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Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The optimal management options for patients with SIH remain uncertain. The aim of this study was to review studies reporting the management of SIH with a special emphasis on the surgical treatment of SIH including clinical trials, case series and case reports related to the issue of various neurosurgical procedures performed for SIH treatment. The clinical outcomes of patients diagnosed with SIH treated with either only surgery or with surgery as the primary method of treatment were analysed. The PubMed, Scopus and Google Scholar databases were searched according to the established criteria. The literature search revealed seven clinical trials, five case series and eight case reports regarding surgical treatment of patients diagnosed with SIH. Manuscripts reporting at least five individuals treated surgically for SIH were considered as case series. In most published articles, surgery provided clinical benefit, resulting in a success rate of 82.6-100% for complete relief of SIH symptoms. Our literature review has revealed that SIH can be diagnosed reliably by MRI and cisternography. The identification of the location of SIH is mandatory for its successful surgical treatment. The clinical outcome is related to the location of SIH in the spinal canal. Most often, cerebrospinal fluid leakage occurs in the thoracic region. Surgical treatment is very effective and the obtained treatment results are complete and permanent. of the location of SIH is mandatory for its successful surgical treatment. The clinical outcome is related to the location of SIH in the spinal canal. Most often, cerebrospinal fluid leakage occurs in the thoracic region. Surgical treatment is very effective and the obtained treatment results are complete and permanent.

ObjectiveSpinal cerebrospinal fluid (CSF) leaks cause spontaneous intracranial hypotension (SIH). Microsurgery can sufficiently seal spinal CSF leaks. Yet, some patients suffer from residual symptoms. Aim of the study was to assess predictors for favorable outcome after surgical treatment of SIH.MethodsWe included consecutive patients with SIH treated surgically from January 2013 to May 2020. Subjects were surveyed by a questionnaire. Primary outcome was resolution of symptoms as rated by the patient. Secondary outcome was postoperative headache intensity on the numeric rating scale (NRS). Association between variables and outcome was assessed using univariate and multivariate regression. A cut-off value for continuous variables was calculated by a ROC analysis.ResultsSixty-nine out of 86 patients (80.2%) returned the questionnaire and were analyzed. Mean age was 46.7 years and 68.1% were female. A significant association with the primary and secondary outcome was found only for preoperative symptom duration (p = 0.001 and p < 0.001), whereby a shorter symptom duration was associated with a better outcome. Symptom duration remained a significant predictor in a multivariate model (p = 0.013). Neither sex, age, type of pathology, lumbar opening pressure, nor initial presentation were associated with the primary outcome. ROC analysis yielded treatment within 12 weeks as a cut-off for better outcome.ConclusionShorter duration of preoperative symptoms is the most powerful predictor of favorable outcome after surgical treatment of SIH. While an initial attempt of conservative treatment is justified, we advocate early definitive treatment within 12 weeks in case of persisting symptoms.

Subdural hematoma is a rare complication after lumbar spine surgeries. Most of the time, the reason for this complication is intracranial hypotension related to an unintended durotomy, and the main symptom is persistent orthostatic headache. The authors present the case of a-38-year-old woman who underwent lumbar discectomy. Three weeks after the surgery, she developed subdural hematoma related to dural tear during the procedure. Emergent hematoma evacuation with craniotomy and dural tear repair was performed simultaneously because of the herniation symptoms of the patient. Clinical findings, radiological assessments, and treatment modalities have been discussed in the presence of a literature review in this case report.

Background and purpose Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. Methods This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. Results Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8–12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. Conclusions Long‐standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time‐dependent manner. Long‐standing untreated ventral spinal cerebrospinal fluid (CSF) leaks can lead to superficial siderosis of the central nervous system. Microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with superficial siderosis in a time‐dependent manner. Therefore, searching for spinal CSF leaks in all patients with superficial siderosis with a thorough workup is strongly recommended.

Abstract BACKGROUND Spinal cerebrospinal fluid (CSF) leaks are the cause of spontaneous intracranial hypotension (SIH). OBJECTIVE To propose a surgical strategy, stratified according to anatomic location of the leak, for sealing all CSF leaks around the 360° circumference of the dura through a single tailored posterior approach. METHODS All consecutive SIH patients undergoing spinal surgery were included. The anatomic site of the leak was exactly localized. We used a tailored hemilaminotomy and intraoperative neurophysiological monitoring (IOM) for all cases. Neurological status was assessed before and up to 90 d after surgery. RESULTS Forty-seven SIH patients had an identified CSF leak between the levels C6 and L1. Leaks, anterior to the spinal cord, were approached by a transdural trajectory (n = 28). Leaks lateral to the spinal cord by a direct extradural trajectory (n = 17) and foraminal leaks by a foraminal microsurgical trajectory (n = 2). The transdural trajectory necessitated cutting the dentate ligament accompanied by elevation and rotation of the spinal cord under continuous neuromonitoring (spinal cord release maneuver, SCRM). Four patients had transient defiticts, none had permanent neurological deficits. We propose an anatomic classification of CSF leaks into I ventral (77%, anterior dural sac), II lateral (19%, including nerve root exit, lateral, and dorsal dural sac), and III foraminal (4%). CONCLUSION Safe sealing (with IOM) of all CSF leaks around the 360° surface of the dura is feasible through a single posterior approach. The exact surgical trajectory is selected according to the anatomic category of the leak.

