Treatment Of Adult Moyamoya Disease Research Articles

Surgical techniques and indications for treatment of adult moyamoya disease

Moyamoya disease (MMD) is a chronic, progressive cerebrovascular disease involving the occlusion or stenosis of the terminal portion of the internal carotid artery (ICA) and the proximal anterior and middle cerebral arteries. Adults with MMD have been shown to progressively accumulate neurological and cognitive deficits without treatment, with a mortality rate double that of pediatric patients with MMD. Surgical intervention is the mainstay of treatment to prevent disease progression and improve clinical outcomes. Several different types of bypasses can be utilized for revascularization in MMD, including indirect, direct, and combined forms of extracranial-to-intracranial (EC-IC) bypass. Overall, the choice of appropriate technique requires consideration of the age of the patient, preoperative hemodynamics, neurologic status, and territories most at risk and in need of revascularization. Here, we will review the indications and surgical techniques for the treatment of adult MMD. Step-by-step instructions for performing several bypass variants with technical pearls are discussed.

Value of Burr‐Hole Surgery as an Adjunct to Encephaloduroarteriosynangiosis in Treatment of Adult Moyamoya Disease: A Case Series

Background Using a single indirect revascularization technique in adult patients with moyamoya disease has been associated with inconsistent outcomes. Herein, we aimed to describe treatment outcomes following combined encephaloduroarteriosynangiosis plus burr‐hole surgery (BHS) in adult moyamoya disease. Methods Encephaloduroarteriosynangiosis combined with 1 anterior and 1 posterior burr hole per hemisphere was performed in 16 adults with moyamoya disease who presented to our center between 2013 and 2019. Patients were followed with clinical reassessments and cerebral angiograms to evaluate for neovascularization. Results Combined encephaloduroarteriosynangiosis and BHS procedures were performed on 24 hemispheres in 16 patients. Follow‐up ranged from 9 to 57 months, during which no new ischemic or hemorrhagic events, perioperative or long‐term complications, or deterioration of symptoms was observed. Follow‐up angiography showed evidence of additional neovascularization provided by the burr holes in 18 of the 24 hemispheres. Of 9 hemispheres with poor revascularization response to encephaloduroarteriosynangiosis, 5 achieved neovascularization at the burr‐hole sites. Older age, smoking, and cerebral infarction or hemorrhage on preoperative magnetic resonance imaging tended to correlate with absence of neovascularization following BHS. Conclusions BHS is a simple and safe procedure that can be done simultaneously with other indirect or direct revascularization procedures. It can provide additional neovascularization even in patients unresponsive to encephaloduroarteriosynangiosis (EDAS) and can be tailored to improve the blood flow in any vascular territory that may not be properly covered by standard revascularization procedures. We find value in adding BHS as an adjunct to other indirect or direct revascularization techniques such as encephaloduroarteriosynangiosis for treatment of adult moyamoya disease.

Surgical Treatment of Adult Moyamoya Disease In Saudi Arabia: A Review Article

Moyamoya disease (MMD) is an isolated chronic, usually bilateral, vasculopathy disease of undetermined etiology. The clinical presentations of MMD include TIA, ischemic stroke, hemorrhagic stroke, seizures, headache, and cognitive impairment. Intra- and extra-cranial revascularization for the prevention of recurrence of bleeding in patients with hemorrhagic MMD is controversial. Surgical revascularization of MMD includes 3 types: Direct revascularization, indirect revascularization and combined revascularization. The surgical goal of cerebral revascularization is to prevent progression of symptomology, alleviate intracranial hemodynamic stress, and reduce the incidence of subsequent ischemic or hemorrhagic stroke. However, surgical treatments pose various complications due to the sudden increase in cerebral blood flow or hemodynamic changes caused by perioperative risk factors and anesthesia, such as HS, cerebral hemorrhage and cerebral infarction, bypass occlusion caused by distal vascular resistance, bypass occlusion caused by compression of the temporalis, and anastomotic aneurysm.

