Cavernous venous malformations in and around the central nervous system. Part 2: Intradural

Walid Ibn Essayed Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

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Joshua D. Bernstock Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

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Jason A. Chen Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

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Patrick R. Ng Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

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Ari D. Kappel Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

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Rose Du Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

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Publication Date:
15 Sep 2023
Page Range:
1–9
Volume/Issue:
Publish Before Print
DOI link:
https://doi.org/10.3171/2023.6.JNS23830
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Cavernous venous malformations (CavVMs) account for a spectrum of lesions with a shared pathogenesis. Their anatomical location dictates their clinical features and surgical treatment. Extradural and dura-based CavVMs were discussed in Part 1 of this review. In this part, intradural CavVMs are discussed, encompassing malformations growing within the intradural space without direct dural involvement. In addition to classic intra-axial CavVMs, cranial nerve CavVMs, intraventricular CavVMs, and intradural extramedullary spinal CavVMs are discussed in this group, given the similar natural history and specific management challenges. Herein the authors focus on critical clinical aspects of and surgical management of these malformations based on their location and discuss optimal surgical approaches at each of these anatomical locations with illustrative cases. The commonalities of the natural history and surgical management that are dictated by anatomical considerations lend to a new location-based taxonomy for classification of CavVMs.

ABBREVIATIONS

CavVM = cavernous venous malformation; CN = cranial nerve; DVA = developmental venous anomaly; GTR = gross-total resection; ISSVA = International Society for the Study of Vascular Anomalies.

Supplementary Materials

    • Supplemental Figs. 1-10 (PDF 13,193)
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Contributor Notes

Correspondence Rose Du: Brigham and Women’s Hospital, Harvard Medical School, Boston, MA. rdu@bwh.harvard.edu.

INCLUDE WHEN CITING Published online September 15, 2023; DOI: 10.3171/2023.6.JNS23830.

Disclosures Dr. Chen reported equity interest in Verge Genomics and Gravity Medical Technology.

  • 1

    Biondi A, Clemenceau S, Dormont D, et al. Intracranial extra-axial cavernous (HEM) angiomas: tumors or vascular malformations? J Neuroradiol. 2002;29(2):91–104.

    • Search Google Scholar
    • Export Citation
  • 2

    Cox EM, Bambakidis NC, Cohen ML. Pathology of cavernous malformations. Handb Clin Neurol. 2017;143:267–277.

  • 3

    Gross BA, Du R, Popp AJ, Day AL. Intramedullary spinal cord cavernous malformations. Neurosurg Focus. 2010;29(3):E14.

  • 4

    Li H, Zhang B, Wang W, Wei MH, Liu BY, Wu Z. Clinical features, intradural transcavernous surgical management, and outcomes of giant cavernous sinus hemangiomas: a single-institution experience. World Neurosurg. 2019;125:e754–e763.

    • Search Google Scholar
    • Export Citation
  • 5

    Sarraf D, Payne AM, Kitchen ND, Sehmi KS, Downes SM, Bird AC. Familial cavernous hemangioma: an expanding ocular spectrum. Arch Ophthalmol. 2000;118(7):969–973.

    • Search Google Scholar
    • Export Citation
  • 6

    Er U, Yigitkanli K, Simsek S, Adabag A, Bavbek M. Spinal intradural extramedullary cavernous angioma: case report and review of the literature. Spinal Cord. 2007;45(9):632–636.

    • Search Google Scholar
    • Export Citation
  • 7

    Pétillon P, Wilms G, Raftopoulos C, Duprez T. Spinal intradural extramedullary cavernous hemangioma. Neuroradiology. 2018;60(10):1085–1087.

    • Search Google Scholar
    • Export Citation
  • 8

    Beechar VB, Srinivasan VM, Reznik OE, et al. Intraventricular cavernomas of the third ventricle: report of 2 cases and a systematic review of the literature. World Neurosurg. 2017;105:935–943.e3.

    • Search Google Scholar
    • Export Citation
  • 9

    Kivelev J, Niemelä M, Kivisaari R, Hernesniemi J. Intraventricular cerebral cavernomas: a series of 12 patients and review of the literature. J Neurosurg. 2010;112(1):140–149.

    • Search Google Scholar
    • Export Citation
  • 10

    Adachi K, Yoshida K, Akiyama T, Kawase T. Cavernous angioma of the vestibular nerve: case report and literature review. Surg Neurol. 2008;70(1):82–86.

