Solitary median maxillary central incisor
The solitary median maxillary central incisor (SMMCI) is a rare dental anomaly. It is usually considered as a minor manifestation of holoprosencephaly (HPE).
Epidemiology
It is estimated to occur in 1:50,000 live births.
Pathology
It is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35 - 38th day(s) from conception. The aetiology is uncertain.
Genetics
A missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI 2.

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