Safety and Efficacy of Superficial Temporal Artery- Middle Cerebral Artery Bypass Graft Combined with Superficial Temporal Artery Branching and Cerebral- Dural-Temporal Vascular Stenosis in the Treatment of Adult Moyamoya Disease

The safety and clinical efficacy of superficial temporal artery-middle cerebral artery bypass grafting combined with superficial temporal artery branching and cerebral-dural-temporal vascular grafting in the treatment of adult moyamoya disease are investigated. A retrospective analysis of the data of 22 adult patients with moyamoya disease who underwent superficial temporal artery-brain artery bypass grafting combined with superficial temporal artery branching and cerebral-dural-muscle vascular stenting from June 2012 to June 2018 is performed to analyze the patient’s treatment outcome, functional recovery, and complications. Among the 22 patients, 17 (77.3 %) patients have improved clinical symptoms 1 w after surgery, and 5 patients (22.7 %) have no significant improvement in symptoms. After 3 mo follow-up, 21 patients (95.5 %) have significant improvement in clinical symptoms and spontaneous living ability compared with preoperative, and 1 patient (5.3 %) has no improvement in symptoms of new cerebral infarction. In patients with ischemic moyamoya disease, the clinical symptoms and quality of life of patients with superficial temporal artery-middle cerebral artery bypass graft combined with superficial temporal artery branching and cerebral-dural-temporal muscle vascularization are significantly improved. It shows that it can improve the prognosis of patients with ischemic moyamoya disease and has important clinical significance for the recovery of neurological function and the improvement of quality of life.

Membrane Retraction Technique in Bypass Surgery for the Treatment of Adult Moyamoya Disease with Deep-Seated Recipient Artery

To introduce a membrane retraction technique that can provide good exposure of deep-seated recipient arteries in cortical sulci and simplify the anastomosis procedure. Only those adult patients with moyamoya disease who underwent superficial temporal artery to middle cerebral artery bypass surgery during which the suitable recipient arteries were located deep in cortical sulci were included in this retrospective study. By placing 10-0 prolene sutures to the arachnoid membrane of the 2 banks of sulcus, arachnoid retraction was applied to pull the sulcus apart and then the deep-seated recipient arteries were exposed. Standard end-to-side anastomosis was completed. The mean occlusion time and successful rate were recorded and compared with those of the normal procedure. From June to November 2019, 124 superficial temporal artery to middle cerebral artery bypass surgeries for the treatment of moyamoya disease were performed in Nanfang Hospital. The membrane retraction technique was used in 5 patients (5/110, 4.5%) and the success rates were 100%. The mean temporary occlusion time was 27.2 minutes. No procedure-related complications were observed. All patients recovered uneventfully. The postoperative angiograms confirmed good patency of bypass grafts in all 5 cases. The membrane retraction technique is an effective and safe method for the treatment of adult patients with moyamoya disease with deep-seated recipient arteries within the sulci.

Diagnosis and treatment of adult Moyamoya disease

Moyamoya disease (MMD) refers to a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery with abnormal collateral vessel formation of unknown etiology. The definite diagnosis of MMD requires cerebral angiography or magnetic resonance angiography and/or magnetic resonance imaging after excluding other underlying diseases, particularly in adult patients. The treatment aims to improve regional cerebral blood flow to prevent cerebral ischemic events and alleviate hemodynamic instability that can provoke cerebral hemorrhage. Although various surgical revascularization methods have been introduced, combined revascularization surgery including direct revascularization is preferred over indirect revascularization only in adult MMD patients. Several recent studies have shown that surgical treatment has better outcomes and prognosis for symptomatic hemodynamically unstable MMD patients with both ischemic and hemorrhagic presentations. For asymptomatic patients, follow up with appropriate imaging is recommended. Surgery should be considered when new symptoms emerge with hemodynamic aggravation.

Clinical efficacy of different vascular reconstruction on adult moyamoya disease

Objective: To investigate the clinical efficacy of superficial temporal artery -middle cerebral artery combined with encephalo-duro-arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) in the treatment of adult moyamoya disease. Methods: The clinical data of 47 adult patients with moyamoya disease who received vascular reconstruction in the Department of Neurosurgery of Taizhou Hospital of Zhejiang Province from January 2014 to January 2018 were retrospectively analyzed. Among them, 21 patients received EDAMS alone (EDAMS group, 14 patients with hemorrhagic moyamoya disease, 7 patients with ischemic moyamoya disease), 26 patients received STA-MCA combined with EDAMS (STA-MCA+EDAMS group, 17 patients with hemorrhagic moyamoya disease, 9 patients with ischemic moyamoya disease). Cerebral hemodynamics at 1 day before surgery and 3 and 6 months after surgery were compared. The clinical efficacy and postoperative complications of the two methods were compared at 3 and 6 months postoperatively in hemorrhagic and ischemic types. Results: For hemorrhagic moyamoya disease, the remission rate (94.1%) at 6 months after surgery in the STA-MCA + EDAMS group was higher than that in the EDAMS group (57.1%), and the difference was statistically significant (P<0.05). The CBF and CBV in the STA-MCA+EDAMS group were higher than those in the EDAMS group at 3 and 6 months after operation, and the MTT and TPP were lower than those in the EDAMS group, but there was no significant difference between the two groups (all P>0.05). For hemorrhagic moyamoya disease and ischemic moyamoya disease, the total incidence of postoperative complications of the two surgical methods was different, but the difference was not statistically significant (both P>0.05). Conclusion: Superficial temporal artery -middle cerebral artery combined with encephalo-duro- arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) can significantly improve neurological function and cerebral hemodynamics in adult moyamoya disease patients with high safety.

