This page will be updated periodically.
* Abstract links are included. (click on the PMID number)
* Note that links to full-text are provided for open access papers (line below PMID).
Digital subtraction myelography in the investigation of post-dural puncture headache in 27 patients: technical note.
Schievink WI, Maya MM, Moser FG.
J Neurosurg Spine. 2017 Jun;26(6):760-764. doi: 10.3171/2016.11.SPINE16968. Epub 2017 Mar 31.
OBJECTIVE Post-dural puncture headaches are common, and the treatment of such headaches can be complex when they become chronic. Among patients with spontaneous spinal CSF leaks, digital subtraction myelography (DSM) can localize the exact site of the leak when an extradural CSF collection is present, and it can also demonstrate CSF-venous fistulas in those without an extradural CSF collection. The authors now report on the use of DSM in the management of patients with chronic post-dural puncture headaches. METHODS The patient population consisted of a consecutive group of 27 patients with recalcitrant post-dural puncture headache that had lasted from 2 to 150 months (mean 26 months). RESULTS The mean age of the 17 women and 10 men was 39.1 years (range 18-77 years). An extensive extradural CSF collection was present in 5 of the 27 patients, and DSM was able to localize the exact site of the dural defect in all 5 patients. Among the 22 patients who did not have an extradural CSF collection, DSM showed a CSF-venous fistula in 1 patient (5%). Three other patients had a small pseudomeningocele at the level of the dural puncture. Percutaneous glue injection or microsurgical repair resulted in resolution of symptoms in 8 of the 9 patients in whom an abnormality had been identified on imaging. CONCLUSIONS Digital subtraction myelography is able to precisely localize the dural puncture site in patients with a post-dural puncture headache and an extensive extradural CSF collection, and it may rarely detect a CSF-venous fistula in such patients without an extradural CSF collection.
Misdiagnosis of Spontaneous Intracranial Hypotension as a Risk Factor for Subdural Hematoma.
Kim YJ, Cho HY, Seo DW, Sohn CH, Ahn S, Lee YS, Kim WY, Lim KS.
Headache. 2017 Jun 26. doi: 10.1111/head.13132. [Epub ahead of print]
This study aimed to evaluate the association between misdiagnosis of spontaneous intracranial hypotension (SIH) and subdural hematoma development.
Although SIH is more prevalent than expected and causes potentially life-threatening complications including subdural hematoma (SDH), the association between misdiagnosis of SIH and SDH development is not yet evaluated.
Retrospective observational study was conducted between January 1, 2005, and December 31, 2014. Adult patients with spontaneous intracranial hypotension (age ≥ 18 years) were enrolled.
Of the 128 patients with SIH, 111 (86.7%) were in no SDH group and 17 (13.3%) were in SDH group. Their clinical presentation did not show significant different between the two groups, except age, the days from symptom onset to correct diagnosis, and the number of misdiagnoses. Age (odds ratio [OR], 1.15; 95% confidence interval [CI], 1.07-1.23) and the number of times SIH was misdiagnosed (OR, 1.82; 95% CI, 1.03-3.21) were independent risk factors for the development of SDH in SIH patients by multivariate logistic analysis. The clinical outcomes, including length of hospital stay and revisit rate, were similar in the two groups.
The number of times SIH was misdiagnosed was associated with the later development of SDH perhaps because of delay in correct diagnosis of SIH. Clinicians would prevent the later complication of SDH in SIH patients by increasing the awareness and a high index of suspicion of SIH.
© 2017 American Headache Society.
emergency department; misdiagnosis; spontaneous intracranial hypotension; subdural hematoma
The status of diagnosis and treatment to intracranial hypotension, including SIH.
Lin JP, Zhang SD, He FF, Liu MJ, Ma XX.
J Headache Pain. 2017 Dec;18(1):4. doi: 10.1186/s10194-016-0708-8. Epub 2017 Jan 13.
Intracranial hypotension, especially spontaneous intracranial hypotension (SIH), is a well-recognized entity associated with cerebrospinal fluid (CSF) leaks, and has being recognized better in recent years, while still woefully inadequate. An increasing number of factors including iatrogenic factors are realized to involve in development and progression of intracranial hypotension. The diagnosis remains difficult due to the various clinical manifestations, some of which are nonspecific and easily to be neglected. Multiple imaging tests are optional in CSF leakage identification while clinicians are still confronted with difficulties when making selection resulting from superiorities and disadvantages of different imaging tests. Treatments for intracranial hypotension are multifarious but evidence is anecdotal. Values of autologous epidural blood patching (EBP), the mainstay of first-line interventional treatment currently, is getting more and more regards while there are no systematic review of its efficacy and risks. Hereby, the purpose of this review was to reveal the present strategy of intracranial hypotension diagnosis and treatment by reviewing literatures, coupled with our experience in clinical work.
Chiari-like displacement due to spontaneous intracranial hypotension in an adolescent: Successful treatment by epidural blood patch.
Schönberger J, Möhlenbruch M, Seitz A, Bußmann C, Bächli H, Kölker S.
Eur J Paediatr Neurol. 2017 Jul;21(4):678-681. doi: 10.1016/j.ejpn.2017.02.004. Epub 2017 Feb 20.
Spontaneous intracranial hypotension is a rarely diagnosed cause of headache, especially in children and adolescents. It is due to cerebrospinal fluid (CSF) leakage via spinal fistulae occurring without major trauma.
An adolescent patient presented with a 3-month history of strictly postural headache. Cranial magnetic resonance imaging (MRI) showed pronounced Chiari-like prolapse of the cerebellar tonsils, narrow ventricles and enlarged cerebral veins. On spinal MRI, myelographic sequences revealed a large collection of CSF around the first sacral roots. CT myelography proved extensive spinal CSF leakage. Hence, we applied epidural patches at multiple levels. Afterwards, symptoms and radiologic findings, including Chiari-like displacement, completely resolved.
