scholarly journals Intraspinal hemorrhage in spontaneous intracranial hypotension: link to superficial siderosis? Report of 2 cases

2016 ◽  
Vol 24 (3) ◽  
pp. 454-456 ◽  
Author(s):  
Wouter I. Schievink ◽  
Philip Wasserstein ◽  
M. Marcel Maya
Keyword(s):  
Differential Diagnosis ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Superficial Siderosis ◽  
Dural Tear ◽  
Epidural Blood Patching ◽  
Intraspinal Hemorrhage ◽  
Csf Leaks

Spontaneous intracranial hypotension due to a spinal CSF leak has become a well-recognized cause of headaches, but such spinal CSF leaks also are found in approximately half of patients with superficial siderosis of the CNS. It has been hypothesized that friable vessels at the site of the spinal CSF leak are the likely source of chronic bleeding in these patients, but such an intraspinal hemorrhage has never been visualized. The authors report on 2 patients with spontaneous intracranial hypotension and intraspinal hemorrhage, offering support for this hypothesis. A 33-year-old man and a 62-year-old woman with spontaneous intracranial hypotension were found to have a hemorrhage within the ventral spinal CSF collection and within the thecal sac, respectively. Treatment consisted of microsurgical repair of a ventral dural tear in the first patient and epidural blood patching in the second patient. The authors suggest that spontaneous intracranial hypotension should be included in the differential diagnosis of spontaneous intraspinal hemorrhage, and that the intraspinal hemorrhage can account for the finding of superficial siderosis when the CSF leak remains untreated.

Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant

2004 ◽  
Vol 100 (6) ◽  
pp. 1098-1100 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Franklin M. Moser
Keyword(s):  
Fibrin Sealant ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Invasive Treatment ◽  
Content Type ◽  
Epidural Blood Patching ◽  
Lower Thoracic Spine ◽  
Fine Print ◽  
Csf Leaks

✓ Spontaneous intracranial hypotension due to a cerebrospinal fluid (CSF) leak in the spine is an important cause of new, daily persistent headaches. Most patients respond well to conservative treatments including epidural blood patching. Limited options for effective treatment are available for patients in whom these treatments fail. The authors treated four patients (mean age 38 years; range 26–43 years) with percutaneous placement of a fibrin sealant. All these patients presented with intractable positional headaches. The CSF leak was located in the lower cervical spine in three patients and in the lower thoracic spine in one patient. Four to 20 milliliters of fibrin sealant was injected at the site of the CSF leak. Two of the four patients became asymptomatic within days of the procedure and thus avoided surgery. There were no complications of this procedure. Percutaneous placement of a fibrin sealant is a safe, minimally invasive treatment for spontaneous spinal CSF leaks and should be considered in patients in whom conservative treatment has failed.

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.

Diffuse non-aneurysmal SAH in spontaneous intracranial hypotension: Sequela of ventral CSF leak?

Cephalalgia ◽  
2015 ◽  
Vol 36 (6) ◽  
pp. 589-592 ◽  
Author(s):  
Wouter I Schievink ◽  
M Marcel Maya
Keyword(s):  
Cerebral Angiography ◽  
Intracranial Hypotension ◽  
Aneurysmal Subarachnoid Hemorrhage ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Superficial Siderosis ◽  
Long Duration ◽  
Spine Mri ◽  
Lateral Sclerosis

Background Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak has become a well-recognized cause of headaches. Recently, various unusual neurological syndromes have been described in such patients with chronic ventral CSF leaks, including superficial siderosis and an amyotrophic lateral sclerosis-like syndrome. The authors now report two patients with spontaneous intracranial hypotension due to a chronic ventral CSF leak who suffered a diffuse non-aneurysmal subarachnoid hemorrhage (SAH). Description of cases A 62-year-old woman underwent uneventful microsurgical repair of a ventral thoracic CSF leak that had been present for 13 years. Seventeen months after surgery, she was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and spine and brain MRI did not reveal a source of the SAH. A 73-year-old woman was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and brain MRI did not reveal a source of the SAH, although superficial siderosis was detected. Spine MRI showed a ventral thoracic CSF leak that by history had been present for 41 years. She underwent uneventful microsurgical repair of the CSF leak. Discussion The authors suggest that patients with a ventral spinal CSF leak of long duration may be at risk of diffuse non-aneurysmal SAH.

