Spinal Arteriovenous Shunts Which are Curable and which Incurable? Yuji Matsumaru, M.D., PhD. 1, Takayuki Hara, M.D. 2, and Akira Matsumur

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1 2 3 1 2 3 Spinal Arteriovenous Shunts Which are Curable and which Incurable? Yuji Matsumaru, M.D., PhD. 1, Takayuki Hara, M.D. 2, and Akira Matsumura, M.D. 3 1 Department of Endovascular Neurosurgery, Toranomon Hospital, 2 Department of Neurosurgery, Toranomon Hospital, 3 Department of Neurosurgery, University of Tsukuba The spinal cord is supplied mainly by an anterior spinal artery with small branches named the sulcal arteries and superficially by the posterior spinal arteries. The spinal cord arterial network has a simple structure with these vessels and connecting vessels such as the vasa corona on the surface of the spinal cord and the arterial basket at the conus medullaris. Spinal angiography under general anesthesia helps to understand this vascular anatomy. Spinal dural arteriovenous fistula AVF presents with spinal cord symptoms due to venous hypertension, and can be treated by embolization or surgery. Spinal cord arteriovenous malformation AVM presents with pain or bleeding. Perimedullary AVF and filum terminale AVM can be treated by embolization or surgery. However, intramedullary AVM is difficult to be cured. AVF at craniocervical junction including perimedullary and dural AVF may present with subarachnoid hemorrhage or spinal cord symptom, and can be treated mainly by surgery. Received August 21, 2012 accepted August 23, 2012 Key words spinal cord, arteriovenous shunt, malformation, fistula, embolization, surgery Jpn J Neurosurg Tokyo 22 44 51, 2013 arteriovenous malformation AVM arteriovenous fistula AVF AVM AVF Table 1 anterior spinal artery ASA posterior spinal artery PSA sulcal artery vasa corona 105 8470 2 2 2 Address reprint requests to Yuji Matsumaru, M.D., PhD., Department of Endovascular Neurosurgery, Toranomon Hospital, 2 2 2 Toranomon, Minato ku, Tokyo 105 8470, Japan 44 22 1 2013 1

Epidural Dural Intra dural Table 1 Spinal arteriovenous shunts Spinal epidural AVF AVM Acquired Spinal dural AVF Acquired Spinal perimedullary AVF Spinal intramedullary AVM Filum terminale AVF AVM Complex lesion Cobb syndrome AVF arteriovenous fistula, AVM arteriovenous malformation VC ASA PSA ASA PSA PSA SA PSA VC VC ASA RMA RPA Fig. 1 Simple figures of the spinal arteries A axial view, B oblique view, C depiction of the conus medullaris. ASA Anterior spinal artery, PSA Posterior spinal artery, SA Sulcal artery, VC Vasa corona, RMA Radiculomedullary artery, RPA Radiculopial artery RPA arterial basket A B C Fig. 1 3 cone beam CT ASA Fig. 2 9 hematomyelia 6 2 PSA arterial basket Fig. 1C Fig. 2 ASA radiculomedullary artery RMA Fig. 1 2 Fig. 2 Spinal angiography AP view Adamkiewicz artery injection reveals the ASA arrows. The ASA joins two PSAs arrow heads at the conus medullaris, which is called arterial basket. ASA ASA vasa corona Jpn J Neurosurg VOL. 22 NO. 1 2013. 1 45

A B A B Fig. 3 Cone bean CT images of an ASA, sagittal image A and axial image B Sulcal arteries toward dorsally in the central sulcas and vasa corona on the surface of the spinal cord are clearly shown. Fig. 4 Superselective injection of the lateral spinal artery A and the subtracted image B It anastomoses with the posterior inferior cerebellar artery in the posterior fossa. A B Fig. 5 Coronal images of an ASA and anterior spinal vein A and posterior spinal vein B with cone bean CT The anterior spinal veins course obliquely for extra dural drainage along with a nerve root A, arrows however, the posterior spinal vein course horizontally B, arrows. Fig. 1 3 ASA 1 1 PSA 6 ASA vasa corona lateral spinal artery Fig. 4 radiculopial artery RPA RMA PSA Vasa corona ASA 2 PSA Fig. 1 3 Fig. 5 Fig. 5B ASA ASA Adamkiewicz artery RMA 1 RMA ASA ASA 2 PSA RPA vasa corona 46 22 1 2013 1

Genesis Table 2 Spinal dural AVF and spinal cord AVM A peak age of onset Symptom Spinal dural AVF Acquired Middle age Paraparesis, sensory disturbance, bladder bowel disturbance Spinal cord AVM Young age Radicular pain or sudden onset paraparesis Course Slowly progressive Stable or recurrence Pathological condition Venous hypertension Mass effect AVF arteriovenous fistula, AVM arteriovenous malformation PSA vasa corona arterial basket ASA A B C D spinal dural arteriovenous fistula SDAVF spinal cord arteriovenous malformation SCAVM Table 2 SDAVF SCAVM SDAVF SCAVM Root sleeve MRI T2 7 Fig. 6A 5 SDAVF 1 Fig. 6 Spinal dural AVF T2 weighted MRI image shows a high signal area in the spinal cord and dilated vessel on the dorsal surface of the spinal cord A. Left lumbar artery injection shows SDAVF with retrograde venous drainage and dilated spinal veins B. Superselective injection shows the shunt and the retrograde drainage vein C, which is occluded with diluted NBCA D. RMA RPA 1 Jpn J Neurosurg VOL. 22 NO. 1 2013. 1 47

