Ophthalmological management in craniosynostosis

Neurochirurgie. 2019 Nov;65(5):310-317. doi: 10.1016/j.neuchi.2019.09.016. Epub 2019 Sep 28.


Introduction: In published series, a large proportion of patients with craniosynostosis show impaired vision.

Materials and methods: A literature review was performed, using the PubMed and Google Scholar databases, to identify original and review articles on the consequences of craniosynostosis on the eyes and visual pathways, and on the ophthalmological management of craniosynostosis.

Results and discussion: Many ophthalmic, potentially sight-threatening, complications, can occur in patients with craniosynostosis, especially when syndromic. Optic neuropathy, mostly resulting from the papilledema-optic atrophy sequence, secondary to raised intracranial pressure (ICP), should be diagnosed early, in order to promptly lower the ICP. Cyclovertical and horizontal strabismus and refractive errors are frequent in unicoronal synostosis (anterior plagiocephaly) and syndromic craniosynostosis. Exorbitism, encountered in some cases of syndromic craniofacial synostosis, leads to exposure keratopathy, which requires aggressive management to avoid severe irremediable corneal complications. Amblyopia can result from optic neuropathy, corneal opacities, strabismus, or refractive errors. If undiagnosed and untreated at a young age, it results in permanent visual impairment.

Conclusion: Children with craniosynostosis require a multidisciplinary care network including a pediatric ophthalmologist. Systematic ophthalmological follow-up enables papilledema to be diagnosed and amblyopia to be diagnosed and treated, in order to avoid visual impairment.

Keywords: Craniosynostosis; Ophthalmology; Papilledema; Raised intracranial pressure; Strabismus.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Craniosynostoses / complications*
  • Craniosynostoses / pathology
  • Craniosynostoses / therapy*
  • Eye Diseases, Hereditary / etiology*
  • Eye Diseases, Hereditary / therapy*
  • Humans
  • Infant
  • Vision Disorders / etiology*
  • Vision Disorders / therapy*
  • Visual Pathways / pathology