Pathogenesis of aneurysms on major vessels in moyamoya disease and management outcome (original) (raw)
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World neurosurgery, 2018
Moyamoya disease (MMD), arterial venous malformations (AVMs), and intracranial aneurysms are distinct cerebrovascular disease processes that most commonly occur in isolation. The literature on the treatment algorithm of each individual condition is well established. An association between MMD and intracranial aneurysms is also known, but MMD in association with AVM is rare. The authors discuss various dilemmas in the multimodality management of these conditions when they coexist with an illustrative case of a 46-year-old woman who presented following a stroke, with 1) left-sided MMD, 2) left-sided frontal AVM, and 3) an aneurysm arising from the A1 segment of the right anterior cerebral artery. These were managed respectively by 1) the left external carotid artery to M2 segment of middle cerebral artery bypass using an autologous radial artery graft and left indirect superior temporal artery-encephaloduroarteriosynangiosis, 2) stereotactic radiosurgery, and 3) endovascular coiling o...
Moyamoya disease is a rare progressive cerebrovascular disease characterised by progressive occlusion of the supraclinoid internal carotid arteries (ICA) and its main branches within the circle of Willis [1]. This occlusion results in the formation of fine vascular network formation in the base of the brain termed as moyamoya vessels [1]. Saccular aneurysms associated with moyamoya disease are commonly located in the vertebrobasilar circulation [2, 3, 4]. Anterior circulation aneurysm associated with moyamoya disease is usually treated by neurosurgical clipping [5]. However with improvements in endovascular treatment (EVT) techniques and devices, EVT offers an alternative. We report a case of ruptured anterior communicating artery aneurysm in a patient with a left middle cerebral artery moyamoya disease treated by EVT. To the best of our knowledge, anterior circulation aneurysm associated with moyamoya disease treated with EVT has not been previously reported in the literature. CASE REPORT A 23 year old gentleman presented with a five day history of headache, diplopia and fever but no neurological deficit. CT scan of the brain showed a large left frontal intraparenchymal haemorrhage associated with subarachnoid and intraventricular haemorrhages [Figure 1]. A reconstructed 3D rotational angiogram (3DRA) of the cerebral circulation showed a bilobed anterior comunicating artery aneurysm measuring 7.2 x 3.9 mm pointing anteroinferiorly with the neck measuring 2.5 mm [Figure 2]. There was also severe M1 segment stenosis of the left middle cerebral artery with multiple collaterals in keeping with moyamoya vessels [Figure 2]. The 3D images showed a better view of the anterior communicating artery aneurysm and the moyamoya vessels, which enabled us to get a good working projection for EVT [Figure 3]. EVT was then chosen as the treatment option. The procedure was done under general anaesthesia. Right femoral access was obtained with 5F guiding catheters (Envoy; Cordis, Miami Lakes, FL). Full heparinasation (5000 IU bolus, followed by continuous infusion [2000-3000 IU/hour]) was achieved and the activated clotting time was maintained between 200-300 seconds. Excelsior SL-10 microcatheter (Boston Scientific ®) and Transcend 0.014" microguidewire (Boston Scientific ®) was used to cannulate the aneurysm and embolisation was done with three detachable platinum microcoils (Microplex ® , Microvention ®). Post embolisation angiogram showed a 10% residual slow filling of the aneurysm's neck and sac [Figure 4].
