title: Clinical manifestations and oral findings in Fraser syndrome.
abstract: This article is the first known case report of Fraser syndrome in the dental literature. Its purpose was to present the clinical manifestations, oral findings, and dental treatment of a 14-year, 10-month-old female patient. Fraser syndrome is a rare recessive autosomal genetic disorder characterized by multisystemic malformation, usually comprising cryptophthalmos, syndactyly, and renal defects. The child presented with: (1) hydrocephaly; (2) face asymmetry; (3) low-inserted ears; (4) flat nose bridge; (5) cryptophthalmos; (6) bilateral absence of eyeballs; (7) hypertelorism; (8) syndactyly on the left fingers and toes; (9) skeletal defects; and (10) lower limb asymmetry. The intraoral examination revealed: (1) complete primary denture; (2) malocclusion; (3) tooth crowding; (4) ogival palate; (5) normal labial frena; (6) absence of lingual frenum (not compromising the tongue movements); (7) parched lips; (8) supragingival calculus adhered to all tooth surfaces; and (9) moderate gingivitis. The dental treatment consisted of periodic monitoring of the patient's oral health status and supragingival scaling associated with topical applications of 0.12% chlorhexidine digluconate gel at 2-week intervals to reduce gingivitis.
authors.affiliation: Department of Pediatric Dentistry, School of Dentistry of Araraquara, São Paulo State University (UNESP), Araraquara, São Paulo, Brazil.
authors.forename: Michele Baffi
authors.initials: MB
authors.lastname: Diniz
authors.forename: Luciana Monti
authors.initials: LM
authors.lastname: Lima
authors.forename: Nancy Tomoko
authors.initials: NT
authors.lastname: Sacono
authors.forename: Andréia Bolzan
authors.initials: AB
authors.lastname: de Paula
authors.forename: Lourdes
authors.initials: L
authors.lastname: dos Santos-Pinto
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology; D002675:Child, Preschool; D003241:Consanguinity; D003728:Dental Calculus / Q000628:therapy; D012534:Dental Scaling; D005124:Eye Abnormalities; D005260:Female; D005891:Gingivitis / Q000628:therapy; D006801:Humans; D006972:Hypertelorism; D008035:Lingual Frenum / Q000002:abnormalities; D008310:Malocclusion; D009056:Mouth Abnormalities; D021362:Palate, Hard / Q000002:abnormalities; D013576:Syndactyly; D013577:Syndrome; D014094:Tooth, Deciduous