Spontaneous Intracranial Hypotension With Brain Sagging Attributable to a Cerebrospinal Fluid–Venous Fistula

2020 ◽  
Vol 77 (10) ◽  
pp. 1320
Author(s):  
Vinil N. Shah ◽  
William P. Dillon
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Brain Sagging ◽  
Venous Fistula

Transvenous embolization of a cerebrospinal fluid–venous fistula for the treatment of spontaneous intracranial hypotension

2021 ◽  
pp. neurintsurg-2021-018160
Author(s):  
Nicholas Borg ◽  
Soliman Oushy ◽  
Luis Savastano ◽  
Waleed Brinjikji
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Spinal Nerve ◽  
Transvenous Embolization ◽  
Neural Foramen ◽  
Content Type ◽  
Venous Fistula ◽  
The Right ◽  
Video Player

Cerebrospinal fluid–venous fistula is an increasingly recognized cause of spontaneous intracranial hypotension.1 The site of the leak is between the dural sleeve around a spinal nerve root and the surrounding foraminal veins. In appropriately investigated patients, transvenous embolization of the draining foraminal and paraspinal veins has been shown to be an effective way of treating the disease, with low periprocedural morbidity, improvement in symptoms and radiological appearances.2 Video 1 shows the technique employed in a typical case using Onyx (Medtronic, Minnesota, USA) to embolize a CSF–venous fistula at the right T10 neural foramen.Video 1

Spontaneous intracranial hypotension presenting with progressive cognitive decline

2021 ◽  
Vol 14 (7) ◽  
pp. e241285
Author(s):  
Sanaz Shoja Gharehbagh ◽  
Birthe Krogh Rasmussen ◽  
Emil Smilkov ◽  
Rigmor Højland Jensen
Keyword(s):  
Intracranial Hypotension ◽  
Brain Mri ◽  
Fluid Collection ◽  
Spontaneous Intracranial Hypotension ◽  
Pituitary Tumour ◽  
Normal Brain ◽  
Thoracic Level ◽  
Mri Scans ◽  
Brain Sagging ◽  
Venous Fistula

A 63-year-old woman presented with headache, progressive somnolence, neurocognitive decline and urinary incontinence through a year. Medical history was unremarkable except for hypertension and hypercholesterolaemia. Neurological examination was normal. Brain MRI showed findings typical for spontaneous intracranial hypotension (subdural fluid collection, pachymeningeal enhancement, brain sagging) and pituitary tumour. The patient’s complaints improved dramatically but temporarily after treatment with each of repeated targeted as well as non-targeted blood patches and a trial with continuous intrathecal saline infusion. Extensive work up including repeated MRI-scans, radioisotope cisternographies, CT and T2-weighted MR myelography could not localise the leakage, but showed minor root-cysts at three levels. Finally, lateral decubitus digital subtraction dynamic myelography with subsequent CT myelography identified a tiny dural venous fistula at the fourth thoracic level. After surgical venous ligation, the patient fully recovered. Awareness of spontaneous dural leaks and their heterogeneous clinical picture are important and demands an extensive workup.

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.

Brain volume changes in spontaneous intracranial hypotension: Revisiting the Monro-Kellie doctrine

Cephalalgia ◽  
2020 ◽  
pp. 033310242095038
Author(s):  
Jr-Wei Wu ◽  
Yen-Feng Wang ◽  
Shu-Shya Hseu ◽  
Shu-Ting Chen ◽  
Yung-Lin Chen ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Brain Tissue ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Fluid Volume ◽  
Tissue Volume ◽  
Fixed Constant ◽  
The Brain ◽  
Cerebrospinal Fluid Volume

Objectives In the application of the Monro-Kellie doctrine in spontaneous intracranial hypotension, the brain tissue volume is generally considered as a fixed constant. Traditionally, cerebral venous dilation is thought to compensate for decreased cerebrospinal fluid. However, whether brain tissue volume is invariable has not yet been explored. The objective of this study is to evaluate whether brain tissue volume is fixed or variable in spontaneous intracranial hypotension patients using automatic quantitative methods. Methods This retrospective and longitudinal study analyzed spontaneous intracranial hypotension patients between 1 January 2007 and 31 July 2015. Voxel-based morphometry was used to examine brain volume changes during and after the resolution of spontaneous intracranial hypotension. Brain structure volume was analyzed using Statistical Parametric Mapping version 12 and FMRIB Software Library v6.0. Post-treatment neuroimages were used as surrogate baseline measures. Results Forty-four patients with spontaneous intracranial hypotension were analyzed (mean [standard deviation] age, 37.8 [8.5] years; 32 female and 12 male). The whole brain tissue volume was decreased during spontaneous intracranial hypotension compared to follow-up (1180.3 [103.5] mL vs. 1190.4 [93.1] mL, difference: −10.1 mL [95% confidence interval: −18.4 to −1.8 mL], p = 0.019). In addition, ventricular cerebrospinal fluid volume was decreased during spontaneous intracranial hypotension compared to follow-up (15.8 [6.1] mL vs. 18.9 [6.9] mL, difference: −3.2 mL [95% confidence interval: −4.5 to −1.8 mL], p < 0.001). Longer anterior epidural cerebrospinal fluid collections, as measured by number of vertebral segments, were associated with greater reduction of ventricular cerebrospinal fluid volume (Pearson’s r = −0.32, p = 0.036). Conclusion The current study found the brain tissue volume and ventricular cerebrospinal fluid are decreased in spontaneous intracranial hypotension patients. The change in ventricular cerebrospinal fluid volume, but not brain tissue volume change, was associated with the severity of spinal cerebrospinal fluid leakage. These results challenge the assumption that brain tissue volume is a fixed constant.