A Chiari-like descent of the cerebellar tonsils alone does not secure the diagnosis of a Chiari I malformation. Especially if other findings indicate spinal CSF leakage, a systematic work-up should be initiated. In most cases, interventional techniques seal the leak successfully, resulting in a favorable outcome.
Watertight Sealing Without Lumbar Drainage for Incidental Ventral Dural Defect in Transthoracic Spine Surgery: A Retrospective Review of 53 Cases.
Jeon SH, Lee SH, Tsang YS, Jung TG, Moon KH, Choi G, Dilip KD.
Clin Spine Surg. 2017 Jul;30(6):E702-E706. doi: 10.1097/BSD.0000000000000249.
A retrospective review.
To evaluate the therapeutic efficacy of the triple layer closure technique to establish watertight sealing without diversion of lumbar drainage, in preventing persistent incidental subarachnoid-pleural fistula and other neurological complications related to excessive drainage of cerebrospinal fluid (CSF) after dural defect in transthoracic ossified posterior longitudinal ligament (OPLL) surgery.
SUMMARY OF BACKGROUND DATA:
CSF leakage into the pleural cavity leads to unfavorable conditions for natural healing of incidental durotomy due to the negative pressure environment of the pleural space and lack of wound healing around the bony cavity near the decompressed spinal cord. This often leads to a persistent incidental subarachnoid-pleural fistula. In addition, diversion of lumbar drainage may lead to excessive CSF drainage resulting in intracranial hypotension. To avoid this, we studied the efficacy of a modified sealing method to establish a more watertight covering at the ventral dural defect without lumbar CSF drainage.
Fifty-three patients who had CSF leakage from the ventral aspect of the spinal cord during transthoracic spine surgery for thoracic OPLL between 2004 and 2013 were retrospectively reviewed. Patients were divided into 2 groups: a conventional group (group A) and a triple layer closure group (group B). In group A (n=33 patients), the dural defect was covered with fibrin glue (Beriplast P) mixed with gelfoam (Spongostan Standard) with subsequent subarachnoid lumbar drainage. In group B (n=20 patients), the dural defect was sealed using the triple layer technique with 2 layers of fibrin glue and gelatin sponge plus a third layer of synthetic hydrogel (Duraseal, Dural Sealant System) without subsequent subarachnoid lumbar drainage. Both groups had chest tubes that drained through an underwater seal. Clinical data including duration and total amount of drainage (chest tube and lumbar drainage), related complications, and duration of hospital stay were compared between the 2 groups.
Compared with the patients in group A, group B had a significantly smaller total volume of drainage and shorter chest tube drainage time (P<0.05) during their hospital stay. In group A, complications occurred in 6 cases (18.2%), including 3 cases of intracranial hypotension combined with transient mental status alteration, postural headache, and dizziness, 1 case of regional atelectasis with pneumonia, and 2 cases of revision thoracotomy. Revision thoracotomy was performed to treat persistent subarachnoid-pleural fistula due to significant and prolonged CSF leakage. In group B, there were no complications and no revision thoracotomy was needed. The mean duration of hospital stay was shorter in group B (15.6 d) compared with group A (22.4 d). CONCLUSIONS: The established watertight closure of the dural defect using the triple layer sealing method without lumbar drainage was more effective and safe.
Intracranial hypotension following traumatic brain injury: a diagnostic and therapeutic challenge.
Low JCM, Shtaya A, Hettige S.
World Neurosurg. 2017 Jun 14. pii: S1878-8750(17)30952-X. doi: 10.1016/j.wneu.2017.06.060. [Epub ahead of print]
Intracranial hypotension (IH) is a recognised cause of coma, however, the diagnosis is often challenging, especially in patients with superimposed traumatic brain injury (TBI).
We report a case of a 67-year-old patient who became comatose following evacuation of bilateral acute subdural haematomas with concurrent respiratory failure. Imaging and intraparenchymal intracranial pressure (ICP) monitoring confirmed secondary IH. She was managed with an epidural blood patch, and a 72 hours period in the trendelenberg position guided by ICP monitoring and clinical assessment. She subsequently made an excellent neurological recovery from an initial Glasgow coma scale (GCS) of 3 to a GCS of 15.
A diagnosis of secondary IH can easily be missed in patients who have suffered a primary brain injury. In patients with a poor neurological recovery, clinicians should rule out secondary IH as a potential cause as immediate treatment can lead to a profound clinical improvement.
Ventral Spontaneous Durotomy Following Vaginal Delivery.
Agarwal N, Kashkoush AI, Prabhu AV, Sekula RF Jr.
World Neurosurg. 2017 Jun;102:697.e5-697.e7. doi: 10.1016/j.wneu.2017.01.133. Epub 2017 Feb 10.
Dural breaches have a diverse etiology, including spontaneous rupture and trauma. Most cases resolve with bed rest; in refractory cases, an epidural blood patch can be placed to obstruct further leakage. We discuss a unique case of a spontaneous ventral durotomy following vaginal delivery that was managed with injections of autologous blood through bilateral transforaminal needles.
A previously healthy, 36-year-old pregnant woman presented to the inpatient maternity ward with positional occipital headaches and neck pain 24 hours after normal spontaneous vaginal delivery. Two dorsally placed epidural blood patches provided only transient relief. Computed tomography myelography revealed ventral cerebrospinal fluid leak. Targeted therapy was provided with computed tomography-guided ventral placement of a blood patch.
Spontaneous durotomy is a rare phenomenon and should be considered in patients who present with positional headaches. Ventrally targeted therapy via an epidural blood patch should be considered to provide optimal relief.
Association of Cerebral Venous Thrombosis and Intracranial Hypotension: Review of 3 Cases.
Sinnaeve L, Vanopdenbosch L, Paemeleire K.
