- Email: [email protected]
Intracranial hypotension secondary to spinal pathology: Diagnosis and treatment
Clinical Neurology and Neurosurgery 143 (2016) 95–98
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Intracranial hypotension secondary to spinal pathology: Diagnosis and treatment Kamyar Sartip a,∗ , Gregory McKenna b , Michael Spina b , Stephen Grahovac c a b c
Department of Radiology, Howard University College of Medicine, 2041 Georgia Ave NW, Washington, DC 20060, United States Department of Radiology, Christiana Care Health System, 4755 Ogletown-Stanton Road, Newark, DE 19718, United States Department of Radiology, Queen’s University, Kidd 1, 76 Stuart Street Kingston, ON K7L 4K6, Canada
a r t i c l e
i n f o
Article history: Received 17 December 2015 Received in revised form 9 February 2016 Accepted 10 February 2016 Available online 16 February 2016 Keywords: Intracranial hypotension Postural headache Orthostatic headache CSF leakage Spine
a b s t r a c t Spinal pathology resulting in cerebrospinal fluid (CSF) leak and intracranial hypotension is an infrequently reported and a potentially severe cause of headaches. We present a case of cerebrospinal fluid (CSF) leak caused by a thoracic disk herniation successfully treated with two targeted epidural blood patches. Although patients typically present with orthostatic headaches, the imaging findings of intracranial hypotension should prompt investigation of the spine for site and cause of the CSF leakage. Treatment includes autologous blood patch and surgery in refractory cases. © 2016 Published by Elsevier B.V.
1. Introduction
2. Methods
Intracranial hypotension from cerebrospinal fluid (CSF) leak is a rare and potentially severe cause of refractory headaches. The condition can be a diagnostic challenge since the chief compliant is headaches while its etiology is elsewhere in the neural axis. Often the CSF leak occurs spontaneously from a suspected inherent weakness in the dura and is referred to as the syndrome of spontaneous intracranial hypotension (SIH) [1,2]. However, spinal pathology such as bone spurs and disc herniations can cause mechanical tears in the dura resulting in significant CSF leakage and intracranial hypotension [1]. While there have been a few case reports of CSF leaks secondary to spinal pathology, majority of the published literature on intracranial hypotension has focused on the diagnosis and treatment of spontaneous CSF leaks. In this paper, we present a review of the world literature on CSF leaks secondary to spinal pathology with a focus on treatment and outcomes.
A pubmed search using the key terms intracranial hypotension, syndrome of intracranial hypotension, CSF leakage, and spine was performed. Literature discussing intracranial hypotension secondary to CSF leakage from spinal pathology from 1990 to 2015 was selected for review.
∗ Corresponding author. E-mail addresses: [email protected] (K. Sartip), [email protected] (G. McKenna), [email protected] (M. Spina), [email protected] (S. Grahovac). http://dx.doi.org/10.1016/j.clineuro.2016.02.017 0303-8467/© 2016 Published by Elsevier B.V.
2.1. Case report A 41 year old female with past medical history significant for obesity, gastric bypass surgery, chronic anemia, and cholecystectomy presented to the emergency department with a ten day history of worsening positional headaches, photophobia, nausea, and vomiting. The symptoms were spontaneous without triggering event or history of trauma. Magnetic resonance imaging (MRI) of the brain demonstrated classic signs of intracranial hypotension including smooth pachymeningeal enhancement and downward displacement of the cerebellum (Fig. 1). Computed tomography (CT) myelography of the entire neural axis was then performed which demonstrated extradural extravasation of contrast consistent with CSF leak associated with a T5-T6 disc herniation (Fig. 2). The disc herniation appeared sharp and likely resulted in a mechanical tear in the dura with flexion over time. After failing conservative treatment including hydration and caffeine, she underwent a 10 ml volume autologous epidural blood patch (EBP) under fluoroscopy
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K. Sartip et al. / Clinical Neurology and Neurosurgery 143 (2016) 95–98 Table 1 Imaging findings of intracranial hypotension on MRI of the brain. Pachymeningeal enhancement Subdural collections Engorgement of the dural sinuses (venous distension sign) Downward displacement of the cerebellum resulting in: • Tonsillar descent into foramen magnum (mimicking chiari 1 malformation) • Effacement of the suprasellar, prepontine, and prechiasmatic cisterns • Flattening or tenting of the optic chiasm Enlargement of the pituitary mimicking macroadenoma Decreased size of ventricles
Table 2 Imaging findings of intracranial hypotension on MRI of the spine. Epidural or subdural collections Spinal dural enhancement Engorgement of epidural venous plexus Prominent spinal cord veins Prominent disk or osteophyte as cause of CSF leakage
2.2. Imaging findings
Fig 1. 41 year old female with intracranial hypotension. (A) Coronal T1 post contrast image demonstrates thin smooth pachymeningeal enhancement along the convexities and falx cerebri. Downward displacement of the brain results in effacement of the suprasellar cistern (arrow). (B) Axial FLAIR image demonstrates hyperintense signal along the convexities and falx corresponding to regions of pachymeningeal enhancement. A subdural hematoma is not present. (C) Sagittal T1 image demonstrates prominence of the dura along the prepontine cistern (arrow) and downward displacement of the cerebellar tonsils resulting in ventral displacement of the pons. (D) Sagittal T1 image demonstrates convex inferior margin of the dominant transverse sinus described as the venous distension sign (arrow).
