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Never visited RIGHT TEMPORAL ARTERIO-VENOUS MALFORMATION (AVM) WITH A PIAL ARTERIO-VENOUS FISTULA (AVF)
Contributed by: patricia burrows, Radiologist, Children's Hospital Boston, Massachusetts, USA.
Patient: 4 month 26 day old male
History: This is a 5-month-old male who was the product of a 38 week pregnancy delivered by cesarean section.  On the first day of life he was noted to have high output cardiac failure and was admitted to the NICU. The patient eventually obtained a work-up for his high output failure which included an MRI of his brain which revealed a large right pial arterial venous fistula. A MRI on 7/23/03 revealed secondary atrophy of the right cerebral hemisphere. An angiogram was performed on 7/25/03 which revealed a large shunt between the anomous connection and large right MCA artery and two horizontal cortical veins that were draining into the right transverse sinus. Prior to this, the patient had a history of failure to thrive and the high output cardiac failure.
Images:[small]larger

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Fig. 1: Cerebral CT dated 10/22/2003 Pre-Embo Patient 5 m.o.

Fig. 2: Cerebral Angio/Embo dated 10/22/2003

Fig. 3: Cerebral Angio/Embo dated 10/22/2003

Fig. 4: Angio dated 10/22/2003 Patient 4 m.o.

Fig. 5: Cerebral Angio/Embo dated 10/22/2003

Fig. 6: Cerebral Angio/Embo dated 10/22/2003

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Fig. 7: MRI dated 10/31/2003 Post Embo Patient 5 m.o.

Fig. 8: Angio dated 10/21/2004 Patient 16 m.o.

Fig. 9: Angio dated 10/21/2004 Patient 16 m.o.

Fig. 10: Angio dated 10/21/2004 Patient 16 m.o.

Fig. 11: Angio dated 10/21/2004 Patient 16 m.o.

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Fig. 12: Follow-up Cerebral Angio dated 10/17/2005 Patient 2.5 y.o.
Findings:

CEREBRAL CT - 10/22/2003:

FINDINGS: There are enlarged vessels identified in the region of the Sylvian fissure, which likely represents an AVM. There are scattered areas of punctate calcifications present in the right cerebral hemisphere, within the right frontal white matter. This may represent dystrophic calcifications from chronic ischemia. There is no evidence of acute intracranial hemorrhage. The visualized osseous structures are unremarkable.

IMPRESSION: FINDINGS CONSISTENT WITH AVM IN RIGHT SYLVIAN FISSURE, WITH SCATTERED AREAS OF DYSTROPHIC CALCIFICATION IN RIGHT CEREBRAL HEMISPHERE.



 

CEREBRAL ANGIOGRAPHY AND EMBOLIZATION - 10/22/2003:

IMAGING FINDINGS: Cerebral angiography shows a large fistula between the second portion of the right middle cerebral artery and a varix of the right middle cerebral vein. The varix drains into two veins that communicates with the right transverse sinus and a smaller vein that communicates with the superior sagittal sinus. All of the dural sinuses are patent with no evidence of narrowing and there is an accessory occipital sinus. Initially, there is no visualization of parenchymal branches in the right hemisphere, as all the contrast shunts into the varix. Selective injection into the opercular branch of the right middle cerebral artery shows normal arterial flow and opacification of cortical veins that trained separately into the superior sagittal sinus. Deep veins are not demonstrated on any of the angiograms. The poster lateral branches of the right posterior cerebral artery are dilated and contribute to the entry venous fistula, although they appear to drain into the same varix. Following embolization, there is improved antegrade flow in the right middle cerebral arterial branches. There is still filling of the two limbs of the draining varix and also an additional single parietal sinus. There appear to be a two additional arteriovenous fistulas, one from the supra clinoid segment of the internal carotid artery, and another from the proximal right middle cerebral artery.

CONCLUSION: Extremely large right pial artery venous fistula. Technically successful occlusion of the main fistula with apparent residual flow through two additional shunts.




