title: A previously unreported association between Nance-Horan syndrome and spontaneous dental abscesses.
abstract: Atypical dentofacial structures may be the first indicator of other anomalies linked to a syndrome. This case describes the management of a 9-year-old girl referred for the routine management of supernumerary teeth. The anomalous form of her teeth, together with multiple supernumerary units and a history of congenital cataracts, were suggestive of a diagnosis of Nance-Horan syndrome. This is an X-linked disorder, in which females usually demonstrate mild expression; this case was unusual in respect to the marked phenotype expressed. Unusually, the girl developed 2 spontaneous abscesses of her noncarious upper incisor teeth; a feature never previously described in this syndrome. This report details the patient's dental management and discusses the possible pathogenesis of the dental abscesses, together with the genetic implications of this syndrome.
authors.affiliation: Department of Paediatric Dentistry, Westmead Centre for Oral Health, Westmead, NSW 2145 Australia. sally_hibbert@wsahs.nsw.gov.au
authors.forename: Sally
authors.initials: S
authors.lastname: Hibbert
mesh_terms: D002386:Cataract / Q000151:congenital; D002648:Child; D003719:Dens in Dente / Q000473:pathology; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000473:pathology; D006801:Humans; D007180:Incisor / Q000002:abnormalities; D008439:Maxillary Diseases / Q000473:pathology; D008963:Molar / Q000002:abnormalities; D010482:Periapical Abscess / Q000473:pathology*; D013577:Syndrome; D014071:Tooth Abnormalities / Q000235:genetics / Q000473:pathology*; D014096:Tooth, Supernumerary / Q000473:pathology