    • Search Google Scholar
    • Export Citation
  • 11

    Samadian M, Maroufi SF, Bakhtevari MH, Borghei-Razavi H. An isolated cavernous malformation of the sixth cranial nerve: a case report and review of literature. Surg Neurol Int. 2021;12:563.

    • Search Google Scholar
    • Export Citation
  • 12

    Tarabay A, Rocca A, Maeder P, Simonin A, Messerer M, Daniel RT. Extra-axial cavernoma of the cerebellopontine angle: a case study and review of literature. World Neurosurg. 2019;128:415–421.

    • Search Google Scholar
    • Export Citation
  • 13

    Gross BA, Du R. Hemorrhage from cerebral cavernous malformations: a systematic pooled analysis. J Neurosurg. 2017;126(4):1079–1087.

  • 14

    Gross BA, Du R. Cerebral cavernous malformations: natural history and clinical management. Expert Rev Neurother. 2015;15(7):771–777.

  • 15

    Josephson CB, Leach JP, Duncan R, Roberts RC, Counsell CE, Al-Shahi Salman R. Seizure risk from cavernous or arteriovenous malformations: prospective population-based study. Neurology. 2011;76(18):1548–1554.

    • Search Google Scholar
    • Export Citation
  • 16

    Ruan D, Yu XB, Shrestha S, Wang L, Chen G. The role of hemosiderin excision in seizure outcome in cerebral cavernous malformation surgery: a systematic review and meta-analysis. PLoS One. 2015;10(8):e0136619.

    • Search Google Scholar
    • Export Citation
  • 17

    Essayed WI, Zhang F, Unadkat P, Cosgrove GR, Golby AJ, O’Donnell LJ. White matter tractography for neurosurgical planning: a topography-based review of the current state of the art. Neuroimage Clin. 2017;15:659–672.

    • Search Google Scholar
    • Export Citation
  • 18

    Serra C, Türe H, Yaltırık CK, Harput MV, Türe U. Microneurosurgical removal of thalamic lesions: surgical results and considerations from a large, single-surgeon consecutive series. J Neurosurg. 2021;135(2):458–468.

    • Search Google Scholar
    • Export Citation
  • 19

    Campero A, Tróccoli G, Martins C, Fernandez-Miranda JC, Yasuda A, Rhoton AL Jr. Microsurgical approaches to the medial temporal region: an anatomical study. Neurosurgery. 2006;59(4 suppl 2):ONS279–ONS308.

    • Search Google Scholar
    • Export Citation
  • 20

    Knerlich-Lukoschus F, Steinbok P, Dunham C, Cochrane DD. Cerebellar cavernous malformation in pediatric patients: defining clinical, neuroimaging, and therapeutic characteristics. J Neurosurg Pediatr. 2015;16(3):256–266.

    • Search Google Scholar
    • Export Citation
  • 21

    Watanabe T, Suematsu Y, Saito K, et al. Selection of surgical approach for cerebellar hemangioblastomas based on venous drainage patterns. Neurosurg Rev. 2021;44(6):3567–3579.

    • Search Google Scholar
    • Export Citation
  • 22

    Baran O, Baydin S, Mirkhasilova M, et al. Microsurgical anatomy and surgical exposure of the cerebellar peduncles. Neurosurg Rev. 2022;45(3):2095–2117.

    • Search Google Scholar
    • Export Citation
  • 23

    Zhang P, Liu L, Cao Y, Wang S, Zhao J. Cerebellar cavernous malformations with and without associated developmental venous anomalies. BMC Neurol. 2013;13:134.

    • Search Google Scholar
    • Export Citation
  • 24

    Cavalcanti DD, Preul MC, Kalani MY, Spetzler RF. Microsurgical anatomy of safe entry zones to the brainstem. J Neurosurg. 2016;124(5):1359–1376.

    • Search Google Scholar
    • Export Citation
  • 25

    Serrato-Avila JL, Paz Archila JA, Silva da Costa MD, et al. Three-dimensional quantitative analysis of the brainstem safe entry zones based on internal structures. World Neurosurg. 2022;158:e64–e74.

    • Search Google Scholar
    • Export Citation
  • 26

    Yagmurlu K, Rhoton AL Jr, Tanriover N, Bennett JA. Three-dimensional microsurgical anatomy and the safe entry zones of the brainstem. Neurosurgery. 2014;10(suppl 4):602–620.

