Vascular Anatomy of the Spinal Cord and Classification of Spinal Arteriovenous Malformations

The arterial and venous anatomy of the spinal cord was described in terms of extrinsic and intrinsic contribution to the cord substance. Based upon anatomical location of an arteriovenous shunt, spinal arteriovenous malformations (AVMs) were classified into intramedullary AVM, perimedullary arteriovenous fistula (AVF) and dural AVF. This simple classification seems practical from the standpoint of endovascular and surgical treatments since it reflects anatomical differences in the shunt location, which is the target in either approach.

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Vascular anatomy, pathophysiology, and classification of vascular malformations of the spinal cord

Seminars in Cerebrovascular Diseases and Stroke ◽

10.1053/scds.2002.127656 ◽

2002 ◽

Vol 2 (3) ◽

pp. 186-200 ◽

Cited By ~ 16

Author(s):

R JAHAN ◽

F VINUELA

Keyword(s):

Spinal Cord ◽

Vascular Malformations ◽

Vascular Anatomy

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Spinal Dural Arteriovenous Fistula Presenting with Myelopathy

Cerebrovascular Neurosurgery ◽

10.1093/med/9780190887728.003.0030 ◽

2019 ◽

pp. 279-286

Author(s):

Vinayak Narayan ◽

Anil Nanda

Keyword(s):

Spinal Cord ◽

Arteriovenous Malformations ◽

Dural Arteriovenous Fistula ◽

Endovascular Embolization ◽

Spinal Dural Arteriovenous Fistula ◽

Arteriovenous Fistulas ◽

Dural Arteriovenous Fistulas ◽

Degenerative Spinal Disease ◽

Spinal Arteriovenous Malformations ◽

Spinal Dural Arteriovenous Fistulas

Abstract: Spinal dural arteriovenous fistulas are a rare cause of congestive myelopathy. Symptoms are insidious in onset and may be confused with degenerative spinal disease. MRI characteristically shows edema of the spinal cord with serpiginous flow voids that follow the surface of the spinal cord. Careful evaluation with spinal angiography is required to ensure accurate diagnosis. Spinal dural arteriovenous fistulas differ from spinal arteriovenous malformations in that most fistulas have only a single fistulous point without a nidus. Spinal dural arteriovenous fistulas may be treated successfully with either surgical resection or endovascular embolization depending on their anatomy. Earlier treatment is associated with better outcomes.

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Spinal arteriovenous malformation in a pediatric patient with a history of congenital syphilis: a case report

BMC Pediatrics ◽

10.1186/s12887-021-02707-y ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Mia J. Bertoli ◽

Kruti Parikh ◽

David Klyde ◽

Catherine A. Mazzola ◽

Shridevi Pandya Shah

Keyword(s):

Spinal Cord ◽

Arteriovenous Malformation ◽

Evoked Potentials ◽

Arteriovenous Malformations ◽

Congenital Syphilis ◽

Lower Extremities ◽

Angiography Suite ◽

Arterial Bleeding ◽

History Of ◽

Spinal Arteriovenous Malformations

Abstract Background Spinal arteriovenous malformations in children are extremely rare and pose great risk for intraoperative hemorrhage. Congenital syphilis sometimes presents with vascular symptoms, however, there is little published on patients with a history of congenital syphilis presenting with spinal arteriovenous malformations. Case presentation A 15-month-old female with a history of congenital syphilis presented with urinary retention, fever, and subacute onset of paraplegia. MRI showed a lesion at T8-L1, angiogram was performed which confirmed the presence of a complex type IVc arteriovenous malformation and fistula from Artery of Adamkiewicz at L1-L2. It also showed peri medullary dilated veins and a pseudoaneurysm that compressed the spinal cord at T8-T10. Somatosensory evoked potentials and motor-evoked potentials were not recordable on the bilateral lower extremities prior to surgery. Once the patient was optimized for surgery, osteoplastic laminotomies from T6-T12 were performed. The dura was opened and the intradural, intramesenchymal hematoma was evacuated. There were two episodes of brisk arterial bleeding with hypotension during resection of the hematoma. The patient was taken to the angiography suite from the OR to successfully coil the large aneurysm. Intraoperative spinal cord monitoring remained undetectable in the bilateral lower extremities. The patient’s paraplegia remained unchanged from preoperative presentation. Conclusion Congenital syphilis may present with vascular changes that might impact surgical approaches and treatment outcomes in patients with spinal arteriovenous malformations. Preparation for massive transfusion and intraoperative monitoring are imperative in ensuring a safe perioperative experience.