J Stroke Cerebrovasc Dis. 2017 Jun 13. pii: S1052-3057(17)30234-3. doi: 10.1016/j.jstrokecerebrovasdis.2017.05.015. [Epub ahead of print]
Cerebral venous thrombosis is a rare complication of intracranial hypotension. We describe 3 cases in which this phenomenon occurred, as a result of a lumbar puncture or due to a spontaneous cerebrospinal fluid leak. We emphasize the importance of early detection of the intracranial hypotension syndrome, the most common clinical manifestation being orthostatic headache. It is not an innocent condition as it is associated with other potential complications such as subdural hygroma/hematoma, cranial nerve palsies, cerebellar tonsillar descent, and even brainstem manifestations. Any change in the typical features of the syndrome should lead to further investigation. Repeat cerebral imaging is important in that situation, including ruling out cerebral venous thrombosis.
Greater Occipital Nerve Treatment in the Management of Spontaneous Intracranial Hypotension Headache: A Case Report.
Niraj G, Critchley P, Kodivalasa M, Dorgham M.
Headache. 2017 Jun;57(6):952-955. doi: 10.1111/head.13095. Epub 2017 May 2.
Clinical presentation of spontaneous intracranial hypotension headache (SIHH) has similarities with postdural puncture headache (PDPH). Recommended treatment for both conditions is an epidural blood patch. Successful outcomes following greater occipital nerve blocks have been reported in the management of PDPH. We present the first report of greater occipital nerve treatment in SIHH.
A 40-year-old male presented with a 2-year history of daily postural headaches having a significant impact on quality of life. Magnetic resonance imaging revealed bilateral convexity subdural collections. Post gadolinium scan revealed pachymeningeal enhancement with reduced pontomesencephalic angle below 50 degrees. The patient was offered an epidural blood patch and greater occipital nerve block with corticosteroids. The patient chose occipital nerve block.
The patient reported significant short-term benefit lasting 4 months. Thereafter, the patient underwent pulsed radiofrequency treatment to bilateral greater occipital nerves. He reported significant benefit lasting 10 months.
Greater occipital nerve treatment may have a role in management of SIHH.
Cerebrospinal fluid outflow resistance as a diagnostic marker of spontaneous cerebrospinal fluid leakage.
Beck J, Fung C, Ulrich CT, Fiechter M, Fichtner J, Mattle HP, Mono ML, Meier N, Mordasini P, Z’Graggen WJ, Gralla J, Raabe A.
J Neurosurg Spine. 2017 Jun 2:1-8. doi: 10.3171/2017.1.SPINE16548. [Epub ahead of print]
OBJECTIVE Spinal CSF leakage causes spontaneous intracranial hypotension (SIH). The aim of this study was to characterize CSF dynamics via lumbar infusion testing in patients with and without proven spinal CSF leakage in order to explore possible discriminators for the presence of an open CSF leak.
METHODS This analysis included all patients with suspected SIH who were treated at the authors’ institution between January 2012 and February 2015. The gold standard for “proven” CSF leakage is considered to be extrathecal contrast accumulation after intrathecal contrast injection. To characterize CSF dynamics, the authors performed computerized lumbar infusion testing to measure lumbar pressure at baseline (opening pressure) and at plateau, as well as pulse amplitude, CSF outflow resistance (RCSF), craniospinal elastance, and pressure-volume index.
RESULTS Thirty-one patients underwent clinical imaging and lumbar infusion testing and were included in the final analysis. A comparison of the 14 patients with proven CSF leakage with the 17 patients without leakage showed a statistically significantly lower lumbar opening pressure (p < 0.001), plateau pressure (p < 0.001), and RCSF (p < 0.001) in the group with leakage. Sensitivity, specificity, and positive and negative predictive values for an RCSF cutoff of ≤ 5 mm Hg/(ml/min) were 0.86, 1.0, 1.0, and 0.89 (area under the curve of 0.96), respectively. The median pressure-volume index was higher (p = 0.003), and baseline (p = 0.017) and plateau (p < 0.001) pulse amplitudes were lower in patients with a proven leak. CONCLUSIONS Lumbar infusion testing captures a distinct pattern of CSF dynamics associated with spinal CSF leakage. RCSF assessed by computerized lumbar infusion testing has an excellent diagnostic accuracy and is more accurate than evaluating the lumbar opening pressure. The authors suggest inclusion of RCSF in the diagnostic criteria for SIH.
Dinosaur Tail Sign: A Useful Spinal MRI Finding Indicative of Cerebrospinal Fluid Leakage.
Sakurai K, Kanoto M, Nakagawa M, Shimohira M, Tokumaru AM, Kameyama M, Shimoji K, Morimoto S, Matsukawa N, Nishio M, Shibamoto Y.
Headache. 2017 Jun;57(6):917-925. doi: 10.1111/head.13075. Epub 2017 Apr 16.
To evaluate the imaging characteristics and diagnostic utility of the “Dinosaur tail sign” in the diagnosis of cerebrospinal fluid (CSF) leakage.
The authors propose the “Dinosaur tail sign,” defined as a combination of the dorsal epidural hyperintensities, fat tissue, spinal cord, and cauda equine on lumbosacral sagittal fat-suppressed T2-weighted image (FST2WI), as a sensitive indicator for diagnosing CSF leakage.
Imaging characteristics of the “Dinosaur tail sign” was evaluated in seven spontaneous intracranial hypotension (SIH) and 23 iatrogenic CSF leakage (ICSFL) patients. Additionally, the diagnostic index was compared between the “Dinosaur tail sign” and other previously reported useful magnetic resonance imaging (MRI) and magnetic resonance myelography (MRM) findings.
In contrast to other imaging findings including the epidural expansion, floating dural sac sign, and distension of the spinal epidural veins on MRI, and paraspinal fluid collections (PFC) on MRM, the “Dinosaur tail sign” was found equally in both SIH and ICSFL patients (6 SIH and 19 ICSFL; 83% of all patients with CSF leakage). The “Dinosaur tail sign” showed sufficient diagnostic utility (sensitivity 83%, specificity 94%, accuracy 89%) that was comparable to that of PFC.
