Patterns of clinical and imaging presentations in patients with spontaneous intracranial hypotension due to spinal cerebrospinal fluid venous fistula: A single-center retrospective cross-sectional study.

OBJECTIVE: To identify distinct clinical or imaging subtypes of spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) venous fistula (CVF). BACKGROUND: Spontaneous intracranial hypotension is classically understood to present clinically with an orthostatic headache and stereotyped brain magnetic resonance imaging (MRI) findings; however, most prior literature examining clinical and brain MRI features of SIH has focused on all types of spinal CSF leaks concurrently. This study aimed to evaluate whether data support the possibility of internally consistent subtypes based on brain imaging features and clinical symptoms analogous to those seen in primary headache syndromes. METHODS: This retrospective cross-sectional single-institution study included 48 consecutive patients meeting the International Classification of Headache Disorders, 3rd edition criteria for SIH due to CVF. Clinical symptoms, pre-treatment brain MRI, and symptom duration were analyzed. Clinical and MRI data were analyzed to identify patterns and associations between symptoms and imaging findings. RESULTS: A total of 20 males and 28 females were evaluated, with a mean (standard deviation) age of 61 (10) years. In all, 44/48 (92%) patients experienced headaches, though 18/48 (40%) did not endorse relief when flat, including six of the 48 (13%) with worsening symptoms when flat. In all, 19/48 (40%) patients reported at least one migraine symptom, and six of the 48 (13%) presented with at least one migraine symptom and had no relief when flat. Clinical symptoms clustered primarily into a "classic" presentation consisting of relief when flat, occipital head pain, comorbid neck pain, a pressure/throbbing headache quality, and an "atypical" presentation that was characterized by having several differences: less relief when flat (nine of 22 (41%) vs. 20/23 (87.0%), p = 0.002; odds ratio [OR] 0.110, 95% confidence interval [CI] 0.016-0.53), more frontal head pain (14/22 (64%) vs. one of 23 (4%), p < 0.001; OR 35.0, 95% CI 4.2-1681.0), less neck pain (two of 21 (4.5%) vs. nine of 13 (69.6%), p < 0.001; OR 0.023, 95% CI 0.0005-0.196), and more stabbing/sharp headache quality (nine of 22 (41%) vs. two of 23 (9%), p = 0.017; OR 7.0, 95% CI 1.18-75.9). Brain MRI findings clustered into three groups: those presenting with most imaging findings of SIH concurrently, those with brain sag but less pachymeningeal/venous engorgement, and those with pachymeningeal/venous engorgement but less brain sag. CONCLUSION: This study highlights the clinical and imaging diversity among patients with SIH due to CVF, challenging the reliance on classic orthostatic headache alone for diagnosis. The findings suggest the existence of distinct SIH subtypes based on clinical and imaging presentations, underscoring the need for comprehensive evaluation in patients with suspected CVF. Future research should further elucidate the relationship between clinical symptoms and imaging findings, aiming to refine diagnostic criteria and enhance understanding of SIH's pathophysiology.

Cranial and Spinal Cerebrospinal Fluid Leaks: Foundations of Identification and Management.

Cerebrospinal fluid (CSF) leaks may occur at the skull base or along the spinal column and can cause a variety of debilitating neurological symptoms for patients. Recognizing the potential presence of a CSF leak and then identifying its source are necessary for accurate diagnosis and definitive treatment. A standardized workflow can be followed for successful leak localization, which often requires several diagnostic studies, and for definitive leak treatment, which can range from minimally invasive, needle-based approaches to a variety of surgical corrections. This review paper provides an overview of epidemiology, pathophysiology, and diagnostic workup for CSF leaks and introduces available treatment options. An illustrative case of a skull base CSF leak demonstrating diagnosis and surgical correction is provided.

Spontaneous Intracranial Hypotension Discovered Following Cerebral Venous Thrombosis: A Case Report and Review of the Literature.

