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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 1 | Page : 195-198
Unilateral moyamoya disease with co-existing arteriovenous malformation
Nishanth Sadashiva, Kannepalli N Rao, Sampath Somanna
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
|Date of Web Publication||12-Jan-2017|
Kannepalli N Rao
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
Source of Support: None, Conflict of Interest: None
|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
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.,,,,,,,,,,,,,,,,,,,, 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. 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. A report suggesting AVM as being secondary to MMD demonstrated the development of AVM in a patient with MMD after 8 years. 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. To the best of our knowledge, the present case is only the seventh case in literature having an AVM with unilateral MMD. 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].
|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. The AVM is clinically silent in most cases, and hence can be managed conservatively. 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. 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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| » References|| |
Kayama T, Suzuki S, Sakurai Y, Nagayama T, Ogawa A, Yoshimoto T. A case of moyamoya disease accompanied by an arteriovenous malformation. Neurosurgery 1986;18:465-8.
Lichtor T, Mullan S. Arteriovenous malformation in moyamoya syndrome. Report of three cases. J Neurosurg 1987;67:603-8.
Okada T, Kida Y, Kinomoto T, Sakurai T, Kobayashi T. Arteriovenous malformation associated with moyamoya disease - Case report. Neurol Med Chir 1990;30:945-8.
Montanera W, Marotta TR, terBrugge KG, Lasjaunias P, Willinsky R, Wallace MC. Cerebral arteriovenous malformations associated with moyamoya phenomenon. AJNR Am J Neuroradiol 1990;11:1153-6.
Akiyama K, Minakawa T, Tsuji Y, Isayama K. Arteriovenous malformation associated with moyamoya disease: Case report. Surg Neurol 1994;41:468-71.
Fuse T, Takagi T, Fukushima T, Hashimoto N, Yamada K. Arteriovenous malformation associated with moyamoya disease. Childs Nerv Syst 1996;12:404-8.
Schmit BP, Burrows PE, Kuban K, Goumnerova L, Scott RM. Acquired cerebral arteriovenous malformation in a child with moyamoya disease. Case report. J Neurosurg 1996;84:677-80.
Halatsch ME, Rustenbeck HH, Jansen J. Progression of arteriovenous malformation in moyamoya syndrome. Acta Neurochir 1997;139:82-5.
Vörös E, Kiss M, Hankó J, Nagy E. Moyamoya with arterial anomalies: Relevance to pathogenesis. Neuroradiology 1997;39:852-6.
Nakashima T, Nakayama N, Furuichi M, Kokuzawa J, Murakawa T, Sakai N. Arteriovenous malformation in association with moyamoya disease. Report of two cases. Neurosurg Focus 1998;5:e6.
Seol HJ, Kim DG, Oh CW, Han DH. Radiosurgical treatment of a cerebral arteriovenous malformation in a patient with moyamoya disease: Case report. Neurosurgery 2002;51:478-81.
Fassett DR, Schloesser PE, Couldwell WT. Hemorrhage from moyamoya-like vessels associated with a cerebral arteriovenous malformation. Case report. J Neurosurg 2004;101:869-71.
O'Shaughnessy BA, DiPatri AJ Jr, Parkinson RJ, Batjer HH. Development of a de novo
cerebral arteriovenous malformation in a child with sickle cell disease and moyamoya arteriopathy. Case report. J Neurosurg 2005;102:238-43.
Nawawi O, Sinnasamy M, Ramli N. Unilateral moyamoya disease with co-existing arteriovenous malformation. Br J Radiol 2006;79:e12-5.
Somasundaram S, Thamburaj K, Burathoki S, Gupta AK. Moyamoya disease with cerebral arteriovenous malformation presenting as primary subarachnoid hemorrhage. J Neuroimaging 2007;17:251-4.
Chen Z, Zhu G, Feng H, Lin J, Wu N. Giant arteriovenous malformation associated with unilateral moyamoya disease in a child: Case report. Surg Neurol 2007;67:89-92.
Deng ZH, Wang S, Li Z, Zhao JZ. Unilateral moyamoya disease associated with cerebellar arteriovenous malformation: One case report. Chin Med J 2008;121:1145-7.
Fujimura M, Kimura N, Ezura M, Niizuma K, Uenohara H, Tominaga T. Development of a de novo
arteriovenous malformation after bilateral revascularization surgery in a child with moyamoya disease. J Neurosurg Pediatr 2014;13:647-9.
Noh JH, Yeon JY, Park JH, Shin HJ. Cerebral arteriovenous malformation associated with moyamoya disease. J Korean Neurosurg Soc 2014;56:356-60.
Yu J, Yuan Y, Zhang D, Xu K. Moyamoya disease associated with arteriovenous malformation and anterior communicating artery aneurysm: A case report and literature review. Exp Ther Med 2016;12:267-71.
Ahn SH, Choo IS, Kim JH, Kim HW. Arteriovenous malformation with an occlusive feeding artery coexisting with unilateral moyamoya disease. J Clin Neurol 2010;6:216-20.
Wu TC, Guo WY, Wu HM, Chang FC, Shiau CY, Chung WY. The rare association of moyamoya disease and cerebral arteriovenous malformations: A case report. Korean J Radiol 2008;9(Suppl):S65-7.
Sadashiva N, Reddy YV, Arima A, Saini J, Shukla D, Pandey P. Moyamoya disease: Experience with direct and indirect revascularization in 70 patients from a nonendemic region. Neurol India 2016 (64 Suppl):S78-86.