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Cavernous malformation of the optic chiasm: Neuro-endoscopic removal
N K Venkataramana1, Shailesh A V Rao1, L N Arun1
1Global Institute of Neurosciences, BGS Global Hospital, Bangalore, Karnataka, India.
Abstract:
Cavernous malformations (CMs) arising from the optic nerve and chiasm are extremely rare. In large autopsy series, CMs were estimated to range from 0.02 to 0.13% in the general population. However, with introduction of MRI, these lesions were found more often than previously thought, ranging from 0.2% to 0.4%. Only 29 cases have been reported according to our knowledge. Most patients present with drop in visual acuity and visual field. Although MRI findings of cavernous malformations have been reported, they may not be diagnostic enough. Among the 29 reported, 16 underwent total resection with good results. In some, resection was complicated by damage to the surrounding neural tissue. Surgical removal is the recommended treatment to restore or preserve vision and to eliminate the risk of future hemorrhage. However, the anatomical location and eloquence of nearby neural structures can make these lesions difficult to access and remove. CMs appear to occur in every age group (range 4 months to 84 years mean-34.6 years) ith an approximately equal male to female ratio. They typically present with chiasmal apoplexy, characterized by sudden visual loss, acute headaches, retro orbital pain, and nausea.
Insights
Cavernous malformations (CMs) of the optic nerve and chiasm are rare, often presenting with vision loss. Surgical removal is recommended for vision preservation and hemorrhage prevention.
Area of Science:
- Neuro-oncology
- Ophthalmology
- Vascular Neurology
Background:
- Cavernous malformations (CMs) are rare vascular lesions.
- Optic nerve and chiasm CMs are exceptionally uncommon, with only 29 reported cases.
- Prevalence in the general population is low, estimated between 0.02-0.13% via autopsy, and 0.2-0.4% via MRI.
Observation:
- Patients typically experience decreased visual acuity and visual field deficits.
- Chiasmal apoplexy, marked by sudden vision loss, headaches, and nausea, is a common presentation.
- Lesions occur across all age groups (4 months to 84 years) with a balanced male-to-female ratio.
Findings:
- MRI findings for CMs may not be definitively diagnostic.
- Surgical resection offers good outcomes in many cases, with 16 of 29 reported patients achieving positive results.
- Complications can arise from damage to surrounding neural tissue during resection.
Implications:
- Surgical removal is the primary treatment to restore/preserve vision and prevent hemorrhage.
- The anatomical location and proximity to eloquent neural structures present surgical challenges.
- Early diagnosis and intervention are crucial for managing optic nerve and chiasm cavernous malformations.
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