The “Dinosaur tail sign” is a useful imaging finding suggestive of CSF leakage. Evaluation of subtle interspinous arched hyperintensities on spinal MRI is mandatory for the diagnosis of SIH and ICSFL.
Spontaneous Intracranial Hypotension: A Review and Introduction of an Algorithm For Management.
Davidson B, Nassiri F, Mansouri A, Badhiwala JH, Witiw CD, Shamji MF, Peng PW, Farb RI, Bernstein M.
World Neurosurg. 2017 May;101:343-349. doi: 10.1016/j.wneu.2017.01.123. Epub 2017 Feb 9.
Spontaneous intracranial hypotension (SIH) is a condition of low cerebrospinal fluid volume and pressure caused by a leak of cerebrospinal fluid through a dural defect. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines for myelography, blood patching, and possible surgical repair. Patients should be monitored closely, because they can deteriorate into a coma or even death. There are no widely accepted guidelines for the management of SIH.
METHODS AND CONCLUSIONS:
We review the existing SIH literature, illustrate management challenges via a case review, and propose an algorithm developed by neurosurgeons, radiologists, and anesthesiologists intended to simplify and streamline the management of SIH.
A Small Leak Will Sink the Brain: Targeted C1-C2 Patching.
Decramer T1, Van Dyck-Lippens PJ2, Franken TP3, Demaerel P4, van Loon J5, Theys T5.
World Neurosurg. 2017 May;101:816.e1-816.e3. doi: 10.1016/j.wneu.2017.02.086. Epub 2017 Feb 27.
Spontaneous intracranial hypotension syndrome results from spontaneous spinal cerebrospinal fluid (CSF) leaks. The first treatment of choice consists of lumbar epidural blood patching. If this fails, further imaging is mandatory to explore the possibility of targeted therapy.
We describe a case of a 50-year-old woman who developed spontaneous intracranial hypotension after minor blunt cervical trauma, complicated with bilateral subdural hematomas. Two lumbar epidural blood patches were unsuccessful. Magnetic resonance imaging with intrathecal gadolinium revealed a CSF leak at the C1-C2 level. A targeted blood patch via a percutaneous high thoracic epidural approach was performed, and symptoms disappeared in the immediate postoperative period with a regression of the subdural hematomas on subsequent imaging.
A targeted epidural blood patch using an epidural catheter represents an elegant approach to a CSF leak at the C1-C2 region and can be successful in treating patients with severe intracranial hypotension syndrome.
Simultaneous Surgical Decompression of Bilateral Subdural Hematoma and an Administration of Epidural Blood Patch for Spontaneous Intracranial Hypotension.
Mariappan R, Philip A, Gandham EJ, Raju K.
J Neurosurg Anesthesiol. 2017 May 19. doi: 10.1097/ANA.0000000000000440. [Epub ahead of print]
Penetrating Osseous Spicules Causing High-Flow Ventral CSF Leaks in the Setting of Relatively Low BMI : A Preliminary Study.
Rosebrock RE, Diehn FE, Luetmer PH, Wald JT, Lane JI, Morris JM, Lehman VT, Carr CM, Mokri B, Thielen KR.
Clin Neuroradiol. 2017 May 16. doi: 10.1007/s00062-017-0596-6. [Epub ahead of print]
We have anecdotally observed patients with high-flow ventral cerebrospinal fluid (CSF) leaks resulting from penetrating osseous spicules or calcified discs to be relatively thin. The purpose of this study was to explore the validity of this observation and determine if a potential association exists between low body mass index (BMI) and high-flow spinal ventral CSF leaks resulting from such dura-penetrating lesions.
Sixteen consecutive patients with precisely localized high-flow ventral spinal CSF leaks on dynamic myelography were identified. The cause of the CSF leak was determined. The BMI on the date nearest to and within 2 weeks of myelography was recorded. Utilizing exact sign test, the body mass index was compared to the average BMI from the National Health and Nutrition Examination Survey (Centers for Disease Control), matched to sex and age-range.
The cohort consisted of 10 males (63%) and 6 females with a mean age of 54 years (range 37-72 years). In all patients, a spiculated osteophyte/calcified disc was identified at the site of the leak. Fourteen patients (88%) had a BMI below the matched national average, while only two patients (13%) had values above the national average (p = 0.004).
Patients with high-flow ventral CSF leaks resulting from spiculated osteophyte or calcified disc as identified by dynamic myelography are more likely to have a BMI below the U.S. national average, matched for gender and age-range. This exploratory analysis requires confirmation as well as further characterization of potential pathophysiologic mechanisms and impact on radiographic and clinical assessments.
Intracranial hypotension causing pituitary enlargement.
Chan DW, Wu AI, Wynne K.
BMJ Case Rep. 2017 May 12;2017. pii: bcr-2017-220057. doi: 10.1136/bcr-2017-220057.
Quantitative Measurement of CSF in Patients with Spontaneous Intracranial Hypotension.
Chen HC, Chen PL, Tsai YH, Chen CH, Chen CC, Chai JW.
AJNR Am J Neuroradiol. 2017 May;38(5):1061-1067. doi: 10.3174/ajnr.A5134. Epub 2017 Apr 6.
BACKGROUND AND PURPOSE:
CSF hypovolemia is a core feature of spontaneous intracranial hypotension. Spontaneous intracranial hypotension is characterized by orthostatic headache and radiologic manifestations, including CSF along the neural sleeves, diffuse pachymeningeal enhancement, and/or venous engorgement. However, these characteristics are only qualitative. Quantifying intraspinal CSF volumes could improve spontaneous intracranial hypotension diagnosis and evaluation of hypovolemic statuses in patients with spontaneous intracranial hypotension. The purpose of this study was to compare intraspinal CSF volumes across spontaneous intracranial hypotension stages and to test the clinical applicability of these measures.
