CC BY-NC-ND 4.0 · J Neurol Surg Rep 2020; 81(04): e59-e65
DOI: 10.1055/s-0040-1722268
Case Report

Spontaneous Intracranial Hypotension Complicated by Subdural Effusions Treated by Surgical Relief of Cranial Venous Outflow Obstruction

J Nicholas Higgins
1   Department of Radiology, Addenbrooke's Hospital, Cambridge, United Kingdom
,
Patrick R. Axon
2   Cambridge Skull Base Unit, Addenbrooke's Hospital, Cambridge, United Kingdom
,
Robert Macfarlane
3   Department of Neurosurgery, Addenbrooke's Hospital, Cambridge, United Kingdom
› Author Affiliations
Funding None.

Abstract

Spontaneous intracranial hypotension describes the clinical syndrome brought on by a cerebrospinal fluid (CSF) leak. Orthostatic headache is the key symptom, but others include nausea, vomiting, and dizziness, as well as cognitive and mood disturbance. In severe cases, the brain slumps inside the cranium and subdural collections develop to replace lost CSF volume. Initial treatment is by bed rest, but when conservative measures fail, attention is focused on finding and plugging the leak, although this can be very difficult and some patients remain bedbound for months or years. Recently, we have proposed an alternative approach in which obstruction to cranial venous outflow would be regarded as the driving force behind a chronic elevation of CSF pressure, which eventually causes dural rupture. Instead of focusing on the site of rupture, therefore, investigation and treatment can be directed at locating and relieving the obstructing venous lesion, allowing intracranial pressure to fall, and the dural defect to heal. The case we describe illustrates this idea. Moreover, since there was a graded clinical response to successive interventions relieving venous obstruction, and eventual complete resolution, it also provides an opportunity to consider particular symptoms in relation to cerebral venous insufficiency in its own right.



