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

Mohammad Khursheed Alam; Ahmed Ali Alfawzan; Kumar Chandan Srivastava; Deepti Shrivastava; Kiran Kumar Ganji; Srinivas Munisekhar Manay

doi:10.1038/s41598-022-09764-y

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

Sci Rep. 2022 Apr 5;12(1):5708. doi: 10.1038/s41598-022-09764-y.

Authors

Mohammad Khursheed Alam¹, Ahmed Ali Alfawzan², Kumar Chandan Srivastava³, Deepti Shrivastava⁴, Kiran Kumar Ganji⁴, Srinivas Munisekhar Manay⁴

Affiliations

¹ Orthodontics, Preventive Dentistry Department, College of Dentistry, Jouf University, Sakaka, Saudi Arabia. mkalam@ju.edu.sa.
² Department of Preventive Dentistry, College of Dentistry in Ar Rass, Qassim University, Ar Rass, Saudi Arabia.
³ Department of Oral and Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Jouf University, Sakaka, Saudi Arabia.
⁴ Preventive Dentistry Department, College of Dentistry, Jouf University, Sakaka, Saudi Arabia.

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.

Publication types

Meta-Analysis
Systematic Review
Research Support, Non-U.S. Gov't

MeSH terms

Acrocephalosyndactylia* / diagnostic imaging
Cephalometry
Cleft Palate*
Humans
Research Report