Clinical manifestations and oral findings in Fraser syndrome.
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.
Diniz
Michele Baffi
MB
Department of Pediatric Dentistry, School of Dentistry of Araraquara, São Paulo State University (UNESP), Araraquara, São Paulo, Brazil.
Lima
Luciana Monti
LM
Sacono
Nancy Tomoko
NT
de Paula
Andréia Bolzan
AB
dos Santos-Pinto
Lourdes
L
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