Introduction:
Limited data exists to guide management of incidentally discovered pediatric moyamoya. Best exemplified in the setting of unilateral moyamoya, in which the unaffected side is monitored, this phenomenon also occurs in populations undergoing routine surveillance of the cerebral vasculature for other conditions, such as sickle cell disease (SCD) or neurofibromatosis type I (NF1). Here we present our experience with specific syndromic moyamoya populations to better characterize the natural history of radiographic and clinical progression in asymptomatic moyamoya.
Methods:
Retrospective review of a consecutive series of 418 patients who underwent surgical revascularization for moyamoya disease at a single institution from 1988-2010.
Results:
Of 418 patients, 83 were asymptomatic at the time of radiographic diagnosis of moyamoya, while also having either unilateral moyamoya or moyamoya in association with either SCD or NF1. Average age at presentation was 9.1 years (range 1-21), with 47 females (60%) and 31 males (40%). Mean follow-up was 5.4 years (SD +/-3.8), with 45 patients (54%) demonstrating radiographic progression and 37 patients (44.6%) becoming symptomatic within this period. The time interval between radiographic progression from the diagnosis of syndromic disease, to evidence of arteriopathy, to signs of slow cortical blood flow, and to stroke was 5.8 years (SD +/-4.7), 0.7 years (SD +/-1.1), and 0.3 years (SD +/-0.5), respectively. SCD patients had the highest incidence of both radiographic (n=15, 75%) and clinical (n=13, 65%) progression, followed by NF1 (n=20, 59% radiographic, n=15, 44% clinical) and unilateral patients (n=10, 34.5% radiographic, n=9, 31%). Transient ischemic attacks were the most frequent symptoms during follow-up (29 patients, 35%), and 10 patients (12%) developed clinical stroke. Overall 49 patients (59%) underwent surgical treatment, and the time between arteriopathy to surgery was 1.1 years (SD +/-1.2).
Conclusion:
Radiographic progression occurred in the majority of asymptomatic patients and generally heralded subsequent clinical symptoms. SCD or NF patients with asymptomatic moyamoya are more likely to progress and require treatment than unilateral moyamoya patients without a syndromic disease. These data demonstrate that moyamoya is a progressive disorder, even in asymptomatic populations, and supports the rationale of early surgical intervention to minimize morbidity from stroke.
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