Syndromic hypodontia and oligodontia: Ectodermal dysplasias

Abstract

The lack of tooth formation or dental dysmorphic morphology is one of the phenotypes of various forms of the ectodermal dysplasias (ED). Teeth that do form are often altered in shape and contour and occasionally manifest with delayed eruption. The oral health-care team needs to work with affected individuals starting around the first year of life with age-appropriate and staged oral health interventions through adulthood. For most patients with ED, definitive rehabilitation (with or without implant treatment) should be delayed until skeletal maturity. Having said this, starting around age 2-3, pediatric removable prosthesis is appropriate with pediatric dentist-guided care through adulthood. As a part of the final reconstruction as a young adult, the team needs to plan for maintenance issues and complications and use a defined set of diagnostic risk factors in the assessment of the affected individual. Through careful assessment, planning, good team communication, and partnership with the affected individual and her/his family, a successful long-term solution can be developed that provides the best in patient-oriented outcomes.