The spectrum of median craniofacial dysplasia

Plast Reconstr Surg. 2011 Feb;127(2):812-821. doi: 10.1097/PRS.0b013e318200aa08.


Given the multiple permutations in craniofacial malformations, classification of median craniofacial dysplasia or midline Tessier no. 0 to 14 clefts has been difficult and disjointed. In this review, the authors present a summary of normal embryology, prior terminology, and their proposed new classification system. Median craniofacial dysplasia has tissue agenesis and holoprosencephaly at one end (the hypoplasias), frontonasal hyperplasia and excessive tissue (the hyperplasias) at the other end, and abnormal splitting or clefting and normal tissue volume (dysraphia) occupying the middle portion of the spectrum. These three distinct subclassifications have different forms of anomalies within their groups.

Publication types

  • Review

MeSH terms

  • Cleft Palate / diagnosis
  • Craniofacial Abnormalities / classification*
  • Craniofacial Abnormalities / diagnosis
  • Craniofacial Dysostosis / classification
  • Craniofacial Dysostosis / diagnosis*
  • Face / embryology
  • Holoprosencephaly / diagnosis
  • Humans
  • Nose / abnormalities
  • Skull / embryology
  • Terminology as Topic