Cerebral venous thrombosis (CVT) is a rare complication of spontaneous intracranial hypotension (SIH). We encountered a case where SIH was discovered after the diagnosis of CVT, suggesting the occurrence of CVT during the acute phase of SIH. We report this rare case of isolated cortical vein thrombosis in the acute phase of SIH. A 48-year-old woman taking low-dose oral contraceptives presented with neck pain, headache, and right-sided weakness. Magnetic resonance imaging and digital subtraction angiography confirmed isolated cortical vein thrombosis. No other specific imaging abnormalities were noted. The patient was initially treated with anticoagulation. Subsequent worsening of her orthostatic headache led to the diagnosis of SIH, with diffuse dural enhancement on gadolinium-enhanced T1-weighted imaging. An epidural blood patch was performed, resulting in a favorable outcome with no neurological deficits. Although CVT can occur in the acute phase of SIH, particularly in patients with thrombophilia, the lack of characteristic imaging findings associated with SIH often complicates the diagnosis.

Improvement of health-related quality of life after closure of spinal CSF leaks in patients with spontaneous intracranial hypotension.

OBJECTIVE: Spontaneous intracranial hypotension (SIH) is an important cause of orthostatic headaches caused by spinal CSF leaks. It has a strong negative impact on patients' socioeconomic status and health-related quality of life (HRQOL). This study aimed to analyze the impact of surgical and endovascular treatments on patients' HRQOL. METHODS: The authors conducted a prospective, observational cohort study that included all patients treated for SIH with microsurgery or embolization, depending on the type of CSF leak, at their institution between April 2022 and May 2023. Patients were asked to complete a specifically designed questionnaire, as well as the 15D HRQOL questionnaire, before and 3 months after treatment. RESULTS: A total of 21 patients (14 female; mean age 51.7 years) were treated in the study period. There were 12 (57%) type 1 leaks, 3 (14%) type 2, and 6 (29%) type 3. While 20 (95.2%) leaks were localized in the thoracic spine, only 1 (4.8%) was found in the lumbar spine. All patients completed the questionnaires. Fifteen (71.4%) patients underwent microsurgery and 6 (28.6%) endovascular embolization. The mean 15D score improved from 0.802 before to 0.889 after treatment (p = 0.013). Compared with an age- and sex-matched general population, HRQOL was significantly impaired in patients with SIH before treatment. After treatment, the authors found no significant difference in the overall HRQOL between patients and the healthy population. Mean headache intensity on a numeric rating scale improved from 8.1 before treatment to 2.3 after treatment (p = 0.003). Patients reported that SIH had a notable impact on their social and working life. CONCLUSIONS: SIH has a considerable negative impact on HRQOL. Microsurgery or embolization can dramatically improve HRQOL, subjective perception of health, and headache intensity. Therefore, surgical or endovascular treatment should be considered given the improvement observed in HRQOL for patients with SIH.

A Low-Volume Epidural Blood Patch for the Treatment of Spontaneous Intracranial Hypotension: A Case Report.

Spontaneous intracranial hypotension (SIH) is a rare neurological syndrome. We report the case of a 47-year-old woman with acute, severe orthostatic headache after surgery, chemotherapy, and radiotherapy for breast cancer. The brain and spine magnetic resonance imaging showed signs of intracranial hypotension. We describe the results of a non-targeted epidural blood patch with 10 mL of the patient's blood administered after unsuccessful conservative treatment. After the procedure, the patient reported gradual headache relief. This effect persisted over one year. The case shows that a single non-targeted low-volume epidural blood patch can be an effective treatment option for a patient with SIH when conservative treatment fails.

Sphenopalatine ganglion block for the treatment of spontaneous intracranial hypotension without demonstrable cerebrospinal fluid leak: A report of two cases.

Spontaneous intracranial hypotension (SIH) is a condition characterized by orthostatic headache associated with nausea, vomiting, tinnitus, vertigo, hypoacusis, neck pain/stiffness, and photophobia. Usual treatment includes bed rest, hydration, caffeine, analgesics, epidural blood patch, steroids, fibrin glue (N-butyl-cyanoacrylate), and surgical repair. In this series, we report two cases, who presented to us with features of SIH and were managed successfully with sphenopalatine ganglion block. This is a novel modality of management of SIH and has not been reported before.