Fig. 2. 41 year old female with intracranial hypotension. Axial (A) and sagittal (B) reformatted CT myelogram images demonstrate a central calcified disc herniation (white arrow) indenting the ventral aspect of the thecal sac and distorting the thoracic cord. Extra-dural contrast material can be seen dorsally and laterally (red arrows). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
at T5-T6 which resulted in resolution of symptoms prior to hospital discharge. She underwent a second 10 ml volume autologous EBP at T5-T6 three weeks later in the outpatient setting secondary to relapse of headaches. Her symptoms have not recurred in the last 9 years.
The classic presentation of intracranial hypotension is orthostatic headaches, low CSF opening pressure, and smooth pachymeningeal enhancement [1,2]. Other brain MRI findings include downward displacement of the cerebellum resulting in effacement of the prepontine and prechiasmatic cisterns, flattening or tenting of the optic chiasm, subdural effusions, enlargement of the pituitary, and engorgement of the dural sinuses (Table 1) [2–4]. In some cases, there can be irregular nodular thickening of the meninges which could mimic an infectious etiology such as tuberculous meningitis. However, imaging findings such as sagging of the brainstem and engorgement of dural sinuses combined with clinical findings such as absence of fever can be used to exclude infectious pathology. MRI findings in the spine (Fig. 3) include epidural fluid collection, dilation of the epidural venous plexus, and prominent spinal cord veins (Table 2) [5]. In rare cases, the CSF tear in the dura may be tamponaded by a herniated cord, a phenomenon referred to as idiopathic thoracic spinal cord herniation [6]. In such instances, there is regression of postural headaches and development of progressing myelopathy [6]. When imaging and clinical findings are suggestive of intracranial hypotension, examination of the entire neural axis should be performed to identify the site of CSF leakage. CT myelography with water soluble contrast is the study of choice for detection of spinal CSF leak [1]. In addition to confirming the presence and location of CSF leak by demonstrating extra-thecal extravasation of contrast, structural abnormalities such as disc herniations, osteophytes, and meningeal diverticula as the cause of dural tear can be identified. Imaging can also reveal additional findings such as disc herniation causing spinal canal stenosis or intramedullary disease such as syringomyelia. Immediate CT after intrathecal injection or careful evaluation under digital subtraction angiography can identify the site of leakage in patients with rapid CSF leak [7]. Valsalva maneuver, walking several minutes after intrathecal injection, delayed imaging up to 4 h, and infusion of artificial CSF can increase sensitivity of finding the leak site in patients with slow flow CSF leaks [3,7,8]. Nuclear cisternography has also been used to detect the presence of a CSF leak; however it often fails to confirm the exact site of leakage [2]. 3. Discussion In a recent review of the literature, we found an additional 19 cases of CSF leak secondary to spinal pathology (Table 3) [9–22]. The mean age was 42.4 years (range: 25–57) and female to male
K. Sartip et al. / Clinical Neurology and Neurosurgery 143 (2016) 95–98
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Table 3 Summary of characteristics, management, and outcomes of intracranial hypotension from spinal pathology. Year
Author
Age, sex
Spinal pathology
EBP lumbar
EBP targeted
Surgery
Headache outcome
Follow-up
1998 2002 2002 2002 2002 2003 2005 2008 2010 2012 2012 2012 2013 2013 2013 2013 2013 2014 2014 2015
Vishteh Eross Eross Eross Winter Rapport Binder Yokota Kim Witiw Hasiloglu Hasiloglu Wilson Argawal Allmendinger Allmendinger Allmendinger Rapoport Hung Sartip
32, m 44, f 39, f 46, m 42, f 37, f 55, f 25, f 31, m 46, f 32, f 42, f 47, m 57, m 56,f 47, m 49, m 45, m 34, f 41, f
C5-C6 osteophyte C5-6 osteophyte + disk C7-T1 osteophyte + disk C4-C5 disk + osteophyte T7-T8 calcified disk T7-T8 disk T2-T3 osteophyte T8-T9 osteophyte L2-L3 disk C4-C5 calcified disk T2-T3 osteophyte with disk T11-12 osteophyte and disk T6-T7 calcified disk T6-7 calcified disk T6-7 calcified disk T6-7 calcified disk T5-6 calcified disc T12-L1 disc T2-T3 osteophyte T5-T6 calcified disk
1 1 3 1 0 0 1 1 0 3 1 0 3 0 1 0 2 0 2 0
0 2 0 0 1 2 0 1 0 0 0 1 0 1 1 2 2 0 1 2
Y Y Y N Y N Y N Y Y N N Y N N N N Y N N
Resolved Remained Improved Improved Resolved Improved Resolved Resolved Resolved Resolved Resolved Resolved Resolved Resolved Resolved Resolved resolved Resolved Resolved Resolved
ND 32 months 1 month 2 months 18 months ND 12 months 24 months 9 days 2 months 4 months 6 months 6 months 5 weeks ND ND ND 3 weeks 20 months 9 years
Fig. 3. 41 year old female with intracranial hypotension. Gradient echo Axial (A) and T2 sagittal (B) MR images demonstrate a central disc herniation indenting the ventral cord at T5-T6 (white arrow). Epidural fluid collection dorsal and lateral to the cord is seen corresponding to region of extra-dural contrast extravasation noted on CT myelogram (red arrows). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
