Abstract MP47: Clinicoradiological Outcomes of Surgical Revascularization for Moyamoya Disease With Minimum 10-Year Follow-Up

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Yiping Li ◽  
Allan R Wang ◽  
Gary K STEINBERG

Introduction: The long-term progression of disease and recurrence rate of symptomatic events after surgical revascularization for moyamoya disease (MMD) are unknown. Method: Patients with MMD operated on between 1991 and 2009 were prospectively enrolled into the departmental database. Results: 230 revascularization (87% combined direct and indirect and 13% indirect alone) procedures were performed on 129 patients for the treatment of MMD with a long-term bypass patency rate of 98%. Unilateral presentation was identified in 39 (30.2%) compared to 90 (69.8%) with bilateral MMD. Posterior circulation (PC) involvement was identified in 34 (26.4%) patients. Over a median follow-up period of 13 years (range 10-26 years), 15 (38.5%) patients experienced radiographic progression of unilateral disease and 14 (10.9%) experienced PC radiographic progression. Delayed surgical revascularization of the untreated contralateral anterior circulation was performed on 11 (28.2%) patients due to symptomatic progression of disease. Progression of PC disease occurred only in patients with PC involvement upon presentation. Aside from those with unilateral progression, fourteen additional patients (10.9%) experienced delayed cerebrovascular accidents (CVAs), 8 of which were ischemic and 6 were hemorrhagic strokes. Twelve delayed CVAs occurred in a previously revascularized hemisphere while one occurred in an un-diseased hemisphere from a micro pseudoaneurysm. Repeat revascularization was performed in 6 patients while 4 were managed conservatively and 4 were deceased. Relative to baseline presenting mRS, 92.2% of patients were improved or unchanged at 10 year follow-up. On multivariate regression, progression of the PC predicted the development of delayed CVA (Odds ratio 8.1, 95% CI 1.1-59.2) while pre-operative stroke (Odds ratio 3.4, 95% CI 1.2-9.8) presentation and progression of disease was predictive of 10-year outcome (Odds ratio 4.0, 95% CI 1.5-10.6). Conclusion: Good functional outcomes over a median follow-up period of 13 years support the strategy of revascularization for MMD patients. Disease progression of the posterior circulation is common and may reduce pial-pial collaterals resulting in delayed CVAs.

Stroke ◽  
2013 ◽  
Vol 44 (suppl_1) ◽  
Author(s):  
Satoshi Kuroda ◽  
Naoki Akioka ◽  
Daina Kashiwazaki ◽  
Hideo Hamada ◽  
Naoya Kuwayama ◽  
...  

Introduction —It is well known that surgical revascularization can improve cerebral hemodynamics and prevent further ischemic cerebrovascular events in moyamoya disease. However, a certain subgroup of patients repeats ischemic attacks even after surgery because of insufficient surgery or disease progression during follow-up periods. Hypothesis —Relevant designs and techniques in additional bypass surgery can resolve ischemic cerebrovascular events in patients with moyamoya disease refractory to previous bypass surgery. Methods —This study included totally 7 patients (9 hemispheres) with moyamoya disease refractory to previous bypass surgery. There were 5 children and 2 adults. They underwent previous bypass surgery in Japan and Europe 6 to 240 months before admission. Based on precise clinical and radiological analysis, cerebrovascular events were considered to occur because of insufficient bypass surgery in 5 patients and disease progression in the ipsilateral posterior cerebral artery in 2. Surgical strategies included wide craniotomy to cover the area where cerebral hemodynamics is still impaired and appropriate bypass procedures such as STA-MCA anastomosis, OA-PCA anastomosis, and indirect bypass. Using [123]I-IMP SPECT or [15]O-gas PET, cerebral hemodynamics was precisely examined before and after surgery Results —Postoperative course was uneventful and cerebral hemodynamics significantly improved in all 7 patients. Postoperative cerebral angiography revealed that additional bypass provided collateral blood flow to ischemic area before surgery. Ischemic cerebrovascular events rapidly resolved in 5 patients and gradually decreased in 2. Conclusion —This study strongly suggests adequate surgical design and procedures can resolve ischemic cerebrovascular events in patients with moyamoya disease refractory to previous bypass surgery.


Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Satoshi Kuroda ◽  
Naoki Nakayama ◽  
Shusuke Yamamoto ◽  
Daina Kashiwazaki ◽  
Haruto Uchino ◽  
...  

Background and Purpose: Surgical revascularization is now known to improve the outcome in patients with moyamoya disease. However, majority of previous studies reported their short-term (<5 years) outcome. Therefore, this study was aimed to evaluate long-term (5 to 20 years) outcome after STA-MCA anastomosis and ultimate indirect bypass, encephalo-duro-myo- arterio-pericranial synangiosis (EDMAPS). Methods: Cumulative incidence of late morbidity/mortality and disease progression were evaluated among 93 patients who underwent STA-MCA anastomosis and EDMAPS. All of them were prospectively followed up for longer than 5 years post-surgery (mean, 10.5±4.4 years). There were 35 pediatric and 58 adult patients. Clinical diagnosis included TIA or ischemic stroke in 80 patients, hemorrhagic stroke in 10, and asymptomatic in 3. STA-MCA anastomosis and EDMAPS were performed onto their 141 hemispheres. MRI and MRA were performed every 6 or 12 years during follow-up periods. Results: During follow-up periods, 92/93 patients were free from any stroke or death, but one recurred hemorrhagic stroke (0.10% per patient-year). Disease progression occurred in the territory of the contralateral carotid or posterior cerebral artery (PCA) in 19 hemispheres of 15 patients (1.5% per patient-year). The interval between initial surgery and disease progression varied from 0.5 to 15 years. Repeat bypass surgery for anterior and posterior circulations resolved ischemic attacks in all 10 patients. Conclusion: STA-MCA anastomosis and EDMAPS would be the best choice to prevent further cerebrovascular events for longer than 10 years by widely providing surgical collaterals to both the MCA and ACA territories. However, regular follow-up would be essential for longer than 10 years post-surgery to identify the disease progression in the territory of contralateral carotid artery and PCA and prevent late cerebrovascular events.


2020 ◽  
Vol 11 ◽  
Author(s):  
Bin Gao ◽  
Kaijiang Kang ◽  
Jia Zhang ◽  
Dong Zhang ◽  
Xingquan Zhao

Background: Headache associated with Moyamoya disease (HAMD) in the Chinese population is not well-described. The long-term outcome of surgical revascularization and natural course of HAMD has not been disclosed either.Methods: A headache screening questionnaire in China based on the ICHD2 and a face-to-face interview performed by an experienced neurologist were used to investigate headache characteristics and frequency and pain intensity in the 3 months before admission, and a telephone interview was used for the follow-up of a large cohort of 119 Chinese patients with HAMD.Results: Headache intensity was rated as scores of 5.9 ± 2.0 on a visual analog scale (VAS), ranging from 0 to 10, in the 3 months before admission. Forty-six patients (38.6%) were categorized as having migraine-like headaches, 29 patients (24.3%) were categorized as having tension type-like headaches, and 44 patients (36.9%) had a combination of both. The majority of patients had migraine-like headaches (n = 34, 73.9%) with a migrainous aura. Both the frequency and intensity of the headache improved significantly in patients treated with surgical revascularization (n = 96, 80.7%) or the conservative treatment (n = 23, 19.3%) in a long-term follow-up.Conclusion: HAMD frequently presented with a migraine-like headache (75.5% in total). A tension type headache was present in 60.9% of patients. The symptom of dizziness is common in patients with HAMD (60.5%), and 19 of them (26.4%) met the diagnose of vestibular migraine. Both intensity and frequency of HAMD show a trend of spontaneous remission in a long-term follow-up, and there is no difference in long-term outcomes of HAMD between surgical revascularization and conservative treatment, which indicates that the effect of bypass intervention on HAMD may be a placebo effect.


2021 ◽  
pp. 135245852110100
Author(s):  
Zoë YGJ van Lierop ◽  
Luuk Wieske ◽  
Marleen JA Koel-Simmelink ◽  
Madhurima Chatterjee ◽  
Iris Dekker ◽  
...  

