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Vascular Malformation

Definition

Non-neoplastic, developmental anomalies of cerebrospinal circulation including arteriovenous malformations(AVM),cavernous hemangiomas, venous angiomas and capillary telangiectasias.

Clinical Features

Capillary Telangiectases

Cavernous Angiomas

  • Presentation:
    • may be childhood
    • usually third and fourth decades8
    • most commonly:
      • seizures:
        • most close to epileptogenic cerebrocortical tissues
    • less frequently:
      • focal neurologic deficits
      • headache
    • catastrophic hemorrhage rare
    • cause of intracranial hematoma, including those that are:
      • acutely symptomatic9
      • ‘encapsulate’ lobar types:10
        • probably from repeated subclinical bleeds and subsequent organization

Pathogenesis

Cavernous Angioma

  • Speculation that may evolve from malformations of capillary telangiectases4

Arteriovenous Fistulas

  • Causes include:
    • trauma:
      • particularly for carotid-cavernous sinus and vertebrovertebral types
    • neurosurgical injury
    • systemic disorder e.g.:
      • fibromuscular dysplasia
      • type IV Ehlers–Danlos syndrome1
  • Many spontaneous

Gross Pathology

Capillary Telangiectases

  • Location:
    • predilection for basis pontis:
      • particularly median raphe region
    • occasionally:
      • cerebral hemispheres
      • spinal cord1
  • Usually incidental finding at autopsy

Venous Angioma

  • Loose collection of dilated veins
  • Location:
    • typically digitate or deep white matter of cerebral or cerebellar hemispheres1

Cavernous Angioma

  • Location:
    • anywhere along neuraxis
    • most above tentorium:8
      • often:
        • in cerebral white matter subjacent to motor strip
      • also:
  • May be multifocal
  • Constituent vessels fashioned into compact, globose mass devoid of intervening neural elements1
  • Spongy core of blood-filled channels:
    • encircled by thin rind of neural parenchyma that is:
      • indurated:
        • because gliotic
      • rusted-appearing:
        • because hemosiderin-laden

AVM

  • Tangle of deformed arterial afferents and draining veins devoid of interposed capillary bed1,20
  • Location:
  • Variable size from:
    • cryptic lesions discovered only on sampling surgically evacuated hematomas,22 to
    • enormous lobar examples spanning full thickness of a cerebral hemisphere
  • Tortuous, cirsoid vascular components:
    • form complex network of blood-filled channels
  • Intervening neural tissues:
    • typically attenuated
  • Rust-brown discoloration:
    • due to previous hemorrhage
  • Involved leptomeninges:
    • thickened
    • opacified
    • frequently siderotic

Histopathology

Capillary Telangiectases

  • Loosely aggregated and variably ectatic capillary-type vessels:
    • i.e. devoid of elastic or muscular mural elements
    • separated by normal or mildly gliotic neuropil
  • A densely mineralized variant:
    • known as calcified telangiectatic hamartoma or hemangioma calcificans
    • rare cause of epilepsy, particularly temporal lobe type23

Cavernous Angioma

  • Closely apposed, engorged vessels composed solely of fibrous tissue (Fig. 1
    Cavernous angioma. The cavernous angioma consists of ectatic and fibrous-walled vascular channels devoid of intervening neuroglial tissue. Neighboring brain parenchyma is present at lower left.

    Fig. 1: Cavernous angioma. The cavernous angioma consists of ectatic and fibrous-walled vascular channels devoid of intervening neuroglial tissue. Neighboring brain parenchyma is present at lower left.

    )
  • Commonly secondary alterations such as:
    • thrombosis
    • dystrophic calcification
  • Sometimes extensive metaplastic ossification
  • Exceptionally hybrid variant:
    • part structure of capillary telangiectases

AVM

  • Variably ectatic and hyalinized veins
  • Abnormally muscularized arteries (Fig. 2
    Arteriovenous malformation. Ectatic, variably muscularized blood vessels with interrupted elastic lamina and fibrotic intimal thickening participate in this malformative lesion (Van Gieson stain).

    Fig. 2: Arteriovenous malformation. Ectatic, variably muscularized blood vessels with interrupted elastic lamina and fibrotic intimal thickening participate in this malformative lesion (Van Gieson stain).

