Manifestações bucais em pacientes com síndrome de Kabuki

AUTOR(ES)
DATA DE PUBLICAÇÃO

2010

RESUMO

Kabuki syndrome (KS, Kabuki make-up syndrome, Niikawa-Kuroki syndrome) is a rare genetic disorder characterized by multiple congenital anomalies and mental retardation. The diagnosis is clinical and based on the findings described simultaneously in independent studies of two groups in Japan. The sample comprised sixteen KS individuals diagnosed by Department of Clinical Genetics, University Hospital of Brasilia, Brasilia, Brazil with ages between 8 to 24 years of both genders. Each individual underwent complete physical examination, as well as intra-oral and radiographic examinations. Extra-oral and intra-oral photographs were also performed. Craniofacial and dental anomalies were observed in all KS patients examined. Seven of the 16 individuals had class III malocclusion, probably due to maxillary recession and mid-face hypoplasia. Also, high arched palate was observed in twelve individuals. Flattening of the condyle was revealed in two individuals. The most common dental anomaly observed was teeth shape abnormalities, mainly screwdriver incisors. Moreover, atypical shape of the premolar s and molar s crowns and root dilaceration were also observed. Hypodontia and microdontia were present in 9 and 4 individuals, respectively. Enamel diffuse opacities were reported in permanent dentition (n=10). A great clinical heterogeneity was observed in KS individuals in line with other previous studies in the literature. Some non previously reported anomalies were also observed in this sample, such as crown anomalies, root shape, flattening of the condyle and fibrosis of raphe palate. Further clinical and molecular studies are necessary in order to better understand the presence of dental anomalies in this syndrome.

ASSUNTO(S)

dentes-anomalias ciencias da saude

ACESSO AO ARTIGO

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