Congenital Opticmeningoceles

Ophthalmic Plast Reconstr Surg. 2021 May-Jun;37(3):e109-e111. doi: 10.1097/IOP.0000000000001864.


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.

Publication types

  • Case Reports

MeSH terms

  • Astrocytes
  • Child
  • Exophthalmos*
  • Glial Fibrillary Acidic Protein
  • Humans
  • Meningocele / diagnosis*
  • Neuroglia
  • Optic Nerve / pathology*
  • Schwann Cells


  • Glial Fibrillary Acidic Protein