There have been few reports described in detail about the dental manifestations of the oral-facial-digital syndrome in adult. A case of an 18-year-old Japanese female with the anomalous dentition is reported here.
In the family history of the patient, no speciality was found except cousinhood of her parents. The extremities were normal in appearance, but prognathism and slight asymmetry of the mandible were observed. As for the intraoral findings, the thick labial frenulumencroached on the alveolar process of the mandible at the midline. Multiple and thick labial frenula were observed on the upper jaw, but cleft of the alveolar process by them was not found. The palate was high-vaulted, but not cleft. Slight ankyloglossia and many odd-shaped teeth were found. All the third molars and the upper lateral incisors were congenitally missing. The marked development was observed on the marginal ridges, labial ridges and the lingual tuberculum of the four lower incisors. The right lower central and lateral incisors were fused. Each incisal edge of the four canines wasflat like an incisor, and the lingual side of the lower canines was shovel-shaped. The lingual tuberculum of the upper canines was markedly accentuated. The slight prominence of the labial and thebuccal collar was observed on the four canines and the lower first premolars. The cusps of the posterior teeth were relatively round, comparing with normal teeth. Theocclusal surface of the posterior teeth was flattened with a various degrees of wrinkling, especially the lower second premolars were almost round and there were supernumerary and small cusps on them. Roentgenographic examination revealed rhizomikry in entire dentition, especially all the first molars had only one root. The irregular thickning of lamina dura was also noticed.
The report of two cases of typical oral-facial-digital syndrome type II (Goldstein, et al.: JADA., 89: 377, 1974) described the anomalies about midline cleft of the lower alveolar process, multiple anomalous crowns and pyramidal molar roots, which were the same as reported here.
Those anomalies may be considered as important signs for the differential diagnosis of oral-facial-digital syndrome.
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