Surgical intervention for spontaneous intracranial hypotension Type 4 CSF leak: A case report

Background: Spontaneous intracranial hypotension (SIH) is a rare condition that can be very debilitating. SIH is well understood to be due to a CSF leak, however, identifying the source of the leak is still a challenge. We are presenting a case of Type 4 CSF leak and reviewing the related literature. Case Description: A 46-year-old female presenting with intractable orthostatic headaches was diagnosed with SIH. She was unable to mobilize due to the severity of her symptoms. MRI scans of the brain and spine did not identify a source of the leak. After failing conservative therapy and multiple epidural blood patches, the patient underwent surgery which resulted in significant improvement in symptoms. Conclusion: This study has shown that surgical intervention improves symptoms in patients who do not have an identifiable source of CSF leak. Further studies need to be done to fully understand the role of surgery in Type 4 CSF leaks.

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

Journal of Neurosurgery ◽

10.3171/2011.12.jns111474 ◽

2012 ◽

Vol 116 (4) ◽

pp. 749-754 ◽

Cited By ~ 32

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.

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Intracranial Hypotension for Anesthesiologists: What We Should Know

Pain Management Case Reports ◽

10.36076/pmcr.2019/3/69 ◽

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

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Spontaneous Intracranial Hypotension and MRI

Rivista di Neuroradiologia ◽

10.1177/197140099801100209 ◽

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.

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

Case Reports in Medicine ◽

10.1155/2016/9809017 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 2

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.

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

Canadian Association of Radiologists Journal ◽

10.1177/0846537119888395 ◽

2020 ◽

Vol 71 (2) ◽

pp. 174-185 ◽

Cited By ~ 1

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.

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Spontaneous intracranial hypotension associated with transdural thoracic osteophyte reversed by primary dural repair

Journal of Neurosurgery Spine ◽

10.3171/spi.2005.2.5.0614 ◽

2005 ◽

Vol 2 (5) ◽

pp. 614-618 ◽

Cited By ~ 31

Author(s):

Devin K. Binder ◽

Vahé Sarkissian ◽

William P. Dillon ◽

Philip R. Weinstein

Keyword(s):

Intracranial Hypotension ◽

Surgical Intervention ◽

Median Sternotomy ◽

Spontaneous Intracranial Hypotension ◽

Imaging Features ◽

Dural Repair ◽

Content Type ◽

Csf Leakage ◽

Upper Thoracic ◽

Csf Leaks

✓ Spontaneous intracranial hypotension (SIH) is an increasingly recognized syndrome associated with a specific set of clinical and imaging findings; however, determining the site of spinal cerebrospinal fluid (CSF) leakage in these patients is often difficult, and indications for surgical intervention need to be better defined. The authors report on a 55-year-old woman who presented with posture-related headache, disorientation, and memory impairment. Imaging features were consistent with SIH. Computerized tomography myelography demonstrated a large T2–3 anterior transdural osteophyte associated with a CSF fistula. After an unsuccessful trial of conservative therapy, the patient underwent median sternotomy, T2–3 discectomy and removal of osteophyte, which allowed adequate exposure for primary dural repair. Postoperatively, there was immediate and prolonged resolution of all of her symptoms. This case of SIH was caused by transdural penetration by an anterior osteophyte and CSF leakage in the upper thoracic spine, which was treated effectively by anterior exposure and primary dural repair. Aggressive surgical intervention may be required to treat upper thoracic CSF leaks refractory to other measures.

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Expert Commentary: Role of Surgery for the Management of CSF Leaks

Cephalalgia ◽

10.1111/j.1468-2982.2008.01777.x ◽

2008 ◽

Vol 28 (12) ◽

pp. 1357-1360 ◽

Cited By ~ 11

Author(s):

B Mokri

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Intracranial Hypotension ◽

Dural Puncture ◽

Spontaneous Intracranial Hypotension ◽

Resonance Imaging ◽

Magnetic Resonance Imaging Mri ◽

Expert Commentary ◽

Csf Leaks

Spontaneous intracranial hypotension (SIH) was described nearly 70 years ago, and for years it was essentially equated with post dural puncture headaches (PDPH) (1). The interest in this entity substantially increased after the magnetic resonance imaging (MRI) abnormalities of this disorder were recognized in the early 1990s (2-6), and consequently many more patients were diagnosed than before and a far broader clinical spectrum of this disorder began to become apparent. It also came to be recognized that the overwhelming majority if not all cases of SIH result from spontaneous cerebrospinal (CSF) leaks and that the independent pathogenic parameter is loss of CSF volume (7).

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Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

Journal of Neurosurgery ◽

10.3171/jns.1996.84.4.0598 ◽

1996 ◽

Vol 84 (4) ◽

pp. 598-605 ◽

Cited By ~ 238

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.

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

Acta Neurochirurgica ◽

10.1007/s00701-021-04868-2 ◽

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.

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Vanishing calcification associated with a spontaneous ventral spinal cerebrospinal fluid leak

Cephalalgia ◽

10.1177/0333102416628468 ◽

2016 ◽

Vol 36 (14) ◽

pp. 1366-1369 ◽

Cited By ~ 2

Author(s):

Wouter I Schievink ◽

Lindsey Ross ◽

Ravi S Prasad ◽

M Marcel Maya

Keyword(s):

Magnetic Resonance Imaging ◽

Cerebrospinal Fluid ◽

Magnetic Resonance ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Uneventful Recovery ◽

Resonance Imaging ◽

Computed Tomographic Myelography ◽

Csf Leaks

Background Some patients with spontaneous intracranial hypotension have a ventral spinal cerebrospinal fluid (CSF) leak and these CSF leaks may be associated with calcified disk herniations. Identifying these calcifications is helpful in directing treatment. We report here the unusual case of a patient with a ventral CSF leak in whom the associated calcification absorbed over a five-month period. Case report A 42-year-old woman developed orthostatic headaches and bilateral abducens nerve palsies. Magnetic resonance imaging of her brain showed typical findings of spontaneous intracranial hypotension. Magnetic resonance imaging of her spine showed an extensive cervicothoracic CSF leak. Computed tomographic myelography showed calcification at the Th1–2 disk space. Three epidural blood patches were performed, but her symptoms persisted. Digital subtraction myelography performed five months later showed an upper thoracic ventral CSF, but the calcification was no longer present. A dural tear, found at surgery at the Th1–2 level, was repaired and the patient made an uneventful recovery. Discussion The resorption of calcifications at the level of a ventral spinal CSF leak could explain the absence of any calcifications in at least some patients with such leaks and demonstrates the usefulness of reviewing previous imaging in patients with ventral CSF leaks if the exact site of the leak remains unknown.

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