J Korean Neurosurg Soc.  2014 Oct;56(4):356-360. 10.3340/jkns.2014.56.4.356.

Cerebral Arteriovenous Malformation Associated with Moyamoya Disease

• Noh JH
• Yeon JY
• Park JH
• Shin HJ

• KMID: 2018090
• DOI: http://doi.org/10.3340/jkns.2014.56.4.356

Abstract

The coexistence of moyamoya disease (MMD) with an arteriovenous malformation (AVM) is exceedingly rare. We report two cases of AVM associated with MMD. The first case was an incidental AVM diagnosed simultaneously with MMD. This AVM was managed expectantly after encephalo-duro-arterio-synangiosis (EDAS) as the main feeders stemmed from the internal carotid artery, which we believed would be obliterated with the progression of MMD. However, the AVM persisted with replacement of the internal carotid artery feeders by new external carotid artery feeders from the EDAS site. The AVM was eventually treated with gamma knife radiosurgery considering an increasing steal effect. The second case was a de novo AVM case. The patient was initially diagnosed with MMD, and acquired an AVM eight years later that was slowly fed by the reconstituted anterior cerebral artery. Because the patient remained asymptomatic, the AVM is currently being closely followed for more than 2 years without further surgical intervention. Possible differences in the pathogenesis and the radiologic presentation of these AVMs are discussed with a literature review. No solid consensus exists on the optimal treatment of MMD-associated AVMs. Gamma knife radiosurgery appears to be an effective treatment option for an incidental AVM. However, a de novo AVM may be managed expectantly considering the possible risks of damaging established collaterals, low flow characteristics, and probably low risks of rupture.

Keyword

Moyamoya disease; Arteriovenous malformation; Pediatric; Children

MeSH Terms

Anterior Cerebral Artery
Arteriovenous Malformations
Carotid Artery, External
Carotid Artery, Internal
Child
Consensus
Humans
Intracranial Arteriovenous Malformations*
Moyamoya Disease*
Radiosurgery
Rupture

Figure

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  Fig. 1 Case 1 images. A : Initial right ICA angiograms obtained in the late arterial phase, showing a small AVM in the right parietal lobe. Left ICA angiogram shows abnormal vascular networks suggestive of MMD. B : Follow-up angiography performed 4.5 years after EDAS, showing progressive stenosis of the right ICA with decreased flow to the AVM. Right ECA angiogram shows surgically established collaterals at the EDAS site. C : Subsequent angiography performed 9.5 years after EDAS, demonstrating newly-developed AVM feeders from the EDAS site. D : Obliteration of the AVM is strongly indicated by MRI taken 2 years after gamma knife radiosurgery. ICA : internal carotid artery, AVM : arteriovenous malformation, MMD : moyamoya disease, EDAS : encephalo-duro-arterio-synangiosis, ECA : external carotid artery.

• 

  Fig. 2 Case 2 images. A : Initial angiography showing steno-occlusive lesions with abnormal vascular networks in the right hemisphere. The left ICA is relatively unaffected. B : Subsequent follow-up MRI taken 8 years after EDAS reveals abnormal signal void structures at the anterior part of the right cingulate gyrus (arrow). C : Follow-up angiography performed 8 years after EDAS, confirming a newly developed AVM with MMD progression. Anomalous arteriovenous shunting (arrow) is more visible on left ICA angiograms. Note the established collateral channels (arrowhead) to the adjacent frontal lobe. ICA : internal carotid artery, EDAS : encephalo-duro-arterio-synangiosis, AVM : arteriovenous malformation, MMD : moyamoya disease.

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