MATERIALS AND METHODS:
A cohort of 23 patients with spontaneous intracranial hypotension and 32 healthy controls was subjected to brain MR imaging and MR myelography with 1.5T imaging. An automatic threshold-based segmentation method was used to calculate intraspinal CSF volumes at initial hospitalization (spontaneous intracranial hypotension-initial), partial improvement (spontaneous intracranial hypotension-intermediate), and complete recovery (spontaneous intracranial hypotension-recovery) stages.
The mean intraspinal CSF volumes observed were the following: 95.31 mL for healthy controls, 72.31 mL for spontaneous intracranial hypotension-initial, 81.15 mL for spontaneous intracranial hypotension-intermediate, and 93.74 mL for spontaneous intracranial hypotension-recovery. Increased intraspinal CSF volumes were related to disease recovery (P < .001). The intraspinal CSF volumes of patients before complete recovery were significantly lower than those of healthy controls. With the estimated intradural CSF volumes as a reference, the intraspinal CSF volume percentage was lower in patients with spontaneous intracranial hypotension with venous engorgement than in those without it (P = .058). CONCLUSIONS: With a threshold-based segmentation method, we found that spinal CSF hypovolemia is fundamentally related to spontaneous intracranial hypotension. Intraspinal CSF volumes could be a sensitive parameter for the evaluation of treatment response and follow-up monitoring in patients with spontaneous intracranial hypotension.
Neurological complications of lumbar and cervical dural punctures with a focus on epidural injections.
Surg Neurol Int. 2017 Apr 26;8:60. doi: 10.4103/sni.sni_38_17. eCollection 2017.
Various types of lumbar dural punctures may contribute to neurological injury. The etiologies of dural injury include; inadvertent dural punctures due to epidurals placed for labor anesthesia, epidural steroid injections (ESI/transforaminal TESI; approximately 9 million ESI performed in the US per year), deliberate placement of intradural pain devices, and spontaneous cerebrospinal fluid (CSF) fistulas. Resulting neurological complications may include; spinal headaches/intracranial hypotension, subdural hematomas, and 6th nerve cranial palsies. Furthermore, uniquely in the cervical spine, inadvertent cervical dural punctures attributed to cervical ESI (CESI) may lead to intramedullary spinal cord injuries (e.g. resulting in monoparesis to quadriplegia) or spinal cord strokes due to intravascular/vertebral artery injections.
In 8 studies, inadvertent lumbar dural punctures contributed to intracranial hypotension, subdural hematomas, and double vision/6th cranial nerve palsies. In 5 of the 6 studies, inadvertent dural punctures occurring during CESI were responsible for intramedullary spinal cord injuries, or direct intravascular/vertebral injections resulting in monoplegia/quadriplegia.
Inadvertent lumbar dural punctures led to multiple neurological complications including intracranial hypotension, subdural hematomas, and double vision/6th cranial nerve palsies. Uniquely, inadvertent cervical dural punctures solely due to CESI directly resulted in intramedullary spinal cord injuries or cord stroked and monoplegia/quadriplegia attributed to intravascular/vertebral artery injections. The potential neurological risks/complications/adverse events attributed to lumbar and cervical ESI must be taken into account before spine surgeons and others order these procedures.
Hypoactive-hypoalert behaviour and thalamic hypometabolism due to intracranial hypotension.
Kearney S, Flynn P, Hughes S, Spence W, McCarron MO.
Pract Neurol. 2017 Apr 22. pii: practneurol-2016-001497. doi: 10.1136/practneurol-2016-001497. [Epub ahead of print]
A 47-year-old man presented with a 9-year history of a hypoalert hypoactive behaviour syndrome, caused by the deep brain swelling variant of spontaneous intracranial hypotension. Along with apathy with retained cognition, he had stable ataxia, impaired upgaze and episodes of central apnoea. MRI brain showed a sagging brainstem, pointed ventricles and reduced angle between the vein of Galen and the straight sinus, but no meningeal enhancement or subdural collections. A dopamine transporter scan showed preganglionic dopamine receptor deficiency; a fluorodeoxy glucose positron emission tomography scan showed bilateral hypothalamic hypometabolism. This variant of spontaneous intracranial hypotension may alter deep brain functioning within the basal ganglia and thalamus, causing the hypoactive-hypoalert behaviour phenotype.
Ventral longitudinal intraspinal fluid collection: Rare presentation as brachial amyotrophy and intracranial hypotension.
Preethish-Kumar V, Vengalil S, Tiwari S, Polavarapu K, Netravathi M, Ramalingaiah AH, Nalini A.
J Spinal Cord Med. 2017 Apr 12:1-6. doi: 10.1080/10790268.2017.1314901. [Epub ahead of print]
Ventral longitudinal intraspinal fluid collection (VLISFC) presenting as hand amyotrophy has been described only in a few cases and there are no reports on associated intracranial CSF hypovolemia (ICH). We describe the clinical and imaging findings of a case with combined brachial amyotrophy and ICH secondary to VLISFC.
A 31 year old man presented with severe positional neck discomfort, radiating pain, progressive asymmetrical wasting and weakness of distal upper limbs. Contrast Magnetic Resonance Imaging (MRI) of the spine demonstrated a ventral extradural intraspinal fluid collection extending from upper border of C6 to lower border of T3 vertebra with pockets of dorsal collection. Three-dimensional constructive interference in steady state (CISS 3D) used in spinal imaging for identification of CSF leak corroborated with the extent seen on T2 sagittal sections; however, the site of the leak was not identified. After a year he developed disturbing postural headache which was relieved in recumbent position. Follow up MRI of brain was normal while spine demonstrated significant cervical cord atrophy and bilateral cord white matter hyperintensities. Conclusion / Clinical Relevance: We report this unusual case where local compression by VLISFC located at the cervical and upper thoracic level not only caused distal bi-brachial amyotrophy mimicking Hirayama disease but also led to secondary intracranial hypotension. An early identification and intervention could possibly have prevented the onset of ICH.
Spontaneous intracranial hypotension diagnosed as Chiari I malformation.
Kingston W, Hoxworth J, Halker-Singh R.
