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Case Reports
. 2021 May-Jun;37(3):e109-e111.
doi: 10.1097/IOP.0000000000001864.

Congenital Opticmeningoceles

Antonio A V Cruz  1 , Patricia M S Akaishi  1 , Francesco Bernardini  2 , Fernando Chahud  3
Affiliations
Case Reports

Congenital Opticmeningoceles

Antonio A V Cruz et al. Ophthalmic Plast Reconstr Surg. 2021 May-Jun.

Abstract

Congenital optic nerve cystic-like malformations associated with normally developed globes are extremely rare. We describe 3 children who presented since birth with proptosis, and eye motility limitation. MRI showed in all cases that the intraorbital segment of the optic nerves was malformed with large cystic-like lesions in the intraconal segment of the orbit. In all cases, biopsies of the wall of the lesions were positive for glial fibrillary acidic protein. Since this protein is a neurobiomarker that exists only in astrocytes in the central nervous system, nonmyelinating Schwann cells of peripheral nerves, and enteric glial cells, we believe that these lesions represent true opticmeningoceles.

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References

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