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Neuro-Ophthalmological Manifestations of Craniosynostosis: Current Perspectives

Authors Duan M, Skoch J, Pan BS, Shah V

Received 16 September 2020

Accepted for publication 12 November 2020

Published 29 January 2021 Volume 2021:13 Pages 29—40

DOI https://doi.org/10.2147/EB.S234075

Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Margaret Wong-Riley


Michael Duan,1 Jesse Skoch,2 Brian S Pan,3 Veeral Shah4,5

1Baylor College of Medicine, School of Medicine, Houton, TX, USA; 2Cincinnati Children’s Hospital Medical Center, Division of Pediatric Neurosurgery, Cinicinnati, OH, USA; 3Cincinnati Children’s Hospital Medical Center, Division of Plastic Surgery, Cinicinnati, OH, USA; 4Cincinnati Children’s Hospital Medical Center, Division of Pediatric Ophthalmology, Cinicinnati, OH, USA; 5University of Cincinnati, Department of Ophthalmology, Cincinnati, OH, USA

Correspondence: Veeral Shah
Cincinnati Children’s Hospital Medical Center, Abrahamson Pediatric Eye Institute/Division of Pediatric Ophthalmology, Cinicinnati, OH, USA
Tel +1 513-803-2230
Fax +1 513-558-351
Email Veeral.Shah@cchmc.org

Abstract: Craniosynostosis, a premature fusion of cranial sutures that can be isolated or syndromic, is a congenital defect with a broad, multisystem clinical spectrum. The visual pathway is prone to derangements in patients with craniosynostosis, particularly in syndromic cases, and there is a risk for permanent vision loss when ocular disease complications are not identified and properly treated early in life. Extensive advancements have been made in our understanding of the etiologies underlying vision loss in craniosynostosis over the last 20 years. Children with craniosynostosis are susceptible to interruptions in visual input arising from strabismus, refractive errors, and corneal damage; any of these aberrations can result in understimulation of the visual cortex during childhood neurodevelopment and permanent amblyopia. Elevated intracranial pressure resulting from abnormal cranial shape or volume can lead to papilledema and, ultimately, optic atrophy and vision loss. A pediatric ophthalmologist is a crucial component of the multidisciplinary care team that should be involved in the care of craniosynostosis patients and consistent ophthalmologic follow-up can help minimize the risk to vision posed by such entities as papilledema and amblyopia. This article aims to review the current understanding of neuro-ophthalmological manifestations in craniosynostosis and explore diagnostic and management considerations for the ophthalmologist taking care of these patients.

Keywords: syndromic craniosynostosis, non-syndromic craniosynostosis, optic nerve atrophy, pediatric ophthalmology, papilledema

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