Posterior circulation involvement and collateral flow pattern in moyamoya disease with the RNF213 polymorphism

2018 ◽  
Vol 35 (2) ◽  
pp. 309-314 ◽  
Author(s):  
Won-Hyung Kim ◽  
Sang-Dae Kim ◽  
Myung-Hyun Nam ◽  
Jin-Man Jung ◽  
Sung-Won Jin ◽  
...  
Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Yiping Li ◽  
Allan R Wang ◽  
Gary K STEINBERG

Introduction: The incidence and natural history of posterior circulation moyamoya disease (PCMMD) is not well-characterized. Methods: Consecutive patients with moyamoya disease prospectively treated at our institution from 2015-2018 were reviewed. Results: 262 consecutive patients with moyamoya disease undergoing 370 revascularization procedures were enrolled. Concurrent PCMMD was identified in 68 (26%) patients – all of which were confined to the anterior circulation. There were no differences in age, sex, race, or rates of diabetes, hypertension, smoking, or symptoms upon presentation between patients with or without PCMMD. Patients with PCMMD were more likely to present with bilateral disease (p<0.001), higher Suzuki grade (p=0.001), more extensive collateral angiopathy (p<0.001), and pial-pial collaterals from the PCA territory (p=0.03). Patients with PCMMD were more likely to suffer from ischemic peri-operative complications in both the anterior (p=0.03) and posterior (p<0.001) circulation territories after anterior circulation revascularization. No differences were observed for hemorrhagic complications (p=1.0). Over a mean follow-up of 1.91±1.5 years, 19 (7.3%) developed new or progressive PCMMD. Patients with PCMMD progression had an Asian predilection and were less likely to present with sentinel symptoms such as headaches (p=0.05 and 0.01 respectively). Patients with bilateral disease (p=0.02), higher Suzuki (p<0.001) and collateral angiopathy grades (p=0.002), and more extensive external carotid artery supply (p=0.03) were also more likely to experience PCMMD progression. There was no association between PCMMD progression and delayed neurological events or functional outcomes, but patients with concurrent PCMMD upon presentation were associated with poor functional outcome (mRS >2) at last follow-up (p=0.02). Conclusions: Posterior circulation disease involvement often presents in the later stages of moyamoya and is associated with higher rates of ischemic peri-operative complications and poor functional outcomes likely due to reduced collateral flow. Progression of PCMMD is not uncommon. Future studies are needed to assess the impact of PCMMD progression on long-term outcomes.


2018 ◽  
Vol 128 (6) ◽  
pp. 1813-1822 ◽  
Author(s):  
Wei Ni ◽  
Hanqiang Jiang ◽  
Bin Xu ◽  
Yu Lei ◽  
Heng Yang ◽  
...  

OBJECTIVEMoyamoya disease (MMD) is occasionally accompanied by intracranial aneurysms. The purpose of this study was to delineate the efficacy of the authors’ current surgical strategy in the management of MMD-associated aneurysms of different types.METHODSBetween January 2007 and March 2016, a consecutive cohort of 34 patients with 36 MMD-associated aneurysms was enrolled in this prospective single-center cohort study. The lesions were classified as peripheral (17 aneurysms) or main trunk aneurysms (13 in the anterior circulation and 6 in the posterior circulation). For the peripheral aneurysms, revascularization with or without endovascular treatment was suggested. For the main trunk aneurysms, revascularization alone, revascularization with aneurysm clipping, or revascularization with aneurysm embolization were used, depending on the location of the aneurysms.RESULTSOf the peripheral aneurysms, 4 were treated endovascularly with staged revascularization, and 13 were treated solely with cerebral revascularization. Of the 13 main trunk aneurysms in the anterior circulation, 10 were clipped followed by revascularization, and 3 were coiled followed by staged cerebral revascularization. Of the 6 main trunk aneurysms in the posterior circulation, 4 underwent endovascular coiling and 2 were treated solely with revascularization. One patient died of contralateral intracerebral hemorrhage 6 months after the operation. No other patients suffered recurrent intracranial hemorrhage, cerebral ischemia, or aneurysm rupture. An angiographic follow-up study showed that all the bypass grafts were patent. Complete occlusion was achieved in all 21 aneurysms that were clipped or embolized. Of the remaining 15 aneurysms that were not directly treated, 12 of 13 peripheral aneurysms were obliterated during the follow-up, whereas 1 remained stable; 1 of 2 posterior main trunk aneurysms remained stable, and the other became smaller.CONCLUSIONSThe authors’ current treatment strategy may benefit patients with MMD-associated aneurysms.


