- Department of Neurosurgery, S.C.B. Medical College and Hospital, Cuttack, Odisha, India
Department of Neurosurgery, S.C.B. Medical College and Hospital, Cuttack, Odisha, India
DOI:10.4103/2152-7806.107891Copyright: © 2013 Panigrahi S This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Panigrahi S, Mishra SS, Das S, Parida DK. Intracerebral pial arteriovenous fistula with large venous varix: A rare case report. Surg Neurol Int 27-Feb-2013;4:22
How to cite this URL: Panigrahi S, Mishra SS, Das S, Parida DK. Intracerebral pial arteriovenous fistula with large venous varix: A rare case report. Surg Neurol Int 27-Feb-2013;4:22. Available from: http://sni.wpengine.com/surgicalint_articles/intracerebral-pial-arteriovenous-fistula-with-large-venous-varix-a-rare-case-report/
Background:Intracerebral pial arteriovenous fistulas (AVFs) are rare vascular lesions of the brain. These lesions are composed of one or more direct arterial connection to a single venous channel without true intervening nidus and usually have associated venous varix or giant venous aneurysms. Intracerebral varices are occasionally associated with high-flow AVF, and usually treated by interrupting the feeding arteries leaving the varices intact.
Case Description:We report a rare case of a 24-year-old male with a single-channel pial AVF of the left cerebral hemisphere, which was fed by the left anterior cerebral artery (ACA) and was associated with large venous varix and continuous varicose venous dilatation. This superficially located varix was over 6 cm in diameter posing significant mass effect and had calcified walls. Direct surgical flow disconnection followed by removal of large varix resulted in complete disappearance of pial AVF without complication.
Conclusion:Though endovascular occlusion of feeding arteries offers a simple and safe option, direct surgical removal should be considered in rare cases of intracerebral superficially located large AVF with calcified wall and mass effect.
Keywords: Direct surgical removal, Intracerebral pial arteriovenous fistulas, large venous varix, left anterior cerebral artery
Intracerebral pial arteriovenous fistulas (AVFs) are rare vascular lesions of the brain and account for 1.6% of all brain vascular malformations.[
Here we report a rare case of intracerebral giant venous varix with calcified walls and mass effect, secondary to a single-channel pial AVF. Because of the superficial location, mass effect and thick calcified wall, direct surgical removal of the AVF mass was performed and the varix removed without complication.
A 24-year-old right-handed male, without history of head trauma or alcohol abuse, was admitted to our department with 6-year history of repeated episodes of generalized tonic-clonic seizure. In spite of anticonvulsant therapy, the attack became more frequent. There was no history suggestive of a prior subarachnoid hemorrhage or transient ischemic events. The patient had no mucocutaneous telangiectasia or episodes of recurrent epistaxis. None of his family members including siblings and parents suffered from such type of illness. On examination, he was alert and behaving normally with no focal neurological deficit. There were no signs of meningism. General physical examination showed normal results. Routine laboratory tests were within normal limits and inter-ictal electroencephalogram revealed no abnormality.