    • Search Google Scholar
    • Export Citation
  • 27

    López JR. Mapping the brainstem: floor of the fourth ventricle. In: Nuwer MR, ed.Intraoperative Monitoring of Neural Function. Handbook of Clinical Neurophysiology. Vol 8. Elsevier; 2008:350-362.

    • Search Google Scholar
    • Export Citation
  • 28

    Catapano JS, Rumalla K, Srinivasan VM, Lawrence PM, Larson Keil K, Lawton MT. A taxonomy for brainstem cavernous malformations: subtypes of pontine lesions. Part 1: basilar, peritrigeminal, and middle peduncular. J Neurosurg. 2022;137(5):1426–1476.

    • Search Google Scholar
    • Export Citation
  • 29

    Catapano JS, Rumalla K, Srinivasan VM, Lawrence PM, Larson Keil K, Lawton MT. A taxonomy for brainstem cavernous malformations: subtypes of pontine lesions. Part 2: inferior peduncular, rhomboid, and supraolivary. J Neurosurg. 2022;137(5):1477–1490.

    • Search Google Scholar
    • Export Citation
  • 30

    Abla AA, Lekovic GP, Turner JD, de Oliveira JG, Porter R, Spetzler RF. Advances in the treatment and outcome of brainstem cavernous malformation surgery: a single-center case series of 300 surgically treated patients. Neurosurgery. 2011;68(2):403–415.

    • Search Google Scholar
    • Export Citation
  • 31

    Kadri PAS, Essayed WIBN, Al-Mefty O. Resection of pontine cavernoma through the anterior transpetrosal approach: 2-dimensional operative video. Oper Neurosurg (Hagerstown). 2021;21(1):E26–E27.

    • Search Google Scholar
    • Export Citation
  • 32

    Morota N, Deletis V. The importance of brainstem mapping in brainstem surgical anatomy before the fourth ventricle and implication for intraoperative neurophysiological mapping. Acta Neurochir (Wien). 2006;148(5):499–509.

    • Search Google Scholar
    • Export Citation
  • 33

    Strauss C, Romstöck J, Nimsky C, Fahlbusch R. Intraoperative identification of motor areas of the rhomboid fossa using direct stimulation. J Neurosurg. 1993;79(3):393–399.

    • Search Google Scholar
    • Export Citation
  • 34

    Erkmen K, Al-Mefty O. Transcondylar approach for resection of medullary cavernous malformation: 2-dimensional operative video. Oper Neurosurg (Hagerstown). 2021;21(3):E233–E234.

    • Search Google Scholar
    • Export Citation
  • 35

    Deshmukh VR, Rangel-Castilla L, Spetzler RF. Lateral inferior cerebellar peduncle approach to dorsolateral medullary cavernous malformation. J Neurosurg. 2014;121(3):723–729.

    • Search Google Scholar
    • Export Citation
  • 36

    Bian LG, Bertalanffy H, Sun QF, Shen JK. Intramedullary cavernous malformations: clinical features and surgical technique via hemilaminectomy. Clin Neurol Neurosurg. 2009;111(6):511–517.

    • Search Google Scholar
    • Export Citation
  • 37

    Jallo GI, Freed D, Zareck M, Epstein F, Kothbauer KF. Clinical presentation and optimal management for intramedullary cavernous malformations. Neurosurg Focus. 2006;21(1):e10.

    • Search Google Scholar
    • Export Citation
  • 38

    Takami T, Yamagata T, Ohata K. Posterolateral sulcus approach for spinal intramedullary tumor of lateral location: technical note. Neurol Med Chir (Tokyo). 2013;53(12):920–927.

    • Search Google Scholar
    • Export Citation
  • 39

    Wu B, Liu W, Zhao Y. Coexistence of extra-axial cavernous malformation and cerebellar developmental venous anomaly in the cerebellopontine angle. World Neurosurg. 2012;78(3-4):375.e5–9.

    • Search Google Scholar
    • Export Citation
  • 40

    Jin SC, Ahn JS, Kwun BD, Kwon DH. Intraventricular cavernous malformation radiologically mimicking meningioma. J Korean Neurosurg Soc. 2008;44(5):345–347.

