Supernumeraries in Nicolaides-Baraitser Syndrome

Int J Paediatr Dent. 2017 Nov;27(6):583-587. doi: 10.1111/ipd.12309. Epub 2017 Jun 21.


Background: Nicolaides-Baraitser Syndrome(NCBRS) is an extremely rare condition which has been reported in only a few cases. NCBRS is a distinct clinical condition with typical clinical features of pre- and post-natal global developmental delay, impaired speech, and seizures. Microcephaly, sparse hair, anteverted alae nasi, undefined philtrum, prominence of distal phalanges and interphalangeal joints, and short metacarpals are also typical of NCBRS.

Case report: There are no reported cases in the literature of patients with NCBRS presenting with multiple dental impactions, and to the authors' knowledge, this is the 28th fully documented case of NCBRS and only 75 cases identified as potentially having NCBRS. The clinical features, diagnosis, and course of management are also described.

Conclusion: Although NCBRS is very rare, it is important to assess dental development in view of the possibility of multiple supernumerary teeth which can have detrimental effects on the occlusion.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Child
  • Face / abnormalities
  • Facies*
  • Foot Deformities, Congenital*
  • Humans
  • Hypotrichosis*
  • Intellectual Disability*
  • Male
  • Tooth, Supernumerary*

Supplementary concepts

  • Nicolaides Baraitser syndrome