Boer
Jan 9, 2020·Archives of Craniofacial Surgery·Se Il LeeHong Sil Joo

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