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Aust Dent J. 2010 Mar;55(1):51-8. doi: 10.1111/j.1834-7819.2009.01178.x.

Van der Woude syndrome: dentofacial features and implications for clinical practice.

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  • 1School of Dentistry, The University of Adelaide, South Australia, Australia.

Abstract

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.

PMID:
20415912
[PubMed - indexed for MEDLINE]
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