Treatment of moyamoya disease in the adult population with pial synangiosis

2014 ◽  
Vol 120 (3) ◽  
pp. 612-617 ◽  
Author(s):  
Ning Lin ◽  
Joshua P. Aronson ◽  
Sunil Manjila ◽  
Edward R. Smith ◽  
R. Michael Scott

Object Surgical treatment of moyamoya disease in the adult population commonly uses direct revascularization, the superficial temporal artery (STA) to middle cerebral artery (MCA) bypass (STA-MCA). Pial synangiosis, a method of indirect revascularization, has been used in adult patients with moyamoya when STA-MCA bypass was not technically feasible. Although the effectiveness of pial synangiosis has been well described in children, only limited reports have examined its role in adult patients with moyamoya disease. In this study the authors report on their experience with pial synangiosis revascularization for this population. Methods The authors reviewed the clinical and radiographic records of all adult patients (≥ 18 years of age) with moyamoya disease who underwent cerebral revascularization surgery using pial synangiosis at a single institution. Results From 1985 to 2010, 66 procedures (6 unilateral, 30 bilateral) were performed on 36 adult patients with moyamoya disease. The mean age at surgery was 28.3 years, and 30 patients were female. Twenty-eight patients (77.8%) presented with transient ischemic attacks (TIAs), 24 (66.7%) with stroke, and 3 (8.3%) with hemorrhage. Preoperative Suzuki stage was III or higher in 50 hemispheres (75.8%) and 3 patients had undergone prior treatments to the affected hemisphere before pial synangiosis surgery. Clinical follow-up was available for an average of 5.8 years (range 0.6–14.1 years), with 26 patients (72.2%) followed for longer than 2 years. Postoperative angiography was available for 24 patients and 46 revascularized hemispheres, and 39 (84.8%) of the 46 hemispheres demonstrated good collateral formation (Matsushima Grade A or B). Postoperative complications included 3 strokes, 5 TIAs, and 2 seizures, and there was no hemorrhage during the follow-up period. One patient required additional revascularization surgery 8 months after pial synangiosis. Conclusions Pial synangiosis is a safe and durable method of cerebral revascularization in adult patients with moyamoya and can be considered as a potential treatment option for moyamoya disease in adults.

Neurosurgery ◽  
2011 ◽  
Vol 68 (1) ◽  
pp. 34-43 ◽  
Author(s):  
Joshua R. Dusick ◽  
Nestor R. Gonzalez ◽  
Neil A. Martin

Abstract BACKGROUND: Several forms of indirect cerebral revascularization have been proposed to promote neovascularity to the ischemic brain. OBJECTIVE: To present clinical and angiographic outcomes of indirect revascularization by encephaloduroarteriosynangiosis and burr holes for the treatment of Moyamoya disease in adults and children. METHODS: Data from 63 hemispheres treated in 42 patients (average age, 30 years; 33 adults; 30 female patients; median follow-up, 14 months) were reviewed. In hemispheres with preoperative and postoperative (6- to 12-month) angiograms available, superficial temporal artery (STA) and middle meningeal artery (MMA) diameters were measured. Preoperative and postoperative corrected arterial sizes were compared. RESULTS: Seven patients (17%) had transient ischemic attacks that resolved within 1 month of surgery. No patients suffered moyamoya-related hemorrhage after treatment. Two patients developed additional symptoms many years after surgery. In 18 hemispheres with preoperative and postoperative angiograms, there was an average postoperative increase in STA and MMA diameters of 51% (P = .003) and 49% (P = .002), respectively. Both children and adults displayed revascularization. Two patients did not demonstrate increased vessel size. STA blush and new branches and MMA blush and new branches were identified in 12, 14, 14, and 16 hemispheres, respectively. Angiographic blush was identified in 59% of frontal and 19% of parietal burr holes (P = .03). Surgical complications included 2 subdural hemorrhages requiring evacuation and 2 new ischemic deficits (1 transient). CONCLUSION: Indirect revascularization by encephaloduroarteriosynangiosis and burr holes for moyamoya results in long-term resolution of ischemic and hemorrhagic manifestations in 95% of adults and children. The MMA appears to contribute significantly to the revascularization on follow-up angiograms with increased size and neovascularity comparable to that of the STA. Angiographically, parietal burr holes do not contribute as significantly as frontal burr holes.


