Mahmoud Zahra MD, Ganesh Krishnamurthy, MD ; Anne Marie Cahill, MD, Hamza Shaikh, MD, Storm, Phillip, MD, Robert W. Hurst, M.D.

 None

Illustrate that spinal AVM should be listed among the differential diagnoses for intracranial subarachnoid hemorrhage in children

 13 year female with no significant past medical history who presented with headache and fall.  The patient reported a history of fall with mild head trauma, without loss of consciousness one day prior to admission

 Clinical examination in the emergency department found the patient to be drowsy but easily arousable and following commands.  The cranial nerves were intact.  There was severe weakness and sensation loss bilaterally in the lower extremities, with only toe movement preserved on the left.  There was a sensory level at T5.  Upper extremity examination was normal bilaterally.

Case History - Imaging CT imaging of the brain demonstrated subarachnoid hemorrhage, primarily around the brain stem and foramen magnum with mild hydrocephalus.

The location of subarachnoid hemorrhage involving the posterior fossa and foramen magnum combined with neurologic deficits involving the bilateral lower extremities and T5 sensory level raised concerns for a lesion involving the spinal cord.

MRI of the spine  Intramedullary spinal hemorrhage at T5 surrounded by edema and multiple enlarged vessels.  Extensive spinal subarachnoid hemorrhage was also present.  The appearance was consistent with rupture of a spinal cord AVM.  An enlarged vessel drained superiorly along the ventral aspect of the cord into the posterior fossa, likely representing venous drainage for the AVM  Following the MRI, the patient was noted to have lost all movement in her lower extremities

Spinal angiogram  Intramedullary spinal arteriovenous malformation supplied primarily by bilateral T5 and T6 intersegmental arteries feeding both anterior and posterior spinal artery supply.  The right T6 intersegmental artery was the largest AVM feeder via an anterior spinal artery directly into the AVM with no normal spinal supply visualized.  The enlarged spinal supply to the AVM also harbored 2 fusiform aneurysms adjacent to the intramedullary hemorrhage.

This AVM supply and the aneurysms were embolized with N-BCA with complete occlusion of the aneurysms and portion of the AVM fed by the vessel On follow up the patient is improving with increasing strength and sensation in lower extremities

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 On follow up the patient is improving  Strength and sensation in lower extremities are better  The sensory level is now around T11

The most commonly used classification for spinal arteriovenous shunt lesions is the following system:  Type I: Dural arteriovenous fistula (dAVF)  Type II: Intramedullary AVM  Type III: Metameric or Juvenile AVM  Type IV: Intradural perimedullary AVF  Other spinal vascular lesions include:  Intramedullary cavernous malformations  Spinal vascular tumors: hemangioma, hemangioblastoma, metastatic tumors, aneurysmal bone cyst, osteoblastoma, angiosarcoma, hemangiopericytoma.

 Second most common kind of spinal arterovenous shunt lesion (20-30% of spinal AV shunt lesions  This lesion contains a nidus within the substance of the spinal cord or on cord surface.  Found along length of cord. Conus AVMs are typically large with multiple arterial feeders  The nidus usually has multiple feeding vessels arising from the anterior and posterolateral spinal arteries)  Spinal AVM associated with neurofibromatosis and the Rendu-Osler-Weber, Klippel-Trenaunay-Weber, and Parkes- Weber syndromes

 Aneurysms are present along the feeding arteries in 20–40% of cases and usually the reason of hemorrhage  Presentation: Hemorrhage is the most common manifestation. It is associated with acute neurologic deterioration  Typically the patient is young, as the hemorrhage is more common in children with spinal AVM than adults  Hemorrhage recurrence is common in spinal AVM  Spinal AVM may also produce symptoms in the myelopathy or radiculopathy secondary to venous congestion

 Presentation: Hemorrhage is the most common manifestation. It is associated with acute neurologic deterioration  Typically the patient is young, as the hemorrhage is more common in children with spinal AVM than adults  Hemorrhage recurrence is common in spinal AVM  Spinal AVM may also produce symptoms in the myelopathy or radiculopathy secondary to venous congestion

Radiologic findings:  MRI: very sensitive and specific tool for detection of spinal AVM, detects the nidus, feeding arteries and draining veins  Spinal AVM manifests as focal distension of the cord often with evidence of edema. Frequently surrounded by hypointense signal on T1 and T2 weighted images secondary to hemosiderin. Multiple serpentine flow voids representing feeding and draining vessels  Subarachnoid hemorrhage may be detected on MRI as T1 shortening, though MRI is more sensitive for intracranial subarachnoid hemorrhage

Radiologic findings:  Spinal angiogram: the modality of choice for evaluation and treatment of spinal AVM  Complete spinal angiogram is required for accurate characterization and treatment planning  All feeding arteries and draining vein should be detected  Complete spinal angiogram includes, in addition to imaging of bilateral vertebral and segmental, bilateral external carotid, subclavian, intercostal, lumber and sacral arteries

Treatment: Multidisciplinary management  surgical resection with preoperative embolization  Embolization: Based on anatomy & hemodynamic of lesion I. Pre-operative presentation is important and best results may be obtained if treated prior to neurologic deterioration. II. Significant risk with ASA embolization, but less so with PSA due to collateralization. III. Treatment and following should be part of multidisciplinary plan of management.  Radiosurgery

 Patsalides A, Knopman J, Santillan A, et al. Endovascular Treatment of Spinal Arteriovenous Lesions: Beyond the Dural Fistula. American Journal of Neuroradiology. 2011; 32:  Mark R. Harrigan, M.D, John P. Deveikis, M.D, Agnieszka Anna Ardelt, M.D., Ph.D. Handbook of Cerebrovascular Disease and Neurointerventional Technique  Biondi A, Merland JJ, Hodes JE, Pruvo JP, Reizine D, Aneurysms of spinal arteries associated with intramedullary arteriovenous malformations. I. Angiographic and clinical aspects.  Lv X, Li Y, Yang X, Jiang C, Wu Z, Endovascular embolization for symptomatic perimedullary AVF and Intramedullary AVM: a series and a literature review  Madhugiri VS, Ambekar S, Roopesk Kumar VR, Sasidharan GM, Nanda A, Spinal aneurysms: clinicoradiological features and management paradigms  Jeffery S Ross, M.D,

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