Coexistence of moyamoya syndrome with arteriovenous malformation. Systematic review and illustrative case report.

The coexistence of Moyamoya Syndrome with Arteriovenous Malformation is exceedingly rare. Here, we present the case of a 37-year-old female patient diagnosed with AVM in the right parietal lobe, accompanied by severe stenosis of the right middle cerebral artery and right anterior cerebral artery, along with moyamoya collateral induction. Our objective was to investigate the frequency and mutual influence of these conditions, and to determine a preferable treatment strategy by conducting a comprehensive review of previous case reports. We conducted a thorough search of PubMed, Scopus, and Web of Science databases, adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Our review encompassed 36 publications, reporting a total of 64 cases of AVM coexisting with Moyamoya Syndrome. Notably, bilateral stenosis was observed in half of the cases. Among patients with unilateral stenosis, AVM was localized in the opposite hemisphere only in 2 cases. Treatment approaches varied, with 18 cases undergoing active treatment for both stenosis and AVM, 11 cases treating stenosis prior to AVM, 5 cases addressing AVM first, and 3 cases treating both AVM and stenosis simultaneously. Embolization, either standalone or supplemented by stereotactic radiosurgery, was employed in six cases. Stereotactic radiosurgery alone was utilized in 12 cases, while 15 patients underwent surgical removal of the AVM. Our findings provide valuable insights for neurosurgeons managing patients with concurrent AVM and Moyamoya Syndrome. The variety of treatment approaches observed in the literature underscores the complexity of these cases, emphasizing the need for individualized strategies. This information may guide future systematic reviews and meta-analyses, contributing to a better understanding of the optimal management of these rare coexisting vascular pathologies.

Sporadic multiple cerebral arteriovenous malformations: case report and systematic review of additional 80 cases.

INTRODUCTION: In absence of hereditary diseases multiple brain arteriovenous malformations are extremely rare. The case series that would include more than 13 patients are unlikely to be found, which causes an obstacle to comprehensively analyzing the peculiarities of epidemiology, symptoms and treatment options for this disorder. We describe patent with two independent arteriovenous malformations in frontal and parietal lobes that have been treated with combination of preoperative embolization, surgical excision and stereotactic radiosurgery. Systematic review of literature was also performed, focusing on epidemiology of sporadic multiple arteriovenous malformations, niduses location, clinical presentation, treatment and outcomes. EVIDENCE ACQUISITION: We systematically analyzed relevant literature using the PubMed database, encompassing studies in English (published between 1956 and 2023) reporting incidence, epidemiological features, symptomatology and treatment of sporadic multiple brain arteriovenous malformations. EVIDENCE SYNTHESIS: Forty-eight studies with a total of 80 sporadic multiple cerebral arteriovenous malformations were extracted from the literature. Twenty-two papers reported incidence of multiple brain arteriovenous malformations. The average incidence (including our data) was 2.4%, varying significantly between children and adults. Hemorrhage from one AVM was the most frequent debut of multiple cerebral arteriovenous malformations. Surgical removal of niduses remains a valuable treatment option even considering the enhancement of embolization techniques and the development of radiosurgery. CONCLUSIONS: Sporadic multiple cerebral arteriovenous malformations represent a difficult problem to solve. The possibility of persistence of multiple brain arteriovenous malformations should be taken into account when diagnosing and following-up.

Predictive Value of Dynamic Cerebral Autoregulation Assessment in Surgical Management of Patients with High-Grade Carotid Artery Stenosis.

Dynamic cerebral autoregulation (DCA) capacity along with the degree of internal carotid artery (ICA) stenosis and characteristics of the plaque can also play an important role in selection of appropriate treatment strategy. This study aims to classify the patients with severe ICA stenosis according to preoperative state of DCA and to assess its dynamics after surgery. Thirty-five patients with severe ICA stenosis having different clinical type of disease underwent reconstructive surgery. DCA was assessed with transfer function analysis (TFA) by calculating phase shift (PS) between Mayer waves of blood flow velocity (BFV) and blood pressure (BP) before and after operation. In 18 cases, regardless of clinical type, preoperative PS on ipsilateral side was within the normal range and did not change considerably after surgery. In other 17 cases preoperative PS was reliably lower both in patients with symptomatic and asymptomatic stenosis. Surgical reconstruction led to restoration of impaired DCA evidenced by significant increase of PS in postoperative period. Our data suggest that regardless clinical type of disease various state of DCA may be present in patients with severe ICA stenosis. This finding can contribute to establishing the optimal treatment strategy, and first of all for asymptomatic patients. Patients with compromised DCA should be considered as ones with higher risk of stroke and first candidates for reconstructive surgery.