Cent Eur Neurosurg 2007; 68(4): 205-213
DOI: 10.1055/s-2007-985852
Review

© Georg Thieme Verlag KG Stuttgart · New York

Spinal Vascular Malformations - Typical and Atypical Findings

Spinale Gefässmalformationen - Typische und Atypische FormenA. Bostroem 1 , 3 , A. Thron 2 , F. J. Hans 1 , T. Krings 2
  • 1Department of Neurosurgery, University Hospital (RWTH), Aachen, Germany
  • 2Department of Neuroradiology, University Hospital (RWTH) Aachen, Aachen, Germany
  • 3Department of Neurosurgery, University Hospital, Bonn, Germany
Weitere Informationen

Publikationsverlauf

Publikationsdatum:
26. Oktober 2007 (online)

Abstract

Vascular malformations of the spinal cord and its meninges are rare diseases which comprise true inborn cavernomas and arteriovenous malformations (AVM), including perimedullary fistulae, glomerular and juvenile AVMs, and presumably acquired dural arteriovenous fistulae. This article gives an overview of the imaging features on magnetic resonance imaging (MRI) and digital subtraction angiography of both typical and atypical findings to describe the wide variety of possible pathological entities encountered. Clinical differential diagnoses, the neurological symptomatology and potential therapeutic approaches of these diseases, which may vary depending on the underlying pathology, are given. Although MRI constitutes the first choice diagnostic modality for suspected spinal vascular malformations, we conclude that the definite diagnosis of the disease and thus the choice of the appropriate therapeutic approach rests on selective spinal angiography which should be performed at a specialized center. Treatment in symptomatic patients offers an improvement in prognosis. Microsurgical treatment is recommended for symptomatic spinal cord cavernomas. Dural arteriovenous shunts can either be treated by microsurgical or endovascular approaches, the former being a simple, quick and secure approach to obliterate the fistula while the latter is technically demanding. In spinal arteriovenous malformations of both the fistulous and the glomerular type, the endovascular approach is the method of first choice; in selected cases, surgery or a combined therapy may be necessary.

Zusammenfassung

Spinale Gefässmalformationen des Myelons und seiner Meningen sind seltene Erkrankungen, zu denen die angeborenen spinalen Kavernome und arteriovenösen Malformationen (AVM) inklusive der perimedullären Fisteln, glomerulären und juvenilen AVMs und die vermutlich erworbenen duralen arteriovenösen Fisteln gehören. Unsere Arbeit gibt eine Übersicht über die bildmorphologischen Charakteristika beider Formen (typisch und atypisch) für die Kernspintomographie (MRT) und die digitalen Substraktionsangiographie (DSA), um das breite Spektrum der Pathologien aufzuzeigen. Die neurologische Symptomatik, klinische Differentialdiagnosen und potentielle therapeutische Vorgehensweisen, welche in Abhängigkeit der grundlegenden Erkrankung sehr variieren können, werden vorgestellt. Obwohl die MRT Mittel der Wahl in der Diagnostik bei Verdacht auf eine Gefäßmalformation ist, schlussfolgern wir, dass eine definitive Diagnose der Erkrankung und damit die Wahl der geeigneten Therapie auf der selektiven spinalen Angiographie beruht. Diese sollte in einem dafür spezialisierten Zentrum durchgeführt werden. Die Behandlung symptomatischer Patienten bietet die Möglichkeit einer Verbesserung der Prognose. Die mikrochirurgische Technik wird bei symptomatischen spinalen Kavernomen empfohlen. Durale arteriovenöse Shunts können sowohl mikrochirurgisch als auch endovaskulär behandelt werden, die erstere ist eine einfache, schnelle und sichere Vorgehensweise, die letztere ist technisch anspruchsvoll. Bei spinalen arteriovenösen Malformationen, sowohl vom fistulösen als auch vom glomerulären Typ, ist die endovaskuläre Technik die Methode der ersten Wahl, aber in selektierten Fällen kann eine chirurgische oder kombinierte Vorgehensweise erforderlich sein.

