Moyamoya disease concurrent with dural arteriovenous fistula: A case report and literature review

Abstract

Moyamoya disease (MMD) and dural arteriovenous fistula (DAVF) are two distinct types of intracranial lesion that share different pathogenic mechanisms. Under rare circumstances, patients with MMD have been reported to have concurrent DAVF. The present case study reports on a 47-year-old male admitted due to sudden headache. Head CT revealed hemorrhage of the right thalamus with ventricular extension. On CT angiography, the normal vasculature in the anterior and posterior circulation disappeared and was replaced by moyamoya-like vessels. The patient received conservative management and was discharged 3 days later. After three months, the patient was readmitted for acute cerebellar hemisphere infarction. Angiogram indicated that the DAVF (Cognard classification Ⅰ) was supplied by the left middle meningeal artery, occipital artery and posterior meningeal artery and drained into the transverse-sigmoid sinus and occipital sinus. Conservative management of the DAVF was adopted. The patient was stable and lived independently during a 4-year follow-up. A literature review of the reported cases was also performed to further characterize this rare entity. The management of DAVF concurrent with MMD depends on its clinical presentation and invasiveness. For patients with symptoms or cortical venous drainage, endovascular intervention should be performed. For asymptomatic DAVFs or those without cerebral venous drainage, close follow-up is a reasonable option.

An Uncommon Case of Moyamoya Syndrome Is Accompanied by an Arteriovenous Malformation with the Involvement of Dural Arteries

“…The prevalence of AVM with involvement of dural arteries linked with MMD is uncertain, with only two previously reported cases of concomitant MMD and dAVF and only two patients characterized as having concurrent MMS and dAVF [ 5 , 9 ]. According to the research, angiogenesis is the mechanism of de novo AVM formation in MMD patients, particularly after stereotactic radiosurgery [ 9 , 11 , 12 ]. However, in this particular case, the patient had no prior history of radiosurgery, which makes it unique.…”

“…Moyamoya disease (MMD) is believed to be caused by a combination of angiogenic factors and hemodynamic stress that leads to the development of arteriovenous malformations (AVMs) [ 1 , 8 , 9 ]. While there is typically no connection between MMD and dural arteriovenous fistulas (dAVFs), there have been uncommon cases of a unilateral "moyamoya" pattern that is associated with an ipsilateral dAVF [ 10 , 11 ]. However, the occurrence of MMD in conjunction with AVMs involving dural arteries has not been reported in the literature, making treatment of these combined conditions particularly challenging.…”

Recent Advances for Global Perspectives on Etiology, Pathophysiology, Clinical Presentations, and Management of Moyamoya Disease

“…Interestingly, imperial embolization is the best for arteriovenous malformation and arteriovenous fistula patients. Nevertheless, still controversial exists, since there is no justifiable evidence that it is necessary to fully treat arteriovenous malformation (AVM) and arteriovenous fistula (AVF), as it is important to consider that the blood supply to the anterior cerebral artery (ACA) and middle cerebral artery (MCA) might be dependent on the AVM [68].…”