Non-targeted serial epidural blood patches for the treatment of intracranial hypotension headache during late pregnancy: a case report.

Intracranial hypotension can cause persistent and severe orthostatic headaches. It is often the result of cerebrospinal fluid leakage secondary to iatrogenic causes. It can also happen spontaneously. Diagnosing and managing severe orthostatic headaches associated with intracranial hypotension can be challenging, particularly during late pregnancy. This case describes a parturient at 30 weeks' gestation who presented with severe headaches due to intracranial hypotension caused by dural defects and cerebrospinal fluid venous fistula. She was managed with serial non-targeted epidural blood patching as a bridging remedy. This allowed further fetal maturity before delivery and definitive management of the dural leakage after delivery.

Spontaneous intracranial hypotension: Exploring the viability of non-contrast FLAIR as a substitute for contrast-enhanced T1WI in assessing pachymeningeal thickening.

PURPOSE: To avoid contrast administration in spontaneous intracranial hypotension (SIH), some studies suggest accepting diffuse pachymeningeal hyperintensity (DPMH) on non-contrast fluid-attenuated inversion recovery (FLAIR) as an equivalent sign to diffuse pachymeningeal enhancement (DPME) on contrast-enhanced T1WI (T1ce), despite lacking thorough performance metrics. This study aimed to comprehensively explore its feasibility. METHODS: In this single-center retrospective study, between April 2021 and November 2023, brain MRI examinations of 43 patients clinically diagnosed with SIH were assessed using 1.5 and 3.0 Tesla MRI scanners. Two radiologists independently assessed the presence or absence of DPMH on FLAIR and DPME on T1ce, with T1ce serving as a gold-standard for pachymeningeal thickening. The contribution of the subdural fluid collections to DPMH was investigated with quantitative measurements. Using Cohen's kappa statistics, interobserver agreement was assessed. RESULTS: In 39 out of 43 patients (90.7%), pachymeningeal thickening was observed on T1ce. FLAIR sequence produced an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 72.1%, 71.8%, 75.0%, 96.6%, and 21.4% respectively, for determining pachymeningeal thickening. FLAIR identified pachymeningeal thickening in 28 cases; however, among these, 21 cases (75%) revealed that the pachymeningeal hyperintense signal was influenced by subdural fluid collections. False-negative rate for FLAIR was 28.2% (11/39). CONCLUSION: The lack of complete correlation between FLAIR and T1ce in identifying pachymeningeal thickening highlights the need for caution in removing contrast agent administration from the MRI protocol of SIH patients, as it reveals a major criterion (i.e., pachymeningeal enhancement) of Bern score.

Factors to predict recurrence after epidural blood patch in patients with spontaneous intracranial hypotension.

OBJECTIVES: This study aimed to identify predictors for the recurrence of spontaneous intracranial hypotension (SIH) after epidural blood patch (EBP). BACKGROUND: Epidural blood patch is the main treatment option for SIH; however, the characteristics of patients who experience relapse after successful EBP treatment for SIH remain understudied. METHODS: In this exploratory, retrospective, case-control study, we included 19 patients with SIH recurrence after EBP and 36 age- and sex-matched patients without recurrence from a single tertiary medical institution. We analyzed clinical characteristics, neuroimaging findings, and volume changes in intracranial structures after EBP treatment. Machine learning methods were utilized to predict the recurrence of SIH after EBP treatment. RESULTS: There were no significant differences in clinical features between the recurrence and no-recurrence groups. Among brain magnetic resonance imaging signs, diffuse pachymeningeal enhancement and cerebral venous dilatation were more prominent in the recurrence group than no-recurrence group after EBP (14/19 [73%] vs. eight of 36 [22%] patients, p = 0.001; 11/19 [57%] vs. seven of 36 [19%] patients, p = 0.010, respectively). The midbrain-pons angle decreased in the recurrence group compared to the no-recurrence group after EBP, at a mean (standard deviation [SD]) of -12.0 [16.7] vs. +1.8[18.3]° (p = 0.048). In volumetric analysis, volume changes after EBP were smaller in the recurrence group than in the no-recurrence group in intracranial cerebrospinal fluid (mean [SD] -11.6 [15.3] vs. +4.8 [17.1] mL, p = 0.001) and ventricles (mean [SD] +1.0 [2.0] vs. +2.0 [2.5] mL, p = 0.003). Notably, the random forest classifier indicated that the model constructed with brain volumetry was more accurate in discriminating SIH recurrence (area under the curve = 0.80 vs. 0.52). CONCLUSION: Our study suggests that volumetric analysis of intracranial structures may aid in predicting recurrence after EBP treatment in patients with SIH.

Don't delay, but don't despair: symptom duration, comorbidity and outcome after closure of spinal cerebrospinal fluid leaks.

OBJECTIVE: Microsurgical sealing of spinal cerebrospinal fluid (CSF) leaks is a viable treatment option in spontaneous intracranial hypotension (SIH). Several factors may influence the outcome, with symptom duration probably the most modifiable variable. METHODS: Patients with closure of spinal CSF leaks between September 2020 and March 2023 and a follow-up period of 6 months were included in this retrospective single-center study. Pre- and postoperative scores for impact of headaches (Headache Impact Test, HIT-6) and quality of life (QoL, EQ-5D-5L) were systematically collected. Multiple regression modelling and subgroup analyses for different symptom durations and comorbidities were performed for these outcomes. RESULTS: One hundred patients (61% female, median age 43.5 years) were included. Six months postoperatively, there was significant improvement in headache impact (HIT-6: 66 (IQR 62-69) to 52 (IQR 40-61, p < 0.001) and QoL (EQ-5D-5L VAS: 40 (IQR 30-60) to 79 (IQR 60-90); EQ-5D-5L Index: 0.67 (IQR 0.35-0.8) to 0.91 (IQR 0.8-0.94, p < 0.001, respectively). Subgroup analysis for a symptom duration above (74%) and below 90 days (26%) and comorbidity, as well as multiple regression analysis, revealed a trend in favor of early treatment and lower comorbidity. However, even after a prolonged symptom duration, improvements were significant. CONCLUSION: As patients with shorter symptom duration show a trend for a better outcome, our results promote a timely diagnosis and treatment in SIH patients. However, a significant postoperative improvement can still be expected even after a prolonged symptom duration.

Headache associated with intracranial hypotension: diagnostic challenges and difficulties in everyday neurological practice.

Low pressure of cerebrospinal fluid (CSF) is a rare cause of headache, except when the patient undergoes a lumbar puncture. Headache associated with a low CSF pressure i.e. intracranial hypotension causes diagnostic difficulties. Headaches related to spontaneous intracranial hypotension (SIH) pose a significant diagnostic challenge in everyday neurological practice. Patients with headaches due to SIH are usually diagnosed only after a long delay. Diagnostic problems may result in unnecessary invasive diagnostic procedures, or even neurosurgical operations. Diagnosing headaches attributed to SIH requires the consideration of several clinical scenarios, and the disease's features causing primary or secondary disturbances. In this review, we discuss the differential diagnosis of SIH-related headaches with reference to accumulated knowledge, including meta-analyses, guidelines, casuistry, and the applicable criteria of the International Classification of Headache Disorders. In addition, we discuss head and spine magnetic resonance imaging abnormalities, which may indicate intracranial hypotension.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. METHODS: A retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. RESULTS: Among the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. CONCLUSIONS: This procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.

Patient-reported symptomatology and its course in spontaneous intracranial hypotension - Beware of a chameleon.

