Apert syndrome without craniosynostosis

Childs Nerv Syst. 2019 Mar;35(3):565-567. doi: 10.1007/s00381-019-04050-1. Epub 2019 Jan 14.

Abstract

Background: Apert syndrome is a rare form of syndromic craniosynostosis, also known as acrocephalosyndactyly, which is a disorder characterized by a unique set of craniofacial, hand, and foot abnormalities. Diagnosis is made through a genetic analysis, where the mutation of FGFR2, Ser252Trp, and Pro253Arg confirms the diagnosis.

Case presentation: Although craniosynostosis is the most common characteristic in clinical presentation, we present an atypical case of a one-and-a-half-year-old girl with Apert syndrome confirmed by genetic testing but without craniosynostosis.

Keywords: Apert syndrome; Atypical case; Craniosynostosis; Fibroblast growth factor receptors (FGFR)2.

Publication types

  • Case Reports

MeSH terms

  • Acrocephalosyndactylia / pathology*
  • Craniosynostoses*
  • Female
  • Humans
  • Infant