Document Detail


Saethre-Chotzen syndrome: review of the literature and report of a case.
MedLine Citation:
PMID:  11314068     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Saethre-Chotzen syndrome is an autosomal acrocephalosyndactyly syndrome whose gene has been assigned to chromosome 7p (TWIST). A case of a 13-year-old girl with Saethre-Chotzen syndrome (ACS III) is described. The features of the syndrome include: turriplagiocephaly with a cranial circumference of 52 cm, facial asymmetry, low hairline, proptosis, antimongoloid slanting of palpebral fissures, nasal deviation with high bridge, angled ears, scoliosis and torticollis, clinodactyly of the fourth and fifth toes, large halluxes, and neurosensorial hypoacusia. For correction of the deformity, a cranioorbital remodeling was performed. The craniofacial approach with remodeling of the frontal bar and reduction of the turricephaly resulted in a satisfactory morphological and functional outcome, with complete three-dimensional reshaping and remodeling of the frontonasoorbital area.
Authors:
L Clauser; M Galiè; A Hassanipour; O Calabrese
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  The Journal of craniofacial surgery     Volume:  11     ISSN:  1049-2275     ISO Abbreviation:  J Craniofac Surg     Publication Date:  2000 Sep 
Date Detail:
Created Date:  2001-04-20     Completed Date:  2001-05-24     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  9010410     Medline TA:  J Craniofac Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  480-6     Citation Subset:  D    
Affiliation:
Department of Craniomaxillofacial Surgery, St. Anna Hospital, Corso Giovecca 203, 44100 Ferrara, Italy.
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MeSH Terms
Descriptor/Qualifier:
Acrocephalosyndactylia / genetics,  pathology*,  surgery
Adolescent
Chromosomes, Human, Pair 7 / genetics
Craniosynostoses / pathology
Exophthalmos / pathology
Eyelids / pathology
Facial Asymmetry / pathology
Female
Frontal Bone / surgery
Humans
Nose / abnormalities,  surgery
Orbit / surgery
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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