References

  • 1 Anson JA, Spetzler RF. Surgical resection of intramedullary spinal cord cavernous malformations.  J Neurosurg. 1993;  78 446-451
  • 2 Backes W, Nijenhuis R, Mull M, Thron A, Wilmink J. Contrast-enhanced MR angiography of the spinal arteries: current possibilities and limitations.  Rivista di Neuroradiologia. 2004;  282-292
  • 3 Behrens S, Thron A. Long-term follow-up and outcome in patients treated for spinal dural arteriovenous fistula.  J Neurol. 1999;  246 181-185
  • 4 Biondi A, Merland JJ, Reizine D, Aymard A, Hodes JE, Lecoz P, Rey A. Embolization with particles in thoracic intramedullary arteriovenous malformations: long-term angiographic and clinical results.  Radiology. 1990;  177 651-658
  • 5 Bradac GB, Daniele D, Riva A, Bracchi M, Stura G, Riccio A, Pagni CA. Spinal dural arteriovenous fistulas: an underestimated cause of myelopathy.  Eur Neurol. 1994;  34 87-94
  • 6 Clemens H, Quast Hv. Untersuchungen über die Gefäße des Rückenmarks.  Acta Anat. 1960;  42 277-306
  • 7 Criscuolo GR, Oldfield EH, Doppman JL. Reversible acute and subacute myelopathy in patients with dural arteriovenous fistulas. Foix-Alajouanine syndrome reconsidered.  J Neurosurg. 1989;  70 354-359
  • 8 Marco JK De, Dillon WP, Halback VV, Tsuruda JS. Dural arteriovenous fistulas: evaluation with MR imaging.  Radiology. 1990;  175 193-199
  • 9 Djindjian R, Cophignon J, Rey A, Theron J, Merland JJ, Houdart R. Superselective arteriographic embolization by the femoral route in neuroradiology. Study of 50 cases. II. Embolization in vertebromedullary pathology.  Neuroradiology. 1973;  6 132-142
  • 10 Doppman JL, Chiro G Di, Dwyer AJ, Frank JL, Oldfield EH. Magnetic resonance imaging of spinal arteriovenous malformations.  J Neurosurg. 1987;  66 830-834
  • 11 Doppman JL, Chiro G, Ommaya A. Obliteration of spinal-cord arteriovenous malformation by percutaneous embolisation.  Lancet. 1968;  1 477
  • 12 Farb RI, Kim JK, Willinsky RA, Montanera WJ, terBrugge K, Derbyshire JA, Dijk JM van, Wright GA. Spinal dural arteriovenous fistula localization with a technique of first-pass gadolinium-enhanced MR angiography: initial experience.  Radiology. 2002;  222 843-850
  • 13 Heros RC, Debrun GM, Ojemann RG, Lasjaunias PL, Naessens PJ. Direct spinal arteriovenous fistula: a new type of spinal AVM.  Case report. J Neurosurg. 1986;  64 134-139
  • 14 Huffmann BC, Gilsbach JM, Thron A. Spinal dural arteriovenous fistulas: a plea for neurosurgical treatment.  Acta Neurochir (Wien). 1995;  135 44-51
  • 15 Hurst RW, Grossman RI. Peripheral spinal cord hypointensity on T2-weighted MR images: a reliable imaging sign of venous hypertensive myelopathy.  AJNR Am J Neuroradiol. 2000;  21 781-786
  • 16 Hurst RW, Kenyon LC, Lavi E, Raps EC, Marcotte P. Spinal dural arteriovenous fistula: the pathology of venous hypertensive myelopathy.  Neurology. 1995;  45 1309-1313
  • 17 Koenig E, Thron A, Schrader V, Dichgans J. Spinal arteriovenous malformations and fistulae: clinical, neuroradiological and neurophysiological findings.  J Neurol. 1989;  236 260-266
  • 18 Krings T, Chng SM, Ozanne A, Alvarez H, Rodesch G, Lasjaunias PL. Hereditary hemorrhagic telangiectasia in children: endovascular treatment of neurovascular malformations: results in 31 patients.  Neuroradiology. 2005;  47 946-954
  • 19 Krings T, Mull M, Bostroem A, Otto J, Hans FJ, Thron A. Spinal epidural arteriovenous fistula with perimedullary drainage. Case report and pathomechanical considerations.  J Neurosurg Spine. 2006;  5 353-358
  • 20 Krings T, Mull M, Gilsbach JM, Thron A. Spinal vascular malformations.  Eur Radiol. 2005;  15 267-278
  • 21 Krings T, Mull M, Reinges MH, Thron A. Double spinal dural arteriovenous fistulas: case report and review of the literature.  Neuroradiology. 2004;  46 238-242
  • 22 Lasjaunias P, Berenstein A, terBrugge K. Surgical Neuroangiography. Springer, Berlin 2001
  • 23 Lee TT, Gromelski EB, Bowen BC, Green BA. Diagnostic and surgical management of spinal dural arteriovenous fistulas.  Neurosurgery. 1998;  43 242-246 , discussion 246-247
  • 24 MacCutcheon IE, Doppman JL, Oldfield EH. Microvascular anatomy of dural arteriovenous abnormalities of the spine: a microangiographic study.  J Neurosurg. 1996;  84 215-220
