Spontaneous spinal CSF–venous fistulas associated with venous/venolymphatic vascular malformations: report of 3 cases

Restricted access

Spontaneous CSF–venous fistulas may be present in up to one-fourth of patients with spontaneous intracranial hypotension. This is a recently discovered type of CSF leak, and much remains unknown about these fistulas. Spinal CSF–venous fistulas are usually seen in coexistence with a spinal meningeal diverticulum, suggesting the presence of an underlying structural dural weakness at the proximal portion of the fistula. The authors now report the presence of soft-tissue venous/venolymphatic malformations associated with spontaneous spinal CSF–venous fistulas in 2 patients with spontaneous intracranial hypotension, suggesting a role for distal venous pathology. In a third patient with spontaneous intracranial hypotension and a venolymphatic malformation, such a CSF–venous fistula is strongly suspected.

ABBREVIATIONS DSM = digital subtraction myelography; SIH = spontaneous intracranial hypotension.
Article Information

Contributor Notes

Correspondence Wouter I. Schievink: Cedars-Sinai Medical Center, Los Angeles, CA. schievinkw@cshs.org.INCLUDE WHEN CITING Published online November 1, 2019; DOI: 10.3171/2019.8.SPINE19716.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Headings
References
  • 1

    Behravesh SYakes WGupta NNaidu SChong BWKhademhosseini A: Venous malformations: clinical diagnosis and treatment. Cardiovasc Diagn Ther 6:5575692016

    • Search Google Scholar
    • Export Citation
  • 2

    Clark MSDiehn FEVerdoorn JTLehman VTLiebo GBMorris JM: Prevalence of hyperdense paraspinal vein sign in patients with spontaneous intracranial hypotension without dural CSF leak on standard CT myelography. Diagn Interv Radiol 24:54592018

    • Search Google Scholar
    • Export Citation
  • 3

    Dobrocky TGrunder LBreiding PSBranca MLimacher AMosimann PJ: Assessing spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension with a scoring system based on brain magnetic resonance imaging findings. JAMA Neurol 76:5805872019

    • Search Google Scholar
    • Export Citation
  • 4

    Ducros ABiousse V: Headache arising from idiopathic changes in CSF pressure. Lancet Neurol 14:6556682015

  • 5

    Farb RINicholson PJPeng PWMassicotte EMLay CKrings T: Spontaneous intracranial hypotension: a systematic imaging approach for CSF leak localization and management based on MRI and digital subtraction myelography. AJNR Am J Neuroradiol 40:7457532019

    • Search Google Scholar
    • Export Citation
  • 6

    Flors LLeiva-Salinas CMaged IMNorton PTMatsumoto AHAngle JF: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics 31:132113412011

    • Search Google Scholar
    • Export Citation
  • 7

    Kranz PGAmrhein TJGray L: CSF venous fistulas in spontaneous intracranial hypotension: Imaging characteristics on dynamic and CT myelograophy. AJR Am J Roentgenol 209:136013662017

    • Search Google Scholar
    • Export Citation
  • 8

    Kranz PGAmrhein TJSchievink WIKarikari IOGray L: The “hyperdense paraspinal vein” sign: a marker of CSF-venous fistula. AJNR Am J Neuroradiol 37:137913812016

    • Search Google Scholar
    • Export Citation
  • 9

    Kumar N: Beyond superficial siderosis: introducing “duropathies”. Neurology 78:199219992012

  • 10

    Kumar NDiehn FECarr CMVerdoorn JTGarza ILuetmer PH: Spinal CSF venous fistula: a treatable etiology for CSF leaks in craniospinal hypovolemia. Neurology 86:231023122016

    • Search Google Scholar
    • Export Citation
  • 11

    Kumar NNeidert NBDiehn FECampeau NGMorris JMBjarnason H: A novel etiology for craniospinal hypovolemia: a case of inferior vena cava obstruction. J Neurosurg Spine 29:4524552018

    • Search Google Scholar
    • Export Citation
  • 12

    Mokri B: Klippel–Trenaunay–Weber syndrome (KTWS) and spontaneous spinal CSF leak: coincidence or link. Headache 54:7267312014

  • 13

    Mokri B: Spontaneous intracranial hypotension. Continuum (Minneap Minn) 21 (4 Headache):108611082015

  • 14

    Mulligan PRPrajapati HJMartin LGPatel TH: Vascular anomalies: classification, imaging characteristics and implications for interventional radiology treatment approaches. Br J Radiol 87:201303922014

    • Search Google Scholar
    • Export Citation
  • 15

    Schievink WI: Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 295:228622962006

  • 16

    Schievink WIMaya MMBarnard ZRMoser FGJean-Pierre SWaxman AD: Behavioral variant frontotemporal dementia as a serious complication of spontaneous intracranial hypotension. Oper Neurosurg (Hagerstown) 15:5055152018

    • Search Google Scholar
    • Export Citation
  • 17

    Schievink WIMaya MMJean-Pierre SNuño MPrasad RSMoser FG: A classification system of spontaneous spinal CSF leaks. Neurology 87:6736792016

    • Search Google Scholar
    • Export Citation
  • 18

    Schievink WIMaya MMMoser FG: False localizing signs of spinal CSF-venous fistulas in spontaneous intracranial hypotension: report of 2 cases. J Neurosurg Spine [epub ahead of print July 5 2019; DOI: 10.3171/2019.4.SPINE19283]

    • Search Google Scholar
    • Export Citation
  • 19

    Schievink WIMaya MMMoser FGJean-Pierre SNuño M: Coma: A serious complication of spontaneous intracranial hypotension. Neurology 90:e1638e16452018

    • Search Google Scholar
    • Export Citation
  • 20

    Schievink WIMaya MMMoser FGPrasad RSCruz RBNuño M: Lateral decubitus digital subtraction myelography to identify spinal CSF–venous fistulas in spontaneous intracranial hypotension. J Neurosurg Spine [epub ahead of print September 13 2019; DOI: 10.3171/2019.6.SPINE19487]

    • Search Google Scholar
    • Export Citation
  • 21

    Schievink WIMaya MMNuño M: Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: association with ventral spinal cerebrospinal fluid leaks: clinical article. J Neurosurg Spine 15:4334402011

    • Search Google Scholar
    • Export Citation
  • 22

    Schievink WIMoser FGMaya MM: CSF-venous fistula in spontaneous intracranial hypotension. Neurology 83:4724732014

  • 23

    Schievink WIMoser FGMaya MMPrasad RS: Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas. J Neurosurg Spine 24:9609642016

    • Search Google Scholar
    • Export Citation
  • 24

    Takai KNiimura MHongo HUmekawa MTeranishi AKayahara T: Disturbed consciousness and coma: diagnosis and management of intracranial hypotension caused by a spinal cerebrospinal fluid leak. World Neurosurg 121:e700e7112019

    • Search Google Scholar
    • Export Citation
  • 25

    Wicklund MRMokri BDrubach DABoeve BFParisi JEJosephs KA: Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD. Neurology 76:137713822011

    • Search Google Scholar
    • Export Citation
TrendMD
Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 373 373 201
Full Text Views 41 41 31
PDF Downloads 26 26 16
EPUB Downloads 0 0 0
PubMed
Google Scholar