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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 1  |  Page : 195-198

Unilateral moyamoya disease with co-existing arteriovenous malformation

Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Date of Web Publication12-Jan-2017

Correspondence Address:
Dr. Kannepalli N Rao
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.198230

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How to cite this article:
Sadashiva N, Rao KN, Somanna S. Unilateral moyamoya disease with co-existing arteriovenous malformation. Neurol India 2017;65:195-8

How to cite this URL:
Sadashiva N, Rao KN, Somanna S. Unilateral moyamoya disease with co-existing arteriovenous malformation. Neurol India [serial online] 2017 [cited 2020 Jul 4];65:195-8. Available from:


We present the history of a 38-year-old male patient who had four episodes of seizures over the last 15 months. He had no neurological deficits on detailed clinical examination. Computed tomogram (CT) of the head was suggestive of a right postero-frontal arteriovenous malformation (AVM) [Figure 1]a. The angiogram revealed stenosis of the right internal carotid artery (ICA) with multiple moyamoya collateral vessels (MMCV) [Figure 1]b,[Figure 1]c,[Figure 1]d. The AVM drained into the superior sagittal sinus without any drainage into the deep venous system [Figure 1]e,[Figure 1]f,[Figure 1]g. The left ICA, anterior cerebral artery (ACA), and middle cerebral artery (MCA) were filling normally [Figure 1]h. The patient was conservatively managed on antiepileptics and was asymptomatic at a 3-year follow-up.
Figure 1: (a) Computed tomogram of the brain shows right frontal region hyperdensities with serpentine vessels suggestive of arteriovenous malformation. (b and c) The figures show an angiogram with the right internal carotid artery (ICA) injection showing the supraclinoid ICA stenosis with basal collaterals. (d and e) The figure shows an arteriovenous malformation fed by the middle cerebral and anterior cerebral arteries. (f) The figure shows a lateral view showing AVM with draining veins. (g) The figure shows an anteroposterior view, AVM with draining vein draining to the superior sagittal sinus. (h) The figure shows the left ICA injection showing the AVM fed by the right ACA which is filling from the left ICA

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The co-existence of moyamoya disease (MMD) with AVM is very rare and 26 such cases have been reported.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21] Some suggest that MMD may progress due to angiogenic factors released by sequestered platelets in the AVM. The progressive vascular occlusions may occur due to stress of increased blood flow causing intimal hyperplasia leading to stenosis.[21] It is also proposed that in MMD, the perforating vessels and end capillaries dilate due to ischemia but the capillary linkage is inadequate to reach the cortex, hence the increased blood flow is diverted into the normal draining veins. As a result, these veins become dilated, mimicking an AVM.[22] A report suggesting AVM as being secondary to MMD demonstrated the development of AVM in a patient with MMD after 8 years.[19] Both arguments exist where AVM is implicated as the causative factor of MMD, and vice versa.

We had previously published the largest Indian series of operated MMD cases from our institute.[23] To the best of our knowledge, the present case is only the seventh case in literature having an AVM with unilateral MMD.[21] Of the 26 cases of MMD accompanied by an AVM reported, 18 presented with cerebral ischemia, whereas six had bleed and two had headache [Table 1].[21]
Table 1: Summary of cases reported with an arteriovenous malformation (AVM) accompanied by moyamoya disease (MMD)

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The principles of managing AVM with MMD are in direct conflict and comprise the most interesting aspect of this condition. Revascularization procedures may lead to the enlargement of the AVM nidus due to recruitment of new collaterals.[19] The AVM is clinically silent in most cases, and hence can be managed conservatively.[22] Radiosurgery has been used to treat AVMs in MMD, but the latent period for the resolution of AVM exposes the patient to further ischemic insults due to progression of MMD. This is especially true when the AVM is fed by MMCV. AVM resection alone has been done in three cases, and 3 patients have undergone AVM resection along with a revascularization procedure.[21] Craniotomy for treatment of AVM may, however, interrupt the MMCVs, exacerbating ischemia. Normal-perfusion pressure breakthrough, seen in AVM surgery, may be problematic in the presence of friable MMCVs. Thus, the timing, ideal treatment modality, and the pathology that needs to be dealt first, is a matter of debate.

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Conflicts of interest

There are no conflicts of interest.

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