False localizing sign of cervico-thoracic CSF leak in spontaneous intracranial hypotension Wouter I. Schievink, MD M. Marcel Maya, MD Ray M. Chu, MD Franklin G. Moser, MD, MMM

Correspondence to Dr. Schievink: [email protected]

ABSTRACT

Objective: Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic junction. The authors undertook a study to determine the significance of these cervico-thoracic CSF leaks.

Methods: The patient population consisted of a consecutive group of 13 patients who underwent surgery for CSF leak repair based on CT myelography showing CSF extravasation at the cervicothoracic junction but without any evidence of an underlying structural lesion.

Results: The mean age of the 9 women and 4 men was 41.2 years. Extensive extrathecal longitudinal CSF collections were demonstrated in 11 patients. At surgery, small leaking arachnoid cysts were found in 2 patients. In the remaining 11 patients, no clear source of CSF leakage could be identified at surgery. Resolution of symptoms was achieved in both patients with leaking arachnoid cysts, but in only 3 of the 11 patients with negative intraoperative findings. Postoperative spinal imaging was performed in 9 of the 11 patients with negative intraoperative findings and showed persistence of the longitudinal intraspinal extradural CSF. Further imaging revealed the site of the CSF leak to be ventral to the thoracic spinal cord. Five of these patients underwent microsurgical repair of the ventral CSF leak with resolution of symptoms in all 5 patients. Conclusions: Cervico-thoracic extravasation of dye on myelography does not necessarily indicate the site of the CSF leak. Treatment directed at this site should not be expected to have a high probability of sustained improvement of symptoms. Neurology® 2015;84:2445–2448

Spontaneous intracranial hypotension has become a routinely diagnosed cause of headaches.1,2 Although numerous other headache types and various other clinical manifestations have been reported, an orthostatic headache is the presenting symptom in more than 95% of patients diagnosed with spontaneous intracranial hypotension. A spontaneous CSF leak in the spine is found in most patients with spontaneous intracranial hypotension. Although the CSF leak can be found at any spinal level (i.e., cervical, thoracic, lumbar, or sacral), CSF leaks at the cervicothoracic junction are reported to be particularly frequent.1–3 We now present evidence that extravasation of CSF at the cervico-thoracic junction, i.e., leakage from within the spinal canal into surrounding tissues, may be a false localizing sign and that the actual site of the CSF leak should be suspected elsewhere in the spine. METHODS Standard protocol approvals, registrations, and patient consents. The study was approved by the Institutional Review Board of Cedars-Sinai Medical Center, Los Angeles, CA. A total of 338 patients with spontaneous intracranial hypotension evaluated at our institution between January 1, 2001, and December 31, 2010, were identified. The diagnosis of spontaneous intracranial hypotension was based on previously established diagnostic criteria.4 We limited the study population to 13 patients who underwent surgery for CSF leak repair based on CT myelography showing CSF extravasation at the cervico-thoracic junction but without any evidence of an underlying structural lesion. Thus, we did not include patients who underwent surgery based on preoperative imaging showing the presence of arachnoid cysts or ventral dural tears at the cervico-thoracic junction as the source of CSF leakage.

The mean age of the 13 patients (9 women and 4 men) was 41.2 years (range 29–58 years). All patients presented with orthostatic headaches. Clinical follow-up ranged from 17 to 147 months (mean 91 months).

RESULTS

From the Departments of Neurosurgery (W.I.S., R.M.C.) and Radiology (M.M.M., F.G.M.), Cedars-Sinai Medical Center, Los Angeles, CA. Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. © 2015 American Academy of Neurology

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Figure 1

Pseudo-cervico-thoracic CSF leak from ventral thoracic dural tear

Postmyelography CT shows dye extravasation into the musculature at C6-7 (A, arrow). Sagittal T2-weighted MRI shows ventral extradural CSF collection extending to Th8 (B, arrow). Digital subtraction myelography shows site of ventral CSF leak at Th5-6 (C, arrow).

Brain MRI showed one or more of the typical changes of spontaneous intracranial hypotension (sagging of the brain, subdural fluid collections, pachymeningeal enhancement, pituitary enlargement, and venous engorgement) in 12 of the 13 patients. CT myelography and MRI myelography showed extradural CSF along the nerve roots at the cervico-thoracic junction in all patients, often extending into the paraspinal musculature (9 patients) (figures 1–4). Extrathecal ventral longitudinal CSF collections extending over at least 12 spinal levels were demonstrated in the cervical and thoracic spine in 11 patients (figures 1– 4), while retrospinal CSF collections were found at the C1-2 level in 4 of these patients.

Figure 2

Before surgery, all patients underwent at least 2 epidural blood patches and 7 patients also underwent percutaneous injections of fibrin sealant directed at the cervico-thoracic junction. At surgery, small leaking arachnoid cysts arising from the axilla of the spinal nerve root sleeve were found in 2 patients (bilateral in 1). These cysts were not detectable on preoperative spinal imaging. These cysts were ligated with aneurysm clips. In the remaining 11 patients, no clear source of CSF leakage could be identified at surgery, although extradural CSF was visible intraoperatively either spontaneously or with a Valsalva maneuver in 7 patients (table). Only extradural exploration was performed in these patients and

Pseudo-cervico-thoracic CSF leak from ventral thoracic dural tear

Magnetic resonance myelogram shows extensive extradural CSF along the cervico-thoracic nerve roots (A, arrows). Sagittal T2-weighted MRI shows ventral extradural CSF collection extending to Th9 (B, arrow). Digital subtraction myelography shows site of ventral CSF leak at Th6-7 (C, arrow). 2446

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Figure 3

Pseudo-cervico-thoracic CSF leak from ventral thoracic dural tear

Postmyelography CT shows dye extravasation along the C7 nerve roots (A, arrows). Sagittal T2-weighted MRI shows ventral extradural CSF collection confined to Th7-8 (B, arrow), confirmed by digital subtraction myelography (C, arrow).

treatment consisted of packing the epidural space with muscle or gelatin sponge. Preoperative imaging demonstrated extensive longitudinal intraspinal extradural CSF collection in all 11 patients in whom no anatomical substrate of the CSF leak could be identified at the time of surgery. Such a longitudinal intraspinal CSF collection was not present on preoperative imaging in the 2 patients with small leaking arachnoid cysts. Complete or near-complete resolution of symptoms was achieved in both patients with small leaking arachnoid cysts, but in only 3 of the 11 patients with negative intraoperative findings. Postoperative spinal imaging was performed months or years after the surgery in 9 (8 with persistent symptoms and 1 with back pain) of the 11 patients with negative intraoperative findings and showed persistence of the longitudinal intraspinal extradural CSF collection in all 9 patients. Further imaging using digital subtraction myelography revealed the exact site of the CSF leak to be ventral to the spinal cord in the thoracic spine in all of these 9 patients (figures 1–3). Through a

Figure 4

posterior transpedicular approach, 5 of these patients underwent microsurgical repair of the ventral CSF leak between 8 and 137 months (mean 59.2 months) after the initial surgery, resulting in complete resolution of symptoms in all 5 patients. At surgery, the dural defects were noted to be at the level of the disc space in all 5 patients (figure 4) and were associated with calcified disc material in 4 patients. DISCUSSION In this study, we demonstrated that CSF extravasation at the cervico-thoracic junction uncommonly corresponds to the actual site of the dural tear, at least among patients undergoing surgery. This is a situation similar to that of CSF extravasation at the C1-2 level, another false localizing sign in spinal CSF leaks, although, in our experience, the C1-2 level never corresponds to the site of the dural tear in patients with spontaneous intracranial hypotension.5 The cervico-thoracic CSF leaks in our study had a characteristic appearance on imaging with extradural CSF along the nerve roots, often extending into the paraspinal musculature. It is

Pseudo-cervico-thoracic CSF leak from ventral thoracic dural tear

Postmyelography CT shows extensive dye extravasation into the musculature at the cervico-thoracic junction (A, arrows) and a midthoracic ventral extradural CSF collection (B, arrow). Intraoperative photograph shows the ventral dural defect at Th6-7 (C, arrow). Neurology 84

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Table

a

Summary of 11 patients with spontaneous intracranial hypotension and a false localizing sign of cervico-thoracic CSF leak

Patient no.

Age, y/sex

Sites of CSF extravasation

Extent of ventral CSF collectiona

Site of CSF leak

1

30/F

C6, C7

C4-Th10

?

