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.

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.

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 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.

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

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.

Postural Headache In A Patient With Marfan's Syndrome

Cephalalgia ◽  
2003 ◽  
Vol 23 (7) ◽  
pp. 552-555 ◽  
Author(s):  
E Ferrante ◽  
A Citterio ◽  
A Savino ◽  
P Santalucia
Keyword(s):  
Intracranial Hypotension ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Postural Headache ◽  
Intracranial Hypotension Syndrome ◽  
Increased Risk ◽  
Diffuse Pachymeningeal Enhancement

A 26-year-old man with Marfan's syndrome had postural headache. Brain MRI with gadolinium showed diffuse pachymeningeal enhancement. MRI myelography revealed bilateral multiple large meningeal diverticula at sacral nerve roots level. He was suspected to have spontaneous intracranial hypotension syndrome. Eight days later headache improved with bed rest and hydration. One month after the onset he was asymptomatic and 3 months later brain MRI showed no evidence of diffuse pachymeningeal enhancement. The 1-year follow-up revealed no neurological abnormalities. The intracranial hypotension syndrome likely resulted from a CSF leak from one of the meningeal diverticula. In conclusion patients with spinal meningeal diverticula (frequently seen in Marfan's syndrome) might be at increased risk of developing CSF leaks, possibly secondary to Valsalva maneuver or minor unrecognizedtrauma.

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.

Spontaneous spinal cerebrospinal fluid leaks as the cause of subdural hematomas in elderly patients on anticoagulation

2010 ◽  
Vol 112 (2) ◽  
pp. 295-299 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Brian K. Pikul ◽  
Charles Louy
Keyword(s):  
Elderly Patients ◽  
Intracranial Hypotension ◽  
Medical Center ◽  
Spontaneous Intracranial Hypotension ◽  
The Elderly ◽  
Cortical Atrophy ◽  
Csf Leak ◽  
Subdural Hematomas ◽  
Maximal Diameter ◽  
Subdural Bleeding

Subdural hematoma is a relatively common complication of long-term anticoagulation, particularly in the elderly. The combination of anticoagulation and cerebral cortical atrophy is believed to be sufficient to explain the subdural bleeding. The authors report a series of elderly patients who were on a regimen of anticoagulation and developed chronic subdural hematomas (SDHs) due to a spontaneous spinal CSF leak. They reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension who were evaluated at Cedars-Sinai Medical Center. Among 141 patients with spontaneous spinal CSF leaks and spontaneous intracranial hypotension, 3 (2%) were taking anticoagulants at the time of onset of symptoms. The mean age of the 3 patients (1 woman and 2 men) was 74 years (range 68–86 years). All 3 patients had chronic SDHs measuring between 12 and 23 mm in maximal diameter. The SDHs resolved after treatment of the underlying spontaneous spinal CSF leak, and there was no need for hematoma evacuation. Epidural blood patches were used in 2 patients, and percutaneous placement of a fibrin sealant was used in 1 patient. The presence of an underlying spontaneous spinal CSF leak should be considered in patients with chronic SDHs, even among the elderly taking anticoagulants.

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