On computed tomography (CT) scan, a large well circumscribed round smooth walled strong and uniformly enhancing hyper dense space occupying lesion with peripheral wall calcification was found in left frontal lobe [
Computed tomography scan of brain showing a left frontal large well circumscribed round smooth walled strong and uniformly enhancing hyper dense space occupying lesion with peripheral wall calcification. The lesion demonstrates no obvious perilesional edema with significant mass effect with midline shift
(a) T2 axial, (b) T2 coronal, (c) T1 sagittal and, (d) MR angiogram sequence of cerebral magnetic resonance imaging reveals a left frontal well defined flow void of size 60 × 66 mm communicating with the superior sagittal sinus through dilated cortical vein with feeder supply from left anterior cerebral artery
128 Multi slice cerebral computed tomographic angiography sagittal (a) and coronal (b) view demonstrates a large left frontal arteriovenous fistula supplied by the distal left anterior cerebral artery draining through a dilated cortical vein into the superior sagittal sinus. A large variceal dilatation of the proximal venous end measuring nearly 6.5 cm in diameter with wall calcification is shown in association with the arteriovenous fistula
The patient underwent a left frontal craniotomy crossing midline. On reflecting the dura to opposite side, a globular soft pinkish pulsatile varix (≈ 6 × 6 cm) was encountered superficially in left frontal lobe [Figure
Postoperative contrast enhancing cerebral computed tomographic axial (a) and sagittal, (b) picture demonstrates a large filling defect in the area of the varix excision. Computed tomographic angiography (c and d) shows complete obliteration of the arteriovenous fistulas and reconstitution of the distal anterior cerebral artery
Intracranial pial single-channel AVFs are rare cerebrovascular lesions, accounting for 1.6% of all lesions.[
Pial AVFs can result from trauma or may be congenital.[
Pial AVFs come to clinical attention with seizures, hemorrhage, headache, focal neurological deficit, symptoms of increased intracranial pressure, and intracranial bruit.[
Pial AVFs can be diagnosed with cerebral CTA, especially 3D angiograms. Three dimensional angiograms can delineate complex angioarchitecture well because of its inherent capability of obtaining reconstruction images at any angles. In this case, the feeder from the left ACA was visualized clearly on 128 multi slice CTA.
Treatment strategies of pial AVFs are different than that of cerebral AVMs. Simple disconnection of arteriovenous shunting is considered enough in most cases, either by microsurgery or endovascular embolization without resection of entire vascular malformation.[
In our case, single channel pial AVF fed directly by left ACA with associated large venus varix was demonstrated in CTA. Because of superficial location, significant mass effect and thick calcified wall, direct surgical removal of the AVF mass was performed after disconnection of single feeder from large venous varix, which was the most proximal part of dilated cortical vein [
Intracerebral pial AVFs with large venous varix are rare vascular malformations that can be successfully managed surgically with good outcome. Although endovascular occlusion of feeding arteries offers a simple and safe option, direct surgical removal should be considered in rare cases of superficially located intracerebral large pial AVF with calcified wall and mass effect.
1. Halbach VV, Higashida RT, Hieshima GB, Hardin CW, Dowd CF, Barnwell SL. Transarterial occlusion of solitary intracerebral arteriovenous fistulas. AJNR Am J Neuroradiol. 1989. 10: 747-52
2. Hoh BL, Putman CM, Budzik RF, Ogilvy CS. Surgical and endovascular flow disconnection of intracranial pial single-channel arteriovenous fistulae. Neurosurgery. 2001. 49: 1351-64
3. Lasjaunias P, Manelfe C, Chiu M. Angiographic architecture of intracranial vascular malformations and fistulas-pretherapeutic aspects. Neurosurg Rev. 1986. 9: 253-63
4. Lawton MT, Jacobowitz R, Spetzler RF. Redefined role of angiogenesis in the pathogenesis of dural arteriovenous malformations. J Neurosurg. 1997. 87: 267-74
5. Lee JY, Son YJ, Kim JE. Intracranial pial arteriovenous fistulas. J Korean Neurosurg Soc. 2008. 44: 101-4
6. Limaye US, Siddhartha W, Shrivastav M, Anand S, Ghatge S. Endovascular management of intracranial pial arterio-venous fistulas. Neurol India. 2004. 52: 87-90
7. Lv X, Li Y, Jiang C, Wu Z. Endovascular treatment of brain arteriovenous fistulas. AJNR Am J Neuroradiol. 2009. 30: 851-6
8. Santosh C, Teasdale E, Molyneux A. Spontaneous closure of an intracranial middle cerebral arteriovenous fistula. Neuroradiology. 1991. 33: 65-6
9. Viñuela F, Drake CG, Fox AJ, Pelz DM. Giant intracranial varices secondary to high-flow arteriovenous fistulae. J Neurosurg. 1987. 66: 198-203