    )
  • Structurally ambiguous vessels:
    • formed solely of fibrous tissue, or
    • both arterial and venous characteristics1,20
  • Conspicuous mural anomalies:
  • Cushions of fibromuscular tissue may appear to project in polypoid fashion into lumens of abnormal vessels
  • Commonly focal duplications and disruptions of internal elastic lamina
  • Superimposed alterations:
    • mural fibroplasia and atheromatosis
    • aneurysmal dilatation
    • calcification
    • thrombosis:
      • if extensive, may preclude visualization of even sizable malformations by angiography22
  • If preoperative embolization to minimize blood loss during resection:
    • frequently an intraluminal foreign body response to occluding material
    • may be focal necrosis:24
      • radiosurgical treatment causes fibrointimal hyperplasia with progressive vaso-occlusion25
  • Entrapped neuropil:
    • usually manifests:
      • dense astrogliosis
      • neuronal depopulation
      • ferruginous encrustation of included neuroglial elements
  • Sometimes oligodendroglioma-like regions in interstices that may be:
    • intrinsic to underlying maldevelopment
    • result of abnormal oligodendroglial aggregation caused by ischemic contraction of entrapped white matter26,27

Other investigations

Venous Angioma

Angiography
  • Radial convergence of ectatic vessels on central draining varix results in diagnostic ‘caput medusae’-like profile

Cavernous Angioma

Angiography
  • Typically not evident
T2-weighted MRI
  • Irregularly hyperdense nodule:
    • no significant edema or mass effect
    • surrounding hypodense penumbra:
      • due to hemosiderin accumulation in adjoining neural tissues

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Vascular Malformation

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Cavernous Angioma

Genetics

Cavernous Angioma

References

1 Kalimo H, Kaste M, Haltia M. Vascular diseases.  Graham DI,  Lantos PL editor. ed. 7. In: Greenfield's Neuropathology. vol. 1:London: Arnold; 2002.

2 Chang SD, Steinberg GK, Rosario M, Crowley RS, Hevner RF. Mixed arteriovenous malformation and capillary telangiectasia. A rare subset of mixed vascular malformations. Case report. J Neurosurg. 1997;86:699–703.

3 Meyer B, Stangl AP, Schramm J. Association of venous and true arteriovenous malformation. A rare entity among mixed vascular malformations of the brain. Case report. J Neurosurg. 1995;83:141–144.

4 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.

5 Farrell DF, Forno LS. Symptomatic capillary telangiectasis of the brainstem without hemorrhage. Report of an unusual case. Neurology. 1970;20:341–346.

6 Román G, Fisher M, Perl DP, Poser CM. Neurological manifestations of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Report of 2 cases and review of the literature. Ann Neurol. 1978;4:130–144.

7 Naff NJ, Wemmer J, Hoenig-Rigamonti K, Rigamonti DR. A longitudinal study of patients with venous malformations. Documentation of a negligible hemorrhage risk and benign natural history. Neurology. 1998;50:1709–1714.

8 Simard JM, Garcia-Bengochea F, Ballinger WE, Mickle JP, Quisling RG. Cavernous angioma. A review of 126 collected and 12 new clinical cases. Neurosurgery. 1986;18:162–172.

9 Tung H, Giannotta SL, Chandrasoma PT, Zee CS. Recurrent intraparenchymal hemorrhages from angiographically occult vascular malformations. J Neurosurg. 1990;73:174–180.

10 Roda JM, Carceller F, Perez-Higueras A, Morales C. Encapsulated intracerebral hematomas: a defined entity. J Neurosurg. 1993;78:829–833.

11 Brown RD, Wiebers DO, Forbes G, O'Fallon WM, Piepgras DG, Marsh WR, et al. The natural history of unruptured intracranial arteriovenous malformations. J Neurosurg. 1988;68:352–357.

12 Ondra SL, Troupp H, George ED, Schwab K. The natural history of symptomatic arteriovenous malformations of the brain. A 24-year follow-up assessment. J Neurosurg. 1990;73:387–391.

13 Benhaiem N, Poirier J, Hurth M. Arteriovenous fistulae of the meninges draining into the spinal veins. A histological study of 28 cases. Acta Neuropathol (Berl). 1983;62:103–111.

14 Rosenblum B, Oldfield EH, Doppman JL, Di Chiro G. Spinal arteriovenous malformations. A comparison of dural arteriovenous fistulas and intradural AVMs in 81 patients. J Neurosurg. 1987;67:795–802.

