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

Abstract

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 CS.in 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.

Crouzon’s syndrome and its dentofacial features

“…The disease is characterised by the premature fusion of the coronal and sagittal sutures, which develop within the first year of life. Once the sutures are closed, further development is compromised, leading to midfacial hypoplasia, a shallow orbit and maxillary hypoplasia 6. Intraoral features consist of mandibular prognathism, oligodontia, overcrowding of the upper teeth, macrodontia, cleft lip and palate, peg teeth, widely spaced teeth, V-shaped maxillary dental arch and occasional upper airway obstruction 5 7.…”

Dynamic Morphological Growth Trajectory of Crouzon Population: A Geometric Morphometric Analysis

“…Lu et al 5 revealed that Crouzon was characterized by a shortened anteroposterior maxillary length and posteriorly shifted mandible compared with healthy controls. Forte et al 6 noted that the nasion-sella pterygomaxillary fissure angle was more obtuse in Alam et al 7 and concluded that Crouzon syndrome is prone to have a retrusive maxilla, shorter and flatter cranial bases, and smaller orbital and mandibular volumes compared to the general population by meta-analysis.…”