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

Behavioral Variant Frontotemporal Dementia as a Serious Complication of Spontaneous Intracranial Hypotension

2018 ◽  
Vol 15 (5) ◽  
pp. 505-515 ◽  
Author(s):  
Wouter I Schievink ◽  
M Marcel Maya ◽  
Zachary R Barnard ◽  
Franklin G Moser ◽  
Stacey Jean-Pierre ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Clinical Criteria ◽  
Epidural Blood Patching ◽  
Early Onset Dementia ◽  
Percutaneous Procedures ◽  
Brain Sagging

Abstract BACKGROUND Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. OBJECTIVE To describe detailed characteristics of a large cohort of patients with SIH and symptoms of bvFTD. METHODS We identified patients with SIH who met clinical criteria for bvFTD. Patients were compared to a cohort of SIH patients without bvFTD. RESULTS The mean age for the 21 men and 8 women was 52.9 yr (range, 37–65 yr). All 29 patients with bvFTD symptoms had hypersomnolence. Magnetic resonance imaging showed brain sagging in all patients, cerebrospinal fluid (CSF) opening pressure low in about half of patients, but a spinal CSF leak could not be detected in any patient. All patients underwent epidural blood patching, but 26 patients eventually underwent 1 or more surgical procedures. Overall, a good outcome was obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to 1 (14%) of 7 patients who did undergo Chiari surgery (P < .003). Compared to SIH patients without symptoms of bvFTD (n = 547), those with bvFTD symptoms were older, more often male, less often demonstrated CSF leak on spinal imaging, and more often underwent surgery (P < .02). CONCLUSION bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured.

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.

Long-term Risks of Persistent Ventral Spinal CSF Leaks in SIH: Superficial Siderosis and Bibrachial Amyotrophy

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012786
Author(s):  
Wouter I. Schievink ◽  
Marcel Maya ◽  
Franklin Moser ◽  
Miriam Nuño
Keyword(s):  
Spinal Cord ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Superficial Siderosis ◽  
Spinal Cord Herniation ◽  
Neurologic Disability ◽  
One Year ◽  
Csf Leaks

Background and Objectives:Superficial siderosis, bibrachial amyotropy, and spinal cord herniation are unusual but serious long-term sequelae of persistent spontaneous spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH), particularly ventral spinal CSF leaks. However, the risk of developing such sequelae has not been established in this population. We undertook this study to determine the risk of these serious complications of persistent ventral spinal CSF leaks.Methods:This cohort study was conducted using data from a prospectively maintained data base of patients who meet the modified International Classification of Headache Disorders (ICHD)-III criteria for SIH. The patient population consisted of a consecutive group of patients with SIH and persistent ventral spinal CSF leaks, who were first seen within one year of onset of SIH symptoms and who had at least one year of follow-up.Results:Among 51 patients with SIH and a persistent ventral spinal CSF leak, superficial siderosis developed in six patients and bibrachial amyotropohy in two patients during 280 patient years of follow-up. The probability of these complications increased from 0% at 48 months, to 4.5% (95% confidence interval (CI): 1.0-28.0%) at 56 months, 10.5% (95% CI: 3.0-36.4%) at 96 months, 32.7% (95% CI: 15.0-62.8%) at 144 months, and 57.9% (95% CI: 30.2-87.6%) at 192 months. None of the patients developed spinal cord herniation.Discussion:Among patients with SIH and a persistent ventral spinal CSF leak, the risk of developing serious long-term sequelae is considerable. This study shows that early treatment of a ventral spinal CSF leak offers a unique opportunity to prevent neurologic disability from superficial siderosis and bibrachial amyotrophy.

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.

scholarly journals Intradural extraarachnoid sutureless technique combined with laminoplasty for indirect repair of ventral dural defects in spontaneous intracranial hypotension: technical note and case series

2021 ◽  
Author(s):  
M. Kamenova ◽  
S. Schaeren ◽  
M-G. Wasner
Keyword(s):  
Surgical Technique ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Treatment Strategies ◽  
Case Series ◽  
Technical Note ◽  
Csf Leak ◽  
Sutureless Technique ◽  
Dural Defects ◽  
Csf Leaks

Abstract Background There is a significant variance in surgical treatment strategies of ventral cerebrospinal fluid (CSF) leaks causing spontaneous intracranial hypotension (SIH). Posterior approaches might represent a preferable alternative to the more invasive anterior and lateral routes, as long as the spinal cord is not exposed to harmful manipulation. The aim of this technical note is to report and illustrate a new surgical technique using an intradural extraarachnoid sutureless technique via laminoplasty for indirect repair of ventral CSF leaks causing intractable SIH symptoms. Methods The surgical technique is described in a step by step fashion. Between May 2018 and May 2020, five patients with ventral spinal CSF leaks were operated on, utilizing this technique. All dural defects were located at the level of the thoracic spine. A retrospective review on demographic and radiological findings, symptoms, outcome, and follow-up was performed. Results The intra- and postoperative course was uneventful in all patients with no surgery-related complications. Three patients recovered completely at discharge, while neurological symptoms significantly improved in two patients. A postoperative MRI of the spine was obtained for all patients, demonstrating regressive signs of CSF leak. Conclusion Based on the presented case series, this intradural extraarachnoid sutureless technique combined with laminoplasty seems to be a safe and effective option for indirect repair of ventral dural defects in SIH. In our opinion, it represents a valid alternative to traditional more aggressive approaches.

Sign in / Sign up

Export Citation Format

Share Document