Cavernous malformations of the optic nerve and chiasm: illustrative case.

backgroundCavernous malformations (CMs) of the optic pathway are rare. In this report, the authors describe a case of a CM affecting bilateral optic nerves that was managed surgically. Additionally, they provide an updated review of the literature focused on the clinical presentation, management, and postoperative outcomes related to this uncommon pathology. OBSERVATIONS: A total of 99 cases have been described in the literature, including the case reported. The patient presented with headache, encephalopathy, and bitemporal hemianopia. He was found to have an optic pathway CM and underwent resection of the lesion via a frontotemporal craniotomy. Based on a literature review, the most common presenting symptoms are visual decline and nonspecific headache. Most optic pathway CMs are managed surgically, with improvement in vision seen 66% of the time. LESSONS: CMs of the optic pathway most often present with clinical manifestations of vision loss and headaches. They are difficult to diagnose and can commonly be confused with other hemorrhagic lesions in this region. With a high index of suspicion guided by the clinical and imaging features, one can narrow the differential to include CM. Surgery remains the primary modality of management when symptomatic, similar to intraparenchymal CMs. https://thejns.org/doi/10.3171/CASE24742.