Improving Indirect Revascularization for Effective Treatment of Adult Moyamoya Disease: A Prospective Clinical, Cerebral Angiographic, and Perfusion Study

Indirect revascularization is simple and safe, but it is not commonly used in adult moyamoya disease owing to its unreliable effect. This prospective study aimed to evaluate the effectiveness of indirect revascularization in treating adult moyamoya disease after refinement of the surgical technique and perioperative care. Adult patients who underwent indirect revascularization as the primary treatment for moyamoya disease between November 2013 and January 2017 were studied. The indirect revascularization procedures included encephalo-duro-arterio-synangiosis and encephalo-myo-synangiosis in the temporal region and encephalo-pericranio-synangiosis in other hypoperfusion areas. The preoperative and postoperative clinical conditions, cerebral angiography findings, and time-to-peak prolongation areas on magnetic resonance perfusion studies were assessed to evaluate the revascularization effect. Refinements of the surgical technique included the selection of surgical area based on perfusion imaging, craniotomy size, and number of affected hemispheres, along with the inclusion of a dural flap in every procedure. Nineteen patients (17 females and 2 males), and a total of 31 cerebral hemispheres, were included. The mean patient age was 32.7 ± 11.4 years. After a mean follow-up of 38.6 ± 11.5 months, all patients showed clinical improvement or stabilization. Available postoperative angiography demonstrated Matsushima grade A or B in 19 of 21 hemispheres. The mean time-to-peak prolongation index of all 31 hemispheres improved from 24.09 ± 9.83% preoperatively to 12.82 ± 6.75% at 3-6 months after surgery (P < 0.001). The complication rate was 12.9%, and all complications were transient. With refinements of surgical techniques and perioperative care, indirect revascularization is more reliable and can be a viable alternative treatment for adult moyamoya disease. In our cohort, outcomes were satisfactory, with mean 38.6-month follow-up.

Surgical Treatment of Adult Moyamoya Disease.

Moyamoya disease (MMD) is being increasingly diagnosed with the development of radiological surveillance technology and increased accessibility to medical care. Accordingly, there have been several recent reports on treatment outcomes in MMD. In this review, we summarize recent advances in surgical treatment and outcomes of adult MMD, while addressing related controversies. Recent studies suggest that revascularization surgery leads to significantly more favorable outcomes for stroke prevention, angiographic and hemodynamic changes, and clinical outcomes than does conservative treatment for adult patients with ischemic MMD. Moreover, direct revascularization methods should be considered as the first-line treatment over indirect methods, although the latter may be considered if a direct method is not possible. In cases of hemorrhagic MMD, several studies have demonstrated that surgical treatment is more effective than conservative treatment in preventing further hemorrhage. In addition to revascularization surgery, endovascular treatment is emerging as a breakthrough therapy for hemorrhagic MMD. Accumulating evidence regarding the surgical treatment of adult MMD suggests the benefit of revascularization over conservative management for both ischemic and hemorrhagic patients. However, the benefit of revascularization in asymptomatic adult MMD remains unclear.

Abstract TP132: The Clinical Role of Microembolic Signals in Adult Moyamoya Disease

Background: Both hemodynamic compromise and artery-to-artery embolism have been thought to be implicated in moyamoya disease (MMD) with ischemic symptoms. The relationship between microembolic signals (MESs) and ischemic stroke remains to be clarified. We aimed to identify associated risk factors of MESs and to explore clinical significance of MESs in adult MMD with ischemic stroke. Methods: A total of 48 patients (89 hemispheres) newly diagnosed with adult MMD were enrolled and angiographically classified according to the Suzuki staging system. For detection of MESs, transcranial doppler (TCD) monitoring was performed within 2 days after first admission. Recent ischemic events, including transient ischemic attack (TIA), which occurred within 3 months of diagnosis, were also recorded. Mean flow velocities in middle cerebral artery using TCD were also evaluated. Results: Eleven hemispheres were found to have MESs, and 78 had no MESs. Among clinical types of MMD in 89 hemispheres, ischemic-type MMD (n = 42, 47%) was most common. Antiplatelet agents were prescribed for 22 patients (43 hemispheres) before evaluation for MES. The incidence of recent ischemic event was associated with the MES status (P = 0.024). Recent ischemic event was found to be predictive of MESs in adult MMD (hazard ratio: 6.294; 95% CI 1.345-29.46). Detection of MESs was significantly higher in the high mean flow velocity of the middle cerebral artery (P = 0.019). After controlling for age, sex, recent ischemic event, and antiplatelets, ‘early stage’ MMD (Suzuki stage I and II) was the independent predictor of MESs at baseline (hazard ratio: 6.172; 95% CI 1.235-31.25). Conclusions: MESs, known to be indicative of artery-to-artery embolism in steno-occlusive cerebrovascular diseases, were observed in ‘early stage’ MMD, particularly with high MFVs and clinically associated with recent ischemic stroke. This necessitates the need for the efficacy of antiplatelets in the treatment of adult MMD with ischemic stroke in a prospective controlled study in the future.

