A 10-year-old boy with bilateral colobomatous cavitary disc anomalies presented with a 3-month history of vision loss in his right eye. MRI of the head and orbit revealed bilateral tubular cystic enlargement of the optic nerve/optic sheath complex with thickening of the optic nerves without inflammation or neoplasm, suggestive of bilateral optic nerve meningocele. An optic nerve sheath fenestration was performed OD, and he experienced an improvement and stabilization of vision in his right eye during a 1-year follow-up period. The authors recommend that surgical decompression, particularly optic nerve sheath fenestration, should be considered in cases with progressive vision loss due to optic nerve meningocele.
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