Co-Existence of Moyamoya Syndrome With Arteriovenous Malformation. Systematic Review and Illustrative Case Report

The co-existence of moyamoya syndrome with arteriovenous malformation is extremely rare. We describe the case of a 37-year-old female patient suffering from arteriovenous malformation in the right parietal lobe combined with severe stenosis of the right middle cerebral artery, and right anterior cerebral artery, accompanied by moyamoya collateral induction. We aimed to investigate the frequency, mutual influence of the diseases on each other, and preferable treatment strategy by reviewing previous case reports. We searched PubMed, Scopus, and Web of Science databases following preferred reporting items for systematic reviews and meta-analyses guidelines and reviewed 36 publications reporting 64 cases of arteriovenous malformation with moyamoya syndrome. In a half of cases, stenosis was bilateral. Among the patients with unilateral stenosis, AVM was localized in the opposite hemisphere only in 2 cases. In 18 cases, both stenosis and AVM were actively treated; in 11 cases, stenosis was treated prior to AVM; in 5 cases, AVM was treated at first; and in three cases, both AVM and stenosis were treated simultaneously. Embolization was used in six cases and was supplemented by stereotactic radiosurgery in two. Stereotactic radiosurgery alone was used in 12 cases. In 15 patients, the AVM was removed surgically. Our findings may benefit neurosurgeons who treat patients with co-existing arteriovenous malformations and moyamoya syndrome and researchers who perform further systematic reviews and meta-analyses on the topic.