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Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting
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Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting
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Journal Article
Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting
2021
Overview
PurposeTo describe the trans-occipital asymmetries of pattern and flash visual evoked potentials (VEPs), in an infant with MRI findings of unilateral optic nerve aplasia and hemi-chiasm dysplasia.MethodsA child with suspected left cystic microphthalmia, left microcornea, left unilateral optic nerve aplasia, and hemi-chiasm underwent a multi-channel VEP assessment with pattern reversal, pattern onset, and flash stimulation at the age of 16 weeks.ResultsThere was no VEP evidence of any post-retinal visual pathway activation from left eye with optic nerve aplasia. The VEP trans-occipital distribution from the functional right eye was skewed markedly across the midline, in keeping with significant misrouting of optic nerve fibres at the chiasm. This was supported by the anatomical trajectory of the optic chiasm and tracts seen on MRI.ConclusionThis infant has chiasmal misrouting in association with unilateral optic nerve aplasia and unilateral microphthalmos. Chiasmal misrouting has not been found in patients with microphthalmos or anophthalmos, but has been reported after early eye loss in animal models. Our findings contribute to our understanding of the discrepancy between the visual pathway physiology of human unilateral microphthalmia and animal models.
Publisher
Springer Nature B.V
Subject
