Spontaneous Intracranial Hypotension: A Systematic Imaging Approach for CSF Leak Localization and Management Based on MRI and Digital Subtraction Myelography

OBJECTIVESpontaneous spinal CSF–venous fistulas are a distinct type of spinal CSF leak recently described in patients with spontaneous intracranial hypotension (SIH). Using digital subtraction myelography (DSM) with the patient in the prone position, the authors have been able to demonstrate such fistulas in about one-fifth of patients with SIH in whom conventional spinal imaging (MRI or CT myelography) showed no evidence for a CSF leak (i.e., the presence of extradural CSF). The authors compared findings of DSM with patients in the lateral decubitus position versus the prone position and now report a significantly increased yield of identifying spinal CSF–venous fistulas with this modification of their imaging protocol.METHODSThe population consisted of 23 patients with SIH who underwent DSM in the lateral decubitus position and 26 patients with SIH who underwent DSM in the prone position. None of the patients had evidence of a CSF leak on conventional spinal imaging.RESULTSA CSF–venous fistula was demonstrated in 17 (74%) of the 23 patients who underwent DSM in the lateral decubitus position compared to 4 (15%) of the 26 patients who underwent DSM in the prone position (p < 0.0001). The mean age of these 16 women and 5 men was 52.5 years (range 36–66 years).CONCLUSIONSAmong SIH patients in whom conventional spinal imaging showed no evidence of a CSF leak, DSM in the lateral decubitus position demonstrated a CSF–venous fistula in about three-fourths of patients compared to only 15% of patients when the DSM was performed in the prone position, an approximately five-fold increase in the detection rate. Spinal CSF–venous fistulas are not rare among patients with SIH.

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False localizing signs of spinal CSF–venous fistulas in spontaneous intracranial hypotension: report of 2 cases

Journal of Neurosurgery Spine ◽

10.3171/2019.4.spine19283 ◽

2019 ◽

Vol 31 (5) ◽

pp. 764-767 ◽

Cited By ~ 2

Author(s):

Wouter I. Schievink ◽

M. Marcel Maya ◽

Franklin G. Moser

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

The Other ◽

Ct Scans ◽

Csf Leak ◽

Digital Subtraction ◽

Glue Injection ◽

Venous Fistula ◽

Digital Subtraction Myelography ◽

Treatment Surgery

A spinal CSF–venous fistula is one of three specific types of spinal CSF leak that can be seen in patients with spontaneous intracranial hypotension (SIH). They are best demonstrated on specialized imaging, such as digital subtraction myelography (DSM) or dynamic myelography, but often they are diagnosed on the basis of increased contrast density in the draining veins (the so-called hyperdense paraspinal vein sign) on early postmyelography CT scans. The authors report on 2 patients who underwent directed treatment (surgery in one patient and glue injection in the other) based on the hyperdense paraspinal vein sign, in whom the actual site of the fistula did not correspond to the level or laterality of the hyperdense paraspinal vein sign. The authors suggest consideration of DSM or dynamic myelography prior to undertaking treatment directed at these fistulas.

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Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas

Journal of Neurosurgery Spine ◽

10.3171/2015.10.spine15855 ◽

2016 ◽

Vol 24 (6) ◽

pp. 960-964 ◽

Cited By ~ 24

Author(s):

Wouter I. Schievink ◽

Franklin G. Moser ◽

M. Marcel Maya ◽

Ravi S. Prasad

Keyword(s):

Intracranial Hypotension ◽

Patient Population ◽

Treatment Options ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Digital Subtraction ◽