2021 ◽  
pp. 1-9
Author(s):  
Peter Kan ◽  
Visish M. Srinivasan ◽  
Aditya Srivatsan ◽  
Ascher B. Kaufmann ◽  
Jacob Cherian ◽  
...  

OBJECTIVE In select patients, extracranial-intracranial (EC-IC) bypass remains an important tool for cerebral revascularization. Traditionally, superficial temporal artery–middle cerebral artery (STA-MCA) bypass was performed using one limb of the STA only. In an attempt to augment flow and to direct flow to different ischemic areas of the brain, the authors adopted a “double-barrel” technique in which both branches of the STA are used to revascularize distinct MCA territories. METHODS A series of consecutive double-barrel STA-MCA bypasses performed between 2010 and 2020 were reviewed. Each anastomosis was directed to augment flow to a territory most at risk based on preoperative perfusion studies, cerebral angiography, and intraoperative indocyanine green data. CT perfusion and CTA were routinely used to evaluate postoperative augmentation and graft patency. Patient perioperative outcomes, surgical complications, and modified Rankin Scale (mRS) scores at the last follow-up were reported. RESULTS Forty-four patients (16 males, 28 females) successfully underwent double-barrel STA-MCA bypass on 54 cerebral hemispheres: 28 operations were for moyamoya disease, 23 for atherosclerotic disease refractory to medical therapy, 2 for complex cerebral aneurysms, and 1 for carotid occlusion as a sequela of cavernous meningioma growth. Ten patients underwent multiple operations, 9 of whom had moyamoya disease/syndrome, with the subsequent operation on the contralateral hemisphere. The average patient age at surgery was 45.1 years (range 14–73 years), with a mean follow-up time of 22.1 months. Intraoperative graft patency was confirmed in 100% of cases, and 101 (98.1%) of the 103 anastomoses with imaging follow-up were patent. Perfusion to the revascularized hemisphere was improved in 88.2% of cases. Perioperative ischemic and hemorrhagic complications occurred in 8 procedures (2 were asymptomatic), whereas remote ischemic and hemorrhagic events occurred in 7 cases. There was no mortality in the series, and the mean patient mRS scores were 1.72 at presentation and 1.15 at the last follow-up. CONCLUSIONS The high rates of intraoperative and postoperative patency support the feasibility of dual-anastomosis STA-MCA bypass for revascularization. The perioperative complication rate is not significantly different from that of single-anastomosis bypass. The functional outcomes at follow-up and perfusion improvement postoperatively support the efficacy and safety of this method as a treatment strategy.


Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Jinbing Zhao ◽  
Hongyi Liu ◽  
Yuanjie Zou ◽  
Shengxue He

Objective: Surgical revascularization is the mainstay of treatment for moyamoya patients. This study was to evaluate the progonosis of combined direct and indirect procedure for moyamoya disease patients. Methods: 76 cerebral hemispheres from 64 adult moyamoya patients undergoing combined superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and encephalo-duro-myo-synangiosis (EDMS) were reviewed retrospectively. The mean follow-up period was 23±14m (6m-70m). CT or MR perfusion and Digital subtraction angiography (DSA) were performed for preoperative diagnosis and postoperative evaluation of cerebral perfusion and revascularization. mRS score, angiographic collateralization score, vessel diameter were recorded to measure neurological dysfunction, revascularization area and vascular compensatory effect respectively. Results: Among the 64 MMD patients, 69 hemispheres received combined direct and indirect operations; 7 hemispheres only received indirect operations. During our follow-up periods, neurological deficits of 57/64 patients (89.1%) were partially alleviated. mRS was significantly decreased after operations not only in all patients, but also in stroke subgroup or hemorrhage subgroup. Generally, good revascularization was established in most of the patients by combined bypass. 92.1% (70/74) sides was scored 2 and 77.6% (59/74) sides was scored 3 determined by angiographic collateralization score. Compared to the preoperative situations (2.60±0.65mm), the calibers of STA main trunk increased profoundly in at postoperative 10 days (3.32±1.05mm, p<0.05 versus pre-operation), and shrinked back to preoperative status at 6 months (2.24±1.00mm, p>0.05 versus pre-operation) and 12 months (2.36±0.73mm, p>0.05 versus pre-operation). Conclusion: Our findings strongly suggested that combined STA-MCA bypass and EDMS provided efficient revascularization and excellent results in preventing strokes and hemorrhage in adult patients. The direct STA-MCA bypass provided early augmentation of cerebral perfusion, whereas the indirect EDMS provided a more durable long-term revascularization, indicating a complementary relationship between the two revascularization methods.


