Abstract

Moyamoya disease is a progressive occlusive arteriopathy for which surgical revascularization is indicated. In this retrospective study, the authors investigated the use of preoperative CT perfusion with the aim of establishing pathological data references. The authors reviewed the medical records of children with moyamoya disease treated surgically at one institution between 2016 and 2019. Preoperative CT perfusion studies were used to quantify mean transit time (MTT), cerebral blood volume (CBV), cerebral blood flow (CBF), and time to peak (TTP) for the anterior, middle, and posterior cerebral artery vascular territories for each patient. CT perfusion parameter ratios (diseased/healthy hemispheres) and absolute differences were compared between diseased and normal vascular territories (defined by catheter angiography studies). Sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for CT perfusion parameters for severe angiographic moyamoya were calculated. Nine children (89% female) had preoperative CT perfusion data; 5 of them had evidence of unilateral hemispheric disease and 4 had bilateral disease. The mean age at revascularization was 77 months (range 40-144 months). The etiology of disease was neurofibromatosis type 1 (3 patients), Down syndrome (2), primary moyamoya disease (2), cerebral proliferative angiopathy (1), and sickle cell disease (1). Five patients had undergone unilateral revascularization. Among these patients, pathological vascular territories demonstrated increased MTT in 66% of samples, increased TTP in 66%, decreased CBF in 47%, and increased CBV in 87%. Severe moyamoya (Suzuki stage ≥ 4) had diseased/healthy ratios ≥ 1 for MTT in 78% of cases, for TTP in 89%, for CBF in 67%, and for CBV in 89%. The MTT and TTP region of interest ratio ≥ 1 demonstrated 89% sensitivity, 67% specificity, 80% PPV, and 80% NPV for the prediction of severe angiographic moyamoya disease. Pathological hemispheres in these children with moyamoya disease demonstrated increased MTT, TTP, and CBV and decreased CBF. The authors' results suggest that preoperative CT perfusion may, with high sensitivity, be useful in deciphering perfusion mismatch in brain tissue in children with moyamoya disease. More severe angiographic disease displays a more distinct correlation, allowing surgeons to recognize when to intervene in these patients. Moyamoya disease is a progressive occlusive arteriopathy for which surgical revascularization is indicated. In this retrospective study, the authors investigated the use of preoperative CT perfusion with the aim of establishing pathological data references. The authors reviewed the medical records of children with moyamoya disease treated surgically at one institution between 2016 and 2019. Preoperative CT perfusion studies were used to quantify mean transit time (MTT), cerebral blood volume (CBV), cerebral blood flow (CBF), and time to peak (TTP) for the anterior, middle, and posterior cerebral artery vascular territories for each patient. CT perfusion parameter ratios (diseased/healthy hemispheres) and absolute differences were compared between diseased and normal vascular territories (defined by catheter angiography studies). Sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for CT perfusion parameters for severe angiographic moyamoya were calculated. Nine children (89% female) had preoperative CT perfusion data; 5 of them had evidence of unilateral hemispheric disease and 4 had bilateral disease. The mean age at revascularization was 77 months (range 40-144 months). The etiology of disease was neurofibromatosis type 1 (3 patients), Down syndrome (2), primary moyamoya disease (2), cerebral proliferative angiopathy (1), and sickle cell disease (1). Five patients had undergone unilateral revascularization. Among these patients, pathological vascular territories demonstrated increased MTT in 66% of samples, increased TTP in 66%, decreased CBF in 47%, and increased CBV in 87%. Severe moyamoya (Suzuki stage ≥ 4) had diseased/healthy ratios ≥ 1 for MTT in 78% of cases, for TTP in 89%, for CBF in 67%, and for CBV in 89%. The MTT and TTP region of interest ratio ≥ 1 demonstrated 89% sensitivity, 67% specificity, 80% PPV, and 80% NPV for the prediction of severe angiographic moyamoya disease. Pathological hemispheres in these children with moyamoya disease demonstrated increased MTT, TTP, and CBV and decreased CBF. The authors' results suggest that preoperative CT perfusion may, with high sensitivity, be useful in deciphering perfusion mismatch in brain tissue in children with moyamoya disease. More severe angiographic disease displays a more distinct correlation, allowing surgeons to recognize when to intervene in these patients.

DOI: 10.3171/2019.10.peds19450