Abstract
ObjectiveMoyamoya vasculopathy is a rare, often bilateral disease characterized by progressive stenosis and occlusion of the distal internal carotid artery, leading to a progressive deterioration of cerebrovascular reactivity (CVR) and increased risk of transient ischemic attacks (TIAs), infarction and hemorrhage. Surgical revascularization is a widely accepted symptomatic treatment, often performed bilaterally in one or two stages. To possibly further optimize treatment strategy, we investigated the effect of unilateral revascularization surgery on the CVR of, and TIA frequency originating from, the contralateral hemisphere. MethodsFrom our database of 143 moyamoya vasculopathy patients we selected those with bilateral disease, who underwent hemodynamic imaging ([15O]H2O positron emission tomography (PET)-CT with acetazolamide challenge) before and 14 months (median) after unilateral revascularization. We evaluated CVR in three regions per hemisphere, and averaged these per hemisphere for statistical comparison. Conservatively treated patients were showed as a comparison group. To examine TIA frequency, we selected patients who presented with TIAs that (also) originated from the contralateral – not to be operated – hemisphere. We scored changes in CVR and TIA frequency of the ipsilateral and contralateral hemisphere over time. ResultsSeven surgical and seven conservative patients were included for CVR comparison. Of the 20 scored contralateral regions in the surgical group, 15 showed improved CVR after unilateral revascularization, while 5 remained stable. The averaged scores improved significantly for both hemispheres. In conservatively treated patients, however, only 3 of the 20 scored regions improved in the least-affected (contralateral) hemispheres, and 9 deteriorated. From the 6 patients with contralateral TIAs at presentation, 4 had a decreased TIA frequency originating from the contralateral hemisphere after unilateral surgery, while 2 patients remained stable. ConclusionBoth CVR and TIA frequency in the contralateral hemisphere can improve after unilateral revascularization surgery in bilateral MMV.
Highlights
Moyamoya Vasculopathy (MMV) is a rare disease characterized by usually bilateral progressive stenosis or occlusion of the distal internal carotid artery and its branches
We used an existing moyamoya vasculopathy (MMV) database, consisting of patients with MMD (moyamoya disease, as defined by the criteria posed by the Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis (2012) and moyamoya syndrome. FU (MMS) (moyamoya syndrome (Scott and Smith, 2009))
Following the strict inclusion criteria for the assessment of changes in contralateral positron emission tomography (PET) findings and Transient Ischemic Attack (TIA) frequency, 12 bilateral MMV pa tients could be included for the PET study, of whom 7 were treated operatively
Summary
Moyamoya Vasculopathy (MMV) is a rare disease characterized by usually bilateral progressive stenosis or occlusion of the distal internal carotid artery and its branches This may cause regional hypoperfusion, which can lead to transient ischemic attacks (TIAs), cerebral infarction and cognitive decline (Scott and Smith, 2009). For surgical decision making and planning, most centers perform extensive preoperative evaluations This includes: digital sub tracting angiography ((DSA) to assess the severity of the arteriopathy and the presence and extent of collateral networks) and some form of cerebrovascular hemodynamic measurements. CVR after a chal lenge is absent or even negative This paradoxical decrease of CBF is the so-called steal phenomenon which is strongly associated with risk of infarction (McKetton et al, 2019; Webster et al, 1995; Yonas et al, 1993) and cortical thinning (Fierstra et al, 2011, 2010). Extra-cranial to intra cranial blood flow improves perfusion and partially restores CVR; reducing the risks of TIAs and infarctions (Jeon et al, 2018)
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