Central incisor syndrome
Solitary median maxillary central incisor (SMMCI) is a rare dental anomaly. It is usually considered as a minor manifestation (variant) of holoprosencephaly (HPE).
It is estimated to occur in 1:50,000 live births.
It is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35-38 day from conception. The etiology is uncertain.
A missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI .
- single, midline central maxillary incisor
- midpalatal vomerine ridge
- v-shaped palate