Neuroepithelial Cyst of the Optic Nerve in a Newborn

Cystic lesions of the optic nerve are uncommon findings that have been associated with neoplasms, systemic disorders, or described as idiopathic. They have been described in a wide range of ages, with few congenital cysts having been described in infants. Surgical excision of these lesions may lead to significant morbidity as previously reported cases often involved transection of the optic nerve. The authors describe a unique case of a newborn with a rare neuroepithelial cyst of the intraorbital optic nerve that was successfully excised while sparing the optic nerve, ultimately resulting in the resolution of an afferent pupillary defect. This case demonstrates that careful surgical excision of a benign optic nerve cyst can be performed without significant morbidity to potentially preserve vision.