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At a glance

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

Synonyms

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

Incidence and genetic inheritance

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

Clinical aspects

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

Anesthetic considerations

Careful laryngoscopy is recommended to avoid dental injuries.

References

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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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