Abstract P568: Incidence and Progression of Posterior Circulation Involvement in Moyamoya Disease
Introduction: The incidence and natural history of posterior circulation moyamoya disease (PCMMD) is not well-characterized. Methods: Consecutive patients with moyamoya disease prospectively treated at our institution from 2015-2018 were reviewed. Results: 262 consecutive patients with moyamoya disease undergoing 370 revascularization procedures were enrolled. Concurrent PCMMD was identified in 68 (26%) patients – all of which were confined to the anterior circulation. There were no differences in age, sex, race, or rates of diabetes, hypertension, smoking, or symptoms upon presentation between patients with or without PCMMD. Patients with PCMMD were more likely to present with bilateral disease (p<0.001), higher Suzuki grade (p=0.001), more extensive collateral angiopathy (p<0.001), and pial-pial collaterals from the PCA territory (p=0.03). Patients with PCMMD were more likely to suffer from ischemic peri-operative complications in both the anterior (p=0.03) and posterior (p<0.001) circulation territories after anterior circulation revascularization. No differences were observed for hemorrhagic complications (p=1.0). Over a mean follow-up of 1.91±1.5 years, 19 (7.3%) developed new or progressive PCMMD. Patients with PCMMD progression had an Asian predilection and were less likely to present with sentinel symptoms such as headaches (p=0.05 and 0.01 respectively). Patients with bilateral disease (p=0.02), higher Suzuki (p<0.001) and collateral angiopathy grades (p=0.002), and more extensive external carotid artery supply (p=0.03) were also more likely to experience PCMMD progression. There was no association between PCMMD progression and delayed neurological events or functional outcomes, but patients with concurrent PCMMD upon presentation were associated with poor functional outcome (mRS >2) at last follow-up (p=0.02). Conclusions: Posterior circulation disease involvement often presents in the later stages of moyamoya and is associated with higher rates of ischemic peri-operative complications and poor functional outcomes likely due to reduced collateral flow. Progression of PCMMD is not uncommon. Future studies are needed to assess the impact of PCMMD progression on long-term outcomes.