title: Hereditary gingival fibromatosis with distinct dental, skeletal and developmental abnormalities.
abstract: A case of a 9-year-old child with hereditary gingival fibromatosis, supernumerary tooth, chest deformities, auricular cartilage deformation, joint laxity and undescended testes is described. The exact mode of inheritance is unclear; a new mutation pattern is possible. These features resemble but differ from the previously reported Laband syndrome. The dental treatment consisted of surgical removal of the fibrous tissue and conservative restorative treatment under general anesthesia. The dental practitioner should be alert for developmental abnormalities such as supernumerary teeth and delayed tooth eruption. A comprehensive medical history and physical systemic evaluation is essential to rule out other systemic abnormalities. Genetic consultation is mandatory for future family planing.
authors.affiliation: Department of Oral and Maxillofacial Surgery, University of Florida, Gainesville, USA. jkatz@dental.ufl.edu
authors.forename: Joseph
authors.initials: J
authors.lastname: Katz
authors.forename: Marcio
authors.initials: M
authors.lastname: Guelmann
authors.forename: Shlomo
authors.initials: S
authors.lastname: Barak
mesh_terms: D000015:Abnormalities, Multiple; D002648:Child; D003456:Cryptorchidism; D004431:Ear, External / Q000002:abnormalities; D005351:Fibromatosis, Gingival / Q000235:genetics* / Q000601:surgery; D030342:Genetic Diseases, Inborn; D006801:Humans; D007593:Joint Instability; D008297:Male; D009154:Mutation; D013249:Sternum / Q000002:abnormalities; D013577:Syndrome; D014096:Tooth, Supernumerary; D014097:Tooth, Unerupted