Ophthalmic Findings in Children With Nonsyndromic Craniosynostosis Treated by Expansion Cranioplasty

Abstract

The ocular and systemic abnormalities of nonsyndromic craniosynostosis are often considered to be less severe than those of syndromic craniosynostosis and are less well described. The purpose of this article was to describe the frequency and nature of ophthalmic abnormalities in children treated for nonsyndromic craniosynostosis by expansion cranioplasty. A retrospective review identified 88 consecutive children with nonsyndromic craniosynostosis who underwent expansion cranioplasty with distraction osteogenesis. Assessment of presence and type of strabismus, refractive error, and amblyopia before and 6 months after surgery was recorded. Children with a mean age of 24.4 months were treated for nonsyndromic craniosynostosis (27 with coronal and 61 with sagittal and/or lambdoid). One-fourth of the patients had a fixation preference. Significant refractive errors were found in 45 (51%) of the 88 patients: hyperopia in 27%, myopia in 5%, and astigmatism in 35%. Anisometropia was present in 20%. Of the 85 patients who completed orthoptic examination, 48 (56%) had strabismus: exodeviation in 26%, esodeviation in 14%, and vertical deviation in 5%. Fourteen patients (16%) had abnormal head posture. Significant refractive error and strabismus were more likely to occur in cases with coronal synostosis. The procedures used for cranial vault expansion improved the abnormal head posture but did not affect the refractive error or ocular misalignment. Of children with nonsyndromic craniosynostosis who need neurosurgical correction, more than half were found to have significant refractive error and strabismus. Our findings support the importance of ophthalmic evaluation in these children.

Ophthalmological outcomes of unilateral coronal synostosis in young children

“…Many papers have discussed the long-term visual outcomes after craniofacial surgery in all kinds of craniosynostosis [26][27][28][29], and few papers have shared experience with lid abnormalities in cases of craniosynostosis.…”

Ophthalmological findings in children with non-syndromic craniosynostosis: preoperatively and postoperatively up to 12 months after surgery

“…Nonetheless, visual dysfunctions, strabismus, refractive errors and amblyopia have been reported. [4][5][6] Orbital anatomy may be affected in the different forms of single-suture craniosynostosis, but to a lesser degree than in the syndromes. Moreover, the surgical treatment in metopic and coronal synostosis entails periorbital dissection and orbital skeleton rearrangement, with risk of iatrogenic visual dysfunction.…”

Ocular biometric features of pediatric patients with fibroblast growth factor receptor-related syndromic craniosynostosis

“…Ametropia is consistently suggested as one of the major ophthalmic manifestations of both syndromic and nonsyndromic craniosynostosis, but age and ethnic group-controlled comparative studies evaluating the refractive errors of syndromic and non-syndromic craniosynostosis patients have not reported yet 6,[8][9][10] . To the best of www.nature.com/scientificreports/ our knowledge, this is the first comparative study of refractive errors in FGFR-related syndromic and nonsyndromic craniosynostosis.…”