Abstract

  Crouzon syndrome is the most common syndrome among the craniosynostosis group. Crouzon syndrome accounts for about 4.8% of all of them. It commonly has autosomal dominant inheritance with complete penetrance and variable expressivity from subtle to severe forms and characterized by craniosynostosis, exophthalmos, and hypoplastic maxilla with relative mandibular prognathism. Mutation of the fibroblast growth factor receptor-2 (FGFR2) gene is responsible for the occurrence of this rare genetic disorder.

Keywords: Craniosynostosis; Exophthalmos; FGFR-2

References

1. Crouzon LE. “Dysostose cranio-faciale héréditaire”. Bulletin de la Société des Médecins des Hôpitaux de Paris 33 (1912): 545-555.
2. Fries PD and Katowitz JA. “Congenital craniofacial anomalies of ophthalmic importance”. Survey of Ophthalmology 35 (1990): 87-119.
3. Pournima G., et al. “Crouzon syndrome: A case report”. European Journal of Dentistry and Medicine 10 (2011): 1-5.
4. Stephanie Gonçalves Carneiro., et al. “Crouzon's syndrome literature review”. International Archives of Otorhinolaryngology Sao Paulo 12 (2008): 436-441.
5. Maloth S., et al. “Diagnosis of Crouzon's syndrome”. Hong Kong Dental Journal 7 (2010): 95-100.
6. Padmanabham V., et al. “Crouzon's syndrome: A review of literature and case report”. Contemporary Clinical Dentistry 2 (2011): 211-214.
7. Babic GS and Babic RR. “Opthalmological and radiological picture of crouzon syndrome: A case report”. Acta Medica Medianae 48 (2009): 37-40.
8. Rani PJ., et al. “Crouzon syndrome: Acase report”. International Journal of Dental Case Reports 2 (2012): 117-122.
9. Singer SL., et al. “Dentofacial features of a family with Crouzon syndrome. Case reports”. Australian Dental Journal 42 (1997): 11-17.
10. Cohen MM. “Craniosynostosis update 1987”. American Journal of Medical Genetics Supply 4 (1988): 99-148.
11. Jarund M and Lauritzen C. “Craniofacial dysostosis: Airway obstruction and craniofacial surgery”. Scandinavian Journal of Plastic and Reconstructive Surgery 30 (1996): 275-279.

Citation

Citation: Priya Agarwal and Andrea Mae L Medriano. “Crouzon Syndrome: Classical Presentation of A Rare Entity”.Acta Scientific Dental Sciences 4.2 (2020): 103-105.

Copyright

Copyright: © 2020 Priya Agarwal and Andrea Mae L Medriano. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.