  • 25 Niimi Y, Berenstein A, Setton A, Neophytides A. Embolization of spinal dural arteriovenous fistulae: results and follow-up.  Neurosurgery. 1997;  40 675-682 , discussion 682-673
  • 26 Pierot L, Vlachopoulos T, Attal N, Martin N, Bert S, Chiras J. Double spinal dural arteriovenous fistulas: report of two cases.  AJNR Am J Neuroradiol. 1993;  14 1109-1112
  • 27 Prestigiacomo CJ, Niimi Y, Setton A, Berenstein A. Three-dimensional rotational spinal angiography in the evaluation and treatment of vascular malformations.  AJNR Am J Neuroradiol. 2003;  24 1429-1435
  • 28 Reinges MH, Thron A, Mull M, Huffmann BC, Gilsbach JM. Dural arteriovenous fistulae at the foramen magnum.  J Neurol. 2001;  248 197-203
  • 29 Rigamonti D, Johnson PC, Spetzler RF, Hadley MN, Drayer BP. Cavernous malformations and capillary telangiectasia: a spectrum within a single pathological entity.  Neurosurgery. 1991;  28 60-64
  • 30 Rodesch G, Hurth M, Alvarez H, Ducot B, Tadie M, Lasjaunias P. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicetre experience on 155 consecutive patients seen between 1981-1999.  Acta Neurochir (Wien). 2004;  146 217-226 , discussion 226-217
  • 31 Rodesch G, Hurth M, Alvarez H, Tadie M, Lasjaunias P. Classification of spinal cord arteriovenous shunts: proposal for a reappraisal- the Bicetre experience with 155 consecutive patients treated between 1981 and 1999.  Neurosurgery. 2002;  51 374-379 , discussion 379-380
  • 32 Rodesch G, Hurth M, Alvarez H, Tadie M, Lasjaunias 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. 2005;  57 973-983 , discussion 973-983
  • 33 Rodesch G, Hurth M, Alvarez H, Tadie M, Lasjaunias PL. 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. 2005;  57 973-983
  • 34 Rosenblum B, Oldfield EH, Doppman JL, Chiro G Di. Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM's in 81 patients.  J Neurosurg. 1987;  67 795-802
  • 35 Russell D, Rubinstein L. Pathology of Tumours of the Nervous System. Williams & Wilkins, Baltimore 1989
  • 36 Saraf-Lavi E, Bowen BC, Quencer RM, Sklar EM, Holz A, Falcone S, Latchaw RE, Duncan R, Wakhloo A. Detection of spinal dural arteriovenous fistulae with MR imaging and contrast-enhanced MR angiography: sensitivity, specificity, and prediction of vertebral level.  AJNR Am J Neuroradiol. 2002;  23 858-867
  • 37 Spetzger U, Gilsbach JM, Bertalanffy H. Cavernous angiomas of the spinal cord clinical presentation, surgical strategy, and postoperative results.  Acta Neurochir (Wien). 1995;  134 200-206
  • 38 Spetzler RF, Detwiler PW, Rinna HA, Porter RW. Modified classification of spinal cord vascular lesions.  J Neurosurg. 2002;  96 145-162
  • 39 Spetzler RF, Zabramski JM, Flom RA. Management of juvenile spinal AVM's by embolization and operative excision. Case report.  J Neurosurg. 1989;  70 628-632
  • 40 Thron A. Spinal dural arteriovenous fistulas.  Radiologe. 2001;  41 955-960
  • 41 Thron A. Vascular Anatomy of the Spinal Cord: Neuroradiological Investigations and Clinical Syndromes. Springer, Berlin, New York 1988
  • 42 Thron A, Caplan L. Vascular malformations and interventional neuroradiology of the spinal cord. In: Brandt T, Caplan LR, Dichgans J, Diener HC, Kennard C (eds). Neurological Disorders: Course and Treatment. Academic Press, Amsterdam, Boston, London 2003: 517-528
  • 43 Thron A, Mull M, Reith W. Spinal arteriovenous malformations.  Radiologe. 2001;  41 949-954
  • 44 Dijk JM, TerBrugge KG, Willinsky RA, Farb RI, Wallace MC. Multidisciplinary management of spinal dural arteriovenous fistulas: clinical presentation and long-term follow-up in 49 patients.  Stroke. 2002;  33 1578-1583
  • 45 Weinzierl MR, Krings T, Korinth MC, Reinges MH, Gilsbach JM. MRI and intraoperative findings in cavernous haemangiomas of the spinal cord.  Neuroradiology. 2004;  46 65-71
  • 46 Zevgaridis D, Medele RJ, Hamburger C, Steiger HJ, Reulen HJ. Cavernous haemangiomas of the spinal cord. A review of 117 cases.  Acta Neurochir (Wien). 1999;  141 237-245

Correspondence

Dr. A. Bostroem

Department of Neurosurgery

University Hospital

Sigmund-Freud-Str.25

53105 Bonn

Germany

Telefon: +228/287/16 52 1

Fax: +228/287/16 57 3

eMail: azize.bostroem@ukb.uni-bonn.de

    >