Abstract
Purpose :
Craniosynostosis (CS) can be associated with afferent visual pathway damage from the retina to the visual cortex. We therefore have incorporated VEP testing as part of our routine evaluation of CS patients since 2013.
Methods :
IRB approved prospective database collection of comprehensive craniofacial team evaluation (clinical, radiological) and LKCR Technologies ETASR, ISCEV standard, VEP testing under monocular conditions.
Results :
Between November 2013 and August 2015, 67 children (66% male; age range: 0.08-15.4 yrs, with mean age [± SD] of 3.00 [± 3.65] yrs) have been recruited. 17 (25%) patients had clinical, radiological or electrophysiological evidence of afferent pathway abnormalities (abnormal VEP, clinical vision loss or retinal, optic nerve or neuroimaging abnormalities of their visual pathway). Qualitative analysis of VEP data showed that 9 (13%) of patients had abnormal responses. Of the 54 patients who had neuroimaging, 6 demonstrated abnormalities of the afferent visual system.
Conclusions :
There was a poor correlation between the clinical and/or neuroimaging abnormalities and qualitatively abnormal VEP’s, which suggests that qualitatively abnormal VEP patterns do not always reflect clinical abnormalities and may not be helpful in interventional decision making in patients with craniosynostosis. Quantitative VEP data (e.g., absolute latencies and intervals between them) may be a more useful diagnostic tool for early detection of visual compromises in CS patients. To test this hypothesis, we are currently performing more quantitative analysis of the VEP responses in CS patients.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.