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.