The scintigraphic appearance of childhood moyamoya disease on cerebral perfusion imaging

This study was performed to evaluate the role of single photon emission computed tomography (SPECT) perfusion imaging in the evaluation of patients with moyamoya disease. Five patients (four female, one male) were studied utilizing iodine-123 iodoamphetamine or technetium-99m hexamethylpropyleneamine oxime SPECT. The data were reconstructed into axial, coronal and sagittal sections for review, and compared with CT, MR and/or angiographic images. All five patients had significant perfusion defects. These areas of vascular compromise were seen to cross normal vascular territories, and were greater in number and extent than seen on anatomic sectional imaging. Patients with moyamoya disease have a recognizable pattern of scintigraphic perfusion deficits which should be identified by pediatric imaging physicians. SPECT perfusion studies should be performed in conjunction with other imaging modalities (CT, MR or angiography).