2019 ◽  
Vol 131 (6) ◽  
pp. 1702-1708 ◽  
Author(s):  
Eric S. Sussman ◽  
Venkatesh Madhugiri ◽  
Mario Teo ◽  
Troels H. Nielsen ◽  
Sunil V. Furtado ◽  
...  

OBJECTIVERevascularization surgery is a safe and effective surgical treatment for symptomatic moyamoya disease (MMD) and has been shown to reduce the frequency of future ischemic events and improve quality of life in affected patients. The authors sought to investigate the occurrence of acute perioperative occlusion of the contralateral internal carotid artery (ICA) with contralateral stroke following revascularization surgery, a rare complication that has not been previously reported.METHODSThis study is a retrospective review of a prospective database of a single surgeon’s series of revascularization operations in patients with MMD. From 1991 to 2016, 1446 bypasses were performed in 905 patients, 89.6% of which involved direct anastomosis of the superficial temporal artery (STA) to a distal branch of the middle cerebral artery (MCA). Demographic, surgical, and radiographic data were collected prospectively in all treated patients.RESULTSSymptomatic contralateral hemispheric infarcts occurred during the postoperative period in 34 cases (2.4%). Digital subtraction angiography (DSA) was performed in each of these patients. In 8 cases (0.6%), DSA during the immediate postoperative period revealed associated new occlusion of the contralateral ICA. In each of these cases, revascularization surgery involved direct anastomosis of the STA to an M4 branch of the MCA. Preoperative DSA revealed moderate (n = 1) or severe (n = 3) stenosis or occlusion (n = 4) of the ipsilateral ICA and mild (n = 2), moderate (n = 4), or severe (n = 2) stenosis of the contralateral ICA. The baseline Suzuki stage was 4 (n = 7) or 5 (n = 1). The collateral supply originated exclusively from the intracranial circulation in 4/8 patients (50%), and from both the intracranial and extracranial circulation in the remaining 50% of patients. Seven (88%) of 8 patients improved symptomatically during the acute postoperative period with induced hypertension. The modified Rankin Scale (mRS) score at discharge was worse than baseline in 7/8 patients (88%), whereas 1 patient had only minor deficits that did not affect the mRS score. At the 3-year follow-up, 3/8 patients (38%) were at their baseline mRS score or better, 1 patient had significant disability compared with preoperatively, 2 patients had died, and 1 patient was lost to follow-up. Three-year follow-up is not yet available in 1 patient.CONCLUSIONSAcute occlusion of the ICA on the contralateral side from an STA-MCA bypass is a rare, but potentially serious, complication of revascularization surgery for MMD. It highlights the importance of the hemodynamic interrelationships that exist between the two hemispheres, a concept that has been previously underappreciated. Induced hypertension during the acute period may provide adequate cerebral blood flow via developing collateral vessels, and good outcomes may be achieved with aggressive supportive management and expedited contralateral revascularization.


2020 ◽  
pp. 1-8
Author(s):  
Ryosuke Tashiro ◽  
Miki Fujimura ◽  
Masahito Katsuki ◽  
Taketo Nishizawa ◽  
Yasutake Tomata ◽  
...  