European Neurology, 2014
Willis circle with absence of the ACAs and of L-MCA and multiple tortuous vessels branching from the R-MCA ('moyamoya vessels'). No aneurysms or vascular malformations were found (fig. 1). Based on these findings, the patient was diagnosed with MMD. No neurological deficits were noted on the patient's physical examination. Upon admission, we performed a Carotid Duplex ultrasound and a TCD, which showed an increase in mean blood flow velocity of R-MCA. This was initially attributed to vasospasm and nimodipine therapy was started. We also performed a progressive TCD follow-up; hemodynamic parameters of the first ultrasound examination as well as those from TCD follow-up are summarized in table 1 (we exclusively reported follow-up values from the R-MCA, which were the only ones that showed significant changes through time). The patient had a very favorable outcome and is currently asymptomatic. Particularly interesting is the monitoring of hemodynamic state of brain vessels done in our case. According to previous studies performed on asymptomatic MMD patients [6] , we found at T0 that both the ICAs showed normal flow velocity and low resistance, while the remaining intracranial vessels showed a consistent hemodynamic pattern in terms of high flow velocity and low resistance. This pattern re
Moyamoya disease:clinical and angiographic features
This is a retrospective study of 12 adults (8 female, 4 male, mean age 45.3; range 26-63) and two children with moyamoya disease (MD) identified among 3400 diagnostic cerebral angiographies performed in a period of 17 years. Diagnosis of MD was made on the basis of features and progression of angiographic findings. Clinical manifestations and angiographic findings were analysed with a review of the literature. Two children and six adult patients had a clinical feature of ischemic cerebral events. The other adult patients had clinical signs of subarachnoid and/or intracerebral and intraventricular hemorrhage. Cerebral angiography showed a typically fine network of vessels at the base of the brain with a hazy, puff-of-smoke appearance, and development of transdural and leptomeningeal anastomoses in children and 8 adults patients. Four adult patients were in the terminal stage, with complete cerebral supply via vertebrobasilar and external carotid branches. Two of the adult patients had accompanying saccular aneurysms with localisations on basilar and internal carotid bifurcations, of which the latter was bleeding. All angiographic changes were bilateral. Low incidence and adult predominance are characteristic of MD in our population. There was no familial occurence among our patients. The clinical features of ischemic stroke were present in both children and adults, and intracranial hemorrhage in adults only. A characteristic angiographic feature of moyamoya was found in both children and adults.
Journal of Stroke and Cerebrovascular Diseases, 2014
Background: Adult moyamoya disease (MMD) is known to have high incidence of cerebral microbleeds (cMBs); however, the clinical significance still remains unclear. We investigated the frequency of cMBs in a large number of patients and analyzed the patterns of MB distribution in association with the location of the hematoma and moyamoya vessels. Methods: We studied 259 consecutive patients with MMD using prospectively collected database. One hundred ninety-one patients were eligible for the present study, and image analysis was performed retrospectively. The presence of cMBs and remains of hemorrhage were determined using gradient-echo T2*weighted sequence (1.5 T). The development of moyamoya vessels was assessed on source images of time-of-flight magnetic resonance angiography. The analysis consists of descriptive assessment of the spatial relationship between cMB, remains of hemorrhage, and moyamoya vessels. Statistical analysis was performed to calculate relative risk ratio in the presence of cMBs in relation to the remains of hemorrhage (macrohematoma), age of onset, and the presence of concomitant moyamoya vessels. Results: Thirty MBs were observed in 20 adult MMD patients (16.9%). MBs were located predominantly in the periventricular white matter (63.3%) followed by the basal ganglia/thalami (20%). Comparing the patients with cMBs from those without, hematoma was more frequently observed in patients with cMBs (odds ratio [OR] 4.29; 95% confidence interval [CI] 1.58-11.62; P 5 .0062). Patients with adult onset was more likely to demonstrate cMBs (14.4%) compared with the patients with pediatric onset (4.1%) (OR 3.93; 95% CI 1.11-13.91). Moyamoya vessels appeared in the lateral part of the trigon, and the periventricular white matter was significantly associated with the presence of cMBs (lateral part of the trigon; OR 3.29 [1.59-6.82], P 5 .0019, periventricle of the body of lateral ventricle; OR 2.40 [1.20-4.79], P 5.0214, respectively). cMBs accompanied concomitant arteries in 23 (76.7%) lesions. The subependymal-leptomeningeal artery anastomosis was the most common pattern (n 5 20, 66.7%). Conclusions: Spatial relationship was demonstrated between the moyamoya vessels and perivascular hemosiderin deposition particularly around the subependymal-leptomeningeal anastomosis, suggesting the mechanism for the development of cMBs in MMD. Present study further supports previous findings that cMBs potentially serve as a marker for the bleeding-prone microangiopathy in MMD. The significance of the present study lies in selecting optimal surgical candidate for preventing future hemorrhage by the presence of the cMBs, whereas current surgical indication relying on the degree From the
Middle Cerebral Artery Occlusion with Moyamoya-Like Vessels and Aneurysms
Interventional Neuroradiology, 2014
We describe two cases of aneurysmal rupture in moyamoya-like vessels in middle cerebral artery occlusion. This phenomenon was previously described in severe steno-occlusive disease and accounts for the hemorrhagic presentation. To our knowledge, these are the second and third clinical cases published in modern neuroradiological literature.