Percutaneous Injection ◽

Spinal Imaging ◽

Venous Fistula ◽

Digital Subtraction Myelography

OBJECTIVE In most patients with spontaneous intracranial hypotension, a spinal CSF leak can be found, but occasionally, no leak can be demonstrated despite extensive spinal imaging. Failure to localize a CSF leak limits treatment options. The authors recently reported the discovery of CSF-venous fistulas in patients with spontaneous intracranial hypotension and now report on the use of digital subtraction myelography in patients with spontaneous intracranial hypotension but no CSF leak identifiable on conventional spinal imaging (i.e., non–digital subtraction myelography). METHODS The patient population consisted of 53 consecutive patients with spontaneous intracranial hypotension who underwent digital subtraction myelography but in whom no spinal CSF leak (i.e., presence of extradural CSF) was identifiable on conventional spinal imaging. RESULTS The mean age of the 33 women and 20 men was 53.4 years (range 29–71 years). A CSF-venous fistula was demonstrated in 10 (19%) of the 53 patients. A CSF-venous fistula was found in 9 (27%) of the 33 women and in 1 (5%) of the 20 men (p = 0.0697). One patient was treated successfully with percutaneous injection of fibrin sealant. Nine patients underwent surgery for the fistula. Surgery resulted in complete resolution of symptoms in 8 patients (follow-up 7–25 months), and in 1 patient, symptoms recurred after 4 months. CONCLUSIONS In this study, the authors found a CSF-venous fistula in approximately one-fifth of the patients with recalcitrant spontaneous intracranial hypotension but no CSF leak identifiable on conventional spinal imaging. The authors suggest that digital subtraction myelography be considered in this patient population.

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The importance of repeat digital subtraction myelography in the diagnosis of cryptogenic CSF-venous fistula causing spontaneous intracranial hypotension

Interventional Neuroradiology ◽

10.1177/1591019921991398 ◽

2021 ◽

pp. 159101992199139

Author(s):

Chinmay P Nagesh ◽

Rashmi Devaraj ◽

Girish Joshi ◽

Peerzada Shafi ◽

KN Krishna ◽

...

Keyword(s):

Intracranial Hypotension ◽

Morphological Changes ◽

False Negative ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Digital Subtraction ◽

Surgical Ligation ◽

Blood Patch ◽

Venous Fistula ◽

Digital Subtraction Myelography

Spontaneous intracranial hypotension (SIH) is a rare disorder that occurs secondary to acquired cerebrospinal fluid (CSF) leaks in the spine. Treatment involves either an epidural blood patch or surgical ligation. Essential to the selecting the optimal management strategy is classifying the type of leak and accurate localization of its level. Hitherto, this has been achieved using conventional imaging methods such as static CT or MR myelography which are adequate for the demonstration of only high flow leaks. Digital subtraction myelography (DSM) is a novel technique which provides superior temporal and spatial resolution in the localization of more challenging slow flow leaks. However, DSM may also be initially non-diagnostic. We report a case of SIH in which repeat DSM revealed a type 3 CSF-venous fistula and demonstrate a possible mechanism of transient CSF leak block resulting in the initial false negative findings based on morphological changes in the culprit nerve sheath diverticulum-pseudomeningocoele complex. The patient underwent successful surgical ligation with clinicoradiological resolution of SIH.

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Safety of Consecutive Bilateral Decubitus Digital Subtraction Myelography in Patients with Spontaneous Intracranial Hypotension and Occult CSF Leak

American Journal of Neuroradiology ◽

10.3174/ajnr.a6765 ◽

2020 ◽

Vol 41 (10) ◽

pp. 1953-1957

Author(s):

M.C. Pope ◽

C.M. Carr ◽

W. Brinjikji ◽

D.K. Kim

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Digital Subtraction ◽

Digital Subtraction Myelography

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Supine Digital Subtraction Myelography for the Demonstration of a Dorsal Cerebrospinal Fluid Leak in a Patient with Spontaneous Intracranial Hypotension: A Technical Note

Journal of Radiology Case Reports ◽

10.3941/jrcr.v6i9.1002 ◽

2012 ◽

Vol 6 (9) ◽

Cited By ~ 1

Author(s):

Michael Carstensen ◽

Navjot Chaudhary ◽

Andrew Leung ◽

Wai Ng

Keyword(s):

Cerebrospinal Fluid ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Cerebrospinal Fluid Leak ◽

Technical Note ◽

Digital Subtraction ◽

Digital Subtraction Myelography ◽

Fluid Leak

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Multiple Spinal CSF Leaks in Spontaneous Intracranial Hypotension. Do They Exist?