Author(s):  
Brian A Tong ◽  
Dan‐Victor Giurgiutiu

Introduction : Rationale: Bilateral vertebral artery occlusion with collateral reconstitution is a rare finding. Compared to patients with acute occlusion, symptom progression may be much slower [1]. Atherosclerotic risk factors lead to occlusion, including hypertension and hyperlipidemia, but it is unclear what leads to collateral reconstitution [2]. These patients may have collateral circulation from anterior and posterior circulation sources that are well developed [1] [2]. Sufficient collateral flow correlates with lower rates of hemorrhagic transformation following recanalization [3] [4]. However, given the risk of spontaneous hemorrhage from microvascular collaterals, the hemorrhagic risk associated with thrombolytic therapy in patients with moyamoya collaterals, due to the fragility of these vessels [5], must be balanced with the benefit of therapy in the presence of severe neurologic deficits along with the mortality and morbidity that may stem from the occlusion. Patient concerns: 67 year old Caucasian male with past medical history of coronary artery disease, abdominal aortic aneurysm, hypertension, history of tobacco use and type 2 diabetes mellitus presents with acute right‐sided weakness. Methods : Diagnoses: On admission, CTA Head and Neck suggested chronic total occlusion of bilateral V4 segments from their origin to the midportion with tandem bilateral high‐grade stenoses throughout the imaged distal V2 and V3 segments bilaterally. MRI could not be obtained because of old lumbar fusion spinal hardware. Cerebral angiography showed microvascular reconstitution, analogous to moyamoya, with slow mid basilar flow, which could be either due to occlusion or competitive flow from top of the basilar collaterals. Interventions: Patient received intra‐arterial integrilin and tPA thrombolysis with TICI 1 reperfusion. Results : Outcomes: Patient presented with NIHSS 18 notable for right sided weakness (2/5 strength in his right upper extremity and 1/5 strength in RLE), bilateral hemianopia, severe dysarthria and right gaze preference. Patient had significant improvement in his exam the next day following thrombolysis. Notably, patient had 5/5 strength in his right upper and right lower extremities compared to his strength on presentation. Repeat head CT on the following day after thrombolysis showed left pontine infarct. Repeat NIHSS was 3 at 24 hours for partial hemianopia, minor nasolabial flattening and mild dysarthria. Conclusions : Conclusion: Bilateral intracranial vertebral artery stenosis and occlusion commonly occurs distal to PICA and near the vertebrobasilar junction [2]. Proximal (specifically areas supplied by PICA) and distal territories within the posterior circulation are often infarcted [2], which can yield a unique exam upon presentation that can help accurately guide diagnosis and treatment when appropriately recognized. The involvement of collateral circulation can play a crucial role in patients undergoing endovascular revascularization therapy [6]. In the setting of bilateral vertebral occlusion with microvascular reconstitution, patients can still undergo catheter based thrombolysis, but not thrombectomy.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Sepideh Amin-Hanjani ◽  
Xinjian Du ◽  
Linda Rose-Finnell ◽  
Dilip Pandey ◽  
DeJuran Richardson ◽  
...  

