A-60 Case of Moyamoya Syndrome in Haitian Female without Known Risk Factors

Objective Moyamoya is a rare, progressive cerebrovascular disorder characterized by carotid artery stenosis or occlusion. Tiny “moyamoya” blood vessels open at the brain base to compensate, often resulting in large vessel ischemic strokes or from hemorrhage of moyamoya vessels. Peak incidence in adults is 30 to 50 years old and 30% progress bilaterally. 54 year-old right-handed Black Haitian female with 13 years of education presented with new stroke, now diagnosed with bilateral moyamoya syndrome. Premorbid history includes hypertension, Type II Diabetes Mellitus, patent foramen ovale, and malignant phyllodes breast tumor treated by resection and local radiation. Presents with no traditional risk factors for moyamoya (e.g., brain radiation, sickle cell disease, neurofibromatosis). Method Five years previously patient experienced multi-focal embolic MCA strokes from left internal carotid occlusion attributed to hypercoagulability of malignancy, returning to previous activities except driving. Present perfusion CT showed complete occlusion of bilateral cavernous carotid arteries. Diagnostic angiogram showed collateral system via PCA-MCA pial-pial collaterals in parieto-occipital-temporal regions, and leptomeningeal collaterals to ACA territory. She underwent bilateral STA-MCA direct bypass. Results Acute neuropsychological testing conducted in Haitian Creole by native-speaking trainee showed expressive aphasia, orientation to person and situation, ability to follow 1-step commands, moderately impaired naming of familiar objects, reduced sustained attention. Also left hemiparesis, right gaze preference, and left homonymous hemianopsia. Conclusions The present case study delineates development of secondary moyamoya in a Black Haitian woman without known risk factors, resulting in significant cognitive-linguistic deficits consistent with brain findings. Limitations include lack of validated measures in Haitian Creole.

Impact of RNF213 Founder Polymorphism (p.R4810K) on the Postoperative Development of Indirect Pial Synangiosis After Direct/indirect Combined Revascularization Surgery for Adult Moyamoya Disease.

BackgroundDirect superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis combined with indirect pial synangiosis provides favorable surgical collaterals for Moyamoya disease (MMD), especially in adults; however, factors leading to the development of each direct and indirect collateral are not well documented.ObjectiveTo investigate the association between RNF213 founder polymorphism (p.R4810K) and each direct and indirect collateral development.MethodsBy qualitative and quantitative evaluations of direct and indirect surgical collaterals using time-of-flight MR angiography, postoperative development of each type of bypass was evaluated independently into two categories. Multivariate logistic regression analysis was performed to study the contributing factors for the development of each surgical collateral.ResultsExcellent development of postoperative direct and indirect bypass was observed in 65 hemispheres (70%) by qualitative evaluation, which was confirmed by quantitative evaluation. Multivariate logistic regression analysis of excellent indirect bypass development revealed a significant positive correlation with the p.R4810K (odds ratio, OR4.0; 95%-confidence interval, CI 1.2-16), advanced MR angiographic stage (OR9.5; 95%CI 1.7-73), and preoperative middle meningeal artery caliber (OR6.8; 95%CI 1.8-35), but a significant negative correlation was found with the excellent direct bypass development (OR0.17; 95%CI 0.03-0.75). No significant correlation was observed between excellent direct bypass development and the p.R4810K (OR0.95; 95%CI 0.37-2.4).ConclusionExcellent development of indirect collaterals after STA-MCA anastomosis combined with indirect pial synangiosis occurs more frequently in adult MMD with the RNF213 founder polymorphism, suggesting a role of the p.R4810K variant for marked in-growth of indirect collaterals and the utility of preoperative genetic analysis.

Risk factors for postoperative ischemic complications in pediatric moyamoya disease

Background Ischemic events are the most common postoperative complication in bypass surgery for moyamoya disease (MMD), but the risk factors for pediatric MMD remain unclear. The goal of the study was to investigate the risk factors for postoperative ischemic complications in pediatric MMD patients. Methods We retrospectively reviewed a consecutive series of pediatric MMD cases at Beijing Tiantan Hospital, Capital Medical University from June 2010 through June 2019. Preoperative clinical variables and radiographic findings were recorded, and logistic regression analysis was carried out to identify the risk factors for postoperative ischemic events. Results A total of 533 operations in 336 patients were included in this study. Postoperative complications occurred after 51 operations (9.6%), including 40/447 indirect bypass procedures, 9/70 direct bypass procedures, and 2/16 combined bypass procedures. Postoperative ischemic events were the most common complication and occurred in 30 patients after 31 procedures (8.9% per patient; 5.8% per operation), including 26/447 indirect bypass procedures, 4/70 direct bypass procedures, and 1/16 combined bypass procedures, and the incidence of these events did not differ significantly between indirect and non-indirect bypass (5.8% vs 5.8%; p = 0.999). Multivariate logistic regression analyses revealed that older age at operation (OR 1.129, 95% CI 1.011–1.260, p = 0.032) and posterior cerebral artery involvement (OR 2.587, 95% CI 1.030–6.496, p = 0.043) were significantly associated with postoperative ischemic events. Conclusion We speculate that older age at operation and posterior cerebral artery involvement are risk factors for postoperative ischemic events in pediatric MMD patients.