Fig. 7 Spinal perimedullary AVF Left lumbar artery injection shows a perimedullary AVF supplied by the PSA A without filling from the ASA B. Superselective injection from the vasa corona shows the fistula C, arrow, which is occluded with diluted NBCA D. A B C D Fig. 6 n buthyl 2 cyanoacrylate NBCA SDAVF 13 1 spinal perimedullary AVF AVF SCAVM PSA vasa corona ASA Fig. 7 1 NBCA ASA PSA vasa corona PSA ASA SDAVF NBCA 2 spinal intramedullary AVM AVM ASA high flow fistula varix 3 filum terminale AVF or AVM SCAVM arterial basket Arterial basket conus medullaris filum terminale Varix AVF SDAVF 48 22 1 2013 1

Fig. 8 Filum terminale AVF The patient presents with progressive paraparesis and bladder bowel disturbance. MRI shows a high signal area in the spinal cord and dilated vessel on the surface of the spinal cord A. Spinal angiography shows the filum terminale AVF being filled by the ASA B and PSA C at the caudal portion beyond the arterial basket, which is occluded with diluted NBCA via the PSA D. A B C D Fig. 8 SAH AVF AVF Fig. 9 3 AVF 4 11 SDAVF AVF AVF 3 AVF SAH 12 AVF AVF AVF AVF NBCA 8 10 AVF AVF 11 Table 3 2000 2011 31 SDAVF 15 73 63 13 7 54 AVF 7 57 42 6 3 50 1 AVF 4 57 2 1 50 2 AVM AVF 3 66 46 2 1 2 1 1 AVM 2 25 SDAVF 1 Jpn J Neurosurg VOL. 22 NO. 1 2013. 1 49

A B C D E F Fig. 9 Craniocervical junction AVF The patient presents with subarachnoid hemorrhage A. The spinal angiographies show a perimedullary AVF at the craniocervical junction with the vasa corona from the ASA B and the lateral spinal artery C. Embolization from the lateral spinal artery with diluted NBCA D occluded the shunt E, F. Table 3 Treatment results of spinal arteriovenous shunts in Toranomon Hospital Cure by embolization Cure by surgery Spinal dural AVF n 15 7 13 54 8 8 100 Perimedullary AVF n 7 3 6 50 1 1 100 Craniocervical junction AVF n 4 1 2 50 2 2 100 Filum terminale AVM AVF n 3 1 2 50 1 1 100 Spinal cord AVM n 2 0 2 0 0 1 0 AVF arteriovenous fistula, AVM arteriovenous malformation, curable rate There are overlapping cases between embolization and surgery. SDAVF SCAVM AVF AVF AVM AVM 50 22 1 2013 1

AVF 1 Dehdashti AR, Da Costa LB, terbrugge KG, Willinsky RA, Tymianski M, Wallace MC Overview of the current role of endovascular and surgical treatment in spinal dural arteriovenous fistulas. Neurosurg Focus 26 E8, 2009. 2 Iwai T, Naito I, Shimaguchi H, Suzuki T, Tomizawa S Angiographic findings and clinical significance of the anterior and posterior spinal arteries in therapeutic parent artery occlusion for vertebral artery aneurysms. Interv Neuroradiol 22 6 299 309, 2000. 3 Kai Y, Hamada J, Morioka M, Yano S, Mizuno T, Kuratsu J Arteriovenous fistulas at the cervicomedullary junction presenting with subarachnoid hemorrhage six case reports with special reference to the angiographic pattern of venous drainage. AJNR Am J Neuroradiol 26 1949 1954, 2005. 4 Kinouchi H, Mizoi K, Takahashi A, Nagamine Y, Koshu K, Yoshimoto T Dural arteriovenous shunts at the craniocervical junction. J Neurosurg 89 755 761, 1998. 5 Klopper HB, Surdell DL, Thorell WE Type spinal dural arteriovenous fistulas historical review and illustrative case. Neurosurg Focus 26 E3, 2009. 6 2011 p.530 7 Krings T, Geibprasert S Spinal dural arteriovenous fistulas. Am J Neuroradiol 30 639 648, 2009. 8 2011 pp.198 200 9 Maillot C Linea splendens. Interv Neuroradiol 2 287, 1996. 10 2011 p.145 11 No Shinkei Geka 4 121 128 2012 12 Ohba S, Onozuka S, Horiguchi T, Kawase T, Yoshida K Perimedullary arteriovenous fistula at the craniocervical junction case report. Neurol Med Chir Tokyo 51 299 301, 2011. 13 Ohta T, Gomi M, Oowaki H, Ishikawa M Chronic venous congestion following embolization of spinal dural arteriovenous fistula. J Neurosurg Spine 9 186 190, 2008. vasa corona arterial basket 22 44 51 2013 Jpn J Neurosurg VOL. 22 NO. 1 2013. 1 51