ratio was 1.5:1. Four were secondary to an osteophyte, eleven were secondary to a disc herniation, and both an osteophyte and disc herniation where found at the level of CSF leakage in the remaining five cases. Fourteen were in the thoracic, five in the cervical spine, and one in the lumbar spine. Of note, nine (45%) were secondary
to pathology between T5 and T9. Time of symptom onset to initial presentation was mentioned in 11 of 20 case reports, and ranged from 1 day to 3 months. Interestingly, the dural tear was caused either by a prominent osteophyte or calcified disc herniation in 17 of 20 cases. This highlights the importance of the morphology and architecture of the spinal pathology in the pathophysiology of this disease. It may also explain why spinal CSF leaks are rare while disk herniations and osteophytes are common. The management of CSF leak occurring from disk herniation or osteophytes is not standardized, however EBP performed in a similar fashion to patients with SIH is first line treatment [1,21]. The EBP is though to create a seal preventing further CSF leakage while allowing the dural tear to heal [1,23]. Other hypothesized mechanism includes increasing epidural pressure thereby reversing the CSF-hematic gradient that exists in the spine, which causes intracranial hypotension to subside [24]. Initial placement can be at the lumbar level as the procedure caries less risk than a target injection at the leakage site [1,11]. However, studies have shown direct EBP injection at site of leakage to be more efficacious in patients with spontaneous intracranial hypotension and this may also hold true in case of CSF leakage from spinal pathology [2,4]. When lumbar injection is performed, the patient can be placed in a Trendelenburg position for a brief period of time similar to cervical myelography so the blood extends in a cephalad direction [1]. Allowing the patient to lay supine for 2–4 h after the injection may improve results. In our review, twelve patients (11 thoracic, 1 cervical) were treated only with EBP, with most (7/12) requiring between two and four injections. Of these twelve patients, four underwent targeted injection(s) after failing lumbar EBP, six were treated only with target injection(s), and two were treated only with lumbar injection. Ten of the patients treated only with EBP showed complete resolution of symptoms, while the other 2 demonstrated improvement. Eight patients (4 cervical, 4 thoracic, 1 lumbar) were treated with spinal surgery. Surgery included either hemilaminectomy or laminectomy, discectomy or removal of osteophytes, and primary closure of the dura. Six patients showed resolution of symptoms while one showed improvement after surgery. Symptoms remained in one of the eight surgical patients. Majority (6/8) of the patients in the surgical group received EBP prior to surgery but only 4 had more than one injection, and only 1 had a target injection at the site of leak. Only 1 of the 4 patients with cervical pathology received a targeted injection. Research has shown that
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K. Sartip et al. / Clinical Neurology and Neurosurgery 143 (2016) 95–98
epidural blood patch may have a limited range of effectiveness and lumbar injection may be out of effective range for dural tears in the cervical and upper thoracic spine [20]. Therefore we advocate a targeted injection before surgery is attempted. Overall, nineteen of the twenty patients showed good outcome, with majority (16/20) showing resolution of symptoms. Total follow-up ranged between 3 weeks and 9 years. However the limited follow-up period in most cases likely introduces bias into the interpretation of the outcomes. 4. Conclusion EBP can be an effective treatment for intracranial hypotension secondary to spinal pathology. Although more technically challenging, most successfully treated cases required a targeted injection for symptoms to resolve. Multiple injections are often necessary. Surgery can be effective in refractory cases. Disclosures None. Reference: [1] J. Inamasu, B.H. Guiot, Intracranial hypotension with spinal pathology, Spine J. 6 (2006) 591–599. [2] T.J. Schwedt, D.W. Dodick, Spontaneous intracranial hypotension, Curr. Pain Headache Rep. 11 (2007) 56–61. [3] B. Mokri, Cerebrospinal fluid volume depletion and its emerging clinical/imaging syndromes, Neurosurg. Focus 9 (2000) e6. [4] W.I. Schievink, Spontaneous spinal cerebrospinal fluid leaks: a review, Neurosurg. Focus 9 (2000) e8. [5] M.T. Burtis, J.L. Ulmer, G.A. Miller, A.C. Barboli, S.A. Koss, W.D. Brown, Intradural spinal vein enlargement in craniospinal hypotension, AJNR Am. J. Neuroradiol. 26 (2005) 34–38. [6] M. Brus-Ramer, W.P. Dillon, Idiopathic thoracic spinal cord herniation: retrospective analysis supporting a mechanism of diskogenic dural injury and subsequent tamponade, AJNR Am. J. Neuroradiol. 33 (2012) 52–56. [7] P.H. Luetmer, B. Mokri, Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks, AJNR Am. J. Neuroradiol. 24 (2003) 1711–1714. [8] W.I. Schievink, M.M. Maya, F.M. Moser, Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. Report of four cases, J. Neurosurg. 100 (2004) 1098–1100.
[9] S.C. Winter, N.F. Maartens, P. Anslow, P.J. Teddy, Spontaneous intracranial hypotension due to thoracic disc herniation. Case report, J. Neurosurg. 96 (2002) 343–345. [10] A.G. Vishteh, W.I. Schievink, J.J. Baskin, V.K. Sonntag, Cervical bone spur presenting with spontaneous intracranial hypotension. Case report, J. Neurosurg. 89 (1998) 483–484. [11] E.J. Eross, D.W. Dodick, K.D. Nelson, P. Bosch, M. Lyons, Orthostatic headache syndrome with CSF leak secondary to bony pathology of the cervical spine, Cephalalgia 22 (2002) 439–443. [12] D.K. Binder, V. Sarkissian, W.P. Dillon, P.R. Weinstein, Spontaneous intracranial hypotension associated with transdural thoracic osteophyte reversed by primary. dural repair. Case report, J. Neurosurg. Spine 2 (2005) 614–618. [13] R.L. Rapport, D. Hillier, T. Scearce, C. Ferguson, Spontaneous intracranial hypotension from intradural thoracic disc herniation. Case report, J. Neurosurg. 98 (2003) 282–284. [14] C.D. Witiw, A. Fallah, P.J. Muller, H.J. Ginsberg, Surgical treatment of spontaneous intracranial hypotension secondary to degenerative cervical spine pathology: a case report and literature review, Eur. Spine J. 21 (Suppl. 4) (2012) S422–7. [15] H. Yokota, K. Yokoyama, H. Noguchi, Y. Uchiyama, S. Iwasaki, T. Sakaki, Thoracic osteophyte causing spontaneous intracranial hypotension, Cephalalgia 28 (2008) 396–398. [16] Z.I. Hasiloglu, B. Abuzayed, A.E. Imal, E. Cagil, S. Albayram, Spontaneous intracranial hypotension due to intradural thoracic osteophyte with superimposed disc herniation: report of two cases, Eur. Spine J. 21 (Suppl. 4) (2012) S383–6. [17] K.T. Kim, Y.B. Kim, Spontaneous intracranial hypotension secondary to lumbar disc herniation, J. Korean Neurosurg. Soc. 47 (2010) 48–50. [18] D. Wilson, J. Christie, R. Ferch, Successful surgical treatment of intractable spontaneous intracranial hypotension due to a calcified thoracic disc prolapse, J. Clin. Neurosci. 20 (2013) 1773–1775. [19] V. Agarwal, G. Sreedher, W.E. Rothfus, Targeted CT-guided epidural blood patch for treatment of spontaneous intracranial hypotension due to calcified intradural thoracic disc herniation, Interv. Neuroradiol. 19 (2013) 121–126. [20] A.M. Allmendinger, T.C. Lee, Spontaneous intracranial hypotension from calcified thoracic disc protrusions causing CSF leak successfully treated with targeted epidural blood patch, Clin. Imaging 37 (2013) 756–761. [21] L.C. Hung, Y.C. Hsu, Spontaneous intracranial hypotension resulting from a thoracic osteophyte, J. Clin. Neurosci. 22 (2015) 1054–1056. [22] B.I. Rapoport, R. Hartl, T.H. Schwartz, Cranial neuropathy due to intradural disc herniation, Neurosurgery 74 (2014) E561–5. [23] F. van Kooten, R. Oedit, S.L. Bakker, D.W. Dippel, Epidural blood patch in post dural puncture headache: a randomised, observer-blind, controlled clinical trial, J. Neurol. Neurosurg. Psychiatry 79 (2008) 553–558. [24] A. Franzini, G. Messina, V. Nazzi, E. Mea, M. Leone, L. Chiapparini, et al., Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and a novel patch method in a series of 28 consecutive patients, J. Neurosurg. 112 (2010) 300–306.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Intracranial hypotension secondary to spinal pathology: Diagnosis and treatment Kamyar Sartip a,∗ , Gregory McKenna b , Michael Spina b , Stephen Grahovac c a b c
Department of Radiology, Howard University College of Medicine, 2041 Georgia Ave NW, Washington, DC 20060, United States Department of Radiology, Christiana Care Health System, 4755 Ogletown-Stanton Road, Newark, DE 19718, United States Department of Radiology, Queen’s University, Kidd 1, 76 Stuart Street Kingston, ON K7L 4K6, Canada
a r t i c l e
i n f o
Article history: Received 17 December 2015 Received in revised form 9 February 2016 Accepted 10 February 2016 Available online 16 February 2016 Keywords: Intracranial hypotension Postural headache Orthostatic headache CSF leakage Spine
a b s t r a c t Spinal pathology resulting in cerebrospinal fluid (CSF) leak and intracranial hypotension is an infrequently reported and a potentially severe cause of headaches. We present a case of cerebrospinal fluid (CSF) leak caused by a thoracic disk herniation successfully treated with two targeted epidural blood patches. Although patients typically present with orthostatic headaches, the imaging findings of intracranial hypotension should prompt investigation of the spine for site and cause of the CSF leakage. Treatment includes autologous blood patch and surgery in refractory cases. © 2016 Published by Elsevier B.V.
1. Introduction
2. Methods
Intracranial hypotension from cerebrospinal fluid (CSF) leak is a rare and potentially severe cause of refractory headaches. The condition can be a diagnostic challenge since the chief compliant is headaches while its etiology is elsewhere in the neural axis. Often the CSF leak occurs spontaneously from a suspected inherent weakness in the dura and is referred to as the syndrome of spontaneous intracranial hypotension (SIH) [1,2]. However, spinal pathology such as bone spurs and disc herniations can cause mechanical tears in the dura resulting in significant CSF leakage and intracranial hypotension [1]. While there have been a few case reports of CSF leaks secondary to spinal pathology, majority of the published literature on intracranial hypotension has focused on the diagnosis and treatment of spontaneous CSF leaks. In this paper, we present a review of the world literature on CSF leaks secondary to spinal pathology with a focus on treatment and outcomes.
A pubmed search using the key terms intracranial hypotension, syndrome of intracranial hypotension, CSF leakage, and spine was performed. Literature discussing intracranial hypotension secondary to CSF leakage from spinal pathology from 1990 to 2015 was selected for review.
∗ Corresponding author. E-mail addresses: [email protected] (K. Sartip), [email protected] (G. McKenna), [email protected] (M. Spina), [email protected] (S. Grahovac). http://dx.doi.org/10.1016/j.clineuro.2016.02.017 0303-8467/© 2016 Published by Elsevier B.V.
2.1. Case report A 41 year old female with past medical history significant for obesity, gastric bypass surgery, chronic anemia, and cholecystectomy presented to the emergency department with a ten day history of worsening positional headaches, photophobia, nausea, and vomiting. The symptoms were spontaneous without triggering event or history of trauma. Magnetic resonance imaging (MRI) of the brain demonstrated classic signs of intracranial hypotension including smooth pachymeningeal enhancement and downward displacement of the cerebellum (Fig. 1). Computed tomography (CT) myelography of the entire neural axis was then performed which demonstrated extradural extravasation of contrast consistent with CSF leak associated with a T5-T6 disc herniation (Fig. 2). The disc herniation appeared sharp and likely resulted in a mechanical tear in the dura with flexion over time. After failing conservative treatment including hydration and caffeine, she underwent a 10 ml volume autologous epidural blood patch (EBP) under fluoroscopy
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K. Sartip et al. / Clinical Neurology and Neurosurgery 143 (2016) 95–98 Table 1 Imaging findings of intracranial hypotension on MRI of the brain. Pachymeningeal enhancement Subdural collections Engorgement of the dural sinuses (venous distension sign) Downward displacement of the cerebellum resulting in: • Tonsillar descent into foramen magnum (mimicking chiari 1 malformation) • Effacement of the suprasellar, prepontine, and prechiasmatic cisterns • Flattening or tenting of the optic chiasm Enlargement of the pituitary mimicking macroadenoma Decreased size of ventricles
Table 2 Imaging findings of intracranial hypotension on MRI of the spine. Epidural or subdural collections Spinal dural enhancement Engorgement of epidural venous plexus Prominent spinal cord veins Prominent disk or osteophyte as cause of CSF leakage
2.2. Imaging findings
Fig 1. 41 year old female with intracranial hypotension. (A) Coronal T1 post contrast image demonstrates thin smooth pachymeningeal enhancement along the convexities and falx cerebri. Downward displacement of the brain results in effacement of the suprasellar cistern (arrow). (B) Axial FLAIR image demonstrates hyperintense signal along the convexities and falx corresponding to regions of pachymeningeal enhancement. A subdural hematoma is not present. (C) Sagittal T1 image demonstrates prominence of the dura along the prepontine cistern (arrow) and downward displacement of the cerebellar tonsils resulting in ventral displacement of the pons. (D) Sagittal T1 image demonstrates convex inferior margin of the dominant transverse sinus described as the venous distension sign (arrow).