OBJECTIVE: Although orthostatic headache is the hallmark symptom of spontaneous intracranial hypotension (SIH), patients can present with a wide range of different complaints and thereby pose a diagnostic challenge for clinicians. Our aim was to describe and group the different symptoms associated with SIH and their course over time. METHODS: We retrospectively surveyed consecutive patients diagnosed and treated for SIH at our institution from January 2013 to May 2020 with a specifically designed questionnaire to find out about their symptomatology and its course. RESULTS: Of 112 eligible patients, 79 (70.5%) returned the questionnaire and were included in the analysis. Of those, 67 (84.8%) reported initial orthostatic headaches, whereas 12 (15.2%) denied having this initial symptom. All except one (98.7%) patients reported additional symptoms: most frequently cephalic pressure (69.6%), neck pain (68.4%), auditory disturbances (59.5%), nausea (57%), visual disturbances (40.5%), gait disturbance (20.3%), confusion (10.1%) or sensorimotor deficits (21.5%). Fifty-seven (72.2%) patients reported a development of the initial symptoms predominantly in the first three months after symptom onset. Age and sex were not associated with the symptomatology or its course (p > 0.1). CONCLUSION: Although characteristic of SIH, a relevant amount of patients present without orthostatic headaches. In addition, SIH can manifest with non-orthostatic headaches at disease onset or during the course of the disease. Most patients report a wide range of associated complaints. A high degree of suspicion is crucial for an early diagnosis and targeted treatment.

Spontaneous Intracranial Hypotension: A Review of Neuroimaging and Current Concepts.

Spontaneous intracranial hypotension (SICH) is the emerging cause of orthostatic headache as it has been better recognized in recent years. SICH happens due to spinal cerebrospinal fluid (CSF) leak; however, the manifestations are predominantly cranial and hence imaging in SICH includes brain and spine. There are few characteristic brain imaging features to be concerned about to diagnose SICH in patients with vague symptoms or low clinical suspicion. Spine screening is recommended in these patients to assess spinal CSF leaks. While neuroradiologists play a significant role from the time of diagnosis to treatment of SICH, there is a need for all the general radiologists to be aware of the condition. Computed tomography myelogram and digital subtraction myelogram are performed for diagnostic and therapeutic management of SICH. There is a known risk for SICH recurrence in patients with sagittal longitudinal epidural collection and hence, targeted blood patch should be used instead of blind patch. Most importantly, slow mobilization is recommended following the patch to avoid recurrence.

Hipotensión intracraneal espontánea. Una puesta al día

Introducción: La hipotensión intracraneal espontánea es un síndrome causado por la disminución del volumen de líquido cefalorraquídeo consecuencia de su fuga al espacio extradural. Aunque la ICHD-3 proporciona un alto nivel de especificidad diagnóstica, esta enfermedad puede manifestarse de forma atípica. Hasta en un 30% no es posible establecer el punto de escape, pero con el refinamiento de los exámenes de imágenes este porcentaje se ha reducido a un 15%-20%. Actualmente, su manejo no se encuentra estandarizado y las recomendaciones se basan en evidencia de limitada calidad metodológica, además de la variabilidad de protocolos entre distintos centros. Desarrollo En esta revisión actualizamos los procedimientos diagnósticos y terapéuticos. Por un lado, analizamos el rol de la resonancia nuclear magnética de encéfalo y médula espinal completa como primer paso diagnóstico y, por otro lado, señalamos los exámenes destinados a determinar la fuga de líquido cefalorraquídeo. Tal es el caso de la mielo-resonancia, la mielo-tomografía computarizada, tanto estándar, dinámica y por sustracción digital, además de la cisternografía con 111-Indium-DPTA. Sin embargo, determinar cuál de estos exámenes es el óptimo es objeto de debate. Lo mismo ocurre con el tratamiento: reposo; parche sanguíneo epidural a ciegas, parche guiado por fluoroscopia o tomografía computarizada, parche de fibrina; o cirugía. Conclusiones Se requiere de una mayor investigación, especialmente con trabajos multicéntricos controlados, para una mejor comprensión de la fisiopatología, el diagnóstico por imágenes, los enfoques terapéuticos y evaluación objetiva de los resultados clínicos. Solo así se establecerán pautas diagnósticas y de tratamiento validadas.

