Comparison of Changes in Retinal Vascular Parameters and Density in Patients with Moyamoya Disease: A Retrospective Study.

INTRODUCTION: This study aimed to compare retinal vascular parameters and density in patients with moyamoya disease using the optical coherence tomography angiography. METHODS: This clinical trial totally enrolls 78 eyes from 39 participants, and all these patients with moyamoya disease (N = 13) are set as experimental group and participants with health who matched with age and gender are considered as the control group (N = 26). Then all these participants receive optical coherence tomography angiography detection. Participants' general data are collected and analyzed. Skeleton density (SD) value, vessel density (VD) value, fractal dimension (FD) value, vessel diameter index (VDI) value, foveal avascular zone (FAZ) value are analyzed. RESULTS: A total of 39 participants are included in this study. The SD value in the experimental group was significantly lower than that in control group (0.175 [0.166, 0.181] vs. 0.184 [0.175, 0.188], p = 0.017). Similarly, the VD value in the experimental group was significantly lower than that in the control group (0.333 [0.320, 0.350] vs. 0.354 [0.337, 0.364], p = 0.024). Additionally, the FD value in the experimental group was significantly lower than that in the control group (2.088 [2.083, 2.094] vs. 2.096 [2.090, 2.101], p = 0.022). As for the VDI and FAZ, VDI and FAZ values in the experimental group were lower than those in the control group, there was no significant difference in VDI and FAZ values between the two groups. CONCLUSIONS: Our study, using non-invasive and rapid OCTA imaging, confirmed decreased retinal vascular parameters and density in patients with moyamoya disease.

Fetal posterior cerebral artery in pediatric and adult moyamoya disease: A single-center experience of 480 patients.

PURPOSE: In past pediatric and adult cohort studies of moyamoya disease, the fetal posterior cerebral artery has received less attention. Its relationship with the clinical manifestations and collateral circulation of moyamoya disease or ipsilateral cerebral hemispheres remains unclear. METHOD: We summarize the clinical features of patients with and without fetal posterior cerebral artery moyamoya disease from consecutive cases.We explored the relationship between fetal posterior cerebral arteries and collateral circulation in the ipsilateral cerebral hemispheres, as well as differences among different subgroups of patients.According to the morphology, the fetal posterior cerebral artery is divided into complete fetal posterior cerebral artery and partial fetal posterior cerebral artery. Clinical features were classified as: infarction,hemorrhage,and non-stroke in unilateral/bilateral cerebral hemispheres. Collateral circulation is divided into extracranial vascular compensation and leptomeningeal collateral circulation. Digital subtraction angiography and CT/MR were used to evaluate the blood flow status and clinical characteristics of patients with moyamoya disease. RESULT: A total of 960 cerebral hemispheres from 142 pediatric patients and 338 adult patients were included in the study. A total of 273 (56.9%) patients had 399 cerebral hemispheres (41.6%) with fetal posterior cerebral arteries. Adults with fetal posterior cerebral arteries had lower rates of infarction (24.6%vs37.3%, P =0.005) and were less likely to have bilateral stroke (8.4%vs11.5%, P =0.038). Cerebral hemispheres with fetal posterior cerebral artery were more likely to have anterior cerebral artery and middle cerebral artery stenosis and less likely to have occlusion (P =0.002, 0.001), and less likely to involve the posterior circulation (P < 0.001). The cerebral hemispheres of the fetal posterior cerebral artery had higher leptomeningeal collateral circulation scores. There are significant differences in extracranial vascular compensation between cerebral hemispheres with and without fetal posterior cerebral artery. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage (P =0.017). CONCLUSIONS: Our results suggest that fetal posterior cerebral artery is associated with infarct manifestations in pediatric and adult moyamoya disease. In the cerebral hemispheres, the fetal posterior cerebral artery is associated with ipsilateral hemispheric anterior and posterior circulation artery injury, extracranial vascular compensation, leptomeningeal collateral circulation compensation, and infarction. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage.

Risk factors for early seizure after revascularization in patients with moyamoya disease.