OBJECTIVESuperficial temporal artery–middle cerebral artery (STA-MCA) anastomosis is the standard surgical management for moyamoya disease (MMD), whereas cerebral hyperperfusion (CHP) is one of the potential complications of this procedure that can result in delayed intracerebral hemorrhage and/or neurological deterioration. Recent advances in perioperative management in the early postoperative period have significantly reduced the risk of CHP syndrome, but delayed intracerebral hemorrhage and prolonged/delayed CHP are still major clinical issues. The clinical implication of RNF213 gene polymorphism c.14576G>A (rs112735431), a susceptibility variant for MMD, includes early disease onset and a more severe form of MMD, but its significance in perioperative pathology is unknown. Thus, the authors investigated the role of RNF213 polymorphism in perioperative hemodynamics after STA-MCA anastomosis for MMD.METHODSAmong 96 consecutive adult patients with MMD comprising 105 hemispheres who underwent serial quantitative cerebral blood flow (CBF) analysis by N-isopropyl-p-[123I]iodoamphetamine SPECT after STA-MCA anastomosis, 66 patients consented to genetic analysis of RNF213. Patients were routinely maintained under strict blood pressure control during and after surgery. The local CBF values were quantified at the vascular territory supplied by the bypass on postoperative days (PODs) 1 and 7. The authors defined the radiological CHP phenomenon as a local CBF increase of more than 150% compared with the preoperative values, and then they investigated the correlation between RNF213 polymorphism and the development of CHP.RESULTSCHP at POD 1 was observed in 23 hemispheres (23/73 hemispheres [31.5%]), and its incidence was not statistically different between groups (15/41 [36.6%] in RNF213-mutant group vs 8/32 [25.0%] in RNF213–wild type (WT) group; p = 0.321). CHP on POD 7, which is a relatively late period of the CHP phenomenon in MMD, was evident in 9 patients (9/73 hemispheres [12.3%]) after STA-MCA anastomosis. This prolonged/delayed CHP was exclusively observed in the RNF213-mutant group (9/41 [22.0%] in the RNF213-mutant group vs 0/32 [0.0%] in the RNF213-WT group; p = 0.004). Multivariate analysis revealed that RNF213 polymorphism was significantly associated with CBF increase on POD 7 (OR 5.47, 95% CI 1.06–28.35; p = 0.043).CONCLUSIONSProlonged/delayed CHP after revascularization surgery was exclusively found in the RNF213-mutant group. Although the exact mechanism underlying the contribution of RNF213 polymorphism to the prolonged/delayed CBF increase in patients with MMD is unclear, the current study suggests that genetic analysis of RNF213 is useful for predicting the perioperative pathology of patients with MMD.


Neurosurgery ◽  
2009 ◽  
Vol 64 (1) ◽  
pp. E195-E196 ◽  
Author(s):  
Toshiaki Hayashi ◽  
Reizo Shirane ◽  
Teiji Tominaga

Abstract OBJECTIVE In patients with moyamoya disease, surgery to revascularize the ischemic brain is a recommended treatment. However, there are a few patients who require additional revascularization surgery because of progression of the disease. Even patients who show no postoperative ischemic symptoms at first may experience late deterioration. We performed additional surgery for such lesions using occipital artery (OA)–posterior cerebral artery (PCA) bypass with indirect revascularization. The efficacy of the procedure is reported. METHODS We treated 3 patients with moyamoya disease who showed a transient ischemic attack after revascularization surgery. Three female patients, ranging in age from 6.0 to 35.2 years (mean age, 23.8 years) at the time of surgery, with ischemic symptoms (leg monoparesis in 2, visual impairment in 1) underwent the additional revascularization procedure. Preoperatively, all patients underwent indirect and/or direct revascularization surgery for initial treatment. All patients showed progression of the disease, especially in the PCA. OA–PCA bypass with encephalogaleodurosynangiosis and burr hole surgery were performed for postoperative ischemic symptoms. RESULTS All patients showed clinical and radiological improvement. The transient ischemic attack was improved in all 3 patients. They did not complain of transient ischemic attack in the recent follow-up period. Follow-up magnetic resonance imaging showed no additional cerebral infarction. Magnetic resonance angiography showed widening of the OA and development of peripheral collateral vessels. Postoperative single-photon emission computed tomographic studies showed marked increase of uptake in both anterior cerebral artery and PCA territories. Cerebral vasodilatory capacity evaluated by an acetazolamide test also showed marked improvement. One patient showed postoperative cerebral edema as a result of focal cerebral hyperperfusion. CONCLUSION OA–PCA anastomosis with indirect revascularization was effective for postoperative ischemia that showed symptoms in the anterior cerebral artery and PCA territories as a result of progression of a PCA lesion.


