Postnatal Progressive Craniosynostosis in Syndromic Conditions: Two Patients With Saethre-Chotzen Due to TWIST1 Gene Deletions and Review of the Literature

Cleft Palate Craniofac J. 2023 Aug;60(8):1021-1028. doi: 10.1177/10556656221090844. Epub 2022 Mar 30.

Abstract

Saethre-Chotzen syndrome (SCS) is a known craniosynostosis syndrome with a variable presentation of craniofacial and somatic involvement. Congenital coronal craniosynostosis is most commonly observed in SCS; however, progressive postnatal craniosynostosis of other sutures has been reported. The authors present 2 infants with progressive postnatal craniosynostosis and SCS caused by chromosome 7p deletions including the TWIST1 gene. The evolution of their clinical features and a literature review of patients with syndromic, postnatal progressive craniosynostosis illustrate the importance of longitudinal observation and management of these patients.

Keywords: Saethre-Chotzen; TWIST; craniosynostosis; progressive postnatal craniosynostosis.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Acrocephalosyndactylia* / genetics
  • Chromosome Deletion
  • Craniosynostoses* / genetics
  • Gene Deletion
  • Humans
  • Infant
  • Nuclear Proteins / genetics
  • Twist-Related Protein 1 / genetics

Substances

  • Twist-Related Protein 1
  • TWIST1 protein, human
  • Nuclear Proteins