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Primary failure of tooth eruption

ORPHA:412206· ICD-10 K00.6

Definition

A rare genetic odontologic disease characterized by failure of eruption of non-ankylosed permanent teeth without evidence of obvious mechanical obstruction. Posterior teeth are preferentially affected (typically with involvement of all teeth distal to the most mesial non-erupted tooth), resulting in a posterior open bite. Non-ankylosed teeth tend to become ankylosed, and orthodontic treatment of affected teeth is generally unsuccessful.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant