Cerebrospinal fluid leak

A cerebrospinal fluid leak (CSF leak) is a medical condition where the cerebrospinal fluid (CSF) surrounding the brain or spinal cord leaks out of one or more holes or tears in the dura mater.[1][2] A cerebrospinal fluid leak can be either cranial or spinal, and these are two different disorders.[3] A spinal CSF leak can be caused by one or more meningeal diverticula or CSF-venous fistulas not associated with an epidural leak.[4][5][6]

Cerebrospinal fluid leak
Other namesCSF leak
SpecialtyNeurology 

CSF leaks are either caused by trauma including that arising from medical interventions, or have no known cause known as spontaneous cerebrospinal fluid leaks (sCSF leaks). Traumatic causes include a lumbar puncture noted by a post-dural-puncture headache, and other trauma such as from a fall or accident. Spontaneous CSF leaks are associated with heritable connective tissue disorders including Marfan syndrome and Ehlers–Danlos syndromes.[7]

Signs and symptoms

The most common symptom of a CSF leak is a fast-onset, extremely painful orthostatic headache or thunderclap headache.[8]

A spinal leak may cause spontaneous intracranial hypotension (low CSF pressure) because the body cannot replenish the CSF fast enough to keep pace with the leak. As a result, the brain may sag inside the skull and into the foramen magnum, which is visible (and measurable) with an MRI of the brain. A cranial leak is more likely to cause intracranial hypertension (high CSF pressure), which carries a risk of meningitis. Both a cranial and spinal leak can fluctuate between high and low CSF pressure.

While high CSF pressure can make lying down unbearable, low CSF pressure due to a leak can be relieved by lying flat on the back.[9]

Other symptoms of a CSF leak can include neck pain, photophobia, dizziness, gait disturbances, tinnitus, visual disturbances, brain fog, nausea, fluid dripping from the nose or ears, and a metallic taste in the mouth. An untreated CSF leak can result in coma or death.[10]

Movement disorders are uncommon in spontaneous CSF leaks but occasionally can be one of the major components of the clinical presentation.[11]

Causes

A cerebrospinal fluid leak can be a rare complication of an anterior cervical discectomy and fusion (ACDF). One study suggested a CSF leak to follow from 0.5% of operations.[12] Another study suggests a CSF leak to follow from 1% of operations. In most of these cases repair is successful.[13]

Diagnosis

CSF leaks are frequently misdiagnosed as migraine, Chiari malformation, dysautonomia or conversion disorder.

Routine imaging assessment uses contrast-enhanced brain MRI with sagittal reformats.:[4][6] Imaging can assess for the following:

  • Subdural fluid collections
  • Enhancement of the meninges
  • Engorgement of venous structures
  • Pituitary swelling
  • Sagging of the brain

For suspected spinal CSF leaks, spine imaging can be used to guide treatment.[6]

Other imaging can be helpful in diagnosing a CSF leak, and in identifying its location, typically using a CT scan or an MRI scan. A myelogram can be used to more precisely identify the location of a CSF leak by injecting a dye to further enhance the imaging. However, CSF leaks are frequently not visible on imaging.

For patients with recalcitrant spontaneous intracranial hypotension and no leak found on conventional spinal imaging, digital subtraction myelography, CT myelography and dynamic myelography (a modified conventional myelography technique) should be considered to rule out a CSF-venous fistula.[14][5] In addition, presence of a hyperdense paraspinal vein should be investigated in imaging as it is highly suggestive of a CSF venous fistula.[15]

Fluid dripping from the nose (CSF rhinorrhoea) or ears (CSF otorrhea) should be collected and tested for the protein Beta-2 transferrin which would be highly accurate in identifying CS fluid and diagnosing a cranial CSF leak.[16]

Treatment

Symptomatic treatment usually involves analgesics for both cranial and spinal CSF leaks. Caffeine and short-term bed rest can alleviate symptoms of low CSF pressure, while elevated rest and acetazolamide can alleviate symptoms of high CSF pressure.

Sometimes a CSF leak will heal on its own. Otherwise, symptoms may last months or even years. An epidural blood patch is the typical treatment for a CSF leak, where up to 20 cubic centimeters of the patient's blood is drawn, then injected into either the lumbar or cervical spine, close to the known or suspected site of the leak. Fibrin glue patching is an alternative where blood patching is unsuccessful. If the site of the leak is known, neurosurgical repair of the dura mater is an option.