Introducction: Spontaneous intracranial hypotension is a syndrome caused by decreased CSF volume secondary to its leakage into the extradural space Although ICHD-3 provides a high level of diagnostic specificity, manifestations may be atypical, making diagnosis challenging. The site of leakage may be undetermined in point Up to 30% of cases, although with recent refinement of imaging, this percentage has been reduced to 15-20%. Currently, management is not standardized and recommendations are based on inconclusive evidence, with variability of protocols between centres. Development. In this review, we update diagnostic and therapeutic procedures. We analyse the role of whole brain and spinal cord MRI as a first investigation and review tests aimed at determining cerebrospinal fluid leakage, such as MRI myelography, conventional CT myelography, dynamic CT myelography, and digital subtraction CT myelography, as well as 111-Indium-DPTA cisternography. Determining optimal use of these investigations remains a matter of debate. The same is true for treatment: rest, blind epidural blood patch, fluoroscopy or CT-guided epidural blood patch, fibrin patch and surgery are discussed. Conclusión: Further research, especially multicentre controlled studies, is required to improve understanding of pathophysiology, diagnostic imaging, therapeutic approaches and to objectively assess clinical outcomes. Only then will diagnostic and treatment guidelines be evidence-based.

Small spheno-ethmoidal meningoencephalocele versus ethmoidal mucocele in spontaneous intracranial hypotension.

Background: Meningoencephalocele is defined as an abnormal sac of brain tissue and meninges extending beyond natural skull margins, often leading to cerebrospinal fluid (CSF) leakage. When this condition arises in the spheno-ethmoidal region, the diagnosis becomes more challenging as it can be mistaken for other nasal pathologies, such as mucocele. Research question: We show in this case report a non-congenital sphenoethmoidal meningoencephalocele causing rhinoliquoral fistula and spontaneous intracranial hypotension. Results: this 65-year-old woman presented with sporadic rhinoliquorrhoea associated with orthostatic headache, nausea and dizziness. Brain MRI revealed a small lesion of an ethmoidal sinus, which was successfully treated with endoscopic endonasal surgery. Histology confirmed the presence of meningoencephalic tissue positive for S100 protein on immunohistochemistry. Conclusions: When dealing with lesions of the paranasal sinuses in contact with the anterior skull base, rhinoliquorrhoea presence suggests meningoencephalocele. In dubious cases, a proper workup, including a thorough clinical history and neurological examination, specific imaging, and a direct search of CSF-like markers, is essential to support the differential diagnosis. In such cases, a transnasal endoscopic surgical approach is recommended to obtain a final histological diagnosis and to perform eventual dural plastic surgery.

Spontaneous intracranial hypotension: a case series and literature review.

Spontaneous intracranial hypotension (SIH) is a condition of negative intracranial pressure resulting from cerebrospinal fluid (CSF) leakage from the dural sac and is a well-known cause of orthostatic headache. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines. Low CSF pressure and diffuse meningeal enhancement on brain MRI are the major instrumental features of the classic syndrome. Neuroimaging plays a key role in diagnosing SIH, particularly in atypical clinical presentations, by recognizing the specific findings of brain sagging on MRI and detecting the level of CSF leak on spinal imaging, thus guiding therapy accordingly. Since SIH could present with such a heterogeneous clinical picture, careful history taking and increased awareness of atypical presentations are of utmost importance. We review the existing SIH literature, illustrate management, clinical and neuroimaging findings of four consecutive patients with atypical SIH, who were recently referred to our hospital for evaluation to simplify and streamline the management of SIH.

Spontaneous Intracranial Hypotension in Pregnancy.

PURPOSE OF REVIEW: Spontaneous intracranial hypotension (SIH) is a debilitating disease typically featuring orthostatic headache and caused by a spinal CSF leak. This review will describe the characteristics of SIH in pregnant patients and the associated unique management and treatment considerations. RECENT FINDINGS: Herein, a novel case is reported of a 41-year-old woman who presented with SIH pre-conception but saw marked improvement of symptoms after 5 weeks antepartum and symptom recurrence 2 months post-partum. A literature review of SIH in pregnancy revealed 14 reported patients across 10 studies since 2000. All the reported cases resulted in delivery of healthy infants and symptomatic improvement with conservative management or a variety of treatment modalities including non-targeted epidural blood patch (EBP). Clinical and imaging features of SIH in pregnancy are reviewed. We hypothesize an antenatal protective mechanism against SIH symptoms through cephalad redistribution of CSF volume from the spinal to intracranial compartments related to uterine growth and decreased CSF volume within the lumbar cistern. Treatment recommendations are discussed including duration of bed rest and decision for non-targeted multi-site EBPs. When required, non-invasive diagnostic spine MRI using fat-suppressed axial T2-weighted imaging may be helpful.