    • Search Google Scholar
    • Export Citation
  • 41

    Paiva A, Lovato RM, Araujo JO, Veiga J. Septum pellucidum cavernoma: a case report and anatomical consideration of an extremely rare lesion. Turk Neurosurg. 2020;30(1):145–148.

    • Search Google Scholar
    • Export Citation
  • 42

    Samireddypalle Y, Hanumaiah R. Cavernous malformations of septum pellucidum and trigeminal nerve: a case report. Global J Med Case Rep. 2021;1(1):1–4.

    • Search Google Scholar
    • Export Citation
  • 43

    Choque-Velasquez J, Colasanti R, Muhammad S, Chioffi F, Hernesniemi J. Vascular lesions of the pineal region: a comprehensive review of the therapeutic options. World Neurosurg. 2022;159:298–313.

    • Search Google Scholar
    • Export Citation
  • 44

    Figueiredo A, Maheshwari S, Goel A. Cavernoma in the pineal region. J Clin Neurosci. 2010;17(5):652–653.

  • 45

    Muzumdar DP, Misra BK, Bhaduri AS. Pineal region cavernoma—case report. Neurol Med Chir (Tokyo). 2000;40(7):372–379.

  • 46

    Ogura T, Kambe A, Sakamoto M, Shinohara Y, Ogawa T, Kurosaki M. Superficial siderosis associated with pineal cavernous malformation. World Neurosurg. 2018;109:230–232.

    • Search Google Scholar
    • Export Citation
  • 47

    Nogueira RM, Cardoso LS, Fonseca L, et al. Hydrocephalus in children—a rare case of pineal cavernoma and literature review. Surg Neurol Int. 2020;11:294.

    • Search Google Scholar
    • Export Citation
  • 48

    Pagni CA, Canavero S, Forni M. Report of a cavernoma of the cauda equina and review of the literature. Surg Neurol. 1990;33(2):124–131.

    • Search Google Scholar
    • Export Citation
  • 49

    D’Onofrio GF, Capo G, Vasiljevic A, Barrey CY. Pure lumbar foraminal cavernous malformation in a patient with Cowden syndrome—a case report. J Spine Surg. 2022;8(3):390–396.

    • Search Google Scholar
    • Export Citation
  • 50

    Harrison MJ, Eisenberg MB, Ullman JS, Oppenheim JS, Camins MB, Post KD. Symptomatic cavernous malformations affecting the spine and spinal cord. Neurosurgery. 1995;37(2):195–205.

    • Search Google Scholar
    • Export Citation
  • 51

    Zhao L, Jiang Y, Wang Y, Bai Y, Sun Y, Li Y. Spinal epidural cavernous hemangiomas: a clinical series of 9 cases and literature review. Front Oncol. 2021;11:572313.

    • Search Google Scholar
    • Export Citation
  • 52

    Labauge P, Denier C, Bergametti F, Tournier-Lasserve E. Genetics of cavernous angiomas. Lancet Neurol. 2007;6(3):237–244.

  • 53

    Khan MJ, Dinkin M, D’Amico DJ. Cerebral cavernous malformation 2 syndrome presenting with retinal cavernous hemangioma and sixth cranial nerve palsy. JAMA Ophthalmol. 2021;139(3):e204104.

    • Search Google Scholar
    • Export Citation
  • 54

    Choudhri O, Feroze AH, Lad EM, et al. Co-occurrence of a cerebral cavernous malformation and an orbital cavernous hemangioma in a patient with seizures and visual symptoms: rare crossroads for vascular malformations. Surg Neurol Int. 2014;5(suppl 4):S148–S154.

    • Search Google Scholar
    • Export Citation
  • 55

    Boockvar JA, Stiefel M, Malhotra N, Dolinskas C, Dwyer-Joyce C, LeRoux PD. Dural cavernous angioma of the posterior sagittal sinus: case report. Surg Neurol. 2005;63(2):178–181.

    • Search Google Scholar
    • Export Citation
  • 56

    Blei F. Nomenclature of vascular anomalies: evolution to the ISSVA 2018 classification system. In: Trenor CC III, Adams DM, eds. Vascular Anomalies. Springer International Publishing; 2020.

    • Search Google Scholar
    • Export Citation
  • 57

    Dasgupta R, Fishman SJ. ISSVA classification. Semin Pediatr Surg. 2014;23(4):158–161.

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