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  • Long Term Outcome of Moyamoya Patients Post Revascularization Surgery

    Final Number:

    Mario Teo MBChB(Hons) BMedSci(Hons) FRCS(SN); Teresa Bell-Stephens RN; Venkatesh S Madhugiri MBBS, MCh; Eric S Sussman MD; Tej Deepak Azad BA; Rohaid Ali; Rogelio Esparza; Michael Zhang BA; Gary K. Steinberg MD, PhD

    Study Design:

    Subject Category:
    Ischemic Stroke

    Meeting: AANS/CNS Cerebrovascular Section 2016 Annual Meeting

    Introduction: Long-term outcomes of moyamoya disease (MMD) patients post revascularization are not well documented. With one of the largest cohorts of treated MMD patients, we investigated their long-term physical, functional, and social well-being.

    Methods: Single institution, combined MMD database and questionnaire study. From 1991–2014, 1307 revascularization procedures (1170 direct bypass, 137 indirect bypass) were performed in 772 patients. We received and analyzed 316 completed patient questionaires.

    Results: 548 females and 224 males, mean age 32 years (range 1–68). We performed 344 revascularizations in 197 pediatric patients (73% direct bypasses), and 963 revascularizations in 572 adults (96% direct bypasses). With a mean follow-up of 7 years (0–25 years), there were 23 deaths, 5 within 30 days postoperatively, and 4 who had died from stroke at long term follow-up. The other 14 deaths were non-MMD related. Of the returned questionaires, 222 patients reported preoperative headache and 182 (82%) of these experienced post-revascularization improvement in their headaches. 101 patients had preoperative hypertension, and postoperatively anti-hypertensives were either discontinued (18) or the dosage reduced (47). 79% (251/316) of patients remained employed or in school at long-term follow up. Excluding children and adults with learning difficulties, 87% (237/273) are self-caring and 75% (204/273) are living independently. Overall, 83% of patients had excellent outcomes (mRS 0–1) at long-term follow up. A limitation of this study is that only 41% of the patients responded to the questionnaire, and it’s possible this may affect the data. Further telephone or clinic visit followup is being performed on the additional patients.

    Conclusions: Headache and hypertension could be the presenting symptoms in some MMD patients, as subgroups experienced improvement postoperatively. About 80% of MMD patients have had excellent long-term physical, social, and functional outcomes post revascularization, with up to 25 years of follow up.

    Patient Care: Due to the rarity of moyamoya disease, the number of patients who had revascularization surgeries, and very little is known on the long-term outcome for these patients in the literature, patients always have many uncertainties and unanswered questions for their future. This long-term outcome data on patients with moyamoya disease post revascularization would help with the patients' long-term expectation of their wellbeing.

    Learning Objectives: By the conclusion of this session, participants should be able to: 1) Describe the long term physical, functional and social outcome of moyamoya patients post revascularization surgery. 2) Discuss the outcome of headache and hypertension post extra cranial-intracranial bypass for moyamoya patients.


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