Características clínicas em sujeitos com síndrome de van der woude: uma revisão integrativa / Clinical characteristics in subjects with van der woude syndrome: an integrative review

Authors

  • Andréa Guedes Barreto Gonçales
  • Carolina Maia Silva
  • Maria Carolina de Moraes Pereira
  • Lucimara Teixeira das Neves

DOI:

https://doi.org/10.34115/basr.v3i4.2648

Keywords:

síndrome de Van der Woude, fenótipos clínicos, fístula labial congênita, fissura de lábio e/ou palato, hipodontia.

Abstract

A síndrome de Van der Woude (SVW) é a forma sindrômica mais comumente descrita em pacientes com fissuras labiopalatinas. Essa anomalia com padrão de herança autossômico dominante apresenta como principais características fenotípicas as fístulas congênitas no lábio inferior, fissura de lábio e/ou palato e hipodontia. Apesar dessas características serem bem fundamentadas no diagnóstico de sujeitos com SVW, pode ocorrer uma gama de variações fenotípicas dessas características, o que dificulta o diagnóstico clínico desses sujeitos. Por essa razão, esse estudo se propôs a realizar essa revisão de literatura integrativa, analisando os artigos publicados que apresentam as descrições dos fenótipos clínicos. Para tal, foram realizadas buscas em bases de dados internacionais, por meio das quais foram selecionados 9 artigos relacionados com o tema. A fístula labial e a fissura de lábio e/ou palato foram relatadas em todos os estudos, porém em alguns estudos, essas anomalias apresentaram formas atípicas. Já a hipodontia foi associada a SVW em 3 estudos. Na presente análise foi possível verificar que a SVW apresenta um amplo espectro de características variáveis, o que pode dificultar o diagnóstico final. A descrição das possibilidades de expressividade variável da síndrome pode contribuir para um diagnóstico mais preciso de sujeitos com SVW.

 

 

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Published

2019-08-09

How to Cite

Gonçales, A. G. B., Silva, C. M., Pereira, M. C. de M., & Neves, L. T. das. (2019). Características clínicas em sujeitos com síndrome de van der woude: uma revisão integrativa / Clinical characteristics in subjects with van der woude syndrome: an integrative review. Brazilian Applied Science Review, 3(4), 1809–1820. https://doi.org/10.34115/basr.v3i4.2648

Issue

Section

Original articles