Operation of superficial temporal artery-middle cerebral artery(STA-MCA)bypass combined with temporal muscle sticking therapy adult moyamoya disease

Objective To investigate the clinical effect of operation of superficial temporal artery-middle cerebral artery(STA-MCA)bypass combined with temporal muscle sticking in the treatment of adult moyamoya disease. Methods A retrospective analysis was the data of operation, including preoperative evaluation, operation method,postoperative follow-up. Results Postoperative complications occurred in 5 cases, including 3 cases of intracranial hemorrhage,2 cases of cerebral infarction. During the follow-up of 6-35 months, no recurrent hemorrhage cases,1 cases of cerebral infarction, TIA remission rate was 93.5%. Postoperative cerebral blood flow of 38 cases significantly improved compared with preoperative by SPET, accounted for 86.3%. Cerebral blood flow of 41 cases was well compensated by vascular bridge, accounted for 91%. Conclusion Operation of STA-MCA bypass combined with temporal muscle sticking can significantly reduce the incidence of stroke, fewer complications, which is an ideal method in the treatment of adult moyamoya disease. Key words: Moyamoya Disease; Surgical Procedures, Operative; Treatment Outcome

An update on the diagnosis and treatment of adult Moyamoya disease taking into consideration controversial issues

Moyamoya disease (MMD) is characterized by a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery (ICA) with abnormal Moyamoya vessel (MMV) development without associated diseases. Recent advances in radiologic tests have increased the number of MMD patients. Beyond detection improvement by magnetic resonance angiography (MRA) or cerebral angiography, predicting factors for disease severity, future hemorrhage, clinical outcome, post-op complications, and technical advances in assessing cerebral hemodynamics have been increasingly reported. Although treatment of pediatric MMD is well established, controversy remains over the treatment in adult patients. In particular, there are debates over the disease entity of adult MMD, contralateral progression in adult unilateral MMD, treatment strategy for asymptomatic adult MMD, and the association of MMD with thyroid disease. The purpose of this review is to provide an update on the diagnosis and treatment of adult MMD while addressing controversial issues.

Angiographic Results of Indirect and Combined Bypass Surgery for Adult Moyamoya Disease

ObjectiveThe aim of this study was to compare the efficacy of indirect and combined bypass surgery for treatment of adult moyamoya disease (MMD). The definition of combined bypass surgery is a combination of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and indirect anastomosis. Development of collateral circulation after surgery was investigated.MethodsForty three patients (58 hemispheres) with MMD were followed by cerebral angiography for at least six months after surgery, between May 2002 and July 2011. Indirect and combined revascularization surgeries were performed in 33 and 25 cases, respectively. Good outcome was defined as more than group B, in accordance with the method suggested by Matsushima.ResultsDevelopment of collateral circulation was not affected by sex (p = 0.493), clinical features (p = 0.206), or Suzuki stage (p = 0.428). Based on postoperative cerebral angiography, the combined bypass surgery group showed a better angiographic outcome, than the encephaloduroarteriomyosynangiosis (EDAMS) group (p = 0.100, odds ratio [OR] 4.107, 95% confidence interval [CI] 0.700 - 24.096). The combined bypass group showed a better response than the encephaloduroarteriogaleosynangiosis (EDAGS) group (p = 0.088, OR 4.600, 95% CI 0.721 - 29.332). Similar responses were observed for EDAGS and EDAMS (p = 0.886, OR 1.120, 95% CI 0.239 - 5.251). The combined bypass group showed a better response than the indirect group (p = 0.064, OR 4.313, 95% CI 0.840 - 22.130).ConclusionResults of this study demonstrate that combined bypass results in better revascularization on angiographic evaluation in adult MMD. Therefore, among surgical procedures, combined bypass is a choice that can be recommended.