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Clinical manifestations and oral findings in fraser syndrome
  • Universidade Estadual Paulista (UNESP)
  • Universidade Estadual de Campinas (UNICAMP)
  • 1551-8949
  • 1935-5068
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.
Issue Date: 
Journal of Dentistry for Children, v. 74, n. 3, p. 231-235, 2007.
Time Duration: 
  • Fraser syndrome
  • Oral findings
  • Tooth anomalies
  • congenital malformation
  • consanguinity
  • dentition
  • eye malformation
  • female
  • gingivitis
  • hard palate
  • human
  • hypertelorism
  • malocclusion
  • mouth disease
  • multiple malformation syndrome
  • pathology
  • preschool child
  • preventive dentistry
  • syndactyly
  • syndrome
  • tongue
  • tooth calculus
  • Abnormalities, Multiple
  • Child, Preschool
  • Consanguinity
  • Dental Calculus
  • Dental Scaling
  • Dentition, Primary
  • Eye Abnormalities
  • Female
  • Gingivitis
  • Humans
  • Hypertelorism
  • Lingual Frenum
  • Malocclusion
  • Mouth Abnormalities
  • Palate, Hard
  • Syndactyly
  • Syndrome
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Appears in Collections:Artigos, TCCs, Teses e Dissertações da Unesp

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