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.