Onyx embolization of a thoracolumbar perimedullary spinal arteriovenous fistula in an infant presenting with subarachnoid and intraventricular hemorrhage

Case report

Restricted access

Identifying a source of spontaneous subarachnoid hemorrhage (SAH) or intraventricular hemorrhage (IVH) in patients with negative results on cranial angiographic imaging can be a diagnostic challenge. The authors present the case of a 14-month-old girl who presented with lethargy and spontaneous SAH and IVH, and later became acutely paraplegic. Except for the SAH and IVH, findings on neuroimages of the brain were normal. Magnetic resonance imaging revealed an intramedullary thoracolumbar spinal cord hemorrhage that was found to be associated with arterialized veins intraoperatively. Catheter-based diagnostic angiography identified a spinal perimedullary macroarteriovenous fistula (macro-AVF) that was completely embolized with Onyx, negating the need for further surgical intervention. The authors believe this to be the first reported case of a thoracolumbar perimedullary macro-AVF presenting with SAH and IVH. In addition, descriptions of Onyx embolization of a spinal AVF in the literature are rare, especially in pediatric patients.

Abbreviations used in this paper: AVM = arteriovenous malformation; ICH = intracranial hemorrhage; IVH = intraventricular hemorrhage; macro-AVF = macroarteriovenous fistula; NBCA = N-butyl-2-cyanoacrylate; SAH = subarachnoid hemorrhage.

Article Information

Address correspondence to: Tyler J. Kenning, M.D., Division of Neurosurgery, Albany Medical Center, 47 New Scotland Avenue, MC 10, Albany, New York 12208. email: kennint@mail.amc.edu.

© AANS, except where prohibited by US copyright law.



  • View in gallery

    Admission cranial CT scans obtained without contrast. A: A hemorrhage within the fourth ventricle and an SAH within the basal cisterns are revealed. B: An SAH within the basal cisterns is revealed. C: A hemorrhage within the third ventricle is demonstrated. D: A hemorrhage within the lateral ventricles is seen.

  • View in gallery

    Spinal MR images obtained after onset of acute paraplegia. A: Sagittal T2-weighted MR image showing T11–12 intramedullary acute hemorrhage with edema extending cranially and a significantly distended bladder. B: Axial T2-weighted MR image with hemorrhage filling a large portion of the spinal cord's transverse diameter and multiple vascular flow voids anterior and to the left of the cord. C: Axial T2-weighted MR image of the spine just caudal to the area of hemorrhage, showing large vascular flow voids to the left of the spinal cord.

  • View in gallery

    Catheter angiography studies showing an AVF fed by the lumbosacral artery and draining into a venous varix at the level of L-1. Left: Preembolization study. Right: After embolization with Onyx-18.



Aviv RIShad ATomlinson GNiemann DTeddy PJMolyneux AJ: Cervical dural arteriovenous fistulae manifesting as subarachnoid hemorrhage: report of two cases and literature review. AJNR Am J Neuroradiol 25:8548582004


Bárzó PVörös EBodosi M: Intraventricular hemorrhage as a false localizing sign of a thoracolumbar arteriovenous malformation: case report. Surg Neurol 51:4304341999


Bemporad JASze G: Magnetic resonance imaging of spinal cord vascular malformations with an emphasis on the cervical spine. Neuroimaging Clin N Am 11:1111292001


Cahan LDHigashida RTHalbach VVHieshima GB: Variants of radiculomeningeal vascular malformations of the spine. J Neurosurg 66:3333371987


Corkill RAMitsos APMolyneux AJ: Embolization of spinal intramedullary arteriovenous malformations using the liquid embolic agent, Onyx: a single-center experience in a series of 17 patients. J Neurosurg Spine 7:4784852007


Do HMJensen MECloft HJKallmes DFDion JE: Dural arteriovenous fistula of the cervical spine presenting with subarachnoid hemorrhage. AJNR Am J Neuroradiol 20:3483501999


Ferbert AHubo IBiniek R: Non-traumatic subarachnoid hemorrhage with normal angiogram. Long-term follow-up and CT predictors of complications. J Neurol Sci 107:14181992


Gonzalez LFZabramski JMTabrizi PWallace RCMasand MGSpetzler RF: Spontaneous spinal subarachnoid hemorrhage secondary to spinal aneurysms: diagnosis and treatment paradigm. Neurosurgery 57:112711312005