2021 ◽  
Author(s):  
Luciano Bambini Manzato ◽  
José Ricardo Vanzin ◽  
Octávio Ruschel Karam ◽  
Victor Emanuel Angeliero ◽  
Artur Eduardo Martio ◽  
...  

Background:Moyamoya disease (MMD) is a steno-occlusive cerebral angiopathy. The incidence of intracranial aneurysms (IA) associated with MMD is high (3.4-14.8%) when compared to the general population (1-3%). IA in the middle cerebral artery (MCA) associated with MMD are rare, with only 25 cases described in the literature. Methods:Search on the PubMed platform, in English, with the MeSH terms “Moyamoya Disease”, “Intracranial Aneurysm” and “Middle Cerebral Artery”. There were 151 results, of which 7 were included in the review. Results:Sumi et al. and Larson et al. reported non-ruptured IA of the M1 segment, treated by surgical clipping and wrapping, respectively. Endo et al. chose to clip a ruptured IA in the M1 segment, followed by anastomosis between the superficial temporal artery and the MCA, excluding the IA . Liu et al. reported a non-ruptured MCA IA in a pediatric patient, treated through an encephalo-duro-arterio-synangiosis; the IA was excluded. Peltier et al. performed an indirect revascularization using the multiple bur-hole technique in a pediatric patient with non-ruptured IA in the M1 segment, excluding the IA. Rivera et al. opted for the conservative approach of a ruptured IA in the M1 segment, which was successful. Yan et al. described 19 IA in the MCA associated with the MMD, without specifying the adopted therapeutic. Conclusion:Due to the low number of cases, no recommendation can be made, and treatment should be individualized.


2019 ◽  
Vol 23 (2) ◽  
pp. 198-203 ◽  
Author(s):  
Gyang Markus Bot ◽  
Jan-Karl Burkhardt ◽  
Nalin Gupta ◽  
Michael T. Lawton

OBJECTIVERevascularization is indicated in the management of moyamoya disease (MMD), with options that include direct and indirect techniques. Indirect bypass is popular in young children because the diminutive caliber of donors and recipients makes direct bypass difficult. The authors reviewed a series of patients treated with direct superficial temporal artery (STA)–to–middle cerebral artery (MCA) bypass in combination with encephalomyosynangiosis (EMS) in children 3 years or younger to demonstrate feasibility and safety.METHODSA retrospective review of all surgeries for MMD over a 19-year period identified 11 procedures in 6 patients. Surgical results, angiographic outcomes, and clinical outcomes were analyzed.RESULTSPatients had a mean age of 22.4 months. The symptomatic hemisphere was revascularized first, and the contralateral hemisphere was revascularized on average 2.8 months later in 5 patients. All direct bypasses were patent postoperatively and remained patent at late follow-up (mean 4.1 years), with both STA and MCA diameters increasing significantly (n = 5, p < 0.03). At last follow-up (mean follow-up duration, 5.0 years), favorable outcomes (modified Rankin Scale scores 0–2) were observed in 5 of the 6 patients (83%), with 1 dependent patient remaining unchanged postoperatively.CONCLUSIONSDirect STA-MCA bypass in combination with EMS for MMD is feasible and safe in patients 3 years or younger, based on favorable clinical and radiological outcomes in this patient cohort. Direct bypass should be considered when immediate revascularization is needed, without the biological delay associated with indirect bypass.


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