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

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.

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.

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.

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.