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