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

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.

Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks

2012 ◽  
Vol 116 (4) ◽  
pp. 749-754 ◽  
Author(s):  
Wouter I. Schievink ◽  
Marc S. Schwartz ◽  
M. Marcel Maya ◽  
Franklin G. Moser ◽  
Todd D. Rozen
Keyword(s):  
Skull Base ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Csf Rhinorrhea ◽  
Spinal Imaging ◽  
Radiological Data ◽  
Single Instance ◽  
Csf Leakage ◽  
Csf Leaks

Object Spontaneous intracranial hypotension is an important cause of headaches and an underlying spinal CSF leak can be demonstrated in most patients. Whether CSF leaks at the level of the skull base can cause spontaneous intracranial hypotension remains a matter of controversy. The authors' aim was to examine the frequency of skull base CSF leaks as the cause of spontaneous intracranial hypotension. Methods Demographic, clinical, and radiological data were collected from a consecutive group of patients evaluated for spontaneous intracranial hypotension during a 9-year period. Results Among 273 patients who met the diagnostic criteria for spontaneous intracranial hypotension and 42 who did not, not a single instance of CSF leak at the skull base was encountered. Clear nasal drainage was reported by 41 patients, but a diagnosis of CSF rhinorrhea could not be established. Four patients underwent exploratory surgery for presumed CSF rhinorrhea. In addition, the authors treated 3 patients who had a postoperative CSF leak at the skull base following the resection of a cerebellopontine angle tumor and developed orthostatic headaches; spinal imaging, however, demonstrated the presence of a spinal source of CSF leakage in all 3 patients. Conclusions There is no evidence for an association between spontaneous intracranial hypotension and CSF leaks at the level of the skull base. Moreover, the authors' study suggests that a spinal source for CSF leakage should even be suspected in patients with orthostatic headaches who have a documented skull base CSF leak.

Lateral decubitus digital subtraction myelography to identify spinal CSF–venous fistulas in spontaneous intracranial hypotension

2019 ◽  
Vol 31 (6) ◽  
pp. 902-905 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Franklin G. Moser ◽  
Ravi S. Prasad ◽  
Rachelle B. Cruz ◽  
...  
Keyword(s):  
Prone Position ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Lateral Decubitus Position ◽  
Csf Leak ◽  
Digital Subtraction ◽  
Spinal Imaging ◽  
Lateral Decubitus ◽  
Venous Fistula ◽  
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.

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

2016 ◽  
Vol 24 (6) ◽  
pp. 960-964 ◽  
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.

scholarly journals Intracranial Hypotension for Anesthesiologists: What We Should Know

2019 ◽  
pp. 69-76
Author(s):  
Maria Eugenia Calvo
Keyword(s):  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Case Series ◽  
Low Flow ◽  
Csf Leak ◽  
Common Denominator ◽  
Invasive Treatment ◽  
Blood Patch ◽  
Mechanism Of Trauma ◽  
Csf Leaks

The common denominator of spontaneous intracranial hypotension (SIH), postsurgical cerebral spinal fluid (CSF) leaks, and postpuncture headache (PPH) is a decrease in CSF volume. The typical presentation is orthostatic headaches, but atypical headaches can be difficult to diagnose and challenging to treat. Management is based on clinical suspicion and characterization of the headache, followed by imaging (noninvasive or invasive). Treatment ranges from conservative to different modalities of epidural blood patches, fibrin glue injections, or surgical exploration and repair. We report 5 cases with great variation in clinical and radiological presentations. Two cases of SIH involved difficult diagnosis and treatment, 2 others featured postsurgical high-flow CSF leaks, and one case presented with a low-flow CSF leak that needed closer evaluation in relation to hardware manipulation. In all cases, recommendations for diagnosis and management of intracranial hypotension were followed, even though in 3 cases the mechanism of trauma was not related to spontaneous hypotension. All cases of headache were resolved. The actual recommendations for SIH are very effective for PPH and postsurgical CSF leaks. With this case series, we illustrate how anatomical and clinical considerations are paramount in choosing appropriate imaging modalities and clinical management. Key words: CSF leak, epidural blood patch, intracranial hypotension, postural headaches, subdural hematomas

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

2019 ◽  
Vol 31 (5) ◽  
pp. 764-767 ◽  
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.

scholarly journals Spontaneous Intracranial Hypotension Treated with a Targeted CT-Guided Epidural Blood Patch

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Inês Correia ◽  
Inês Brás Marques ◽  
Rogério Ferreira ◽  
Miguel Cordeiro ◽  
Lívia Sousa
Keyword(s):  
Intracranial Hypotension ◽  
Epidural Blood Patch ◽  
Spontaneous Intracranial Hypotension ◽  
Complete Recovery ◽  
Csf Leak ◽  
Ct Guided ◽  
Blood Patch ◽  
History Of ◽  
Persistent Headache ◽  
Csf Leaks

Spontaneous intracranial hypotension (SIH) is an important cause of new daily persistent headache. It is thought to be due to spontaneous spinal cerebrospinal fluid (CSF) leaks, which probably have a multifactorial etiology. The classic manifestation of SIH is an orthostatic headache, but other neurological symptoms may be present. An epidural blood patch is thought to be the most effective treatment, but a blind infusion may be ineffective. We describe the case of a young man who developed an acute severe headache, with pain worsening when assuming an upright posture and relief gained with recumbency. No history of previous headache, recent cranial or cervical trauma, or invasive procedures was reported. Magnetic resonance imaging showed pachymeningeal enhancement and other features consistent with SIH and pointed towards a cervical CSF leak site. After failure of conservative treatment, a targeted computer tomography-guided EBP was performed, with complete recovery.

Imaging of the Spontaneous Low Cerebrospinal Fluid Pressure Headache: A Review

2020 ◽  
Vol 71 (2) ◽  
pp. 174-185 ◽  
Author(s):  
P. Martineau ◽  
S. Chakraborty ◽  
Khunsa Faiz ◽  
Jai Shankar
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Fluid Pressure ◽  
Spontaneous Intracranial Hypotension ◽  
Nuclear Imaging ◽  
Csf Leak ◽  
Resonance Imaging ◽  
Pressure Headache ◽  
Csf Leaks

Spontaneous intracranial hypotension (SIH) is a significant cause of chronic, postural headaches. Spontaneous intracranial hypotension is generally believed to be associated with cerebrospinal fluid (CSF) leaks, and these leaks can be posttraumatic, iatrogenic, or idiopathic in origin. An integral part of the management of patients with this condition consists of localizing and stopping the leaks. Radiologists play a central role in the workup of this condition detecting leaks using computed tomography, magnetic resonance imaging, or nuclear imaging. In this article, we briefly review SIH and the various imaging modalities, which can be used to identify and localize a spontaneous CSF leak.

Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

1996 ◽  
Vol 84 (4) ◽  
pp. 598-605 ◽  
Author(s):  
Wouter I. Schievink ◽  
Fredric B. Meyer ◽  
John L. D. Atkinson ◽  
Bahram Mokri
Keyword(s):  
Cerebrospinal Fluid ◽  
Thoracic Spine ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Cervicothoracic Junction ◽  
Ct Myelography ◽  
Content Type ◽  
Fine Print ◽  
Csf Leaks

✓ Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients. The mean age of the six women and five men included in the study was 38 years (range 22–51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age. In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.

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

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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