Abstract WP156: Moyamoya Syndrome - Comparison of Long Term Outcome of Conservative and Surgical treatment in India.

Abstract

Background: Revascularization surgery (RS) is the therapy of choice in Moyamoya disease (MMD). Due to rarity of disease and ethical concerns, randomized controlled trials about the treatment options are lacking. Very little information is available on the outcome of conservatively treated moyamoya patients. Aim: We compared the long term outcome of moyamoya patients treated conservatively to those who underwent RS. Methods: Our study population included all patients with moyamoya from 2002-2012. The demographic and clinical characteristics were obtained by retrospective chart review. The imaging details were reviewed. The follow-up information and outcome were obtained prospectively. Results: Of the 36 patients, 26( 72.2%) had MMD and 10 ( 27.8 %) had moyamoya syndrome. Of the patients with moyamoya syndrome, 6 had a well recognized risk factors( 2-Downs syndrome,1-Neurofibromatosis,2- atherosclerosis,1-vasculitis) and 4 had unilateral disease. The median age at onset of symptoms was 17.5years (range,10 months- 55 years). Fifteen patients belonged to pediatric group and 21 were adults. All the paediatric patients had ischemic events at onset and 10 (47.6 %) of the adults presented with haemorrhage. Twenty (55.6%) patients received conservative treatment and 16(44.4 %) underwent revascularization procedures. The median duration of follow-up was 28 months (range,3-90 months). Two of the surgically treated patients had recurrent ischemic events on follow up, but none of the conservatively treated patients had events. An excellent outcome (Modified rankin scale of ≤ 2) was seen in 12 (75%) of surgically treated patients and all of the conservatively treated patients. Of the 4 patients in the surgically treated group with poor outcome, one was due to recurrent stroke ,one due to postoperative stroke and 2 had severe stroke at onset. Conclusion: Compared to East Asians, our patients had a lower stroke recurrence rate and better functional outcome even with conservative treatement. The impact of RS in modifying the patients clinical course in Indian patients is difficult to evaluate. Future studies should focus on clinical and imaging predictors of progression to select moyamoya patients for RS.