Background: Natalizumab treatment provides a model for non-inflammation-induced disease progression in multiple sclerosis (MS). Objective: To study serum contactin-1 (sCNTN1) as a novel biomarker for disease progression in natalizumab-treated relapsing-remitting MS (RRMS) patients. Methods: Eighty-nine natalizumab-treated RRMS patients with minimum follow-up of 3 years were included. sCNTN1 was analyzed at baseline (before natalizumab initiation), 3, 12, 24 months (M) and last follow-up (median 5.2 years) and compared to 222 healthy controls (HC) and 15 primary progressive MS patients (PPMS). Results were compared between patients with progressive, stable, or improved disability according to EDSS-plus criteria. Results: Median sCNTN1 levels (ng/mL,) in RRMS (baseline: 10.7, 3M: 9.7, 12M: 10.4, 24M: 10.8; last follow-up: 9.7) were significantly lower compared to HC (12.5; p ⩽ 0.001). It was observed that 48% of patients showed progression during follow-up, 11% improved, and 40% remained stable. sCNTN1 levels were significantly lower in progressors both at baseline and at 12M compared to non-progressors. A 1 ng/mL decrease in baseline sCNTN1 was consistent with an odds ratio of 1.23 (95% confidence interval 1.04–1.45) ( p = 0.017) for progression during follow-up. Conclusion: Lower baseline sCNTN1 concentrations were associated with long-term disability progression during natalizumab treatment, making it a possible blood-based prognostic biomarker for RRMS.


2019 ◽  
Vol 23 (5) ◽  
pp. 586-592 ◽  
Author(s):  
Coleman P. Riordan ◽  
Armide Storey ◽  
David J. Cote ◽  
Edward R. Smith ◽  
R. Michael Scott

OBJECTIVEThere are limited data on the long-term outcomes for children undergoing surgical revascularization for moyamoya disease (MMD) in North America. The authors present a series of pediatric MMD patients who underwent a standard revascularization procedure, pial synangiosis, more than 20 years previously at a single institution by a single surgical team.METHODSThis study is a retrospective review of all patients aged 21 years or younger treated for MMD at Boston Children’s Hospital who were operated on more than 20 years previously by the senior author (R.M.S.). Radiographic and operative reports, outpatient clinical records, and communications with patients and families were reviewed to document current clinical status, ability to perform daily activities, and concurrent or new medical conditions.RESULTSA total of 59 patients (38 female [64.4%], 21 male [35.6%]; median age at surgery 6.2 years [IQR 0.5–21 years]) were identified who were diagnosed with MMD and underwent surgical revascularization procedures more than 20 years previously. Clinically, all but 2 patients (96.6%) presented with the following symptoms alone or in combination: 43 (73%) presented with stroke, 22 (37%) with transient ischemic attack, 12 (20%) with seizures, 7 (12%) with headache, 3 (5%) with choreiform movements, and 2 (3%) with hemorrhage; MMD was incidentally detected in 2 patients (3%). Five patients had unilateral MMD at presentation, but 3 of these ultimately progressed to develop bilateral MMD after an average of 16 months; therefore, pial synangiosis was ultimately performed in a total of 116 hemispheres during the study period. Clinical follow-up was available at a median interval of 20.6 years (IQR 16.1–23.2 years). Modified Rankin Scale scores were stable or improved in 43 of 50 patients with evaluable data; 45 of 55 are currently independent. There were 6 patient deaths (10.2%; 3 due to intracranial hemorrhage, 2 due to tumor-related complications, and 1 due to pulmonary artery stenosis), 4 of whom had a history of previous cranial radiation. One patient (1.7%) experienced a late stroke. Synangiosis vessels remained patent on all available late MRI and MRA studies. Four patients reported uneventful pregnancies and vaginal deliveries years following their revascularization procedures.CONCLUSIONSRevascularization for MMD by pial synangiosis appears to confer protection from stroke for pediatric patients over long-term follow-up. A history of cranial radiation was present in 4 of the 6 patients who died and in the lone patient with late stroke. Most patients can expect productive, independent lives following revascularization surgery in the absence of significant preoperative neurological deficits and comorbidities.