BRAIN MRI AND MRA - 10/31/2003:


FINDINGS: The corpus callosum is diffusely thin. Multiple flow voids are noted in a suprasellar location and also anterior to the brainstem, representing enlarged vessels. Additional flow voids are noted within the right Sylvian fissure and the adjacent region. There are multiple areas of low signal on the axial T2 and flair sequences that correspond to areas of high signal on the axial T1 sequence, within the lumen of vessels in the right Sylvian fissure. The patient has a history of placement of multiple vascular coils in a large arterial venous malformation in this location, and this finding is consistent with clotted blood within these vessels that have been thrombosed, the coils themselves can best be appreciated on the axial T2 sequence. The post-gadolinium images and the MRA images demonstrate a prominent right MCA, and prominent right PCA with large branches to the vascular malformation in the right frontal lobe. There are multiple small vessels surrounding the area of previous embolization, with collateral vessels noted around the temporal tip and into the sylvian fissure. Contrast enhancement may be appreciated within multiple enlarged vessels within this region, indicating residual flow to this malformation. There are multiple prominent dural vessels, and dural enhancement adjacent to the vascular deformity. There is T1 shortening mostly in the white matter adjacent to the vascular lesion, and also more superiorly in the centrum semi ovale, consistent with mineralization, as also seen on the CT, secondary to chronic ischemia. There is volume loss of the adjacent right cerebral brain parenchyma.

IMPRESSION: Evidence of residual multiple collateral vessels, from both MCA and PCA origin, enhancing within the right Sylvian fissure and adjacent regions, S/P coil embolization.



CEREBRAL ANGIOGRAPHY - 10/21/04:

CLINICAL HISTORY: Right temporal AVM status post resection.
FINDINGS: Injection of the right internal carotid artery shows interval removal of the endovascular coils. There are new surgical clips present. There is no obvious arteriovenous shunting in the region of the previously seen AVM. There is a parenchymal filling defect associated with the surgical bed. Again demonstrated is marked tortuosity of the superficial cortical veins. This is similar to the prior exam.

Injection of the vertebral arteries shows a prominent midline meningeal branch originating from the left vertebral artery. However, there is no arteriovenous shunting apparent on the current exam. The dural venous sinuses posteriorly appear patent although the transverse sinuses are somewhat narrowed especially on the right.

Injection of the left internal carotid artery shows a normal arterial phase apart from the branch occlusion noted above. There is a parenchymal filling defect distal to the area of occlusion, however, there is also opacification of this region on delayed images through leptomeningeal collaterals. Again demonstrated are extremely tortuous superficial cortical veins throughout the left cerebral hemisphere.

CONCLUSION:

1. Interval resection of right temporal arteriovenous malformation with no appreciable arteriovenous shunting seen on the present exam.

2. Postoperative changes in the right temporal lobe region.

3. Prominent meningeal branch originating from the left vertebral artery without evidence of arteriovenous shunting.

4. Abnormal cortical veins throughout both cerebral hemispheres as on the prior exam. This is of uncertain etiology. but, in conjunction with narrowing of the transverse sinuses, could be related to venous hypertension.





CEREBRAL ANGIOGRAPHY - 10/17/05:


FINDINGS: Comparison is made to the prior study from 10/21/04. Injection of the right internal carotid artery shows surgical clips present, stable in appearance since 10/21/04. There is no obvious arteriovenous shunting in the region of the previously seen AVM. There is a parenchymal filling defect associated with the surgical bed. Again demonstrated is marked tortuosity of the superficial cortical veins. This is similar to the prior exam. Injection of the left vertebral artery shows a prominent midline meningeal branch originating from the left vertebral artery. This branch is less prominent on today's exam with no apparent arteriovenous shunting. Tortuous superficial draining veins are again demonstrated. The dural venous sinuses posteriorly appear patent although the transverse sinuses are somewhat narrowed especially on

the right.

CONCLUSION:

1. Interval resection of right temporal arteriovenous malformation with no appreciable arteriovenous shunting seen on the present exam.

2. Postoperative changes in the right temporal lobe region.

3. Decreased prominence of the meningeal branch originating from the left vertebral artery without evidence of arteriovenous shunting.

4. Abnormal cortical veins throughout both cerebral hemispheres as on the prior exam. This is of uncertain etiology. but, in conjunction with narrowing of the transverse sinuses, could be related to venous hypertension.

Diagnosis: Congenital right sylvian fissure/temporal arteriovenous malformation (AVM) with pial arteriovenous fistula (AVF).

The patient was treated with embolization X2 to embolize feeders to the AVM originating from the MCA. Flow continued to be seen post embo so the patient underwent a subtotal resection (11/03/03) and a second procedure to completely resect the AVM (10/19/04) when flow voids were still appreciable and spontaneous thrombosis (despite large amounts of embolitic material) failed to occur.  Follow-up angio showed no residual AV shunting.
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Additional Details:

Case Number: 7736261Last Updated: 03-15-2007
Anatomy: Cranium and Contents   Pathology: Vascular
Modality: CT, MR, AngiographyAccess Level: Readable by all users
Keywords: chf

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