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Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis
Parinaz Rostamzad1, Zehra F Arslan2, Irene M J Mathijssen1
1Department of Plastic and Reconstructive Surgery, Erasmus MC, University Medical Center, 3000 CA Rotterdam, The Netherlands.
Insights
Ocular abnormalities like strabismus and astigmatism are common in craniosynostosis before surgery. Early screening and a multidisciplinary approach are crucial for timely treatment of these vision-impacting conditions.
Area of Science:
- Ophthalmology
- Pediatric Surgery
- Medical Genetics
Background:
- Craniosynostosis, a condition involving premature fusion of skull sutures, is associated with various ophthalmic abnormalities.
- Understanding the prevalence of these ocular issues before surgical intervention is critical for patient management.
Purpose of the Study:
- To systematically review and quantify the prevalence of ophthalmic abnormalities in patients with non-syndromic and syndromic craniosynostosis before craniofacial surgery.
Main Methods:
- A comprehensive systematic literature search was performed across major databases (Medline OVID, Embase, Cochrane, Google Scholar, Web of Science).
- Included studies focused on pediatric patients (<18 years) with craniosynostosis, encompassing case reports, series, and case-control studies.
- A meta-analysis of single proportions was conducted using a random effects model to calculate pooled prevalence estimates with 95% confidence intervals (CI).
Main Results:
- Thirty-two studies involving 2027 patients were analyzed.
- Strabismus was the most frequent anomaly: in non-syndromic craniosynostosis, horizontal strabismus (19%) and vertical strabismus (17%) were most common in unicoronal craniosynostosis (UCS).
- In syndromic craniosynostosis, horizontal strabismus was highest in Crouzon (52%) and Apert (50%) syndromes. Astigmatism was also prevalent, particularly in UCS (35%) and Crouzon syndrome (43%). Visual acuity reduction was noted in 5-65% of syndromic cases.
Conclusions:
- This review highlights the significant prevalence of ocular anomalies in both syndromic and non-syndromic craniosynostosis.
- A coordinated, multidisciplinary approach is essential for the timely screening and management of ophthalmic conditions in these patients.
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.
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