Introduction: Atherosclerotic vertebrobasilar disease (VBD) is a significant etiology of posterior circulation stroke. In addition to thromboembolism, regional hypoperfusion is considered an important potential contributor to stroke risk. To examine the role of hemodynamic compromise in VBD, a prospective observational multi-center study, Vertebrobasilar Flow Evaluation and Risk of Transient Ischemic Attack and Stroke (VERiTAS), has recently been conducted. Here we report baseline features and vessel flow measurements from the study cohort. Methods: Baseline demographic and clinical data was collected in patients with recent vertebrobasilar TIA or stroke and ≥50% atherosclerotic stenosis or occlusion in vertebral and/or basilar arteries. Large vessel flow in the vertebrobasilar territory was assessed using quantitative MRA (QMRA). Results: The cohort (n=75, 56% male) had a mean age of 65.5 (range 40 to 90) years; two thirds presented with ischemic stroke. Hypertension (93%) and hyperlipidemia (79%) were the most prevalent vascular risk factors. Vertebral and basilar artery flows correlated negatively with degree of stenosis in the affected vessel, and positively to the minimal diameter at the site of stenosis (p<0.01). A threshold effect was evident, with affected vessel flows dropping significantly in patients with ≥70% stenosis or occlusion (p<0.05). Tandem disease involving the basilar and either or both the vertebrals had the greatest impact on immediate downstream flow in the basilar artery (38 ml/min vs. 74 ml/min, p<0.01). Assessment of distal flow status, incorporating collateral flow, however correlated neither with multifocality of disease nor severity of the maximal stenosis. Conclusions: Flow in stenotic posterior circulation vessels correlate with residual diameter and stenosis and drop significantly in the setting of tandem disease. However, distal flow status, incorporating collateral capacity, is not well predicted by the severity or location of the disease. Final clinical outcome results from the ongoing VERiTAS study will further clarify the relevance of anatomic stenosis and hemodynamic assessment to predicting stroke risk in patients with vertebrobasilar disease.


2020 ◽  
Vol 133 (5) ◽  
pp. 1450-1459
Author(s):  
Yu Chen ◽  
Li Ma ◽  
Junlin Lu ◽  
Xiaolin Chen ◽  
Xun Ye ◽  
...  

OBJECTIVEPostoperative hemorrhage during the acute phase is rarely observed after revascularization surgery for moyamoya disease (MMD) but can have severe complications. Its risk factors and outcomes are still unclear. The aim of this study was to investigate the predictors of postoperative hemorrhage during the acute phase in MMD and examine the outcomes of the hemorrhage.METHODSThe authors reviewed the preoperative clinical characteristics and radiographic features of 465 consecutive MMD cases (518 procedures) that had undergone direct or combined bypass surgery at their institution between 2009 and 2015. Patients with postoperative intracerebral hemorrhage (ICH) or ICH plus intraventricular hemorrhage (IVH) during the acute phase were screened, and then the incidence, location, and risk factors of hemorrhage in these patients were analyzed. Short-term and long-term outcomes (modified Rankin Scale scores) for these patients were also collected. Outcomes were compared between patients with and those without postoperative ICH using propensity score analysis to reduce the between-group differences in baseline characteristics.RESULTSPostoperative hemorrhage occurred in 11 (2.1%; ICH = 9, IVH = 2) of 518 procedures (mean patient age 39.82 ± 8.8 years). Hemorrhage occurred in the first 24 hours after the operation in 8 cases (72.7%). In the ICH group, most of the hemorrhage sites (77.8%) were located beneath the anastomosed area, and the mean hematoma volume was 16.98 ± 22.45 ml (range 3–57 ml). One case from the ICH group required hematoma evacuation. Among the adult patients (463 procedures [89.4%]), preoperative hypertension (p = 0.008), CT perfusion (CTP) stage > III (p = 0.013), and posterior circulation involvement (p = 0.022) were significantly associated with postoperative ICH. No significant differences between the postoperative ICH group and the no-hemorrhage group were detected in terms of postoperative neurofunctional status at discharge (p = 0.569) or at the last follow-up (p = 1.000). Neither was there a significant difference in future stroke risk (p = 0.538) between these two groups.CONCLUSIONSPreoperative hypertension, CTP stage > III, and posterior circulation involvement are independent risk factors for postoperative ICH after direct or combined revascularization for MMD. After appropriate perioperative management, postoperative ICH has no significant correlations with the postoperative short-term and long-term neurofunctional status.