Fig. 2. 41 year old female with intracranial hypotension. Axial (A) and sagittal (B) reformatted CT myelogram images demonstrate a central calcified disc herniation (white arrow) indenting the ventral aspect of the thecal sac and distorting the thoracic cord. Extra-dural contrast material can be seen dorsally and laterally (red arrows). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
at T5-T6 which resulted in resolution of symptoms prior to hospital discharge. She underwent a second 10 ml volume autologous EBP at T5-T6 three weeks later in the outpatient setting secondary to relapse of headaches. Her symptoms have not recurred in the last 9 years.
The classic presentation of intracranial hypotension is orthostatic headaches, low CSF opening pressure, and smooth pachymeningeal enhancement [1,2]. Other brain MRI findings include downward displacement of the cerebellum resulting in effacement of the prepontine and prechiasmatic cisterns, flattening or tenting of the optic chiasm, subdural effusions, enlargement of the pituitary, and engorgement of the dural sinuses (Table 1) [2–4]. In some cases, there can be irregular nodular thickening of the meninges which could mimic an infectious etiology such as tuberculous meningitis. However, imaging findings such as sagging of the brainstem and engorgement of dural sinuses combined with clinical findings such as absence of fever can be used to exclude infectious pathology. MRI findings in the spine (Fig. 3) include epidural fluid collection, dilation of the epidural venous plexus, and prominent spinal cord veins (Table 2) [5]. In rare cases, the CSF tear in the dura may be tamponaded by a herniated cord, a phenomenon referred to as idiopathic thoracic spinal cord herniation [6]. In such instances, there is regression of postural headaches and development of progressing myelopathy [6]. When imaging and clinical findings are suggestive of intracranial hypotension, examination of the entire neural axis should be performed to identify the site of CSF leakage. CT myelography with water soluble contrast is the study of choice for detection of spinal CSF leak [1]. In addition to confirming the presence and location of CSF leak by demonstrating extra-thecal extravasation of contrast, structural abnormalities such as disc herniations, osteophytes, and meningeal diverticula as the cause of dural tear can be identified. Imaging can also reveal additional findings such as disc herniation causing spinal canal stenosis or intramedullary disease such as syringomyelia. Immediate CT after intrathecal injection or careful evaluation under digital subtraction angiography can identify the site of leakage in patients with rapid CSF leak [7]. Valsalva maneuver, walking several minutes after intrathecal injection, delayed imaging up to 4 h, and infusion of artificial CSF can increase sensitivity of finding the leak site in patients with slow flow CSF leaks [3,7,8]. Nuclear cisternography has also been used to detect the presence of a CSF leak; however it often fails to confirm the exact site of leakage [2]. 3. Discussion In a recent review of the literature, we found an additional 19 cases of CSF leak secondary to spinal pathology (Table 3) [9–22]. The mean age was 42.4 years (range: 25–57) and female to male
K. Sartip et al. / Clinical Neurology and Neurosurgery 143 (2016) 95–98
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Table 3 Summary of characteristics, management, and outcomes of intracranial hypotension from spinal pathology. Year
Author
Age, sex
Spinal pathology
EBP lumbar
EBP targeted
Surgery
Headache outcome
Follow-up
1998 2002 2002 2002 2002 2003 2005 2008 2010 2012 2012 2012 2013 2013 2013 2013 2013 2014 2014 2015
Vishteh Eross Eross Eross Winter Rapport Binder Yokota Kim Witiw Hasiloglu Hasiloglu Wilson Argawal Allmendinger Allmendinger Allmendinger Rapoport Hung Sartip
32, m 44, f 39, f 46, m 42, f 37, f 55, f 25, f 31, m 46, f 32, f 42, f 47, m 57, m 56,f 47, m 49, m 45, m 34, f 41, f
C5-C6 osteophyte C5-6 osteophyte + disk C7-T1 osteophyte + disk C4-C5 disk + osteophyte T7-T8 calcified disk T7-T8 disk T2-T3 osteophyte T8-T9 osteophyte L2-L3 disk C4-C5 calcified disk T2-T3 osteophyte with disk T11-12 osteophyte and disk T6-T7 calcified disk T6-7 calcified disk T6-7 calcified disk T6-7 calcified disk T5-6 calcified disc T12-L1 disc T2-T3 osteophyte T5-T6 calcified disk
1 1 3 1 0 0 1 1 0 3 1 0 3 0 1 0 2 0 2 0
0 2 0 0 1 2 0 1 0 0 0 1 0 1 1 2 2 0 1 2
Y Y Y N Y N Y N Y Y N N Y N N N N Y N N
Resolved Remained Improved Improved Resolved Improved Resolved Resolved Resolved Resolved Resolved Resolved Resolved Resolved Resolved Resolved resolved Resolved Resolved Resolved
ND 32 months 1 month 2 months 18 months ND 12 months 24 months 9 days 2 months 4 months 6 months 6 months 5 weeks ND ND ND 3 weeks 20 months 9 years
Fig. 3. 41 year old female with intracranial hypotension. Gradient echo Axial (A) and T2 sagittal (B) MR images demonstrate a central disc herniation indenting the ventral cord at T5-T6 (white arrow). Epidural fluid collection dorsal and lateral to the cord is seen corresponding to region of extra-dural contrast extravasation noted on CT myelogram (red arrows). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
