title: Features of oligodontia in three generations.
abstract: Congenitally missing teeth of three generations of a family and the spousal influence on the oligodontia of the third generation is investigated. For clarification, a review of commonly used terms is included in the paper. Although there are a number of factors affecting the failure to develop permanent tooth buds, most researchers believe that an autosomal dominant trait is responsible for the oligodontia in this family. Many syndromes are associated with oligodontia, but none seem to be prevalent in the direct descendants of the first generation. A member of the second generation married a person with Witkop's (tooth-and-nail) syndrome. Subsequently, their child exhibits the same manifestations. A feature noted in this three generation pedigree is missing permanent first molars. This is considered rare, especially when few abnormal ectodermal findings are reported. Additional findings include an increased number of missing teeth, decreased tooth size and a prominent maxillary labial frenum. The dental treatment for patients affected will likely involve a multidisciplinary approach. The treatment is particularly important since the lack of a full complement of teeth impacts the emotional and physical well-being of the individual. Children who are missing permanent teeth should be screened for other ectodermal abnormalities to rule out syndromes associated with congenitally missing teeth. Oligodontia is predicted to affect fifty percent of the fourth generation. It may vary in penetrance and expression.
authors.affiliation: Department of Dental Hygiene, University of Nebraska Medical Center College of Dentistry, Lincoln 68583-0740, USA.
authors.forename: J M
authors.initials: JM
authors.lastname: Stimson
authors.forename: J E
authors.initials: JE
authors.lastname: Sivers
authors.forename: G L
authors.initials: GL
authors.lastname: Hlava
mesh_terms: D000848:Anodontia / Q000145:classification / Q000235:genetics*; D004476:Ectodermal Dysplasia / Q000235:genetics; D005260:Female; D015870:Gene Expression; D006801:Humans; D008297:Male; D008963:Molar; D009260:Nail Diseases / Q000151:congenital; D010375:Pedigree; D019683:Penetrance; D013577:Syndrome; D009626:Terminology as Topic; D014083:Tooth Germ