A 1-Donor 2-Recipient Superficial Temporal Artery-Middle Cerebral Artery Bypass for Moyamoya Syndrome: 2-Dimensional Operative Video

We present the case of a 34-yr-old male who suffered repeated ischemic events resulting in right-sided weakness. He was found to have left M1 segment near occlusion on angiography with a large area of uncompensated hypoperfusion. The patient underwent a direct superficial temporal artery-middle cerebral artery (STA-MCA) bypass. Direct bypass in the acute setting of ischemia has been previously described.1-5 Moyamoya ischemic disease can be treated with either direct or indirect surgical revascularization. There have been several techniques developed for direct bypasses in moyamoya ischemic disease. These include the standard 1-donor 1-recipient (1D1R) end-to-side (ES) bypass, the “double-barrel” 2-donor 2-recipient (2D2R) ES bypass, and the more recently developed 1-donor 2-recipient (1D2R)6,7 utilizing both an ES and a side-to-side (SS) bypass with a 1-donor vessel. The case presentation, surgical anatomy, decision-making, operative nuances, and postoperative course and outcome are reviewed. The patient gave verbal consent for participating in the procedure and surgical video.

The history of coronary surgery development

The leading place among cardiovascular diseases in the world – 50% – belongs to the ischaemic heart disease. Coronary bypass grafting is the golden standard for treatment of patients with multivessel ischaemic heart disease. The modern level of coronary surgery makes it possible to perform safe and efficacious direct revascularisation with hospital lethality not exceeding 1-3%. This article presents a review of literature devoted to the history of coronary surgery development including analysis of relevant sources dated 2010-2020 published in PubMed and Google Scholar databases: from first experimental procedures in the beginning of the 20th century and indirect myocardial revascularisation methods to direct bypass grafting of the impaired heart vessels actively developed since early 1960s. The article describes types of grafts applied with description of advantages and disadvantages of each one as well as contemporary methods and conditions for coronary bypass grafting and further prospects in development of ischaemic heart disease surgery.

iStent: Trabecular Micro-Bypass Stent

Within the class of procedures termed minimally invasive glaucoma surgery (MIGS), the iStent Trabecular Micro-Bypass Stent (Glaukos Corporation, San Clemente, CA, USA) is an intraocular implant that resides within Schlemm’s canal and allows for direct bypass of the trabecular meshwork by aqueous fluid. The acceptance of MIGS is growing due to its role in lowering intraocular pressure in mild-to-moderate glaucoma combined with its favorable safety profile. With transscleral glaucoma-filtering surgery, there is an increased potential for significant complications including hypotony, suprachoroidal hemorrhage, and long-term risk of endophthalmitis. By contrast, the iStent minimizes these risks and has demonstrated similarities to cataract surgery in terms of associated complications. Multiple publications have described both the safety and efficacy of the implant. These studies are reviewed in addition to a description of the implantation technique and postoperative management pearls. The second-generation model, the iStent inject, may ease implantation and the use of multiple stents may potentially play a role in more advanced disease.

Direct double bypass using the posterior auricular artery as initial surgery for moyamoya disease: technical note

Surgical treatments for moyamoya disease (MMD) include direct revascularization procedures with proven efficacy, for example, superficial temporal artery (STA) to middle cerebral artery (MCA) bypass, STA to anterior cerebral artery bypass, occipital artery (OA) to MCA bypass, or OA to posterior cerebral artery bypass. In cases with poor development of the parietal branch of the STA, the posterior auricular artery (PAA) is often developed and can be used as the bypass donor artery. In this report, the authors describe double direct bypass performed using only the PAA as the donor in the initial surgery for MMD.In the authors’ institution, MMD is routinely treated with an STA-MCA double bypass. Some patients, however, have poor STA development, and in these cases the PAA is used as the donor artery. The authors report the use of the PAA in the treatment of 4 MMD patients at their institution from 2013 to 2016. In all 4 cases, a double direct bypass was performed, with transposition of the PAA as the donor artery. Good patency was confirmed in all cases via intraoperative indocyanine green angiography and postoperative MRA or cerebral angiography. The mean blood flow measurement during surgery was 58 ml/min. No patients suffered a stroke after revascularization surgery.