ratio was 1.5:1. Four were secondary to an osteophyte, eleven were secondary to a disc herniation, and both an osteophyte and disc herniation where found at the level of CSF leakage in the remaining five cases. Fourteen were in the thoracic, five in the cervical spine, and one in the lumbar spine. Of note, nine (45%) were secondary
to pathology between T5 and T9. Time of symptom onset to initial presentation was mentioned in 11 of 20 case reports, and ranged from 1 day to 3 months. Interestingly, the dural tear was caused either by a prominent osteophyte or calcified disc herniation in 17 of 20 cases. This highlights the importance of the morphology and architecture of the spinal pathology in the pathophysiology of this disease. It may also explain why spinal CSF leaks are rare while disk herniations and osteophytes are common. The management of CSF leak occurring from disk herniation or osteophytes is not standardized, however EBP performed in a similar fashion to patients with SIH is first line treatment [1,21]. The EBP is though to create a seal preventing further CSF leakage while allowing the dural tear to heal [1,23]. Other hypothesized mechanism includes increasing epidural pressure thereby reversing the CSF-hematic gradient that exists in the spine, which causes intracranial hypotension to subside [24]. Initial placement can be at the lumbar level as the procedure caries less risk than a target injection at the leakage site [1,11]. However, studies have shown direct EBP injection at site of leakage to be more efficacious in patients with spontaneous intracranial hypotension and this may also hold true in case of CSF leakage from spinal pathology [2,4]. When lumbar injection is performed, the patient can be placed in a Trendelenburg position for a brief period of time similar to cervical myelography so the blood extends in a cephalad direction [1]. Allowing the patient to lay supine for 2–4 h after the injection may improve results. In our review, twelve patients (11 thoracic, 1 cervical) were treated only with EBP, with most (7/12) requiring between two and four injections. Of these twelve patients, four underwent targeted injection(s) after failing lumbar EBP, six were treated only with target injection(s), and two were treated only with lumbar injection. Ten of the patients treated only with EBP showed complete resolution of symptoms, while the other 2 demonstrated improvement. Eight patients (4 cervical, 4 thoracic, 1 lumbar) were treated with spinal surgery. Surgery included either hemilaminectomy or laminectomy, discectomy or removal of osteophytes, and primary closure of the dura. Six patients showed resolution of symptoms while one showed improvement after surgery. Symptoms remained in one of the eight surgical patients. Majority (6/8) of the patients in the surgical group received EBP prior to surgery but only 4 had more than one injection, and only 1 had a target injection at the site of leak. Only 1 of the 4 patients with cervical pathology received a targeted injection. Research has shown that
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epidural blood patch may have a limited range of effectiveness and lumbar injection may be out of effective range for dural tears in the cervical and upper thoracic spine [20]. Therefore we advocate a targeted injection before surgery is attempted. Overall, nineteen of the twenty patients showed good outcome, with majority (16/20) showing resolution of symptoms. Total follow-up ranged between 3 weeks and 9 years. However the limited follow-up period in most cases likely introduces bias into the interpretation of the outcomes. 4. Conclusion EBP can be an effective treatment for intracranial hypotension secondary to spinal pathology. Although more technically challenging, most successfully treated cases required a targeted injection for symptoms to resolve. Multiple injections are often necessary. Surgery can be effective in refractory cases. Disclosures None. Reference: [1] J. Inamasu, B.H. Guiot, Intracranial hypotension with spinal pathology, Spine J. 6 (2006) 591–599. [2] T.J. Schwedt, D.W. Dodick, Spontaneous intracranial hypotension, Curr. Pain Headache Rep. 11 (2007) 56–61. [3] B. Mokri, Cerebrospinal fluid volume depletion and its emerging clinical/imaging syndromes, Neurosurg. Focus 9 (2000) e6. [4] W.I. Schievink, Spontaneous spinal cerebrospinal fluid leaks: a review, Neurosurg. Focus 9 (2000) e8. [5] M.T. Burtis, J.L. Ulmer, G.A. Miller, A.C. Barboli, S.A. Koss, W.D. Brown, Intradural spinal vein enlargement in craniospinal hypotension, AJNR Am. J. Neuroradiol. 26 (2005) 34–38. [6] M. Brus-Ramer, W.P. Dillon, Idiopathic thoracic spinal cord herniation: retrospective analysis supporting a mechanism of diskogenic dural injury and subsequent tamponade, AJNR Am. J. Neuroradiol. 33 (2012) 52–56. [7] P.H. Luetmer, B. Mokri, Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks, AJNR Am. J. Neuroradiol. 24 (2003) 1711–1714. [8] W.I. Schievink, M.M. Maya, F.M. Moser, Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. Report of four cases, J. Neurosurg. 100 (2004) 1098–1100.
