Optic Nerve Cavernous Malformation

Cavernous malformation (CM) is cluster of abnormal tortuous, thin wall vascular channel lined by a single layer of endothelium. CMs may be calcified and contain blood stains in varying degrees of thrombosis and degradation. The prevalence of intracranial cavernous malformation is calculated to be 0.4 to 0.8% of the population. The occurrence of optic nerve cavernoma is less than 1% of all central nervous system cavernous malformations. Cavernoma of the anterior visual pathway is extremely rare. In one literature review of all CM cases reported worldwide until September 2020, there were only 84 cases reported. The nature of CM is that it displaces and does not infiltrate neural tissue.

Cavernous malformation of optic nerve (CMON) manifests as sudden onset visual disturbance, or less likely gradual progressive visual decline, with or without headache. Management options are many including emergent surgery, urgent or elective, and observation. Due to the challenge of diagnosis and identifying the pathology and acute visual deterioration, most of the studies recommend urgent surgical decompression of the optic nerve. Surgical intervention is recommended rather than observation.

Case: A 23-year-old male patient presented with a history of acute left eye visual loss associated with mild headache for 2 days, not known to have chronic illness before. The patient denied drug use. . The patient was clinically alert and oriented, neurologically remarkable of left eye hand motion, right eye temporal field deficit with extraocular movement, and no nuchal rigidity. General examination demonstrates right scapula hyperkeratosis 5 × 3 cm.

Computed tomography (CT) of the brain revealed left round regular suprasellar hyperdensity lesion, and CT angiography excluded aneurysm or vascular lesion. Magnetic resonance imaging of the brain revealed heterogeneous, lobulated, oval-shaped solid-cystic mass lesion seen in the suprasellar region involving the central and left-side of the optic chiasm, measuring 2 × 1.7 × 1.5 cm. The lesion is surrounded by a rim of low signal intensity due to hemorrhage or calcification and showed mild heterogeneous enhancement. The cerebral arteries are of normal caliber and in their expected location with no aneurysmal dilatation seen.

The patient underwent left pterional craniotomy and optic nerve lesion resection; it was brown/red solid and hard attaching to the wall. Histopathological evaluation demonstrates brown hemorrhagic lesion. Hemangioma/vascular malformation was found with old and new hemorrhages, with no evidence of glioma.

Conclusion: Cavernous malformation of the optic nerve (CMON) is a rare clinical disorder. Evaluation of CMON is challenging, as it mimics many neurosurgical disorders. The gold standard of diagnosis is surgical biopsy. With acute presentation, visual fiber displacenot invade in favor surgical decompression over observation strategy.

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Artikel online veröffentlicht:
07. Februar 2025