Noninvasive vascular imaging of moyamoya: Diagnosis, followup, and surgical planning
Moyamoya disease and syndrome are typically described by their imaging features on cerebral angiography, however advances in noninvasive imaging has allowed improved anatomic and physiologic characterization of this vascular abnormality. Advanced imaging complements the role of cerebral angiography, and when used appropriately can provide valuable information regarding the diagnosis, treatment planning, and surveillance in patients with moyamoya. Techniques to be aware of include computed tomography, CT-angiography, Magnetic Resonance Imaging, MR Angiography (time-of-flight and dynamic contrast enhancemed angiography), and perfusion imaging (CT perfusion, arterial spin labeling perfusion, and nuclear medicine perfusion). With an appropriate understanding and implementation of noninvasive techniques, this disease process can be better understood allowing for improved patient management.
Surgical Neurology International, 2014
Background: Management of aneurysms associated with deep collateral vessels in moyamoya disease is challenging both from an endovascular and a surgical standpoint. Diffi culties with access or localization, and compromise of the collateral circulation with subsequent ischemia are the primary concerns, making direct obliteration potentially unfeasible or risky. Alternatively, superfi cial temporal artery-middle cerebral artery bypass is another potential strategy for resolution of these aneurysms. Case Description: Presented are the fi ndings and management for a patient with moyamoya disease and bilateral deep collateral vessel aneurysms, successfully treated with endovascular obliteration following a right-sided hemorrhage and subsequently with bypass for an unruptured but growing contralateral aneurysm. Conclusions: A rationale and approach to management is outlined, as derived from review of the current literature and the illustrative case with bilateral collateral vessel aneurysms.
Moyamoya disease: Diagnostic imaging
Polish journal of radiology / Polish Medical Society of Radiology, 2011
Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. The incidence of moyamoya disease is high in Asian countries; in Europe and North America, the prevalence of the disease is considerably lower. Clinically, the disease may be of ischaemic, haemorrhagic and epileptic type. Cognitive dysfunction and behavioral disturbance are atypical symptoms of moyamoya disease.Characteristic angiographic features of the disease include stenosis or occlusion of the arteries of the circle of Willis, as well as the development of collateral vasculature. Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease.The most common surgical treatment combines the direct arterial anastomosis between the superficial temporal artery and middle cerebral, and the indirect synangiosis involving placement of vascularised tissue in the b...
Moyamoya Disease: A Rare Vascular Disease of the CNS
Vascular Malformations of the Central Nervous System [Working Title]
Moyamoya disease (MMD) is a rare disease affecting the cerebral vasculature of the central nervous system (CNS) with a reported incidence of 0.35-0.94 per 100,000 populations. It was first reported from Japan and later from other parts of the world. The pathology is narrowing of blood vessels supplying anterior circulation and rarely posterior circulation. It was believed that the disease is genetic in origin, but environmental factors also play a role. Patients with this rare disease may present with ischemic or hemorrhagic symptoms. Ischemic symptoms account for the disease in most of the pediatric patients, whereas in adults, hemorrhage is more common. Diagnostic imaging like CT angiogram and magnetic resonance angiogram helps in demonstrating the narrowing or the collateral vessels like "a puff of smoke" (moyamoya) formed at the base of the brain. Moyamoya disease is treated medically and/or surgically. Aspirin is the main medication used. Surgical options are direct or indirect revascularization techniques to bypass the stenosis. The disease is progressive in majority of the patients, but if treated early, they can have good prognosis especially children.