Craniosynostosis

Eur J Hum Genet. 2011 Apr;19(4):369-76. doi: 10.1038/ejhg.2010.235. Epub 2011 Jan 19.

Abstract

Craniosynostosis, defined as the premature fusion of the cranial sutures, presents many challenges in classification and treatment. At least 20% of cases are caused by specific single gene mutations or chromosome abnormalities. This article maps out approaches to clinical assessment of a child presenting with an unusual head shape, and illustrates how genetic analysis can contribute to diagnosis and management.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Chromosome Aberrations
  • Cranial Sutures
  • Craniosynostoses / diagnosis*
  • Craniosynostoses / genetics*
  • Craniosynostoses / pathology
  • Ephrin-B1 / genetics
  • Female
  • Genetic Counseling
  • Humans
  • Male
  • Mutation / genetics
  • Nuclear Proteins / genetics
  • Prenatal Diagnosis
  • Receptor, Fibroblast Growth Factor, Type 1 / genetics
  • Receptor, Fibroblast Growth Factor, Type 2 / genetics
  • Receptor, Fibroblast Growth Factor, Type 3 / genetics
  • Risk Assessment
  • Tomography Scanners, X-Ray Computed
  • Twist-Related Protein 1 / genetics

Substances

  • EFNB1 protein, human
  • Ephrin-B1
  • Nuclear Proteins
  • TWIST1 protein, human
  • Twist-Related Protein 1
  • FGFR1 protein, human
  • FGFR2 protein, human
  • FGFR3 protein, human
  • Receptor, Fibroblast Growth Factor, Type 1
  • Receptor, Fibroblast Growth Factor, Type 2
  • Receptor, Fibroblast Growth Factor, Type 3