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The oculo-auriculo-fronto-nasal syndrome (OAFNS) - Description of a rare and complex craniofacial deformity and its interdisciplinary management before school age.
MedLine Citation:
PMID:  22245432     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Craniofacial clefts are certainly among the most challenging congenital malformations with respect to functional, aesthetic and psychosocial consequences. The aetiology is still under discussion, recent molecular genetic findings suggest defects in the ciliary function of neural crest cells during facial development. The severity of craniofacial clefting is known to be extremely variable. Different classifications have been proposed however nomenclature is not uniform. If vertical, median craniofacial clefting of fronto-naso-maxillary structures is accompanied by auriculo-mandibular malformations the term oculo-auriculo-fronto-nasal syndrome (OAFNS) has been proposed. Extreme craniofacial abnormalities have to be expected in this rare disorder. Adequate correction is a surgical challenge and interventions have to be adapted individually to patient's needs with respect to general condition, age and growth. This case report describes both the underlying pathology as well as the interdisciplinary management of a female patient from birth to 6years of age affected by this rare combination of vertical craniofacial clefting and bilateral auriculo-mandibular dysplasia.
Authors:
Nicolai Adolphs; Eric Arnaud; Ernst-Johannes Haberl; Luitgard Graul-Neumann; Gül Schmidt; Horst Menneking; Bodo Hoffmeister
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-1-12
Journal Detail:
Title:  Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery     Volume:  -     ISSN:  1878-4119     ISO Abbreviation:  -     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-1-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8704309     Medline TA:  J Craniomaxillofac Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 European Association for Cranio-Maxillofacial Surgery. Published by Elsevier Ltd. All rights reserved.
Affiliation:
Department of Oral and Maxillofacial Surgery (Head: Prof. Dr. med. Dr. med. dent. Bodo Hoffmeister), Clinical Navigation, University Hospital Charité, Campus Virchow-Klinikum, Augustenburger Platz 1, 13353 Berlin, Germany.
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