Craniofacial characteristics in Crouzon's syndrome: A systematic review and meta-analysis

Sci Prog. 2023 Jan-Mar;106(1):368504231156297. doi: 10.1177/00368504231156297.


The purpose of this meta-analysis was to strengthen the credibility of primary research results by combining open-source scientific material, namely a comparison of craniofacial features (Cfc) between Crouzon's syndrome (CS) patients and non-CS populations. All articles published up to October 7, 2021, were included in the search of PubMed, Google Scholar, Scopus, Medline, and Web of Science. The PRISMA guidelines were followed to conduct this study. PECO framework was applied in the following ways: Those who have CS are denoted by the letter P, those who have been diagnosed with CS via clinical or genetic means by the letter E, those who do not have CS by the letter C, and those who have a Cfc of CS by the letter O. Independent reviewers collected the data and ranked the publications based on their adherence to the Newcastle-Ottawa Quality Assessment Scale. A total of six case-control studies were reviewed for this meta-analysis. Due to the large variation in cephalometric measures, only those published in at least two previous studies were included. This analysis found that CS patients had a smaller skull and mandible volumes than those without terms of SNA° (MD = -2.33, p = <0.001, I2 = 83.6%) and ANB°(MD = -1.89, p = <0.005, I2 = 93.1%)), as well as ANS (MD = -1.87, p = 0.001, I2 = 96.5%)) and SN/PP (MD = -1.99, p = 0.036, I2 = 77.3%)). In comparison to the general population, people with CS tend to have shorter and flatter cranial bases, smaller orbital volumes, and cleft palates. They differ from the general population in having a shorter skull base and more V-shaped maxillary arches.

Keywords: Crouzon's syndrome; craniofacial characteristics; electronic databases; forest plot; meta-analysis; systematic review.

Publication types

  • Meta-Analysis
  • Systematic Review
  • Review

MeSH terms

  • Case-Control Studies
  • Craniofacial Dysostosis* / genetics
  • Humans