2021 ◽  
pp. 1-7
Author(s):  
Constantin Roder ◽  
Uwe Klose ◽  
Helene Hurth ◽  
Cornelia Brendle ◽  
Marcos Tatagiba ◽  
...  

<b><i>Background and Purpose:</i></b> Hemodynamic evaluation of moyamoya patients is crucial to decide the treatment strategy. Recently, CO<sub>2</sub>-triggered BOLD MRI has been shown to be a promising tool for the hemodynamic evaluation of moyamoya patients. However, the longitudinal reliability of this technique in follow-up examinations is unknown. This study aims to analyze longitudinal follow-up data of CO<sub>2</sub>-triggered BOLD MRI to prove the reliability of this technique for long-term control examinations in moyamoya patients. <b><i>Methods:</i></b> Longitudinal CO<sub>2</sub> BOLD MRI follow-up examinations of moyamoya patients with and without surgical revascularization have been analyzed for all 6 vascular territories retrospectively. If revascularization was performed, any directly (by the disease or the bypass) or indirectly (due to change of collateral flow after revascularization) affected territory was excluded based on angiography findings (group 1). In patients without surgical revascularization between the MRI examinations, all territories were analyzed (group 2). <b><i>Results:</i></b> Eighteen moyamoya patients with 39 CO<sub>2</sub> BOLD MRI examinations fulfilled the inclusion criteria. The median follow-up between the 2 examinations was 12 months (range 4–29 months). For 106 vascular territories analyzed in group 1, the intraclass correlation coefficient was 0.784, <i>p</i> &#x3c; 0.001, and for group 2 (84 territories), it was 0.899, <i>p</i> &#x3c; 0.001. Within the total follow-up duration of 140 patient months, none of the patients experienced a new stroke. <b><i>Conclusions:</i></b> CO<sub>2</sub> BOLD MRI is a promising tool for mid- and long-term follow-up examinations of cerebral hemodynamics in moyamoya patients. Systematic prospective evaluation is required prior to making it a routine examination.


Lupus ◽  
2019 ◽  
Vol 29 (1) ◽  
pp. 83-91
Author(s):  
G Vajgel ◽  
C B L Oliveira ◽  
D M N Costa ◽  
M A G M Cavalcante ◽  
L M Valente ◽  
...  

Objective We analyzed baseline and follow-up characteristics related to poorer renal outcomes in a Brazilian cohort of admixture race patients with lupus nephritis. Methods Overall, 280 outpatients with a diagnosis of systemic lupus erythematosus and previous kidney biopsy of lupus nephritis were recruited from August 2015 to December 2018 and had baseline laboratory and histologic data retrospectively analyzed; patients were then followed-up and data were recorded. The main outcome measure was the estimated glomerular filtration rate at last follow-up. Secondary analyses assessed the impact of initial kidney histology and treatment in long-term kidney survival. Results Median duration of lupus nephritis was 60 months (interquartile range: 27–120); 40 (14.3%) patients presented progressive chronic kidney disease (estimated glomerular filtration rate <30 and ≥10 ml/min/1.73 m2) or end-stage kidney disease at last visit. Adjusted logistic regression analysis showed that class IV lupus nephritis (odds ratio 14.91; 95% confidence interval 1.77–125.99; p = 0.01) and interstitial fibrosis ≥25% at initial biopsy (odds ratio 5.87; 95% confidence interval 1.32–26.16; p = 0.02), lack of complete or partial response at 12 months (odds ratio 16.3; 95% confidence interval 3.74–71.43; p < 0.001), and a second renal flare (odds ratio 4.49; 95% confidence interval 1.10–18.44; p = 0.04) were predictors of progressive chronic kidney disease. In a Kaplan-Meier survival curve we found that class IV lupus nephritis and interstitial fibrosis ≥25% were significantly associated with end-stage kidney disease throughout follow-up (hazard ratio 2.96; 95% confidence interval 1.3–7.0; p = 0.036 and hazard ratio 4.96; 95% confidence interval 1.9–12.9; p < 0.0001, respectively). Conclusion In this large cohort of admixture race patients, class IV lupus nephritis and chronic interstitial damage at initial renal biopsy together with non-response after 1 year of therapy and relapse were associated with worse long-term renal outcomes.


