Abstract
Purpose :
Cerebral vascular malformations (CVMs) may result in hemorrhage, seizure, neurologic dysfunction, and death. CVMs include capillary telangiectasias, venous malformations, cavernous malformations, and arteriovenous malformations. Cavernous and arteriovenous malformations carry the highest risk of complications. Retinal venous malformations (RVMs) have been proposed as an associated finding. Our objective was to determine the prevalence of RVMs in patients with high risk CVMs.
Methods :
We retrospectively reviewed patients diagnosed with cerebral cavernous or arteriovenous malformations (high risk CVMs) who were evaluated by the ophthalmology service at Northwestern University between 2017 and 2020. Patients were stratified into three cohorts based on level of certainty: dilated funduscopic exam (DFE) alone, DFE with any form of ocular imaging, and DFE with complete imaging of the macula. We recorded ophthalmic examination abnormalities, ocular imaging findings, and major CVM complications. The main outcome measure was the prevalence of RVMs in patients with high risk CVMs. Secondary outcomes included rates of hemorrhage or seizure, and prevalence of neuro-ophthalmic abnormalities.
Results :
We evaluated 157 patients with high risk CVMs who had undergone DFE. Ocular imaging of any type had been performed in 56 patients, of whom 46 had complete imaging of the macula. Zero RVMs were identified in any cohort (95% confidence interval: 0-1.9% for the entire cohort, 0-5.4% for any ocular imaging cohort, 0-6.5% for the complete macular imaging cohort). Cerebral hemorrhage or seizure occurred in 15-33% of patients. Associated visual field defects or cranial nerve palsies were found in 13-19% of patients.
Conclusions :
Zero RVMs were identified in patients with high risk CVMs. Current recommendations include neuro-imaging for all RVM patients. In our proposed clinical algorithm for the management of patients with RVMs, we suggest neuro-ophthalmic examination be performed due to the large percentage of patients with high risk CVMs who have visual field defects or cranial nerve palsies. Any abnormal neuro-ophthalmic finding should prompt neuro-imaging. If neuro-ophthalmic evaluation is unremarkable, we recommend neuro-imaging for patients < 40 years of age, as CVMs carry a cumulative annual risk of complications.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.