scholarly journals Spontaneous Intracranial Hypotension Without CSF Leakage—Concept of a Pathological Cranial to Spinal Fluid Shift

2021 ◽  
Vol 12 ◽  
Author(s):  
Johannes Goldberg ◽  
Levin Häni ◽  
Christopher Marvin Jesse ◽  
Irena Zubak ◽  
Eike I. Piechowiak ◽  
...  
Keyword(s):  
Intracranial Hypotension ◽  
Venous Pressure ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Imaging Findings ◽  
Dural Sac ◽  
Csf Leakage ◽  
Medical Hypothesis ◽  
Orthostatic Symptoms ◽  
Venous Fistula

Objective: Spontaneous intracranial hypotension (SIH) is typically caused by CSF leakage from a spinal dural tear, a meningeal diverticulum, or a CSF venous fistula. However, some patients present with classic orthostatic symptoms and typical intracranial imaging findings without evidence of CSF leakage despite repeated diagnostic work-up. This article aims to elaborate a hypothesis that would explain a pathologically increased orthostatic shift of CSF from the cranial to the spinal compartment in the absence of a CSF leak.Medical Hypothesis: The symptoms of SIH are caused by a decrease in intracranial CSF volume, intracranial hypotension, and downward displacement of intracranial structures. A combination of pathologically increased spinal compliance, decreased intracranial CSF volume, low CSF outflow resistance, and decreased venous pressure might result in a pathological orthostatic cranial-to-spinal CSF shift. Thus, in rare cases, intracranial hypotension may occur in the absence of CSF leakage from the dural sac.Conclusion: We propose a pathophysiological concept for the subgroup of SIH patients with typical cranial imaging findings and no evidence of CSF leakage. In these patients, reducing the compliance or the volume of the spinal compartment seems to be the appropriate therapeutic strategy.

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

2020 ◽  
Vol 32 (2) ◽  
pp. 305-310 ◽  
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.

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.

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.

Severe acute orthostatic headache: spontaneous intracranial hypotension (SIH)

2021 ◽  
Vol 14 (6) ◽  
pp. e243179
Author(s):  
Pushpendra Nath Renjen ◽  
Dinesh Mohan Chaudhari ◽  
Nidhi Goyal ◽  
Kamal Ahmed
Keyword(s):  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Signs And Symptoms ◽  
Csf Leak ◽  
Lumbosacral Spine ◽  
Ct Guided ◽  
Mri Features ◽  
Blood Patch ◽  
Csf Leakage ◽  
Underlying Mechanisms

The most common cause of spontaneous intracranial hypotension headache is a cerebrospinal fluid (CSF) leakage, but the underlying mechanisms remain unknown. Intracranial hypotension is characterised by diffuse pachymeningeal enhancement on cranial MRI features, low CSF pressure and orthostatic headaches mostly caused by the dural puncture. We report a 31-year-old woman who presented to our services with reports of continuous severe bifrontal headache, which increased on sitting up and resolved on lying down. MRI of the cervical and lumbosacral spine showed signs of CSF leak; hence, patient was diagnosed with spontaneous intracranial hypotension headache. A CT-guided epidural blood patch was done at L4–5 with fibrin glue injected at the site of leak. The patient’s signs and symptoms improved after the procedure.

Spontaneous Intracranial Hypotension and MRI

1998 ◽  
Vol 11 (2) ◽  
pp. 203-206
Author(s):  
I. Muras ◽  
A. Scuotto ◽  
M. Maisto ◽  
F.P. Bernini
Keyword(s):  
Intracranial Hypotension ◽  
Medical Condition ◽  
Spontaneous Intracranial Hypotension ◽  
Bed Rest ◽  
Csf Leak ◽  
Meningeal Enhancement ◽  
Csf Leakage ◽  
Downward Displacement ◽  
Fluid Collections ◽  
The Brain

Postural headache due to low intracranial pressure is a well-known entity and is most commonly encountered following lumbar puncture. It may occur as a consequence of a medical condition (dehydratation, uremia, etc.) but in some cases no precipitating event is apparent and the intracranial hypotension is believed to have developed spontaneously. In such cases the underlying cause of the syndrome is rarely established and treatment is non specific. We describe three patients with spontaneous intracranial hypotension examined with MRI of the brain. Women are more commonly affected than men in the third or fourth decades of life. Schaltenbrand (1938) proposed three mechanisms by which spontaneous intracranial hypotension may be explained: diminished CSF production; CSF hyperabsorption, CSF leakage. The defect causing a CSF leak usually remains obscure. Several cases of diffuse meningeal enhancement on MRI have recently been described, probably due to meningeal hyperaemia resulting from the low CSF pressure. Subdural fluid collections have also been detected as a result of rupture of bridging veins due to the decrease in CSF volume and downward displacement of the brain. In our cases, MRI showed a diffuse dural thickening, hyperintense in T2. Spontaneous intracranial hypotension is often a self-limiting disease, responding well to bed rest and a generous intake of oral or parenteral fluid and salt.