2021 ◽  
Vol 39 (3_suppl) ◽  
pp. 207-207
Author(s):  
Ken Kato ◽  
Yuichiro Doki ◽  
Takashi Ura ◽  
Yasuo Hamamoto ◽  
Takashi Kojima ◽  
...  

207 Background:ATTRACTION-1/ONO-4538-07 (AT-1), an open-label, single-arm, multicenter phase 2 clinical trial conducted in Japan, evaluated the clinical activity and safety of nivolumab in patients with advanced esophageal squamous cell carcinoma (ESCC) refractory/intolerant to fluoropyrimidine-, platinum-, and taxane-based chemotherapy. We previously reported the 2-year follow-up findings of AT-1, in which nivolumab demonstrated antitumor activity with a manageable safety profile for these patients. Here we report the final findings from AT-1 at a minimum follow-up of 5 years. Methods:Patients aged ≥20 years with an Eastern Cooperative Oncology Group Performance Status (ECOG PS) of 0-1 received 3 mg/kg nivolumab intravenously every 2 weeks in 6-week cycles until disease progression or unacceptable toxicity. The primary endpoint was centrally-assessed objective response rate (ORR), defined as the proportion of patients whose best overall response was either a complete or partial response. Secondary endpoints included overall survival (OS), investigator-assessed ORR, progression-free survival (PFS), change in tumor burden, time to response, time to disease progression, and duration of response. Results:Between February 25 and November 14, 2014, a total of 65 patients were enrolled. Sixty-four patients were evaluated for the efficacy, and all patients were evaluated for the safety. At the final database lock on August 6, 2020, 11 (17.2%, 95% confidence interval [CI] 9.9-28.2) of 64 patients had an objective response by central assessment. The median OS was 10.8 months (95% CI, 7.4-13.9), and the estimated 5-year OS rate was 6.3% (95% CI, 2.0-14.0). The median PFS was 1.5 months (95% CI, 1.4-2.8), and the estimated 5-year PFS rate was 6.8% (95% CI, 2.2-15.1). Treatment-related adverse events that occurred with a frequency of > 10% were diarrhea and rash. The presentation will include characteristics of long-term survivors as well as detailed efficacy and safety data of nivolumab. Conclusions:This final assessment represents the longest follow-up of patients with advanced ESCC treated with nivolumab. Nivolumab demonstrated continued long-term efficacy in these patients based on a minimum of 5-year long-term survival update of AT-1. Furthermore, no new safety signals with nivolumab were identified during long-term follow-up. These findings are consistent with those of nivolumab monotherapy for various types of cancer. Clinical trial information: No.142422.


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.


2020 ◽  
Author(s):  
Lixia Xia ◽  
Zhongfei Hao ◽  
Hongjun Wang ◽  
Yan Feng ◽  
Yongxuan Zhan ◽  
...  

Abstract Background: Ruptured tiny intracranial aneurysm (RTIA) is particularly rare seen. Treatment of RTIA is more difficult and not under control.Methods: We hereby present twelve rare cases of RTIA that were diagnosed based on DSA. These patients were found due to spontaneous or traumatic SAH, 11 of whom were treated only by electrocoagulation, and one of whom was treated by stent-assisted coiling and electrocoagulation.Results: There were eight patients with aneurysms located in posterior circulation and four patients with aneurysms located in anterior circulation. 11 patients were cured only by electrocoagulation merely: ten patient’ RTIA disappeared completely and immediately and another patient’s RTIA was thrombosed two months later. The twelfth patient was carried out stent-assisted coiling and electrocoagulation. The twelve patients’ average follow-up time was 12.5 months and the outcomes were all surprisingly excellent. No revascularization of aneurysm was found. Conclusions: Electrocoagulation with guidewire manipulation is a suitable method to treat RTIA. While large-scale studies with long-term follow-up are required to validate these promising results.


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