15 Porter RW, Detwiler PW, Spetzler RF, Lawton MT, Baskin JJ, Derksen PT, et al. Cavernous malformations of the brainstem. Experience with 100 patients. J Neurosurg. 1999;90:50–58.

16 Deutsch H, Jallo GI, Faktorovich A, Epstein F. Spinal intramedullary cavernoma. Clinical presentation and surgical outcome. J Neurosurg. 2000;93:65–70.

17 Pagni CA, Canavero S, Forni M. Report of a cavernoma of the cauda equina and review of the literature. Surg Neurol. 1990;33:124–131.

18 Matias-Guiu X, Alejo M, Sole T, Ferrer I, Noboa R, Bartumeus F. Cavernous angiomas of the cranial nerves. Report of two cases. J Neurosurg. 1990;73:620–622.

19 Zevgaridis D, Buttner A, Weis S, Hamburger C, Reulen HJ. Spinal epidural cavernous hemangiomas. Report of three cases and review of the literature. J Neurosurg. 1998;88:903–908.

20 Mandybur TI, Nazek M. Cerebral arteriovenous malformations. A detailed morphological and immunohistochemical study using actin. Arch Pathol Lab Med. 1990;114:970–973.

21 Carleton CC, Cauthen JC. Vascular (“arteriovenous”) malformations of the choroid plexus. Arch Pathol. 1975;99:286–288.

22 Lobato RD, Perez C, Rivas JJ, Cordobes F. Clinical, radiological, and pathological spectrum of angiographically occult intracranial vascular malformations. Analysis of 21 cases and review of the literature. J Neurosurg. 1988;68:518–531.

23 Vaquero J, Manrique M, Oya S, Cabezudo JM, Bravo G. Calcified telangiectatic hamartomas of the brain. Surg Neurol. 1980;13:453–457.

24 Schweitzer JS, Chang BS, Madsen P, Vinuela F, Martin NA, Marroquin CE, et al. The pathology of arteriovenous malformations of the brain treated by embolotherapy. II. Results of embolization with multiple agents. Neuroradiology. 1993;35:468–474.

25 Schneider BF, Eberhard DA, Steiner LE. Histopathology of arteriovenous malformations after gamma knife radiosurgery. J Neurosurg. 1997;87:352–357.

26 Lombardi D, Scheithauer BW, Piepgras D, Meyer FB, Forbes GS. “Angioglioma” and the arteriovenous malformation-glioma association. J Neurosurg. 1991;75:589–596.

27 Nazek M, Mandybur TI, Kashiwagi S. Oligodendroglial proliferative abnormality associated with arteriovenous malformation. Report of three cases with review of the literature. Neurosurgery. 1988;23:781–785.

28 Tomlinson FH, Rufenacht DA, Sundt TM, Nichols DA, Fode NC. Arteriovenous fistulas of the brain and the spinal cord. J Neurosurg. 1993;79:16–27.

29 Heckl S, Aschoff A, Kunze S. Radiation-induced cavernous hemangiomas of the brain. A late effect predominantly in children. Cancer. 2002;94:3285–3291.

30 Larson JJ, Ball WS, Bove KE, Crone KR, Tew JM. Formation of intracerebral cavernous malformations after radiation treatment for central nervous system neoplasia in children. J Neurosurg. 1998;88:51–56.

31 Zabramski JM, Wascher TM, Spetzler RF, Johnson B, Golfinos J, Drayer BP, et al. The natural history of familial cavernous malformations. Results of an ongoing study. J Neurosurg. 1994;80:422–432.

32 Dobyns WB, Michels VV, Groover RV, Mokri B, Trautmann JC, Forbes GS, et al. Familial cavernous malformations of the central nervous system and retina. Ann Neurol. 1987;21:578–583.

33 Labauge P, Enjolras O, Bonerandi JJ, Laberge S, Dandurand M, Joujoux JM, et al. An association between autosomal dominant cerebral cavernomas and a distinctive hyperkeratotic cutaneous vascular malformation in 4 families. Ann Neurol. 1999;45:250–254.

34 Amin-Hanjani S, Robertson R, Arginteanu MS, Scott RM. Familial intracranial arteriovenous malformations. Case report and review of the literature. Pediatr Neurosurg. 1998;29:208–213.

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Last updated: 1 Jan 2007

Vascular Malformation

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