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Ruptured Conus Medullaris Arteriovenous Malformation

Cerebrovascular Neurosurgery ◽

10.1093/med/9780190887728.003.0032 ◽

2019 ◽

pp. 297-306

Author(s):

Michaela H. Lee ◽

Brandon D. Liebelt ◽

Peter Nakaji ◽

Robert F. Spetzler

Keyword(s):

Spinal Cord ◽

Arteriovenous Malformation ◽

Sexual Function ◽

Neurological Deficit ◽

Arteriovenous Malformations ◽

Vascular Supply ◽

Conus Medullaris ◽

Proper Diagnosis ◽

Spinal Arteriovenous Malformations ◽

Posterior Spinal Arteries

Abstract: Conus medullaris arteriovenous malformations are an exceedingly rare cause of neurological deficit. They occupy the tip of the spinal cord and can generate motor, sensorty, bowel, bladder, or sexual function deficits. Because of the complex vascular supply of the caudal aspect of the spinal cord, which usually includes multiple feeders from both the anterior and posterior spinal arteries, management may be endovascular, microsurgical, or both. Making the proper diagnosis and distinguishing it from other similar pathologies such as arteriovenous fistula is critical. As with other spinal arteriovenous malformations, it is usually not necessary to pursue the nidus into the spinal cord parenchyma at the time of surgery. This chapter discusses the diagnosis and management of this rare disorder.

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Microsurgical management of glomus spinal arteriovenous malformations: pial resection technique

Journal of Neurosurgery Spine ◽

10.3171/2012.3.spine11982 ◽

2012 ◽

Vol 16 (6) ◽

pp. 523-531 ◽

Cited By ~ 25

Author(s):

Gregory J. Velat ◽

Steve W. Chang ◽

Adib A. Abla ◽

Felipe C. Albuquerque ◽

Cameron G. McDougall ◽

...

Keyword(s):

Spinal Cord ◽

Arteriovenous Malformations ◽

Signs And Symptoms ◽

Vascular Lesions ◽

Morbidity Rate ◽

Surgical Morbidity ◽

Surgical Interventions ◽

Spinal Arteriovenous Malformations

Object Intramedullary, or glomus, spinal arteriovenous malformations (AVMs) are rare vascular lesions amenable to resection with or without adjuvant embolization. The authors retrospectively reviewed the senior author's (R.F.S.'s) surgical series of intramedullary spinal AVMs to evaluate clinical and radiographic outcomes. Methods Detailed chart and radiographic reviews were performed for all patients with intramedullary spinal AVMs who underwent surgical treatment between 1994 and 2011. Presenting and follow-up neurological examination results were obtained and graded using the modified Rankin Scale (mRS) and McCormick Scale. Surgical technique, outcomes, complications, and long-term angiographic studies were reviewed. Results During the study period, 20 patients (10 males and 10 females) underwent resection of glomus spinal AVMs. The mean age at presentation was 30 ± 17 years (range 7–62 years). The location of the AVMs was as follows: cervical spine (n = 10), thoracic spine (n = 9), and cervicothoracic junction (n = 1). The most common presenting signs and symptoms included paresis or paralysis (65%), paresthesias (40%), and myelopathy (40%). Perioperative embolization was performed in the majority (60%) of patients. Pial AVM resection was performed in 17 cases (85%). Angiographically verified AVM obliteration was achieved in 15 patients (75%). At a mean follow-up duration of 45.4 ± 52.4 months (range 2–176 months), 14 patients (70%) remained functionally independent (mRS and McCormick Scale scores ≤ 2). One perioperative complication occurred, yielding a surgical morbidity rate of 5%. Three symptomatic spinal cord tetherings occurred at a mean of 5.7 years after AVM resection. No neurological decline was observed after endovascular and surgical interventions. No deaths occurred. Long-term angiographic follow-up data were available for 9 patients (40%) at a mean of 67.6 ± 60.3 months (range 5–176 months) following AVM resection. Durable AVM obliteration was documented in 5 (83%) of 6 patients. Conclusions Intramedullary AVMs may be safely resected with satisfactory clinical and angiographic results. The pial resection technique, which provides subtotal AVM nidus resection, effectively devascularized these lesions, as confirmed on postoperative angiography, without violating the spinal cord parenchyma, thereby potentially reducing iatrogenic injury.

