Cranial nerves involvement in craniosynostosis: a systematic review
Renzo Manara1,2, Anna Tietze3, Roberto Faggin4
1Neurosciences Department, University of Padova, Padua, Italy.
Summary
Cranial nerve involvement is common in craniosynostosis, particularly affecting the optic nerve, leading to vision issues. Further research and guidelines are needed for comprehensive management of these complex cases.
Area of Science:
- Neurology
- Pediatric Surgery
- Genetics
Background:
- Craniosynostosis management reveals later-life cranial nerve issues, especially in syndromic cases.
- These involvements stem from bone foramen stenosis, intracranial pressure, or genetic factors.
- Affected nerves impact vision, smell, hearing, feeding, and facial function, affecting child development.
Purpose of the Study:
- To systematically review literature on cranial nerve involvement in craniosynostosis.
- To identify patterns, affected nerves, and associated syndromes.
- To highlight gaps in current knowledge and management strategies.
Main Methods:
- Systematic literature review following PRISMA criteria.
- Searched PubMed and Scopus up to February 2025 for case series and reports.
- Included 63 English-language papers, excluding duplicates; statistical analysis focused on the optic nerve.
Main Results:
- Optic nerve involvement (papilledema, atrophy, hypoplasia) reported in 140 patients, often with reduced vision.
- Syndromic craniosynostosis (Crouzon, Apert) common; male predominance noted.
- Upper cranial nerves (III, IV, VI) frequently involved; inferior cranial nerves rarely reported despite posterior fossa issues.
Conclusions:
- Cranial nerve involvement is frequent but under-investigated in craniosynostosis.
- Involvement patterns vary by syndrome and suture involvement.
- Guidelines for assessment and treatment are needed, requiring collaborative efforts from specialized centers.
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