Neurology. 2017 Mar 28;88(13):1294. doi: 10.1212/WNL.0000000000003775.
No Abstract Available.
Intracranial hypotension: diagnosis by trial of Trendelenburg positioning and imaging.
Tipirneni A, Shah NH, Atchaneeyasakul K, Berry AC, Adams DJ.
Intern Emerg Med. 2017 Mar;12(2):259-261. doi: 10.1007/s11739-016-1456-0. Epub 2016 Apr 28.
Reversal of Progressive Conscious Disturbance with Epidural Blood Patch for Cerebrospinal Fluid Leakage at C2 Level.
Lai YC, Chia YY, Lien WH.
Pain Physician. 2017 Mar;20(3):E465-E468.
Intracranial hypotension syndrome (IHS) is generally caused by cerebrospinal fluid (CSF) leakage. Complications include bilateral subdural hygroma or haematoma and herniation of the cerebellar tonsils. Epidural blood patch (EBP) therapy is indicated if conservative treatment is ineffective. We reported the case of a 46-year-old man with a history of postural headache and dizziness. The patient was treated with bed rest and daily hydration with 2000 mL of fluid for 2 weeks. However, dizziness and headache did not resolve, and he became drowsy and disoriented with incomprehensible speech. Magnetic resonance imaging demonstrated diffuse dural enhancement on the postcontrast study, sagging of the midbrain, and CSF leakage over right lateral posterior thecal sac at C2 level. We performed EBP at the level of T10-T11. We injected 14 mL of autologous blood slowly in the Trendelenburg position. Within 30 minutes, he became alert and oriented to people, place, and time. We chose thoracic EBP as first line treatment in consideration of the risk of cervical EBP such as spinal cord and nerve root compression or puncture, chemical meningitis. Also we put our patient in Trendelenburg position to make blood travel towards the site of the leak. Untreated IHS may delay the course of resolution and affect the patient’s consciousness. Delivery of EBP via an epidural catheter inserted from the thoracic spine is familiar with most of anesthesiologists. It can be a safe and effective treatment for patients with IHS caused by CSF leak even at C2.Key words: Anaesthetic techniques, regional, thoracic; cerebrospinal fluid leakage; epidural blood patch; heavily T2-weighted magnetic resonance myelography; intracranial hypotension syndrome; Trendelenburg position.
Open Access: painphysicianjournal.com/current/pdf?article=NDMyNA%3D%3D&journal=104
Postural Headaches Due to Cerebrospinal Fluid Leakage Through Subarachnoid-Pleural Fistula: A Case Report.
Headache. 2017 Mar;57(3):467-471. doi: 10.1111/head.12974. Epub 2016 Nov 10.
Lin SF, Weng HY.
Postural headaches are commonly associated with spontaneous intracranial hypotension and cerebrospinal fluid (CSF) leakage from the spine. A subarachnoid-pleural fistula (SPF) is a very rare and serious type of CSF fistula that has mostly been reported following traumatic causes.
Here, the case of a 36-year-old woman who suffered from postural headaches after chiropractic manipulation of her neck was reported. Brain and spinal magnetic resonance (MR) imaging showed brain sagging, a C7-T1 dural defect, and overt CSF leakage. Heavy T2-weighted MR myelography revealed paravertebral fluid collections, communicating with pleural effusions through bilateral SPFs. The postural headaches were relieved by 3 courses of epidural blood patches.
From the patient’s history, her severe SPFs were attributed to rupture of the spinal leptomeninges during vigorous chiropractic manipulation of her neck. Heavy T2-weighted MR myelography could delineate the route of SPFs and CSF leakages.
Spontaneous Intracranial Hypotension Presenting as a “Pseudo-Chiari 1.
Haider AS1, Sulhan S1, Watson IT1, Leonard D1, Arrey EN2, Khan U3, Nguyen P1, Layton KF4.
Cureus. 2017 Feb 16;9(2):e1034. doi: 10.7759/cureus.1034.
Spontaneous intracranial hypotension (SIH) is classified as a decrease in cerebrospinal fluid (CSF) pressure secondary to a CSF leakage and consequent descent of the brain into the foramen magnum. Diagnosing SIH can be difficult due to its overlapping findings with Arnold-Chiari type 1 Malformation (CM1) where the cerebellar tonsils herniate into the foramen magnum. The similarity of both conditions calls for a more reliable imaging technique to localize the CSF leak which could narrow the differential diagnosis and aid in choosing the correct treatment. Here, we present a case of a 28-year-old female, ten weeks post-partum with symptoms similar to SIH. MRI of the brain was remarkable for tonsillar herniation below the foramen magnum. Literature was reviewed for additional neuroradiology techniques that would aid in narrowing our differential diagnosis. Interestingly, computed tomography-, digital subtraction-, and magnetic resonance myelography with intrathecal gadolinium are the preferred techniques for diagnosis of high flow and low flow CSF leaks, respectively. These modalities further aid in choosing the correct treatment while avoiding complications. Literature suggests that treatment for CM1 involves posterior fossa decompression, whereas the mainstay of treatment for SIH involves an epidural blood patch (EBP). Thus, our patient was treated with an EBP and recovered without complication.
Cranial nerve palsy following central neuraxial block in obstetrics – a review of the literature and analysis of 43 case reports.
Chambers DJ, Bhatia K.
Int J Obstet Anesth. 2017 Feb 20. pii: S0959-289X(16)30180-7. doi: 10.1016/j.ijoa.2017.02.003. [Epub ahead of print]
Cranial nerve palsy is a rarely reported complication of central neuraxial block in obstetrics. The aetiology is diverse and includes both decreased and increased intracranial pressure.
Medline, CINAHL, and EMBASE databases were searched to identify cases of cranial nerve palsy following obstetric central neuraxial block. Possible aetiology, clinical symptoms and signs, treatment, and time to resolution were assessed.
