Pediatric moyamoya disease complicated by cerebral infarction in the contralateral hemisphere 1 month after two-staged vascular reconstructive surgery of each hemisphere: A case report

Abstract

The authors report a child with moyamoya disease who repeated acute progression of major arterial stenosis on the contralateral side after two staged vascular reconstructive surgery. An 8-year-old boy had frequent transient ischemic attacks involving the left extremities starting at 1 years of age. Cerebral angiogram revealed right internal carotid artery (ICA) occlusion and typical moyamoya vessels in the right basal ganglia, while left anterior cerebral artery (ACA) and middle cerebral artery (MCA) showed only stenotic configurations without moyamoya vessels. Cerebral blood flow (CBF) measurement using 133Xe SPECT demonstrated low CBF and negative response to Diamox loading in the territories of the right MCA, ACA, part of the posterior cerebral artery (PCA) and the left ACA. We initially performed indirect reconstructive surgery on the affected right MCA and ACA territories, followed by cerebral infarct in the left frontal lobe 34 days after surgery due to the left ICA occlusion. One month later, we added left STA MCA anastomosis, followed again by cerebral infarct in the right occipital lobe 1 month after surgery due to right PCA occlusion. Thus, we underscore the necessity for careful postoperative observation of progressive major arterial stenosis using magnetic resonance angiography in pediatric patients with moyamoya disease undergoing indirect or direct reconstructive surgery of 1 hemisphere. In such patients, performing one staged extensive indirect reconstructive surgery may be prudent, covering whole areas where preoperative CBF measurement revealed low CBF at rest and impairment of vascular reactivity on Diamox loading.