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Visish M. Srinivasan, Phiroz E. Tarapore, Stefan W. Koester, Joshua S. Catapano, Caleb Rutledge, Kunal P. Raygor, and Michael T. Lawton


Rare arteriovenous malformations (AVMs) of the optic apparatus account for < 1% of all AVMs. The authors conducted a systematic review of the literature for cases of optic apparatus AVMs and present 4 cases from their institution. The literature is summarized to describe preoperative characteristics, surgical technique, and treatment outcomes for these lesions.


A comprehensive search of the English-language literature was performed in accordance with established Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to identify all published cases of AVM in the optic apparatus in the PubMed, Web of Science, and Cochrane databases. The authors also searched their prospective institutional database of vascular malformations for such cases. Data regarding the clinical and radiological presentation, visual acuity, visual fields, extent of resection, and postoperative outcomes were gathered.


Nine patients in the literature and 4 patients in the authors’ single-surgeon series who fit the inclusion criteria were identified. The median age at presentation was 29 years (range 8–39 years). Among these patients, 11 presented with visual disturbance, 9 with headache, and 1 with multiple prior subarachnoid hemorrhages; the AVM in 1 case was found incidentally. Four patients described prior symptoms of headache or visual disturbance consistent with sentinel events. Visual acuity was decreased from baseline in 10 patients, and 11 patients had visual field defects on formal visual field testing. The most common visual field defect was temporal hemianopia, found in one or both eyes in 7 patients. The optic chiasm was affected in 10 patients, the hypothalamus in 2 patients, the optic nerve (unilaterally) in 8 patients, and the optic tract in 2 patients. Six patients underwent gross-total resection; 6 patients underwent subtotal resection; and 1 patient underwent craniotomy, but no resection was attempted. Postoperatively, 9 of the patients had improved visual function, 1 had no change, and 3 had worse visual acuity. Eight patients demonstrated improved visual fields, 1 had no change, and 4 had narrowed fields.


AVMs of the optic apparatus are rare lesions. Although they reside in a highly eloquent region, surgical outcomes are generally good; the majority of patients will see improvement in their visual function postoperatively. Microsurgical technique is critical to the successful removal of these lesions, and preservation of function sometimes requires subtotal resection of the lesion.