Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

Abstract

BackgroundWe aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG).MethodsA 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH.ResultsSIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided.ConclusionsThis multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

Spontaneous intracranial hypotension

“…3 A survey of UK healthcare professionals identified a lack of awareness of SIH among non-specialists, variability in management pathways and difficulty accessing investigations such as myelography and treatments such as epidural blood patches. 4 Guidelines for the diagnosis and management of SIH have recently been published by a multidisciplinary group of neurologists, neuroradiologists, neurosurgeons, anaesthetists and patient representatives, 5 with input from international experts and UK-based professional bodies. The guideline addresses the most important steps in the patient pathway, including clinical diagnosis, radiological diagnosis, identification of the spinal CSF leak site, non-targeted and targeted treatment, symptomatic management, management of complications and follow-up.…”

“…delaying epidural blood patch treatment with conservative management for any longer than 2 weeks from symptom onset 5 for three reasons: 72% of patients do not respond to symptom management alone; symptoms can improve without radiological resolution of the leak (which remains associated with long-term risks such as superficial siderosis) and early treatment of SIH gives greater likelihood of treatment response. 17 Bed rest is usually the main factor that relieves patients' symptoms and should be encouraged where possible while waiting for spontaneous resolution, or for specific investigation and treatments to be performed.…”

“…The evidence suggests that using over 20 mL of blood is more effective, 19 20 and guidelines recommend giving 20-40 mL, as tolerated by the patient. 5 Non-targeted epidural blood patch for SIH can be performed at a single (lumbar) level or divided between two levels (lumbar and thoracic); the blood spreads throughout the entire epidural space and so can successfully treat spinal CSF leaks even if they are in the cervical region. 21 Patients undergoing a non-targeted epidural blood patch should be informed of its potential risks and complications and advised to seek urgent medical attention if they develop any new symptoms.…”

“…If there is no response to a first non-targeted epidural blood patch, then it may be repeated 2-4 weeks later. 5 After successful treatment of SIH, some patients develop a rebound headache, which often has a reverse orthostatic quality (ie, worse on lying flat and improved on being upright) suggesting intracranial hypertension. Usually, there are no objective signs of intracranial hypertension (such as papilloedema) and the headache is self-limiting, but occasionally clinicians use medications such as acetazolamide.…”

Progressive superficial siderosis despite complete remission of intracranial hypotension symptoms following epidural patching: Case report

“…Persistent xanthochromia, persistent extradural fluid, or radiographic evidence of accumulating hemosiderin pigments might necessitate more aggressive treatment approaches, including surgery 19. Given the risks of causing additional CSF leaks with further lumbar punctures to check for xanthochromia, a non-invasive imaging approach to monitor such patients may be most appropriate.This case should inform guidelines on monitoring after treatment of a spinal CSF leak 20.…”