Hashimoto HIida JShin YHironaka YSakaki T: Spinal dural arteriovenous fistula with perimesencephalic subarachnoid haemorrahge. J Clin Neurosci 7:64662000


Iwanaga HWakai SOchiai CNarita JInoh SNagai M: Ruptured cerebral aneurysms missed by initial angiographic study. Neurosurgery 27:45511990


Kim MSHan DHHan MHOh CW: Posterior fossa hemorrhage caused by dural arteriovenous fistula: case reports. Surg Neurol 59:5125162003


Kinouchi HMizoi KTakahashi ANagamine YKoshu KYoshimoto T: Dural arteriovenous shunts at the craniocervical junction. J Neurosurg 89:7557611998


Koch CGottschalk SGiese A: Dural arteriovenous fistula of the lumbar spine presenting with subarachnoid hemorrhage. J Neurosurg 4 Suppl100:3853912004


Krings TChng SMOzanne AAlvarez HRodesch GLasjaunias PL: Hereditary hemorrhagic telangiectasia in children: endovascular treatment of neurovascular malformations. Neuroradiology 47:9469542005


Lavoie PRaymond JRoy DGuilbert FWeill A: Selective treatment of an anterior spinal artery aneurysm with endosaccular coil therapy. J Neurosurg Spine 6:4604642007


Little ASGarrett MGermain RFarhataziz NAlbuquerque FCMcDougall CG: Evaluation of patients with spontaneous subarachnoid hemorrhage and negative angiography. Neurosurgery 61:113911502007


Morimoto TYoshida SBasugi N: Dural arteriovenous malformation in the cervical spine presenting with subarachnoid hemorrhage: case report. Neurosurgery 31:1181201992


Pinto ANFerro JMCanhao PCampos J: How often is perimesencephalic subarachnoid hemorrhage CT pattern caused by ruptured aneurysms?. Acta Neurochir (Wien) 124:79811993


Rinkel GJWijdicks EFHasan DKienstra GEFranke CLHageman LM: Outcome in patients with subarachnoid hemorrhage and negative angiography according to pattern of haemorrhage on computed tomography. Lancet 338:9649681991


Rodesch GHurth MAlvarez HTadie MLasjaunias P: Classification of spinal cord arteriovenous shunts: proposal for a reappraisal—the Bicêtre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery 51:3743792002


Rodesch GHurth MAlvarez HTadie MLasjaunias P: Spinal cord intradural arteriovenous fistulae: anatomic, clinical, and therapeutic considerations in a series of 32 consecutive patients seen between 1981 and 2000 with emphasis on endovascular therapy. Neurosurgery 57:9739832005


Topcuoglu MAOgilvy CSCarter BSBuonanno FSKoroshetz WJSinghal AB: Subarachnoid hemorrhage without evident cause on initial angiography studies: diagnostic yield of subsequent angiography and other neuroimaging tests. J Neurosurg 98:123512402003


van Gijn JRinkel GJ: Subarachnoid hemorrhage: diagnosis, causes and management. Brain 124:2492782001


Vates GEQuinones-Hinojosa AHalbach VVLawton MT: Conus perimedullary arteriovenous fistula with intracranial drainage: case report. Neurosurgery 49:4574612001


Vermeer SERinkel GJAlgra A: Circadian fluctuations in onset of subarachnoid hemorrhage. New data on aneurismal and perimesencephalic hemorrhage and a systematic review. Stroke 28:8058081997


Warakaulle DRAviv RINiemann DMolyneux AJByrne JVTeddy P: Embolisation of spinal dural arteriovenous fistulae with onyx. Neuroradiology 45:1101122003


Willinsky RTerBrugge KLasjaunias PMontanera W: The variable presentations of craniocervical and cervical dural arteriovenous malformations. Surg Neurol 34:1181231990


Zozulya YPSlin'ko EIAl-Qashqish II: Spinal arteriovenous malformations: new classification and surgical treatment. Neurosurg Focus 20:5E72006




All Time Past Year Past 30 Days
Abstract Views 38 38 19
Full Text Views 78 78 6
PDF Downloads 108 108 7
EPUB Downloads 0 0 0


Google Scholar