Surgery to treat a CSF-venous fistula in CSF leak patients is highly effective.[17]

The use of antibiotics to prevent meningitis in those with a CSF leak due to a skull fracture is of unclear benefit.[18]

References
  1. MedlinePlus Encyclopedia: CSF leak
  2. Cerebrospinal Fluid Leak Imaging at eMedicine
  3. "Are all CSF leaks similar?". Spinal CSF Leak Foundation. 29 April 2018.
  4. Kranz, Peter G.; Luetmer, Patrick H.; Diehn, Felix E.; Amrhein, Timothy J.; Tanpitukpongse, Teerath Peter; Gray, Linda (2015-12-23). "Myelographic Techniques for the Detection of Spinal CSF Leaks in Spontaneous Intracranial Hypotension". American Journal of Roentgenology. 206 (1): 8–19. doi:10.2214/AJR.15.14884. ISSN 0361-803X. PMID 26700332.
  5. Kranz, Peter G.; Amrhein, Timothy J.; Gray, Linda (December 2017). "CSF Venous Fistulas in Spontaneous Intracranial Hypotension: Imaging Characteristics on Dynamic and CT Myelography". AJR. American Journal of Roentgenology. 209 (6): 1360–1366. doi:10.2214/AJR.17.18351. ISSN 1546-3141. PMID 29023155.
  6. Kranz, Peter G.; Gray, Linda; Malinzak, Michael D.; Amrhein, Timothy J. (2019-11-01). "Spontaneous Intracranial Hypotension: Pathogenesis, Diagnosis, and Treatment". Neuroimaging Clinics of North America. Minimally Invasive Image-Guided Spine Interventions. 29 (4): 581–594. doi:10.1016/j.nic.2019.07.006. ISSN 1052-5149. PMID 31677732.
  7. Reinstein, E; Pariani, M; Bannykh, S; Rimoin, D; Schievink, WI (April 2013). "Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study". European Journal of Human Genetics. 21 (4): 386–390. doi:10.1038/ejhg.2012.191. PMC 3598315. PMID 22929030.
  8. "Thunderclap headaches - Symptoms and causes". Mayo Clinic. Retrieved 2018-08-01.
  9. "CSF Leak: A Curable Cause of Headache". www.cedars-sinai.edu. Retrieved 2018-08-01.
  10. "Headache Secondary to Intracranial Hypotension, Schievink, W and Deline, C". www.researchgate.net. Retrieved 2018-09-22.
  11. Mokri, Bahram (December 2014). "Movement disorders associated with spontaneous CSF leaks: a case series". Cephalalgia: An International Journal of Headache. 34 (14): 1134–1141. doi:10.1177/0333102414531154. ISSN 1468-2982. PMID 24728303. S2CID 3100453.
  12. Yee, TJ; Swong, K; Park, P (March 2020). "Complications of anterior cervical spine surgery: a systematic review of the literature". Journal of Spine Surgery. 6 (1): 302–322. doi:10.21037/jss.2020.01.14. PMC 7154369. PMID 32309668.
  13. Syre, P; Bohman, LE; Baltuch, G; Le Roux, P; Welch, WC (15 July 2014). "Cerebrospinal fluid leaks and their management after anterior cervical discectomy and fusion: a report of 13 cases and a review of the literature". Spine. 39 (16): E936-43. doi:10.1097/BRS.0000000000000404. PMID 25010015.
  14. Schievink, Wouter I.; Moser, Franklin G.; Maya, M. Marcel; Prasad, Ravi S. (June 2016). "Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas". Journal of Neurosurgery. Spine. 24 (6): 960–964. doi:10.3171/2015.10.SPINE15855. ISSN 1547-5646. PMID 26849709.
  15. Clark, Michael S.; Diehn, Felix E.; Verdoorn, Jared T.; Lehman, Vance T.; Liebo, Greta B.; Morris, Jonathan M.; Thielen, Kent R.; Wald, John T.; Kumar, Neeraj; Luetmer, Patrick H. (January 2018). "Prevalence of hyperdense paraspinal vein sign in patients with spontaneous intracranial hypotension without dural CSF leak on standard CT myelography". Diagnostic and Interventional Radiology (Ankara, Turkey). 24 (1): 54–59. doi:10.5152/dir.2017.17220. ISSN 1305-3612. PMC 5765931. PMID 29217497.
  16. O'Cearbhaill, Roisin M.; Kavanagh, Eoin C. (March 2018). "Beta-2 Transferrin and IR". Journal of Vascular and Interventional Radiology: JVIR. 29 (3): 439. doi:10.1016/j.jvir.2017.10.002. ISSN 1535-7732. PMID 29455884.
  17. Wang, Timothy Y.; Karikari, Isaac O.; Amrhein, Timothy J.; Gray, Linda; Kranz, Peter G. (2020-03-01). "Clinical Outcomes Following Surgical Ligation of Cerebrospinal Fluid-Venous Fistula in Patients With Spontaneous Intracranial Hypotension: A Prospective Case Series". Operative Neurosurgery (Hagerstown, Md.). 18 (3): 239–245. doi:10.1093/ons/opz134. ISSN 2332-4260. PMID 31134267.
  18. Ratilal, BO; Costa, J; Pappamikail, L; Sampaio, C (28 April 2015). "Antibiotic prophylaxis for preventing meningitis in patients with basilar skull fractures". The Cochrane Database of Systematic Reviews (4): CD004884. doi:10.1002/14651858.CD004884.pub4. PMID 25918919.
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