BACKGROUND: To investigate the risk factors for early seizure after revascularization in patients with moyamoya disease (MMD). METHODS: A total of 298 patients with MMD diagnosed in our hospital from 2015 to 2018 were analyzed retrospectively. We summarized the characteristics of seizure after revascularization in patients with MMD and analyzed the predictors of early postoperative seizure. RESULTS: We identified 15 patients with MMD who developed seizures within 1 week after revascularization. According to logistic regression analysis, age (OR: 1.04, 95% CI 0.998-1.086; P = 0.060) and infarct side (OR: 1.92, 95% CI 0.856-4.290; P = 0.113) were not significantly associated with incident early seizure. Postoperative infarction (OR: 12.89, 95% CI 4.198-39.525; P = 0.000) and preoperative cerebral infarction (OR: 4.08, 95% CI 1.267-13.119; P = 0.018) were confirmed as risk factors for early seizure. CONCLUSIONS: We believe that a history of preoperative infarction and new infarction are independent risk factors of early seizure in patients with MMD after revascularization.

Intraoperative transit-time ultrasonography combined with FLOW800 predicts the occurrence of cerebral hyperperfusion syndrome after direct revascularization of Moyamoya disease: a preliminary study.

BACKGROUND: Cerebral hyperperfusion syndrome (CHS) is a common complication after direct bypass surgery in patients with Moyamoya disease (MMD). Since preventive measures may be inadequate, we assessed whether the blood flow difference between the superficial temporal artery (STA) and recipient vessels (△BF) and the direct perfusion range (DPR) are related to CHS. METHODS: We measured blood flow in the STA and recipient blood vessels before bypass surgery by transit-time probe to calculate △BF. Perfusion changes around the anastomosis before and after bypass were analyzed with FLOW800 to obtain DPR. Multiple factors, such as △BF, DPR, and postoperative CHS, were analyzed using binary logistic regression. RESULTS: Forty-one patients with MMD who underwent direct bypass surgery were included in the study. Postoperative CHS symptoms occurred in 13/41 patients. △BF and DPR significantly differed between the CHS and non-CHS groups. The optimal receiver operating characteristic (ROC) curve cut-off value was 31.4 ml/min for ΔBF, and the area under the ROC curve (AUC) was 0.695 (sensitivity 0.846, specificity 0.500). The optimal cut-off value was 3.5 cm for DPR, and the AUC was 0.702 (sensitivity 0.615, specificity 0.750). CONCLUSION: Postoperative CHS is caused by multiple factors. △BF is a risk factor for CHS while DPR is a protective factor against CHS.

Intraoperative local hemodynamic quantitative analysis of direct revascularization in patients with moyamoya disease.

Direct bypass surgery improves blood flow in patients with moyamoya disease (MMD) as well as causes local flow relocation and disturbance. This study aimed to describe the characteristics and changes of quantitative blood flow in MMD patients. Sixty-four consecutive MMD patients (67 hemispheres) who underwent STA-MCA anastomosis between August 2013 and September 2017 were included in this study. Intraoperative blood flow of superficial temporal artery and middle cerebral artery was measured with a noninvasive, ultrasound flowprobe, before and after the bypass. All data were collected prospectively. Before the bypass, 67 hemispheres were measured and 45 hemispheres acquired post-bypass measurements. Thirty-three (49.3%) had anterograde flow and 34 (50.7%) had retrograde. Retrograde blood flow was significantly higher than anterograde blood flow in MCA-M4 section (3.71 ± 2.78 ml/min vs 2.39 ± 1.71 ml/min, P = 0.023). Thirty-eight (56.7%) patients had local cerebrovascular reactivity, whereas 29 (43.3%) lost it. After bypass, blood flow was changed into three patterns: anterograde flow (1, 2.2%), retrograde flow (3, 6.7%), and bi-direction flow (41, 91.1%). Flow of M4 was significantly improved in both proximal (13.64 ± 9.16 vs 3.28 ± 2.57, P < 0.001) and distal (7.17 ± 7.21 vs 3.28 ± 2.57, P = 0.002) sides to the bypass. Postoperative infarctions occurred in 4 (8.9%) patients. All postoperative infarctions (n = 4) happened in those patients who did not have change of flow direction in M4 section distal to the bypass (P = 0.040). Direct bypass surgery improves local blood flow after bypass surgery in both sides of the anastomosis. Flow direction was usually changed after the surgery into a bi-direction pattern: anterograde flow in M4 distal to the bypass, and retrograde flow in M4 proximal to the bypass. Proximal side acquired around 5.4 times of flow than the distal side. Patients with change of flow direction in distal M4 were related with lower risks of postoperative infarctions.

Characteristics of cognitive impairment in adult asymptomatic moyamoya disease.