2

39/M

C6, C7, C8

C1-Th12

Th4-5

3

43/M

C6, C7, C8

C1-Th8

?

4

39/M

C6, C7, C8

C3-L3

Th2-3

5

39/M

C6, C7

C1-Th12

Th7-8

6

55/M

C8

C1-Th12

Th2-3

cervico-thoracic junction and the other patients was the presence of an extensive longitudinal intraspinal CSF collection in the patients in whom cervicothoracic CSF extravasation was a false localizing sign. It is important to recognize that CSF extravasation at the cervico-thoracic junction generally does not correspond to the actual site of the dural tear responsible for the CSF leak. Treatments directed at this site, including targeted epidural blood patching, percutaneous fibrin glue injections, and surgery, should not be expected to have a high probability of sustained improvement of symptoms.

7

29/F

C6, C7, C8, Th1

C1-L5

Th6-7

8

50/F

C6, C7

C3-Th8

Th5-6

AUTHOR CONTRIBUTIONS

9

31/F

C7, C8

C6-Th10

Th4-5

10

40/M

C8

C2-Th11

Th2-3

11

58/F

C7, C8

C1-Th9

Th6-7

Wouter I. Schievink: drafting/revising the manuscript for content, including medical writing for content; study concept or design; analysis or interpretation of data; acquisition of data; study supervision or coordination. M. Marcel Maya: drafting/revising the manuscript for content, including medical writing for content; study concept or design; analysis or interpretation of data; acquisition of data. Ray M. Chu: drafting/revising the manuscript for content, including medical writing for content; analysis or interpretation of data. Franklin G. Moser: drafting/revising the manuscript for content, including medical writing for content; analysis or interpretation of data.

At time of initial spinal imaging.

likely that these commonly observed extrathecal cervico-thoracic CSF collections are the result of CSF leaking into the epidural space from another site (thoracic in our patient population), extending rostrally within the spinal canal along the spinal gutters, and eventually escaping through the cervico-thoracic neural foramina. It is unclear why the cervico-thoracic junction is another preferential site of CSF extravasation, but it may be related to the location of the cervico-thoracic junction at the transition zone between the mobile cervical spine and the immobile thoracic spine. We limited our study to patients undergoing surgery, because this allowed a correlation between radiographic findings and intraoperative examination. Among the 13 patients with cervico-thoracic CSF extravasation but no evidence for an anatomical substrate on preoperative imaging, the source of the CSF leak (a small ruptured arachnoid cyst) was found at this level in only 2 patients. Postoperative imaging using digital subtraction myelography performed months or years after the surgery eventually demonstrated the actual site of the CSF leak to be in the thoracic spine.6 The one difference between the patients who had an actual source of CSF leakage at the

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STUDY FUNDING No targeted funding reported.

DISCLOSURE The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

Received December 17, 2014. Accepted in final form March 9, 2015. REFERENCES 1. Mokri B. Spontaneous intracranial hypotension. Curr Neurol Neurosci Rep 2001;1:109–117. 2. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;295:2286–2296. 3. Cho KI, Moon HS, Jeon HJ, Park K, Kong DS. Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology 2011;29:1139–1144. 4. Schievink WI, Maya MM, Louy C, Moser FG, Tourje J. Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension. AJNR Am J Neuroradiol 2008;29:853–856. 5. Schievink WI, Maya MM, Tourje J. False localizing sign of C1-2 cerebrospinal fluid leak in spontaneous intracranial hypotension. J Neurosurg 2004;100:639–644. 6. Hoxworth JM, Patel AC, Bosch EP, Nelson KD. Localization of a rapid CSF leak with digital subtraction myelography. AJNR Am J Neuroradiol 2009;30:516–519.

June 16, 2015

ª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

False localizing sign of cervico-thoracic CSF leak in spontaneous intracranial hypotension Wouter I. Schievink, M. Marcel Maya, Ray M. Chu, et al. Neurology 2015;84;2445-2448 Published Online before print May 15, 2015 DOI 10.1212/WNL.0000000000001697 This information is current as of May 15, 2015 Updated Information & Services

including high resolution figures, can be found at: http://www.neurology.org/content/84/24/2445.full.html

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Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 2015 American Academy of Neurology. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.

False localizing sign of cervico-thoracic CSF leak in spontaneous intracranial hypotension.

Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic ...
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