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.

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.

scholarly journals Radioisotope Cisternography: A Potentially Useful Tool for Headache Diagnosis in Patients with an IDD Pump

2014 ◽  
Vol 17;1 (1;17) ◽  
pp. E89-E94 ◽  
Author(s):  
Dr. Jose De Andres
Keyword(s):  
Drug Delivery ◽  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Chronic Headache ◽  
Spontaneous Intracranial Hypotension ◽  
Diagnostic Technique ◽  
Csf Leak ◽  
Cerebrospinal Fluid Leakage ◽  
Common Procedure ◽  
Csf Leakage

Background: Spontaneous intracranial hypotension (SIH) and related headache after intrathecal pump implantation is a possibility. Knowing the exact location of the cerebrospinal fluid (CSF) leak is mandatory for managing with interventional therapies. Objectives: Radioisotope cisternography (RICG) is a common procedure in nuclear medicine and may be used as an additional procedure in the differential diagnosis of headaches due to intracranial liquor hypotension syndrome caused by microleakage of CSF. Study Design: We report an imaging case of complex chronic headache in a patient with an implanted drug delivery pump. The symptomatology of the patient was considered typical of spontaneous intracranial hypotension (SIH). Therefore, the initial suspicion was the loss of CSF due to the implanted pump catheter. The negativity of the routine headache test can exclude any leakage of CSF at the site of catheter insertion. Setting: Anesthesia, Critical Care, and Multidisciplinary Pain Management Department, General University General Hospital, Valencia, Spain. Results: Many patients are treated with implanted intrathecal infusion systems. In these patients, any neurological symptoms are readily attributed to the invasive neuroaxial system, especially severe cephalalgia. RICG was helpful in this particular case to make a diagnosis of exclusion of the diagnosis of SIH due to CSF leakage. Conclusion: RICG may be considered as an additional diagnostic technique in patients with chronic pain and implanted spinal infusion devices for whom standard detection procedures have been exhausted. Key words: Intrathecal drug delivery (IDD), chronic headache (CH), radiosotope cisternography (RICG), cerebrospinal fluid leakage, spontaneous intracranial hypotension (SIH)

scholarly journals Intrathecal preservative-free normal saline challenge magnetic resonance myelography for the identification of cerebrospinal fluid leaks in spontaneous intracranial hypotension

2015 ◽  
Vol 123 (3) ◽  
pp. 732-736 ◽  
Author(s):  
Julius Griauzde ◽  
Joseph J. Gemmete ◽  
Aditya S. Pandey ◽  
Neeraj Chaudhary
Keyword(s):  
Normal Saline ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Case Series ◽  
Csf Leak ◽  
Csf Leakage ◽  
Leakage Site ◽  
Safe Technique ◽  
Preservative Free ◽  
Mr Myelography

OBJECT A CSF leak can be difficult to locate in patients who present with spontaneous intracranial hypotension (SIH). The purpose of this case series was to describe the authors’ experience with intrathecal preservative-free normal saline challenge coupled with contrast-enhanced MR myelography (CEMRM), which was used to provoke and detect a CSF leakage site in patients with SIH. METHODS The authors performed a retrospective review of the records of patients who underwent preservative-free normal saline challenge followed by intrathecal gadolinium (Gd) contrast infusion and MR myelography from 2010 to 2012. RESULTS The records survey identified 5 patients who underwent 6 procedures. Intrathecal preservative-free normal saline challenge followed by CEMRM identified a CSF leak during 5 of the 6 procedures. Previous CT myelograms were available from 4 patients, which did not reveal a leakage site. A CT myelogram of 1 patient showed a single leak, but the authors’ saline challenge-CEMRM technique identified multiple additional leakage sites. Three patients exhibited transient postprocedural symptoms related to the saline infusion, but no long-term or permanent adverse effects related to the procedure were observed. CONCLUSIONS Instillation of preservative-free normal saline into the thecal sac followed by intrathecal Gd infusion is a safe technique that may increase the detection of a CSF leak on MR myelography images in patients with SIH.

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