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

BACKGROUND: We 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). METHODS: A 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. RESULTS: SIH 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. CONCLUSIONS: This 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.

Treatment of spontaneous intracranial hypotension: experiences in a UK regional neurosciences Centre.

A robust treatment paradigm for spontaneous intracranial hypotension has yet to be agreed upon. We present retrospective data from the patient cohort at our UK regional neurosciences centre from 2010-2020 and describe our locally developed treatment pathway.Seventy-three patients were identified: 31 men and 42 women; mean age was 42 years. The majority presented with a headache of variable duration, and most had positive imaging. Very few patients (7%) responded to conservative treatment. Sixty-six underwent epidural blood patching, with 39 (59%) having a good response. Twenty-three patients underwent myelography and targeted treatment (injection of fibrin sealant at the leak site), with 13 (57%) showing a good response. One patient had successful surgery. The relapse rate after response to epidural blood patching was 10%, and after response to targeted treatment was 23%. Most patients who relapsed responded to repeated treatments.The outcome data for our diverse patient cohort shows the success of a staged approach to treatment. Relapse rates are low, and surgery is only rarely required. We use these data to inform our discussions with patients, and present them here to enable other centres to develop robust investigation and treatment paradigms of their own.