Teeth, Supernumerary

Finn (1967) presented a pedigree in which all females (numbering 14) were affected and all males (numbering 3) were unaffected, in 5 sibships in 3 generations. The author suggested X-linked dominant inheritance with female limitation, or female-limited autosomal dominant inheritance. In some of the affected females the supernumerary teeth occurred in the midline of the maxilla, so-called mesiodens.

Babu et al. (1998) reported a mother and daughter with nonsyndromic multiple impacted normal and supernumerary teeth (308280).

Chan et al. (2009) described the occurrence of semilobar holoprosencephaly (HPE; see 236100) in the child of a mother with mesiodens and suggested that the supernumerary maxillary tooth may be a microform of HPE.