FGFR2 gene related Apert and Crouzon Syndrome with Different Craniofacial Dysmorphism A Systematic Review and Meta-analysis - Biomedical Review: Journal of Basic and Applied Medical Sciences (JBAMS) by Siddharth Health Research and Social Welfare Society

FGFR2 gene related Apert and Crouzon Syndrome with Different Craniofacial Dysmorphism: A Systematic Review and Meta-analysis

Shalini Dhiman
India

31-Dec-2024 12:00 AM 3227

Dhiman S1*, Panigrahi I2, Padhi BK3, Guptaa S4, Satapathy P5, Khatib MN6, Gaidhane S7, Kaur H8, Sharma M9, Zahiruddin QS10, Sah R11, Kaur K12, Garg M13

Background: This study aims to compare Craniofacial dysmorphism like maxillary, mandibular, and dental arch dimensions, and cranial suture fusion prevalence in Apert and Crouzon Syndrome from the publicly available scientific information and also give insights to improve the findings of further studies. The protocol was submitted to the International Prospective Register of Systematic Reviews CRD42023395454 accessed 11 February 2023.

Material and Methods: On a large scale interval from January 2000 to January 2023 a comprehensive search on different database platforms: PubMed, Google Scholar, Cochrane, Web of Science, and Wiley online library. PRISMA (Preferred reporting item for Systematic Review and Meta-Analyses) guidelines were followed to conduct this systematic review. The meta-analysis was carried out by calculating the random effects model and pooled mean proportions with 95% confidence intervals (CI).

Results: A total of 53 studies were considered worthy, but 39 were excluded due to unusable data formats. The I2 index provides a better way of assessing effect size heterogeneity. Forest plots were generated to visualize the heterogeneity of the individual outcome. Subgroup analyses were performed for each outcome to assess the potential, differences in effect sizes. Effect size and heterogeneity of the dental arch are more in CS (I2: 58%, 95%CI 0.01,0.29, P=0.12) and least in AS (I2: 52%;95%CI 0.01;0,27, P=0.15). Effect size and heterogeneity of maxilla of AS patients (I2: 91%, 95%CI 0.09;0.47, P<0.01) and CS (I2: 94%,95%CI 0.07;0.64, P<0.01). We observed significant heterogeneity in AS and CS patients.

Conclusion: This review demonstrates the large variation in cephalometric measurements in CS and AS patients. The CS patient had a smaller skull and mandible volume than the AS patient. AS had an anterior crossbite (p<0.001) and CS had an edge-to-edge bite (p<0.011). CS tends to have short and flat cranial bases, smaller orbital volumes, and cleft palates.

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