A Cavernous Sinus Meningioma in a Child with Progressive Bilateral Visual Loss Ultimately Attributed to Unsuspected...

Pediatric optic nerve sheath meningiomas (ONSMs) are rare and have an aggressive presentation often resulting in rapid vision decline. Here, we report a case of a right cavernous sinus meningioma (CSM) with a delayed diagnosis of concomitant bilateral ONSMs, ultimately leading to permanent bilateral blindness.

A 7-year-old boy with a history of a right trochlear nerve palsy and amblyopia presented with worsening right eye vision. An MRI revealed a right CSM that was resected; however, the patient experienced a progressive visual acuity decline from 20/60 right eye and 20/40 left eye to light perception right eye and 20/400 left eye, along with bilateral optic atrophy appreciated on optical coherence tomography. Repeat evaluation of the brain MRIs revealed bilateral ONSMs characterized by a tram-track appearance. The visual acuity remained unimproved 3 years after optic canal decompression with optic nerve sheath fenestration followed by proton beam radiation.

Pediatric ONSM is rare, with a high risk of permanent blindness in children. Intracranial meningioma and unexplained painless vision loss should prompt a careful evaluation of orbital neuroimaging for this condition.