Introdução: A síndrome de Van der Woude (SVW) consiste em uma anormalidade genética atribuída, em uma grande parcela de casos, a variantes no gene IRF6, sendo caracterizada pela presença de fístulas paramedianas no lábio inferior, fissura de lábio e/ou palato e hipodontia. O fenótipo hipodontia vem sendo considerado uma característica da síndrome devido à sua alta prevalência. No entanto, outros fenótipos dentários têm sido pouco explorados na caracterização da síndrome de Van der Woude. Objetivo: Investigar se existe e qual seria o padrão de ocorrência de taurodontismo associado ou não à hipodontia na síndrome de Van der Woude Metodologia: Foram selecionados 110 sujeitos com diagnóstico confirmado da síndrome matriculados no Hospital de Reabilitação de Anomalias Craniofaciais (HRAC/USP) e analisadas a presença de taurodontismo e/ou hipodontias nas radiografias panorâmicas arquivadas. Resultados: 92,72% dos sujeitos apresentaram hipodontia, 42,72% apresentaram algum grau de taurodontismo. Em 40% da amostra essas duas anomalias cursavam juntas no fenótipo dentário. Houve uma maior prevalência de hipodontia dos incisivos laterais superiores, e para o taurodontismo uma maior prevalência dos primeiros molares superiores. Conclusão: o taurodontismo é um fenótipo frequentemente associado a hipodontia na SVW e deve ser considerado no detalhamento fenotípico para o diagnóstico e planejamento de reabilitação dessa síndrome.

Introduction: Van der Woude syndrome (SVW) is a genetic abnormality attributed, in a large number of cases, to variants in the IRF6 gene, characterized by the presence of paramedian fistulas in the lower lip, cleft lip and/or palate and hypodontia.The hypodontia phenotype has been considered a characteristic of SVW due to its high prevalence. However, other dental phenotypes have been comparatively underexplored in the characterization of the syndrome. Objective: To investigate whether there is and what would be the pattern of occurrence of taurodontism associated or not with hypodontia in Van der Woude syndrome. Methodology: 110 subjects with a confirmed diagnosis of the syndrome were selected at the Hospital for Rehabilitation of Craniofacial Anomalies (HRAC/USP) and analyzed for the presence of taurodontism and/or hypodontia in the archived panoramic x-rays. Results: 92.72% of the subjects had hypodontia, 42.72% had some degree of taurodontism. In 40% of the sample, these two anomalies co-occurred in the dental phenotype. There was a higher prevalence of hypodontia of maxillary lateral incisors, and for taurodontism a higher prevalence of maxillary first molars. Conclusion: Hypodontia was the most frequently found dental anomaly, which was expected, as it is already considered a characteristic of SVW. However, in almost half of the cases with hypodontia, this phenotype did not occur in isolation, but along with taurodontism. Thus, it is concluded that taurodontism is a phenotype frequently associated with hypodontia in SVW and should be considered in the phenotypic detail for the diagnosis and rehabilitation planning of this syndrome.