Forty-one articles containing 43 case reports of cranial nerve palsy following obstetric central neuraxial block were identified. Four cranial nerve palsies were bilateral; the remainder being unilateral. The cranial nerves most commonly affected were the abducens (17 case reports) and facial (12 case reports) nerves. Epidural block was implicated in 25 cases. Classical post-dural puncture headache preceded cranial nerve palsy in 27 cases. Subdural haematomas were reported in six cases and cortical venous or transverse sinus thrombosis in one case. Epidural blood patch was administered for treatment of cranial nerve palsy in 17 cases. Thirty-five patients had complete resolution of symptoms but in eight the cranial nerve palsy was permanent. No case reports of olfactory, oculomotor, glossopharyngeal, accessory or hypoglossal nerve palsy were identified.
Intracranial hypotension is the most common aetiology of cranial nerve palsy after central neuraxial block in obstetrics. Neuroimaging is recommended in every case, to exclude other neurological causes. Epidural blood patch was the most utilised treatment for post-dural puncture cranial nerve palsy, but outcomes were variable. The majority of cranial nerve palsies resolved over the subsequent weeks and months.
Surgical Management of Spontaneous Spinal Cerebrospinal Fluid Epidural Fistula.
Siedler DG, Ibbett IM, Thani NB.
World Neurosurg. 2017 Mar;99:810.e5-810.e10. doi: 10.1016/j.wneu.2016.12.106. Epub 2017 Jan 3.
Intracranial hypotension secondary to spontaneous spinal cerebrospinal fluid (CSF) fistula is a rare condition that can have serious sequelae. Early diagnosis and treatment can be challenging.
We present the case of a 17-year-old male who presented with a history of sudden-onset, postural headaches associated with upper thoracic back pain. Magnetic resonance imaging (MRI) demonstrated a thoracic extradural fluid collection and slumping of the brain within the posterior fossa. The patient was initially managed with a period of bed rest, followed by a thoracic epidural blood patch. Symptoms recurred and subsequent operative exploration found a large arachnoid cyst with CSF egress through a linear split in the axilla of the right T7 nerve root. The arachnoid cyst was resected, and the defect was closed primarily. All symptoms completely resolved. MRI at 3 months postoperatively demonstrated normal spinal configuration and resolution of brain sagging.
Spontaneous CSF leaks are a rare cause of postural headache. Although epidural blood patching is an easy and safe intervention, early serial imaging to ascertain the evolution of the pathology may identify cases that are amenable to early surgical management.
Spinal meningeal diverticula, spontaneous intracranial hypotension, and superficial siderosis.
Schievink WI, Maya MM.
Neurology. 2017 Feb 28;88(9):916-917. doi: 10.1212/WNL.0000000000003665. Epub 2017 Jan 27.
No Abstract Available.
Reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome associated with intracranial hypotension.
Feil K, Forbrig R, Thaler FS, Conrad J, Heck S, Dorn F, Pfister HW, Straube A.
Neurocrit Care. 2017 Feb;26(1):103-108. doi: 10.1007/s12028-016-0320-4.
Reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) are both rare disorders. The pathophysiology of both diseases is not yet fully understood.
We report the unique case of a 19-year-old comatose woman who was brought to the ER after a series of generalized tonic-clonic seizures 6 days post peridural anesthesia for cesarean section. Vital signs and initial laboratory testing including urine analysis and drug screening were unremarkable. Initial cranial CT scan showed an acute small subdural hematoma (17 mm length × 6 mm width × 30 mm height), cerebral edema with slit ventricles, and slight cerebellar tonsillar herniation as signs of intracranial hypotension. CT angiography depicted narrowing of the proximal intracranial vessels consistent with RCVS. MR imaging was also suggestive of both intracranial hypotension and RCVS and showed, in addition, vasogenic edema consistent with PRES. An extensive CSF leakage involving T1 to L2/L3 was confirmed by spinal MRI.
The patient underwent conservative therapy for intracranial hypotension (e.g., head-down position) as well as epidural blood patch, which led to regression of the clinical symptoms within a few days. Follow-up MRI showed complete resolution of all radiological changes.
In summary, our patient developed clinical and neuroradiological signs of intracranial hypotension and a combination of PRES and RCVS associated with a CSF leakage caused by peridural anesthesia; by treating the intracranial hypotension, the other syndromes resolved. From a clinical point of view, it is important to look for CSF leakage as a treatable possible cause of PRES and/or RCVS triggered by intracranial hypotension as in our patient postpartum. Moreover, it is vital to obtain a good history as, in cases of suspected CSF leakage with classic postural headache, a recent spinal/cranial procedure is typically present.
The Monro-Kellie Doctrine in Action: Posterior Reversible Leukoencephalopathy Syndrome Caused by Intracranial Hypotension from Lumboperitoneal Shunt Placement.
Karakis I, Nuccio AH, Amadio JP, Fountain AJ Jr.
World Neurosurg. 2017 Feb;98:868.e11-868.e15. doi: 10.1016/j.wneu.2016.12.046. Epub 2016 Dec 23.
Posterior reversible leukoencephalopathy syndrome (PRES) is linked to various etiologies, including most importantly systemic hypertension. Its association with intracranial hypotension (IH), a potential sequela of various neurosurgical procedures, is underrecognized. We report a case of lumboperitoneal shunt-induced IH resulting in PRES with the goal to increase awareness and elaborate on the potential biologic mechanism, based on the Monro-Kellie hypothesis.
A 26-year-old woman with acquired immunodeficiency syndrome and epilepsy was admitted for recurrent cryptococcal meningitis and breakthrough seizures. There was radiologic evidence of ventricular enlargement, and opening pressure on serial lumbar punctures was constantly elevated. Owing to persistently elevated, symptomatic intracranial pressure and transient relief with serial lumbar punctures, a lumboperitoneal shunt was placed. The patient subsequently had a breakthrough seizure and became encephalopathic. Repeat head imaging showed reduced ventricular size, engorged venous sinuses, and tonsillar herniation in keeping with IH, coupled with extensive white matter abnormalities in bilateral parieto-occipital lobes indicative of PRES. The patient had an emergent programmable valve placed in the lumboperitoneal shunt to prevent excessive cerebrospinal fluid drainage, leading to clinical and radiologic improvement. Subsequent cerebrospinal fluid leak resulted in recurrent presentation.