2018 ◽  
Vol 21 (6) ◽  
pp. 632-638 ◽  
Author(s):  
Tomomi Kimiwada ◽  
Toshiaki Hayashi ◽  
Reizo Shirane ◽  
Teiji Tominaga

OBJECTIVESome pediatric patients with moyamoya disease (MMD) present with posterior cerebral artery (PCA) stenosis before and after anterior circulation revascularization surgery and require posterior circulation revascularization surgery. This study evaluated the factors associated with PCA stenosis and assessed the efficacy of posterior circulation revascularization surgery, including occipital artery (OA)–PCA bypass, in pediatric patients with MMD.METHODSThe presence of PCA stenosis before and after anterior circulation revascularization surgery and its clinical characteristics were investigated in 62 pediatric patients (< 16 years of age) with MMD.RESULTSTwenty-three pediatric patients (37%) with MMD presented with PCA stenosis at the time of the initial diagnosis. A strong correlation between the presence of infarction and PCA stenosis before anterior revascularization was observed (p < 0.001). In addition, progressive PCA stenosis was observed in 12 patients (19.4%) after anterior revascularization. The presence of infarction and a younger age at the time of initial diagnosis were risk factors for progressive PCA stenosis after anterior revascularization (p < 0.001 and p = 0.002, respectively). Posterior circulation revascularization surgery, including OA-PCA bypass, was performed in 9 of the 12 patients with progressive PCA stenosis, all of whom showed symptomatic and/or radiological improvement.CONCLUSIONSPCA stenosis is an important clinical factor related to poor prognosis in pediatric MMD. One should be aware of the possibility of progressive PCA stenosis during the postoperative follow-up period and consider performing posterior circulation revascularization surgery.


2014 ◽  
Vol 156 (9) ◽  
pp. 1745-1751 ◽  
Author(s):  
Tomohito Hishikawa ◽  
Koji Tokunaga ◽  
Kenji Sugiu ◽  
Isao Date

2014 ◽  
Vol 20 (4) ◽  
pp. 403-412 ◽  
Author(s):  
Gerasimos Baltsavias ◽  
Nadia Khan ◽  
Venko Filipce ◽  
Anton Valavanis

The anastomotic network of the posterior circulation in children with moyamoya disease has not been analyzed. We aimed to investigate the angiographic anatomy of this unique vascular network in patients with childhood moyamoya disease. Selective and superselective injections of the posterior circulation were performed in six children with newly diagnosed moyamoya disease. The arterial branches feeding the moyamoya anastomotic network, their connections and the recipient vessels were demonstrated. Depending on the level of the steno-occlusive lesion, the feeding vessels were the thalamoperforators, the posterior choroidals, the splenic artery, parietoccipital artery, other cortical posterior cerebral artery (PCA) branches, the dural branch of the PCA, the premamillary artery and other posterior communicating artery perforators. Through connections, which are described, the recipient vessels were the striate and medullary arteries, other thalamic arteries with or without medullary extensions, the pericallosal artery, medial parietoccipital cortical branches of the PCA and the anterior choroidal artery. High quality selective and superselective angiography helped in demonstrating the angiographic anatomy of the moyamoya posterior anastomotic network previously either vaguely or incompletely described, as well as connections within the posterior circulation but also its relevance as a collateral to the anterior circulation.


1986 ◽  
Vol 65 (4) ◽  
pp. 454-460 ◽  
Author(s):  
Susumu Miyamoto ◽  
Haruhiko Kikuchi ◽  
Jun Karasawa ◽  
Izumi Nagata ◽  
Ikuo Ihara ◽  
...  

✓ The involvement of the posterior circulation in moyamoya disease was studied in 178 patients. Forty-three had several types of disturbance such as visual field defect, decreased visual acuity, episodes of blindness, and scintillating scotomata. Most of these symptoms were attributed to occlusive lesions in the posterior circulation. Visual disturbances were seen more often in patients with a juvenile onset than in cases of adult onset. Superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis and encephalomyosynangiosis (EMS) improved the cerebral perfusion both in the anterior and posterior circulation by redistribution of blood. In most cases, the visual symptoms subsided or were stabilized after STA-MCA anastomosis and EMS. These surgical procedures did not, however, lead to direct revascularization in cases of ischemia in the visual cortex. In five patients with impending blindness, transplantation of the omentum to the occipital lobe led to improved vision.


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