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Polymalformative syndrome characterized by dental malformations and various distal skeletal anomalies.

Mesomelic Dysplasia, Thai Type; Mesomelic Dysplasia with Ankle, Carpal, and Tarsal Synostosis; Mesomelic Dysplasia, Kantaputra Type; Molarization of Anterior Teeth with Deafness.

Incidence and mode of inheritance are unknown. Only one case report of an affected female.

Molarization and premolarization of anterior teeth, possibly as a consequence of a mesenchymal defect. Histologically, tooth structure was normal; sensorineural deafness; flared nares.

Careful laryngoscopy is recommended to avoid dental injuries.

Kantaputra PN, Gorlin RJ: Double dens invaginatus of molarized maxillary central incisors, premolarization of maxillary lateral incisors, multituberculism of the mandibular incisors, canines and first premolar, and sensorineural hearing loss. Clin Dysmorphol 1:128, 1992.  [PubMed: 1285271]

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