Symptomatic hyperperfusion after combined revascularization surgery in patients with pediatric moyamoya disease: patient series

Abstract

BACKGROUND Symptomatic hyperperfusion after cerebral revascularization for pediatric moyamoya disease (MMD) is a rare phenomenon. The authors report a series of patients with this condition. OBSERVATIONS In all three patients in this case series, the combined revascularization was on the left side, the patency of bypass grafts was confirmed after surgery, and focal hyperemia around the anastomotic site was observed on single photon emission computed tomography (SPECT). On the first to eighth days after surgery, all of the patients developed neurological manifestations, including motor aphasia, cheiro-oral syndrome, motor weakness of their right upper limbs, and severe headaches. These symptoms disappeared completely approximately 2 weeks after surgery, and all patients were discharged from the hospital. Quantitative SPECT was performed to determine the proportional change in cerebral blood flow (ΔRCBF) (to ipsilateral cerebellar ratio (denoted ΔRCBF) in the region of interest around the anastomoses, and the mean value was 1.34 (range, 1.29–1.41). LESSONS This rare condition, which develops soon after surgery, requires an accurate diagnosis by SPECT. One indicator is that the ΔRCBF has risen to 1.3 or higher. Subsequently, strategic blood pressure treatment and fluid management could prevent the development of hemorrhagic stroke.