BACKGROUND: Cognitive impairment in adult moyamoya disease (MMD) is thought to be the result of ischemic stroke; however, the presence and extent of cognitive decline in asymptomatic patients is unclear. METHODS: After classification using T2-weighted fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI), a total of 19 MMD patients with a history of cerebral infarction, 21 asymptomatic MMD patients, and 20 healthy controls matched for age, sex, and years of education were prospectively included in this study. A detailed neuropsychological evaluation of two moyamoya subgroups and normal controls was conducted. RESULTS: Asymptomatic patients showed varying degrees of decline in intelligence (Raven's Standard Progressive Matrices, P = 0.001), spatial imagination (mental rotation, P = 0.014), working memory (verbal working memory-backward digit span, P = 0.011), and computational ability (simple subtraction, P = 0.014; complex subtraction, P < 0.001) compared with normal controls. MMD patients with cerebral infarction had more severe impairment in complex arithmetic (P = 0.027) and word short-term memory (P = 0.01) than those without symptoms. CONCLUSION: In asymptomatic MMD patients, a variety of cognitive impairment precedes the onset of clinical symptoms such as cerebral infarction, which may be a long-term complication of conservative treatment.

Abnormal Embryonic Development of Cerebral Arteries as a Potential Cause of Moyamoya Disease.

OBJECTIVE: Moyamoya disease (MMD) is a cerebrovascular disorder, currently defined as progressive stenosis of intracranial internal carotid artery and its main branches with secondary formation of netlike vessels. Its precise formation mechanism, however, is unknown. We propose that MMD is caused by abnormal embryonic development of the cerebral arteries and also propose a mechanism for MMD formation. METHODS: The anterior, middle, and posterior cerebral arteries, anterior choroidal arteries (AChoA) and posterior choroidal arteries, and posterior corpus callosum arteries were analyzed separately for each patient with MMD to determine whether the arteries exhibited the following characteristics, which we regarded as remnants of primitive vessels: 1) plexiform arteries rather than normal artery trunks; 2) clustered arteries converging at locations of normal artery trunks; and 3) dilated AChoA. RESULTS: We retrospectively analyzed 39 consecutive patients with MMD, of whom 30 had anterior cerebral arteries, 31 middle cerebral arteries, 10 posterior cerebral arteries, 30 AChoAs, 18 posterior choroidal arteries, and 20 posterior corpus callosum arteries and had the characteristics of primitive vessel remnants. Altogether, 82.05% of the patients had the remnants of primitive vessels. CONCLUSIONS: MMD is more likely to be caused by abnormalities of cerebral artery development, which mainly occur in the embryonic period or postnatally. The abnormality developing processes include sprouting angiogenesis, vessel fusion, and pruning, which primarily affect cranial ramus of primitive internal carotid artery, may occasionally affect the caudal ramus of the primitive internal carotid artery, and rarely affect the vertebrobasilar artery system. So-called moyamoya vessels comprise unfused primitive small vessels; to compensate, enlarged AChoAs remain undegenerated but are not dilated.

Posterior Circulation Moyamoya Disease versus Primitive Vertebral-Basilar Artery System Moyamoya Disease: New Classification of Moyamoya Disease from the Perspective of Embryology.

BACKGROUND AND PURPOSE: Moyamoya disease (MMD) is a chronic cerebrovascular disorder with little known etiology. We aim to propose a new classification system for MMD from the perspective of embryology. METHODS: MMD patients' digital subtraction angiograms were retrospectively analyzed. Every angiogram was analyzed to find the abnormal vessels and from which part of the posterior cerebral artery (PCA) the lesions begin. RESULTS: In 262 MMD cases, 32 pediatric patients had PCA involvement, of which 17 were male and 15 were female; 68 adults had PCA involvement, of which 33 were male and 35 were female. The initially affected part of the PCA was compared between sexes and between pediatric and adult patients, and the findings are not statistically significant (P = 0.233, P = 0.855, P = 0.343, respectively). However, of the 100 cases with PCA involvement, only 4 had the lesions begin from the first part of the PCA, and all of the 4 cases had the basilar artery lesions. All the other 96 cases had the lesions begin from the second part of the PCA or from the posterior communication artery, which is derived from the caudal ramus of the primitive intracarotid artery, leaving the first part of the PCA and basilar artery excluded from affection. CONCLUSION: MMD should be classified into primitive intracarotid artery system-involved type and primitive vertebral basilar artery system-involved type. The reason that the vertebral basilar artery is so rarely involved in MMD might be because of its late development in the brain.