title: The tricho-rhino-phalangeal syndrome: oral manifestations and management.
abstract: Tricho-rhino-phalangeal Syndrome (TRPS) is a rare inherited dysplasia affecting hair, nasal structure and fingers. A literature review indicated that since first described, three types of manifestations have been identified. A Table summarising the oral manifestations demonstrates the variety of presentations. A South African male child presented with the syndrome and was found to show premature eruption of permanent teeth, a finding that has not been previously reported. His oro-facial manifestations also included malaligned and unerupted crowded teeth, bulbosity of the nasal tip and an elongated philtrum and evidence of mild intellectual impairment. A protocol has been developed to guide the future management of these cases.
authors.forename: T
authors.initials: T
authors.lastname: Roberts
authors.forename: M
authors.initials: M
authors.lastname: Chetty
authors.forename: L
authors.initials: L
authors.lastname: Stephen
authors.forename: K
authors.initials: K
authors.lastname: Fieggen
authors.forename: P
authors.initials: P
authors.lastname: Beighton
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology; D002675:Child, Preschool; D005385:Fingers / Q000002:abnormalities*; D005500:Follow-Up Studies; D005892:Gingivitis, Necrotizing Ulcerative / Q000175:diagnosis; D006197:Hair / Q000002:abnormalities*; D006209:Halitosis / Q000175:diagnosis; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000175:diagnosis*; D009666:Nose / Q000002:abnormalities*; D011378:Prognathism / Q000175:diagnosis; D013577:Syndrome; D014076:Tooth Diseases / Q000175:diagnosis*; D014078:Tooth Eruption / Q000502:physiology