| Van der Woude syndrome: dentofacial features and implications for clinical practice. | |
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MedLine Citation:
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PMID: 20415912 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Van der Woude syndrome (VWS) is the most common clefting syndrome in humans. It is characterized by the association of congenital lower lip fistulae with cleft lip and/or cleft palate. VWS individuals have a high prevalence of hypodontia. Although caused by a single gene mutation, VWS has variable phenotypic expression. This study aimed to describe the range of clinical presentations in 22 individuals with VWS to facilitate its diagnosis. METHODS: A retrospective study of 22 patients with a diagnosis of VWS was undertaken at the Australian Craniofacial Unit (ACFU) in Adelaide. Three extended families with affected members were included in the study cohort. RESULTS: The overall prevalence of lip pits in this study cohort was 86%. Cleft phenotypes included bilateral cleft lip and palate (32%); unilateral cleft lip and palate (32%); submucous cleft palate (23%); and isolated cleft hard and soft palate (9%). Missing permanent teeth were reported in 86% of affected individuals. CONCLUSIONS: Submucous cleft palate in VWS may go undiagnosed if the lower lip pits are not detected. Associated hypodontia and resultant malocclusions will also require management by a dental team. |
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Authors:
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A K Lam; D J David; G C Townsend; P J Anderson |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Australian dental journal Volume: 55 ISSN: 1834-7819 ISO Abbreviation: Aust Dent J Publication Date: 2010 Mar |
Date Detail:
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Created Date: 2010-04-26 Completed Date: 2010-07-15 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0370612 Medline TA: Aust Dent J Country: Australia |
Other Details:
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Languages: eng Pagination: 51-8 Citation Subset: D; IM |
Affiliation:
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School of Dentistry, The University of Adelaide, South Australia, Australia. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Anodontia
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epidemiology Cleft Lip / epidemiology*, genetics Cleft Palate / epidemiology*, genetics Cohort Studies Female Genes, Dominant / genetics Humans Lip / abnormalities* Lip Diseases / congenital*, epidemiology, genetics Male Oral Fistula / congenital*, epidemiology, genetics Palate, Soft / abnormalities Pedigree Penetrance Phenotype Prevalence Retrospective Studies South Australia / epidemiology Syndrome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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