Epidural venous engorgement resulting in progressive cervical myelopathy from shunt-related intracranial hypotension

Case report and review of the literature

View More View Less
  • 1 Department of Neurosurgery, University of Utah School of Medicine, Primary Children’s Medical Center, Salt Lake City, Utah
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00
Print or Print + Online

✓The authors report an unusual case of engorged epidural veins causing progressive cervical myelopathy after long-term cerebrospinal fluid (CSF) shunt therapy and intracranial hypotension. An 18-year-old woman, who had previously undergone shunt placement with a distal slit valve for a porencephalic cyst when 2 years of age, presented with progressive spastic quadriparesis, numbness, and gait difficulty. Postural headaches were absent and a lumbar puncture revealed low CSF pressure. Neuroimaging disclosed markedly engorged anterior epidural veins causing compression of the cervical spinal cord. The slit-valve shunt system was surgically removed and an external drain was placed. The patient’s CSF pressure was gradually raised to clinically tolerable levels. Once the optimal pressure was identified, a programmable shunt was placed with the valve set at the same level. The patient’s neurological status improved, and the epidural veins had returned to their normal size on follow-up imaging. The authors describe the unique treatment strategy used in this patient and review the literature on epidural venous engorgement as it relates to intracranial hypotension.

Abbreviations used in this paper:CSF = cerebrospinal fluid; CT = computed tomography; MR = magnetic resonance; SIH = spontaneous intracranial hypotension.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

Contributor Notes

Address reprint requests to: Douglas L. Brockmeyer, M.D., Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Utah School of Medicine, Primary Children’s Medical Center, 100 North Medical Drive, Salt Lake City, Utah 84113. email: douglas.brockmeyer@hsc.utah.edu.
  • 1

    Agrawal D, & Durity FA: Seizure as a manifestation of intracranial hypotension in a shunted patient. Pediatr Neurosurg 42:165167, 2006

  • 2

    Albayram S, , Wasserman BA, , Yousem DM, & Wityk R: Intracranial hypotension as a cause of radiculopathy from cervical epidural venous engorgement: case report. AJNR Am J Neuroradiol 23:618621, 2002

    • Search Google Scholar
    • Export Citation
  • 3

    Alvarez-Linera J, , Escribano J, , Benito-León J, , Porta-Etessam J, & Rovira A: Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology 55:18951897, 2000

    • Search Google Scholar
    • Export Citation
  • 4

    Aoki N, , Sakai T, & Oikawa A: Spontaneous intracranial hypotension associated with subdural hematoma: diagnostic usefulness of percutaneous subdural tapping and magnetic resonance imaging. Acta Neurochir (Wien) 140:4750, 1998

    • Search Google Scholar
    • Export Citation
  • 5

    Arai M, , Takada T, & Nozue M: Orthostatic tinnitus: an otological presentation of spontaneous intracranial hypotension. Auris Nasus Larynx 30:8587, 2003

    • Search Google Scholar
    • Export Citation
  • 6

    Arnautović KI, , al-Mefty O, , Pait TG, , Krisht AF, & Husain MM: The suboccipital cavernous sinus. J Neurosurg 86:252262, 1997

  • 7

    Bakshi R, , Mechtler LL, , Kamran S, , Gosy E, , Bates VE, & Kinkel PR, : MRI findings in lumbar puncture headache syndrome: abnormal dural-meningeal and dural venous sinus enhancement. Clin Imaging 23:7376, 1999

    • Search Google Scholar
    • Export Citation
  • 8

    Blank SC, , Shakir RA, , Bindoff LA, & Bradey N: Spontaneous intracranial hypotension: clinical and magnetic resonance imaging characteristics. Clin Neurol Neurosurg 99:199204, 1997

    • Search Google Scholar
    • Export Citation
  • 9

    Burtis MT, , Ulmer JL, , Miller GA, , Barboli AC, , Koss SA, & Brown WD: Intradural spinal vein enlargement in craniospinal hypotension. AJNR Am J Neuroradiol 26:3438, 2005

    • Search Google Scholar
    • Export Citation
  • 10

    Carrero EJ, , Agusti M, , Fabregas N, , Valldeoriola F, & Fernandez C: Unilateral trigeminal and facial nerve palsies associated with epidural analgesia in labor. Can J Anaesth 45:893897, 1998

    • Search Google Scholar
    • Export Citation
  • 11

    Chen CJ, , Lee TH, , Hsu HL, , Tseng YC, , Wong YC, & Wang LJ: Spinal MR findings in spontaneous intracranial hypotension. Neuroradiology 44:9961003, 2002

    • Search Google Scholar
    • Export Citation
  • 12

    Chiapparini L, , Ciceri E, , Nappini S, , Castellani MR, , Mea E, & Bus-sone G, : Headache and intracranial hypotension: neuroradiological findings. Neurol Sci 25:3 Suppl S138S141, 2004