IH appears to be a distinct cause of PRES not previously reported in the neurosurgical literature. It occurs in susceptible patients, on average 1-5 days after the IH trigger, and seems clinically and radiologically similar to more common hypertensive cases in terms of initial presentation and prognosis. Increased vigilance is required for prompt recognition and management.
Headache Rounds: Orthostatic Headache Complicated by Transient Right Hemisensory Loss.
Planas-Ballvé A, Da Silva C, Martin L, Broto J, Crespo-Cuevas AM, López-Cancio E, Munuera J, Millán M, Dávalos A.
Headache. 2017 Feb;57(2):302-308. doi: 10.1111/head.13015.
Novel orbital findings of intracranial hypotension.
Holbrook JF, Hudgins PA, Bruce BB, Saindane AM.
Clin Imaging. 2017 Jan – Feb;41:125-131. doi: 10.1016/j.clinimag.2016.10.019. Epub 2016 Oct 29.
To determine whether orbital findings on routine brain MRI can be used to differentiate patients with intracranial hypotension from controls.
The authors evaluated axial T2-weighted images for the amount of optic nerve sheath CSF and 3D-T1-weighted images for optic nerve angle of sixteen patients with intracranial hypotension and 60 controls.
Patients with intracranial hypotension demonstrated significantly decreased CSF in the optic nerve sheath. Optic nerve angle was higher in the intracranial hypotension group compared to controls.
Decreased optic nerve sheath CSF and straightened optic nerve angle are significantly more common in the setting of intracranial hypotension.
Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension.
Wu JW, Hseu SS, Fuh JL, Lirng JF, Wang YF, Chen WT, Chen SP, Wang SJ.
Brain. 2017 Jan 2. pii: aww328. doi: 10.1093/brain/aww328. [Epub ahead of print]
Spontaneous intracranial hypotension results from cerebrospinal fluid leakage. Currently, the treatment of choice for spontaneous intracranial hypotension is the epidural blood patch, which has a variable response rate and no clear outcome predictors. This study aimed to identify predictors for response rate of a first targeted epidural blood patch in patients with spontaneous intracranial hypotension. We reviewed cases of patients with spontaneous intracranial hypotension who received targeted epidural blood patch at our hospital between 1 January 2007 and 1 July 2014. The outcome measure was first epidural blood patch response. We analysed demographics, clinical manifestations, neuroimaging findings (non-contrast heavily T2-weighted magnetic resonance myelography and brain magnetic resonance imaging), and blood volume as potential outcome predictors. Significant predictors were tested and a decision tree was used to construct a predictive model. In total, 150 patients with spontaneous intracranial hypotension were included for final analyses. Their overall first targeted epidural blood patch response rate was 58.7%. Among patients with a greater injected blood volume (≥22.5 versus <22.5 ml), the response rate was higher (67.9% versus 47.0%, P = 0.01). In brain and spinal magnetic resonance imaging studies, significant predictors included anterior epidural cerebrospinal fluid collection length (<8 versus ≥8 segments; 72.5% versus 37.3%, odds ratio = 4.4, 95% confidence interval: 2.2-8.9, P < 0.001) and midbrain-pons angle (≥40° versus <40°; 71.3% versus 37.5%, odds ratio = 4.1, 95% confidence interval 2.1-8.3, P < 0.001). Decision tree analyses showed that patients with anterior epidural CSF collection involving <8 segments and an injected blood volume ≥22.5 ml had an 80.0% response rate. Patients with anterior epidural cerebrospinal fluid collection involving ≥8 segments and a midbrain-pons angle <40° had a 21.2% response rate. These three variables predicted first epidural blood patch response in 71.3% of patients. Brain and spinal neuroimaging findings and epidural blood patch blood volume can be used to predict targeted first epidural blood patch response in patients with spontaneous intracranial hypotension.
Intracranial structural alteration predicts treatment outcome in patients with spontaneous intracranial hypotension.
Choi H, Lee MJ, Choi HA, Cha J, Chung CS.
Cephalalgia. 2017 Jan 1:333102417690106. doi: 10.1177/0333102417690106. [Epub ahead of print]
Background Intracranial structural dislocation in spontaneous intracranial hypotension (SIH) can be measured by various intracranial angles and distances. We aimed to identify the clinical significance of structural dislocation in relation to treatment outcome in patients with SIH. Methods In this retrospective analysis, we identified patients with SIH who received an epidural blood patch (EBP) at Samsung Medical Center from January 2005 to March 2015. Structural dislocation in pretreatment MRIs of SIH patients was assessed by measuring tonsillar herniation, mamillopontine distance, the angle between the vein of Galen and straight sinus (vG/SS angle), the pontomesencephalic angle, and the lateral ventricular angle. After the first EBP, poor response was defined as the persistence of symptoms that prompted a repeat EBP. Results Out of the 95 patients included, 31 (32.6%) showed poor response. Among the radiological markers of structural dislocation, the vG/SS angle was associated with poor response (49.82 ± 16.40° vs 66.58 ± 26.08°, p = 0.002). Among clinical variables, premorbid migraine ( p = 0.036) was related to poor response. In multivariate analysis, reduced vG/SS angle was independently associated with poor response (OR 1.04 [95% CI 1.01 – 1.07] per 1° decrease, p = 0.006). In 23 patients who underwent MRI after successful treatment, the vG/SS angle significantly increased after the EBP ( p < 0.001, by paired t-test), while two patients with aggravation or recurrence showed a further reduction of their vG/SS angles. Conclusions Intracranial structural dislocation, measured by the vG/SS angle, is associated with poor response to the first EBP in patients with SIH. Successful treatment can reverse the structural dislocation. PMID: 28110544