Publication History

Received: 10 July 2020

Accepted: 28 October 2020

Article published online:
31 December 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache 2013; 53 (07) 1034-1053
  • 2 Schievink WI, Maya MM, Jean-Pierre S, Nuño M, Prasad RS, Moser FG. A classification system of spontaneous spinal CSF leaks. Neurology 2016; 87 (07) 673-679
  • 3 Schievink WI, Deline CR. Headache secondary to intracranial hypotension. Curr Pain Headache Rep 2014; 18 (11) 457
  • 4 Urbach H. Intracranial hypotension: clinical presentation, imaging findings, and imaging-guided therapy. Curr Opin Neurol 2014; 27 (04) 414-424
  • 5 Kumar N, Diehn FE, Carr CM. et al. Spinal CSF venous fistula: a treatable etiology for CSF leaks in craniospinal hypovolemia. Neurology 2016; 86 (24) 2310-2312
  • 6 Mokri B. Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. Mayo Clin Proc 2002; 77 (11) 1241-1246
  • 7 Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006; 115 (07) 495-500
  • 8 Pérez MA, Bialer OY, Bruce BB, Newman NJ, Biousse V. Primary spontaneous cerebrospinal fluid leaks and idiopathic intracranial hypertension. J Neuroophthalmol 2013; 33 (04) 330-337
  • 9 Woodworth BA, Prince A, Chiu AG. et al. Spontaneous CSF leaks: a paradigm for definitive repair and management of intracranial hypertension. Otolaryngol Head Neck Surg 2008; 138 (06) 715-720
  • 10 Owler BK, Allan R, Parker G, Besser M. Pseudotumour cerebri, CSF rhinorrhoea and the role of venous sinus stenting in treatment. Br J Neurosurg 2003; 17 (01) 79-83
  • 11 King JO, Mitchell PJ, Thomson KR, Tress BM. Manometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology 2002; 58 (01) 26-30
  • 12 Higgins N, Trivedi R, Greenwood R, Pickard J. Brain slump caused by jugular venous stenoses treated by stenting: a hypothesis to link spontaneous intracranial hypotension with idiopathic intracranial hypertension. J Neurol Surg Rep 2015; 76 (01) e188-e193
  • 13 Higgins JN, Macfarlane R, Axon PR. et al. Headache, cerebrospinal fluid leaks, and pseudomeningoceles after resection of vestibular schwannomas: efficacy of venous sinus stenting suggests cranial venous outflow compromise as a unifying pathophysiological mechanism. J Neurol Surg B Skull Base 2019; 80 (06) 640-647
  • 14 Ray BS, Dunbar HS. Thrombosis of the dural venous sinuses as a cause of pseudotumor cerebri. Ann Surg 1951; 134 (03) 376-386
  • 15 King JO, Mitchell PJ, Thomson KR, Tress BM. Cerebral venography and manometry in idiopathic intracranial hypertension. Neurology 1995; 45 (12) 2224-2228
  • 16 Karahalios DG, Rekate HL, Khayata MH, Apostolides PJ. Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies. Neurology 1996; 46 (01) 198-202
  • 17 Higgins N, Pickard J, Lever A. What do lumbar puncture and jugular venoplasty say about a connection between chronic fatigue syndrome and idiopathic intracranial hypertension?. EJMINT Original Article 2014; 2014: 1448000223
  • 18 Dashti SR, Nakaji P, Hu YC. et al. Styloidogenic jugular venous compression syndrome: diagnosis and treatment: case report. Neurosurgery 2012; 70 (03) E795-E799
  • 19 Higgins JN, Garnett MR, Pickard JD, Axon PR. An evaluation of styloidectomy as an adjunct or alternative to jugular stenting in idiopathic intracranial hypertension and disturbances of cranial venous outflow. J Neurol Surg B Skull Base 2017; 78 (02) 158-163
  • 20 Zhou D, Ding JY, Ya JY. et al. Understanding jugular venous outflow disturbance. CNS Neurosci Ther 2018; 24 (06) 473-482
  • 21 Chazen JL, Talbott JF, Lantos JE, Dillon WP. MR myelography for identification of spinal CSF leak in spontaneous intracranial hypotension. AJNR Am J Neuroradiol 2014; 35 (10) 2007-2012
  • 22 Akbar JJ, Luetmer PH, Schwartz KM, Hunt CH, Diehn FE, Eckel LJ. The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol 2012; 33 (03) 535-540
  • 23 Hoxworth JM, Trentman TL, Kotsenas AL, Thielen KR, Nelson KD, Dodick DW. The role of digital subtraction myelography in the diagnosis and localization of spontaneous spinal CSF leaks. AJR Am J Roentgenol 2012; 199 (03) 649-653
  • 24 Schievink WI, Maya MM, Moser FG. Digital subtraction myelography in the investigation of post-dural puncture headache in 27 patients: technical note. J Neurosurg Spine 2017; 26 (06) 760-764
  • 25 Luetmer PH, Mokri B. Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol 2003; 24 (08) 1711-1714
  • 26 Zhang X, Li C, Wang Q. Stent placement for treatment of internal jugular vein stenosis: report of 3 cases. Int J Clin Exp Med 2017; 10 (09) 13881-13887
  • 27 Zhou D, Meng R, Zhang X. et al. Intracranial hypertension induced by internal jugular vein stenosis can be resolved by stenting. Eur J Neurol 2018; 25 (02) 365-e13
  • 28 Li M, Sun Y, Chan CC, Fan C, Ji X, Meng R. Internal jugular vein stenosis associated with elongated styloid process: five case reports and literature review. BMC Neurol 2019; 19 (01) 112
  • 29 Michel P, Angelillo-Scherrer A, Maeder P, Binaghi S, Meyer IA, Correia P. Stenting as a treatment for exercise-induced intracranial hypertension from bilateral jugular vein obstruction. Neurol Clin Pract 2016; 6 (02) e10-e13
  • 30 Brinjikji W, Graffeo CS, Perry A. et al. Moving target: transient rotsational stenosis precipitating jugular bow hunter's syndrome. J Neurointerv Surg 2017; 9 (07) e28