[9] S.C. Winter, N.F. Maartens, P. Anslow, P.J. Teddy, Spontaneous intracranial hypotension due to thoracic disc herniation. Case report, J. Neurosurg. 96 (2002) 343–345. [10] A.G. Vishteh, W.I. Schievink, J.J. Baskin, V.K. Sonntag, Cervical bone spur presenting with spontaneous intracranial hypotension. Case report, J. Neurosurg. 89 (1998) 483–484. [11] E.J. Eross, D.W. Dodick, K.D. Nelson, P. Bosch, M. Lyons, Orthostatic headache syndrome with CSF leak secondary to bony pathology of the cervical spine, Cephalalgia 22 (2002) 439–443. [12] D.K. Binder, V. Sarkissian, W.P. Dillon, P.R. Weinstein, Spontaneous intracranial hypotension associated with transdural thoracic osteophyte reversed by primary. dural repair. Case report, J. Neurosurg. Spine 2 (2005) 614–618. [13] R.L. Rapport, D. Hillier, T. Scearce, C. Ferguson, Spontaneous intracranial hypotension from intradural thoracic disc herniation. Case report, J. Neurosurg. 98 (2003) 282–284. [14] C.D. Witiw, A. Fallah, P.J. Muller, H.J. Ginsberg, Surgical treatment of spontaneous intracranial hypotension secondary to degenerative cervical spine pathology: a case report and literature review, Eur. Spine J. 21 (Suppl. 4) (2012) S422–7. [15] H. Yokota, K. Yokoyama, H. Noguchi, Y. Uchiyama, S. Iwasaki, T. Sakaki, Thoracic osteophyte causing spontaneous intracranial hypotension, Cephalalgia 28 (2008) 396–398. [16] Z.I. Hasiloglu, B. Abuzayed, A.E. Imal, E. Cagil, S. Albayram, Spontaneous intracranial hypotension due to intradural thoracic osteophyte with superimposed disc herniation: report of two cases, Eur. Spine J. 21 (Suppl. 4) (2012) S383–6. [17] K.T. Kim, Y.B. Kim, Spontaneous intracranial hypotension secondary to lumbar disc herniation, J. Korean Neurosurg. Soc. 47 (2010) 48–50. [18] D. Wilson, J. Christie, R. Ferch, Successful surgical treatment of intractable spontaneous intracranial hypotension due to a calcified thoracic disc prolapse, J. Clin. Neurosci. 20 (2013) 1773–1775. [19] V. Agarwal, G. Sreedher, W.E. Rothfus, Targeted CT-guided epidural blood patch for treatment of spontaneous intracranial hypotension due to calcified intradural thoracic disc herniation, Interv. Neuroradiol. 19 (2013) 121–126. [20] A.M. Allmendinger, T.C. Lee, Spontaneous intracranial hypotension from calcified thoracic disc protrusions causing CSF leak successfully treated with targeted epidural blood patch, Clin. Imaging 37 (2013) 756–761. [21] L.C. Hung, Y.C. Hsu, Spontaneous intracranial hypotension resulting from a thoracic osteophyte, J. Clin. Neurosci. 22 (2015) 1054–1056. [22] B.I. Rapoport, R. Hartl, T.H. Schwartz, Cranial neuropathy due to intradural disc herniation, Neurosurgery 74 (2014) E561–5. [23] F. van Kooten, R. Oedit, S.L. Bakker, D.W. Dippel, Epidural blood patch in post dural puncture headache: a randomised, observer-blind, controlled clinical trial, J. Neurol. Neurosurg. Psychiatry 79 (2008) 553–558. [24] A. Franzini, G. Messina, V. Nazzi, E. Mea, M. Leone, L. Chiapparini, et al., Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and a novel patch method in a series of 28 consecutive patients, J. Neurosurg. 112 (2010) 300–306.