Spontaneous Spinal Cerebrospinal Fluid Leaks

Cephalalgia ◽  
2008 ◽  
Vol 28 (12) ◽  
pp. 1345-1356 ◽  
Author(s):  
WI Schievink
Keyword(s):  
Cerebrospinal Fluid ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Epidural Blood Patching ◽  
Delay In Diagnosis ◽  
Facial Numbness ◽  
Cerebrospinal Fluid Leaks ◽  
Brain Sagging ◽  
Glue Injection ◽  
Fluid Collections

Spontaneous intracranial hypotension is an uncommon but not rare cause of new onset daily persistent headaches. A delay in diagnosis is the norm. Women are affected more commonly than men and most are in the fifth or sixth decade of life. The underlying cause is a spontaneous spinal cerebrospinal fluid (CSF) leak. Typically the headache is orthostatic in nature but other headache patterns occur as well. Associated symptoms are common and include neck pain, a change in hearing, diplopia, facial numbness, cognitive abnormalities and even coma. Typical imaging findings consist of subdural fluid collections, pachymeningeal enhancement, pituitary hyperaemia and brain sagging, but magnetic resonance imaging may be normal. Myelography is the study of choice to identify the CSF leak but is not always necessary to make the diagnosis. Treatment consists of bedrest, abdominal binder, epidural blood patching, percutaneous fibrin glue injection or surgical CSF leak repair. Outcomes have been poorly studied.

Sonography of the optic nerve sheath diameter before and after microsurgical closure of a dural CSF fistula in patients with spontaneous intracranial hypotension – a consecutive cohort study

Cephalalgia ◽  
2018 ◽  
Vol 39 (2) ◽  
pp. 306-315 ◽  
Author(s):  
Jens Fichtner ◽  
Christian T Ulrich ◽  
Christian Fung ◽  
Debora Cipriani ◽  
Jan Gralla ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Optic Nerve ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Optic Nerve Sheath Diameter ◽  
Optic Nerve Sheath ◽  
Cerebrospinal Fluid Leakage ◽  
Nerve Sheath ◽  
Fluid Leak

Objective Spontaneous intracranial hypotension is caused by spinal cerebrospinal fluid leakage. Patients with orthostatic headaches and cerebrospinal fluid leakage show a decrease in optic nerve sheath diameter upon movement from supine to upright position. We hypothesized that the decrease in optic nerve sheath diameter upon gravitational challenge would cease after closure of the leak. Methods We included 29 patients with spontaneous intracranial hypotension and refractory symptoms admitted from 2013 to 2016. The systematic workup included: Optic nerve sheath diameter sonography, spinal MRI and dynamic myelography with subsequent CT. Microsurgical sealing of the cerebrospinal fluid leak was the aim in all cases. Results Of 29 patients with a proven cerebrospinal fluid leak, one declined surgery. A single patient was lost to follow-up. In 27 cases, the cerebrospinal fluid leak was successfully sealed by microsurgery. The width of the optic nerve sheath diameter in supine position increased from 5.08 ± 0.66 mm before to 5.36 ± 0.53 mm after surgery ( p = 0.03). Comparing the response of the optic nerve sheath diameter to gravitational challenge, there was a significant change from before (−0.36 ± 0.32 mm) to after surgery (0.00 ± 0.19 mm, p < 0.01). In parallel, spontaneous intracranial hypotension-related symptoms resolved in 26, decreased in one and persisted in a single patient despite recovery of gait. Conclusions The sonographic assessment of the optic nerve sheath diameter with gravitational challenge can distinguish open from closed spinal cerebrospinal fluid fistulas in spontaneous intracranial hypotension patients. A response to the gravitational challenge, that is, no more collapse of the optic nerve sheath while standing up, can be seen after successful treatment and correlates with the resolution of clinical symptoms. Sonography of the optic nerve sheath diameter may be utilized for non-invasive follow-up in spontaneous intracranial hypotension.

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