RESUMO
The aim of this article is to review possible cranio-maxillofacial deformative consequences associated with hypohidrotic ectodermal dysplasia and embryonic malformations, which include dental ageneses, and describe the oral habilitation. Hypohidrotic ectodermal dysplasia patients had a clinical examination and underwent radiographic and Steiner's analyses and a respiratory capability test before assessment and treatment. Fifteen patients (eight males and seven females, aged 5-45 years) had tooth ageneses (from hypodontia to anodontia) associated with cutaneous dyshydrosis and hair and nail dystrophy. Most patients had sparse or absent hair, a short face with an unusual facial concavity, a maxillary retrusion and a relative mandibular protrusion. Dentists must conduct a comprehensive and multidisciplinary approach to these patients in order to improve their dental, masticatory, growth and orthognathic conditions.