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Review
. 2022 Feb 18;11(4):1060.
doi: 10.3390/jcm11041060.

Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis

Parinaz Rostamzad  1 , Zehra F Arslan  2 , Irene M J Mathijssen  1 , Maarten J Koudstaal  3 , Mieke M Pleumeekers  1 , Sarah L Versnel  1 , Sjoukje E Loudon  2
Affiliations
Review

Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis

Parinaz Rostamzad et al. J Clin Med. .

Abstract

Background: The aim of this study was to describe the ophthalmic abnormalities and their prevalence in craniosynostosis prior to craniofacial surgery.

Methods: A systematic search was conducted on Medline OVID, Embase, Cochrane, Google Scholar, Web of Science Core Collection. Inclusion criteria were English papers, children aged <18 years with non-syndromic and syndromic craniosynostosis, case reports, case series, and case-control studies. A system of domains was established consisting of an anatomic and functional ophthalmic domain. A meta-analysis of single proportions was carried out using random effects model and pooled mean proportions with 95% confidence intervals (CI) were calculated.

Results: Thirty-two papers analyzing 2027 patients were included. Strabismus was the most common anomaly in non-syndromic craniosynostosis: Horizontal strabismus was highest prevalent in unicoronal craniosynostosis (UCS) 19% (95% CI 9-32), followed by vertical strabismus 17% (95% CI 5-33). In syndromic craniosynostosis, horizontal strabismus was most prevalent in Crouzon syndrome 52% (95 CI 26-76), followed by Apert syndrome 50% (95% CI 42-58). Vertical strabismus was most prevalent in Saethre-Chotzen 60% followed by Muenke's syndrome 36%. Furthermore, astigmatism was the second most reported outcome in non-syndromic craniosynostosis and highest prevalent in UCS 35% (95% CI 21-51). In syndromic craniosynostosis, astigmatism was most frequently seen in Crouzon syndrome 43% (95% CI 22-65), followed by Apert syndrome 34% (95% CI 14-58). Moreover, in syndromic craniosynostosis, 5-40% had a decrease in visual acuity (VA) ≤ 0.3 LogMAR in the better eye and 11-65% had a VA ≤ 0.3 LogMAR in at least one eye.

Discussion: This review demonstrates the high prevalence of ocular anomalies in non-syndromic and syndromic craniosynostosis. A multidisciplinary and systematic approach is needed for the screening and optimal treatment of these conditions in a timely manner.

Keywords: craniofacial disorders; non-syndromic craniosynostosis; ocular anomalies; orbital malformations; syndromic craniosynostosis.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
PRISMA diagram of the record selection process.
Figure 2
Figure 2
Risk of bias of included studies.
See this image and copyright information in PMC

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