Recurrent Spontaneous Intracranial Hypotension (SIH) and the Durability of Repeat Epidural Blood Patch (EBP).

OBJECTIVE: Spontaneous intracranial hypotension (SIH) remains a rare and difficult clinical entity to diagnose and treat. Epidural blood patch (EBP) of the dural sac is the mainstay definitive treatment for refractory cases and has mixed efficacy. We sought to evaluate the recent efficacy and outcomes of EBP for SIH at our institution. METHODS: Twenty-three patients (14 women, 9 men, mean age 49) were seen and treated for SIH between Summer 2009 and Spring 2018 at the same institution. All patients underwent brain MRI with and without gadolinium contrast and T2-weighted spine MRI. Targeted EBP was placed one or two vertebral levels below areas of suspected leak, while the patient was positioned in the lateral decubitus position. Patients were seen in the outpatient setting within a week following initial EBP and repeat EBP was offered to patients with persistent symptoms. Patients were followed if symptoms persisted or for 6 months following clinical relief of symptoms. RESULTS: 22/23 (95.7%) patients presented with complaints of orthostatic headache, and 3 (13%) patients presented with altered mental status (AMS) or focal neurologic deficit. Brain MRI demonstrated pachymeningeal enhancement in 16/23 (69.6%) patients, and 5/23 (21.7%) patients had a subdural hematoma (SDH) present. Dural leaks were successfully identified in 18/23 (78.3%) patients. 12/23 (52.2%) patients had symptomatic relief with initial EBP, and 5/23 (21.7%) patients received further EBPs for persistent disease with all achieving relief after repeat EBP. 5/12 (41.7%) of patients had recurrent symptoms after initial relief with EBP, and 4/5 (80%) were successfully treated with a second EBP. The mean initial EBP volume and number of EBPs per patient were 21.7 mL (median 20 mL, 7-40 mL) and 3.54 (median 1, 1-13) respectively. There was one complication from initial EBP (cervical dural tear requiring operative closure) treated with open surgical management successfully. In total, 18/23 (78.2%) patients are currently asymptomatic with regard to their SIH. The mean follow-up in this cohort was 2.6 years (median 1.8 years, 1.8 months-9.27 years). CONCLUSIONS: EBP is a viable and effective option for the treatment of recurrent SIH caused by cerebrospinal fluid (CSF) leaks.

Recovery and long-term outcome after neurosurgical closure of spinal CSF leaks in patients with spontaneous intracranial hypotension.

INTRODUCTION: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid leak causes orthostatic headaches and impacts quality of life. Successful closure rates are often reported, whereas data on long-term outcome are still scarce. METHODS: Between April 2020 and December 2022 surgically treated patients completed the Headache Impact Test-6 prior to surgery and at 14 days, three months, six months, and 12 months postoperatively. In addition to the Headache Impact Test-6 score, we extracted data related to orthostatic symptoms. RESULTS: Eighty patients were included. Median Headache Impact Test-6 score preoperatively was 65 (IQR 61-69), indicating severe and disabling impact of headaches. At three months headache impact significantly improved to 49 (IQR 44-58) (p < 0.001) and remained stable up to 12 months (48, IQR 40-56), indicating little to no impact of headaches on quality of life. The need to lie down "always" or "very often" was reduced from 79% to 23% three months postoperatively (p < 0.001). CONCLUSIONS: Surgical closure of spinal CSF leaks significantly improves the impact of headaches in the long term. At least three months should be expected for recovery. Despite permanent closure of the CSF-leak, a quarter of patients still have relevant long-term impairment, indicating the need for further research on its cause and possible treatment.