Craniofacial morphology in Apert syndrome: a systematic review and meta-analysis

Abstract

This meta-analysis aims to compare Apert syndrome (AS) patients with non-AS populations (not clinically or genetically diagnosed) on craniofacial cephalometric characteristics (CCC) to combine publicly available scientific information while also improving the validity of primary study findings. A comprehensive search was performed in the following databases: PubMed, Google Scholar, Scopus, Medline, and Web of Science, an article published between 1st January 2000 to October 17th, 2021. PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed to carry out this systematic review. We used the PECO system to classify people with AS based on whether or not they had distinctive CCC compared to the non-AS population. Following are some examples of how PECO has been used: People with AS are labeled P; clinical or genetic diagnosis of AS is labeled E; individuals without AS are labeled C; CCC of AS are labeled O. Using the Newcastle–Ottawa Quality-Assessment-Scale, independent reviewers assessed the articles' methodological quality and extracted data. 13 studies were included in the systematic review. 8 out of 13 studies were score 7–8 in NOS scale, which indicated that most of the studies were medium to high qualities. Six case–control studies were analyzed for meta-analysis. Due to the wide range of variability in CCC, we were only able to include data from at least three previous studies. There was a statistically significant difference in N-S-PP (I2: 76.56%; P = 0.014; CI 1.27 to − 0.28) and Greater wing angle (I2: 79.07%; P = 0.008; CI 3.07–1.17) between AS and control subjects. Cleft palate, anterior open bite, crowding in the upper jaw, and hypodontia occurred more frequently among AS patients. Significant shortening of the mandibular width, height and length is the most reported feature in AS patients. CT scans can help patients with AS decide whether to pursue orthodontic treatment alone or to have their mouth surgically expanded. The role of well-informed orthodontic and maxillofacial practitioners is critical in preventing and rehabilitating oral health issues.

Oral Health Status in Marfan Syndrome: A Systematic Review and Meta-Analysis of 353 Cases

“…This study's goal was to compile all of the existing research on a particular issue and organize it into manageable categories [34]. This was conducted to characterize the available data and recommend future studies that are as objective as possible [35]. Research into the role of MFS as an independent risk factor in the development, progression, and severity of oral diseases in healthy people will be necessary to validate or reject our findings.…”

Cleft Palate in Apert Syndrome

“…Apert syndrome patients present with anomalies of the palate, which may or may not include cleft palate ( Table 1 and Table 2 ). Cleft palate ( Figure 1 A) is present in a subset of affected individuals with Apert syndrome [ 8 ] and the frequency is higher than control subjects [ 31 ]. However, the incidence of cleft palate in patients with Apert syndrome varies between different studies.…”