Neurology Clinical Practice ◽

10.1212/cpj.0000000000001084 ◽

2021 ◽

pp. 10.1212/CPJ.0000000000001084

Author(s):

Wouter Ingmar Schievink ◽

M. Marcel Maya ◽

Franklin Moser ◽

Ravi Prasad ◽

Vikram Wadhwa ◽

...

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Spinal Imaging ◽

Recent Group ◽

Using Data ◽

Digital Subtraction Myelography ◽

Type 3 ◽

Csf Leaks

AbstractObjective:To determine the frequency of multiple spinal CSF leaks in a recent group of patients with spontaneous intracranial hypotension (SIH) who were investigated with digital subtraction myelography (DSM).Methods:This observational study was conducted using data from a prospectively maintained data base of patients who meet the International Classification of Headache Disorders (ICHD)-III criteria for SIH. The patient population consisted of a consecutive group of 745 patients with SIH who underwent DSM between March 2009 and February 2020. Based on the results of DSM, participants were classified according to type and number of spinal CSF leaks.Results:Among 398 patients with SIH and extradural CSF on spinal imaging, multiplicity of CSF leaks was observed in none of 291 patients with type 1a ventral leaks and in four (6.2%) of 65 patients with type 1b (postero-) lateral leaks. Among 97 patients with SIH from spinal CSF-venous fistulas (type 3 leaks) who did not have extradural CSF on spinal imaging, nine patients (9.3%) had multiple fistulas (p<0.0001 for comparison between groups). Type 3 and type 1a or 1b CSF leaks coexisted in an additional five patients.Conclusions:Among patients with SIH, multiplicity of CSF leaks was observed radiographically in none of the patients with ventral leaks, in 6% of patients with lateral leaks, and in 9% of patients with CSF-venous fistulas. These results suggest that patients with SIH can be reassured that the occurrence of multiple CSF leaks is negligible to uncommon at most, depending on type of CSF leak.

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

Journal of Neurosurgery Spine ◽

10.3171/2019.8.spine19716 ◽

2020 ◽

Vol 32 (2) ◽

pp. 305-310 ◽

Cited By ~ 3

Author(s):

Wouter I. Schievink ◽

Marcel M. Maya ◽

Franklin G. Moser ◽

Alexander Tuchman ◽

Rachelle B. Cruz ◽

...

Keyword(s):

Soft Tissue ◽

Intracranial Hypotension ◽

Vascular Malformations ◽

Spontaneous Intracranial Hypotension ◽

Proximal Portion ◽

Csf Leak ◽

Venous Fistula

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.

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690 Intracranial Hypotension Due to CSF Leak Secondary to Ventral Thoracic Osteophyte Complex: Resolution After Transdural Thoracic Discectomy and Dural Repair

British Journal of Surgery ◽

10.1093/bjs/znab134.313 ◽

2021 ◽

Vol 108 (Supplement_2) ◽

Author(s):

B Kewlani ◽

I Hussain ◽

J Greenfield

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Bed Rest ◽

Structural Defects ◽

Csf Leak ◽

History Of ◽

Surgical Cure ◽

Thoracic Discectomy ◽

Herniated Discs

Abstract The hallmark symptom of spontaneous intracranial hypotension (SIH) is orthostatic headaches which manifests secondary to cerebrospinal fluid (CSF) hypovolaemia. Well-recognised aetiologies include trauma which includes procedures such as lumbar punctures and spinal surgery. More recently, structural defects such as bony osteophytes and calcified or herniated discs have been attributed to mechanically compromising dural integrity consequently resulting in CSF leak and symptom manifestation. A thorough literature review noted only a handful of such cases. We report the case of a thirty-two-year-old Asian female who presented with a one-month history of new-onset progressively worsening orthostatic headaches. Workup included MRI of the thoracic spine which revealed an epidural collection of CSF consequently prompting a dynamic CT-myelogram of the spine which not only helped to confirm severe cerebral hypotension but also suggested the underlying cause as being a dorsally projecting osteophyte-complex at level T2-3. Conservative and medical management including bed rest, analgesia, mechanical compression, and epidural blood patches failed to alleviate symptoms and a permanent surgical cure was eventually sought. The surgery involved T2-T3 laminectomy and osteophytectomy and at a 3-month follow-up, complete resolution of symptoms was noted.

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