BackgroundCerebrospinal fluid (CSF) loss in spontaneous intracranial hypotension (SIH) is accompanied by volume shifts between the intracranial compartments. This study investigated tricompartimental and longitudinal volume shifts after closure of a CSF leak.MethodsPatients with SIH and suitable pre-therapeutic and post-therapeutic imaging for volumetric analysis were identified from our tertiary care center between 2020 and 2023. The Bern SIH score was calculated. Pre-interventional and post-interventional volumetry encompassed the CSF, parenchymal and venous compartments (ie, venous sinus and choroid plexus volumes).ResultsIn total, 32 patients with SIH (49.7±16.0 years, 22 women) met inclusion criteria. The mean SIH score decreased between baseline (4.5±2.7) and early (2.7±2.3, <7 days after intervention), and also late follow-up (1.4±1.7, follow-up ≥7 days) after leak closure. This was accompanied by a significant increase in ventricular volume from 22.1 to 25.0 mL (P=0.01) at early follow-up, and 23.9 mL at later follow-up (P=0.080). In contrast, venous sinus volumes decreased from 13.8 to 9.6 mL (P=0.016) at early follow-up, and 10.0 mL (P=0.007) at late follow-up. No significant change in mean choroid plexus, total gray or total white matter volume was observed.ConclusionsClosure of a spinal CSF leak leads to an early increase in ventricular CSF volume and a decrease in venous sinus volume. The results reflect the long-term convergence of the SIH score to normal values and indicate that permanent closure of a CSF leak induces a stable recompensation of the intracranial compartments without involving significant volume shifts within the cerebral parenchyma.

Background There is a significant variance in surgical treatment strategies of ventral cerebrospinal fluid (CSF) leaks causing spontaneous intracranial hypotension (SIH). Posterior approaches might represent a preferable alternative to the more invasive anterior and lateral routes, as long as the spinal cord is not exposed to harmful manipulation. The aim of this technical note is to report and illustrate a new surgical technique using an intradural extraarachnoid sutureless technique via laminoplasty for indirect repair of ventral CSF leaks causing intractable SIH symptoms. Methods The surgical technique is described in a step by step fashion. Between May 2018 and May 2020, five patients with ventral spinal CSF leaks were operated on, utilizing this technique. All dural defects were located at the level of the thoracic spine. A retrospective review on demographic and radiological findings, symptoms, outcome, and follow-up was performed. Results The intra- and postoperative course was uneventful in all patients with no surgery-related complications. Three patients recovered completely at discharge, while neurological symptoms significantly improved in two patients. A postoperative MRI of the spine was obtained for all patients, demonstrating regressive signs of CSF leak. Conclusion Based on the presented case series, this intradural extraarachnoid sutureless technique combined with laminoplasty seems to be a safe and effective option for indirect repair of ventral dural defects in SIH. In our opinion, it represents a valid alternative to traditional more aggressive approaches.

Background Spontaneous intracranial hypotension (SIH) is a debilitating condition requiring effective treatment; however, objective data on treatment response are scarce. Purpose To assess the suitability of the brain MRI-based SIH score (bSIH) for monitoring treatment success in SIH patients with a proven spinal cerebrospinal fluid (CSF) leak after microsurgical closure of the underlying dural breach. Methods This retrospective cohort study included consecutive SIH patients with a proven spinal CSF leak, investigated at dedicated referral centre January 2012 to March 2020. The bSIH score integrates 6 imaging findings; 3 major (2 points) and 3 minor (1 point), and ranges from 0 to 9, with 0 indicating low and 9 high probability of spinal CSF loss. The score was calculated using brain magnetic resonance imaging (MRI) before and after surgical treatment of the underlying CSF leak. Headache intensity was registered on a numeric rating scale (NRS) (range 0–10). Results In this study 52 SIH patients, 35 (67%) female, mean age 45.3 years, with a proven spinal CSF leak were included. The mean bSIH score decreased significantly from baseline to after surgical closure of the underlying dural breach (6.9 vs. 1.3, P  < 0.001). A decrease in the NRS score was reported (8.6 vs. 1.2, P  < 0.001). Conclusion The bSIH score is a simple tool which may serve to monitor treatment success in SIH patients after surgical closure of the underlying spinal dural leak. Its decrease after surgical closure of the underlying spinal dural breach indicates restoration of an equilibrium within the CSF compartment.

A 49-year-old woman developed symptoms of syringomyelia 3 years after having presented with spontaneous intracranial hypotension (SIH). She had previously undergone two unsuccessful non-targeted epidural blood patches. The MR scan showed features of cervicothoracic syringomyelia and ongoing intracranial features of SIH. Following two further ineffective non-targeted epidural blood patches, a cerebrospinal fluid venous fistula was identified on myelography, which was surgically disconnected. At subsequent follow-up, she showed marked and progressive clinical and radiological improvement. This case shows that offering curative treatment for the underlying cause of SIH is an effective—and the preferred—way of managing SIH-associated syrinx.

Abstract BACKGROUND Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. OBJECTIVE To describe detailed characteristics of a large cohort of patients with SIH and symptoms of bvFTD. METHODS We identified patients with SIH who met clinical criteria for bvFTD. Patients were compared to a cohort of SIH patients without bvFTD. RESULTS The mean age for the 21 men and 8 women was 52.9 yr (range, 37–65 yr). All 29 patients with bvFTD symptoms had hypersomnolence. Magnetic resonance imaging showed brain sagging in all patients, cerebrospinal fluid (CSF) opening pressure low in about half of patients, but a spinal CSF leak could not be detected in any patient. All patients underwent epidural blood patching, but 26 patients eventually underwent 1 or more surgical procedures. Overall, a good outcome was obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to 1 (14%) of 7 patients who did undergo Chiari surgery (P < .003). Compared to SIH patients without symptoms of bvFTD (n = 547), those with bvFTD symptoms were older, more often male, less often demonstrated CSF leak on spinal imaging, and more often underwent surgery (P < .02). CONCLUSION bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured.