EDITORIAL

Management of spontaneous intracranial hypotension Alok Tyagi Correspondence to Dr Alok Tyagi, Department of Neurology, Institute of Neurological Sciences, Queen Elizabeth University Hospital, 1345 Govan Road, Glasgow G51 4TF, UK; [email protected] Accepted 6 January 2016

▸ http://dx.doi.org/10.1136/ practneurol-2015-001231

To cite: Tyagi A. Pract Neurol Published Online First: [ please include Day Month Year] doi:10.1136/practneurol2015-001343

Intracranial hypotension from a spontaneous dural CSF leak is being diagnosed more frequently. Patients can present acutely with characteristic orthostatic headaches and with exertional or thunderclap headaches. Often the diagnosis is delayed and the headache disorder can evolve into a ‘chronic daily headache’ pattern. MR brain imaging can show classical features of intracranial hypotension such as subdural collections, pituitary hyperaemia, brain sagging, engorged venous sinuses with pachymeningeal enhancement with contrast being the hallmark finding. The MR brain scan is normal in up to 20% cases1 and a lumbar puncture or an MR scan of spine may be needed to confirm the diagnosis. CT or MR myelography can identify the site of CSF leak, though none is found in half of cases.2 For those with a fast CSF leak and large spinal CSF collection, dynamic CT and digital subtraction myelography may help. Intracranial hypotension often resolves spontaneously and needs no further treatment. Strict bed rest, increased oral fluid intake, oral or intravenous caffeine, abdominal binders, theophylline, or corticosteroids are often tried but with only anecdotal success. In patients with disabling symptoms that persist despite conservative measures, an autologous epidural blood patch is the mainstay of treatment. Blood patches have been very successful for postlumbar puncture headaches, and this provides the rationale for using them in spontaneous intracranial hypotension. A blood patch can lead to a rapid improvement due to changes in the craniospinal CSF mechanics, with a more sustained effect probably due to tamponade and sealing of the dural defect.2 When there is no identified site of CSF leak, a ‘blind’ autologous lumbar epidural blood patch (15–20 mL of blood) can be performed. Injecting a larger volume of blood into the lumbar area can allow

blood to track up to multiple spinal levels, sometimes resolving the CSF leak even at a distant site. If the initial procedure is unsuccessful or if the headache recurs, a repeat lumbar epidural blood patch may help and their effects can be cumulative. Using the Trendelenburg position during and after the procedure has been reported to give success in over half of cases.2 Alternatively, a blood patch can be ‘targeted’, either at the site of the CSF leak if identified, or at the cervicothoracic junction where most CSF leaks occur. Fluoroscopic or CT guidance might improve the success of targeted blood patching. The success rate is best in those receiving a targeted blood patch at the outset but this needs to be balanced against the higher potential of complications. A reasonable approach, albeit unproven by clinical trials, is to do a blind lumbar epidural blood patch after diagnosis and, if unsuccessful, to search for the site of a CSF leak with a view to performing a targeted blood patch. The management becomes increasingly difficult and complex in patients whose symptoms persist despite two or more blood patches. The priority is to identify clearly the site of the CSF leak site in order to direct treatment appropriately. Finding multiple sites of CSF leaks in occasional patients complicates matters further. Some authors have reported innovative techniques with blood patching at multiple sites using a single catheter access3 or the use of percutaneous fibrin glue sealant.1 If a patient’s symptoms remain disabling, and there is a clearly identified site of CSF leak, and all treatment measures have failed, it is worth considering surgery. This might include ligation of leaking meningeal diverticula, direct repair of dural tears, packing of the epidural space with fibrin glue1 or strengthening the dura with a duroplasty. There is little evidence for these treatments, which are perhaps best

Tyagi A. Pract Neurol 2016;0:1–2. doi:10.1136/practneurol-2015-001343

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EDITORIAL carried out by specialists with experience in managing refractory spontaneous intracranial hypotension. Very rarely, patients present with or develop alterations in consciousness levels leading to coma or even death, from complications including severe sagging of the brain, subdural haematomas or venous sinus thrombosis. The surgical evacuation of subdural haematomas in spontaneous intracranial hypotension is controversial and must only be done in close proximity to the surgical correction of the CSF leak. Intrathecal saline infusion can be a useful temporising measure4 to normalise the intracranial pressure and so allow time to investigate and correct the CSF leak, as in Stephen et al’s instructive case report.5 The prevalence and natural history of spontaneous intracranial hypotension remains unclear and merits further investigation. We need greater clarity regarding the best imaging techniques to identify the site of the CSF leak as well as studies to compare the response rates of the various invasive therapeutic options. Competing interests None declared.

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Provenance and peer review Commissioned; externally peer reviewed. This paper was reviewed by Manjit Matharu, London, UK

REFERENCES 1 Graff-Radford SB, Schievink WI. High-pressure headaches, low-pressure syndromes, and CSF leaks: diagnosis and management. Headache 2014;54:394–401. 2 Williams EC, Buchbinder BR, Ahmed S, et al. Spontaneous intracranial hypotension: presentation, diagnosis, and treatment. Anesthesiology 2014;121:1327–33. 3 Griauzde J, Gemmete JJ, Chaudhary N, et al. Large-volume blood patch to multiple sites in the epidural space through a single-catheter access site for treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol 2014;35:1841–6. 4 Ghavanini AA, Scott CA, Chan DK, et al. Management of patients with spontaneous intracranial hypotension causing altered level of consciousness: report of two cases and review of literature. Cephalalgia 2013;33:43–51. 5 Stephen CD, Rojas R, Lioutas VA, et al. Complicated spontaneous intracranial hypotension treated with intrathecal saline infusion. Pract Neurol Published Online First: 14 Oct 2015 doi:10.1136/practneurol-2015-001231.

Tyagi A. Pract Neurol 2016;0:1–2. doi:10.1136/practneurol-2015-001343

Management of spontaneous intracranial hypotension.

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