Diniz, Michele Baffi [UNESP]Lima, Luciana Monti [UNESP]Sacono, Nancy Tomoko [UNESP]De Paula, Andréia BolzanSantos-Pinto, Lourdes Aparecida Martins dos [UNESP]2014-05-272014-05-272007-09-01Journal of Dentistry for Children, v. 74, n. 3, p. 231-235, 2007.1551-89491935-5068http://hdl.handle.net/11449/69861This 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.231-235engFraser syndromeOral findingsTooth anomaliescongenital malformationconsanguinitydentitioneye malformationfemalegingivitishard palatehumanhypertelorismmalocclusionmouth diseasemultiple malformation syndromepathologypreschool childpreventive dentistrysyndactylysyndrometonguetooth calculusAbnormalities, MultipleChild, PreschoolConsanguinityDental CalculusDental ScalingDentition, PrimaryEye AbnormalitiesFemaleGingivitisHumansHypertelorismLingual FrenumMalocclusionMouth AbnormalitiesPalate, HardSyndactylySyndromeArtigoAcesso restrito2-s2.0-479491134980000-0003-2386-842X