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Successful treatment of a group of spinal cord arteriovenous malformations by interruption of dural fistula

Journal of Neurosurgery ◽

10.3171/jns.1983.59.6.1019 ◽

1983 ◽

Vol 59 (6) ◽

pp. 1019-1030 ◽

Cited By ~ 152

Author(s):

Edward H. Oldfield ◽

Giovanni Di Chiro ◽

Eugene A. Quindlen ◽

Kenneth G. Rieth ◽

John L. Doppman

Keyword(s):

Spinal Cord ◽

Arteriovenous Fistula ◽

Arteriovenous Malformations ◽

Venous Hypertension ◽

Current Therapy ◽

Type I ◽

Content Type ◽

Symptoms And Signs ◽

Extensive Surgery ◽

Fine Print

✓ As demonstrated by selective spinal cord arteriography, over 80% of spinal cord arteriovenous malformations (AVM's) occupy a predominantly extramedullary position. Current therapy frequently requires surgical stripping of the long dorsal intradural vessel(s) from the underlying spinal cord over many cord segments. The authors report six patients with a dural arteriovenous fistula fed by a cluster of abnormal epidural arteries. These vessels, which surrounded and were embedded into the dural covering of a thoracic nerve root, drained into a long sinuous intrathecal paramedullary vein(s). The angiographic and surgical appearance of the intradural component of these lesions was identical to that of lesions previously classified as Type I AVM's of the spinal cord. All patients had symptoms and signs of myelopathy. In five patients, surgery was limited to coagulation and excision of the extradural vessels and division of the intradural arterialized vein. Progressive improvement began within days following surgery. No residual abnormality was demonstrated by postoperative selective spinal cord arteriography, which was performed in all five patients. The findings support those of Kendall and Logue, that surgery restricted to elimination of the arteriovenous fistula at the intervertebral foramen is curative, and that more extensive surgery is unnecessary for this subgroup of AVM's of the spinal cord. These lesions comprise a sizable percent of all spinal AVM's. Resolution of myelopathy in these patients supports the hypothesis that venous hypertension causes chronic progressive myelopathy.

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Acute thrombosis of a giant perimedullary arteriovenous fistula in a pediatric HHT patient

Interventional Neuroradiology ◽

10.1177/15910199211022499 ◽

2021 ◽

pp. 159101992110224

Author(s):

Janaki Devara ◽

Vivek N Iyer ◽

Deepti M Warad ◽

Waleed Brinjikji ◽

Ahmad Aljobeh ◽

...

Keyword(s):

Spinal Cord ◽

Arteriovenous Fistula ◽

Arteriovenous Malformations ◽

Autosomal Dominant ◽

Clinical Symptoms ◽

Acute Thrombosis ◽

Acute Paraparesis ◽

Spinal Arteriovenous Shunt ◽

Preventive Role

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder that results in arteriovenous malformations (AVMs) in the nose, mucocutaneous surfaces and visceral organs, including lung, brain, liver, bowel and rarely spinal cord. We describe a case of a young child with HHT who presented with acute paraparesis due to acute thrombosis of a spinal perimedullary arteriovenous fistula. Patient underwent coil embolization of spinal arteriovenous shunt with resolution of clinical symptoms. This case highlights the possibility of catastrophic complications in young children with HHT, the potential preventive role of screening for spinal AVMs in HHT and the importance of timely intervention.

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Classification of Spinal Cord Arteriovenous Shunts: Proposal for a Reappraisal—The Bicêtre Experience with 155 Consecutive Patients Treated between 1981 and 1999

Neurosurgery ◽

10.1227/00006123-200208000-00013 ◽

2002 ◽

Vol 51 (2) ◽

pp. 374-380 ◽

Cited By ~ 10

Author(s):

Georges Rodesch ◽

Michel Hurth ◽

Hortensia Alvarez ◽

Marc Tadié ◽

Pierre Lasjaunias

Keyword(s):

Spinal Cord ◽

Hereditary Hemorrhagic Telangiectasia ◽

Arteriovenous Malformations ◽

Morphological Features ◽

Fully Integrated ◽

Multiple Lesions ◽

Arteriovenous Shunts ◽

Weber Syndrome ◽

Klippel Trenaunay Syndrome

Abstract OBJECTIVE Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances? METHODS We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions. RESULTS All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease. CONCLUSION It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.

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