    • Search Google Scholar
    • Export Citation
  • 13

    Chiapparini L, , Farina L, , D’Incerti L, , Erbetta A, , Pareyson D, & Carriero M, : Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology 44:143150, 2002

    • Search Google Scholar
    • Export Citation
  • 14

    Christoforidis GA, , Mehta BA, , Landi JL, , Czarnecki EJ, & Pias-kowski RA: Spontaneous intracranial hypotension: report of four cases and review of the literature. Neuroradiology 40:636643, 1998

    • Search Google Scholar
    • Export Citation
  • 15

    Clarot F, , Callonnec F, , Douvrin F, , Hannequin D, , Simonet J, & Proust B, : Giant cervical epidural veins after lumbar puncture in a case of intracranial hypotension. AJNR Am J Neuroradiol 21:787789, 2000

    • Search Google Scholar
    • Export Citation
  • 16

    Evan RW, & Mokri B: Spontaneous intracranial hypotension resulting in coma. Headache 42:159160, 2002

  • 17

    Förderreuther S, , Yousry I, , Empl M, & Straube A: Dilated cervical epidural veins and extra arachnoid fluid collection in orthostatic headaches. Neurology 57:527529, 2001

    • Search Google Scholar
    • Export Citation
  • 18

    Inoue T, , Cohen-Gadol AA, & Krauss WE: Low-pressure headaches and spinal cord herniation. Case report. J Neurosurg 98:1 Suppl 9395, 2003

  • 19

    Miyazaki T, , Chiba A, , Nishina H, , Uesaka Y, , Nakase H, & Kana-zawa I: Upper cervical myelopathy associated with low CSF pressure: a complication of ventriculoperitoneal shunt. Neurology 50:18641866, 1998

    • Search Google Scholar
    • Export Citation
  • 20

    Miyazawa K, , Shiga Y, , Hasegawa T, , Endoh M, , Okita N, & Higano S, : CSF hypovolemia vs intracranial hypotension in “spontaneous intracranial hypotension syndrome. Neurology 60:941947, 2003

    • Search Google Scholar
    • Export Citation
  • 21

    Moayeri NN, , Henson JW, , Schaefer PW, & Zervas NT: Spinal du-ral enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. J Neurosurg 88:912918, 1998

    • Search Google Scholar
    • Export Citation
  • 22

    Mokri B: The Monro–Kellie hypothesis: applications in CSF volume depletion. Neurology 56:17461748, 2001

  • 23

    Mokri B: Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia—evolution of a concept. Mayo Clin Proc 74:11131123, 1999

    • Search Google Scholar
    • Export Citation
  • 24

    Mokri B: Spontaneous intracranial hypotension. Curr Pain Headache Rep 5:284291, 2001

  • 25

    Mokri B: Spontaneous low cerebrospinal pressure/volume headaches. Curr Neurol Neurosci Rep 4:117124, 2004

  • 26

    Nakahara K, , Iida H, , Mitomi T, , Osawa S, , Utsuki S, & Shimizu S, : Dilation of spinal epidural veins caused by spontaneous intracranial hypotension. Case report. Neurol Med Chir 45:404406, 2005

    • Search Google Scholar
    • Export Citation
  • 27

    Nowak DA, , Rodiek SO, , Zinner J, , Guhlmann A, & Topka H: Broadening the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia. Case report. J Neurosurg 98:903907, 2003

    • Search Google Scholar
    • Export Citation
  • 28

    O’Carroll CP, & Brant-Zawadzki M: The syndrome of spontaneous intracranial hypotension. Cephalalgia 19:8087, 1999

  • 29

    Pakiam ASI, , Lee C, & Lang AE: Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol 56:869872, 1999

  • 30

    Paldino M, , Mogilner AY, & Tenner MS: Intracranial hypotension syndrome: a comprehensive review. Neurosurg Focus 15:6 ECP2, 2003

  • 31

    Rabin BM, , Roychowdhury S, , Meyer JR, , Cohen BA, , LaPat KD, & Russell EJ: Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol 19:10341039, 1998

    • Search Google Scholar
    • Export Citation
  • 32

    Sharma P, , Sharma A, & Chacko AG: Syringomyelia in spontaneous intracranial hypotension. Case report. J Neurosurg 95:905908, 2001

  • 33

    Wingerchuk DM, , Patel NP, , Patel AC, , Dodick DW, & Nelson KD: Progressive cervical myelopathy secondary to chronic ventricu-loperitoneal CSF overshunting. Neurology 65:171172, 2005

    • Search Google Scholar
    • Export Citation
  • 34

    Yousry I, , Förderreuther S, , Moriggl B, , Holtmannspötter M, , Naidich TP, & Straube A, : Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol 22:12391250, 2001

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 929 374 44
Full Text Views 252 77 8
PDF Downloads 200 28 0
EPUB Downloads 0 0 0