Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

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.

CSF-Venous Fistula of the Clival Skull Base: A Unique Case Study and Literature Review.

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024.

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.

Intraoperative Visualization of Flow in Direct Cerebrospinal Fluid-Venous Fistulas Using Intrathecal Fluorescein.

BACKGROUND: Cerebrospinal fluid-venous fistulas (CVFs) are a rare cause of spontaneous intracranial hypotension. Intraoperatively, CVFs are not readily identifiable and difficult to differentiate from normal veins. OBJECTIVE: To assess the utility of intrathecal fluorescein injection intraoperatively to visualize and identify the CVF. METHODS: We report a case series of patients treated surgically for a CVF. After surgical exposure, we injected intrathecal fluorescein (5-10 mg) through a lumbar catheter, which we placed immediately before surgery. RESULTS: Four patients with spontaneous intracranial hypotension with a suspected CVF underwent surgical ligation with adjunctive intrathecal fluorescein application. Intraoperative fluorescein injection confirmed the presence of a CVF in 3 cases. In 2 cases, we observed rapid fluorescein filling of a single epidural vein constituting the CVF. Other epidural vessels did not fill with fluorescein. In 1 case, fluorescein helped to identify a residual CVF after previous incomplete embolization. In the fourth case, no CVF was found intraoperatively. By contrast, a meningeal nerve root diverticulum was visualized, wrapped, and clipped. CONCLUSION: We demonstrate for the first time the direct intraoperative visualization of CVF using intrathecal fluorescein. CVF can be identified intraoperatively using fluorescein dye, which can be a valuable adjunct for the surgeon confronted with this disease.

Postoperative Spinal Cerebrospinal Fluid-Venous Fistulas Associated With Dural Tears in Patients With Intracranial Hypotension or Superficial Siderosis-A Digital Subtraction Myelography Study.

BACKGROUND: Postoperative spinal cerebrospinal fluid (CSF) leaks are common but rarely cause extensive CSF collections that require specialized imaging to detect the site of the dural breach. OBJECTIVE: To investigate the use of digital subtraction myelography (DSM) for patients with extensive extradural CSF collections after spine surgery. METHODS: A retrospective review was performed to identify a consecutive group of patients with extensive postoperative spinal CSF leaks who underwent DSM. RESULTS: Twenty-one patients (9 men and 12 women) were identified. The mean age was 46.7 years (range, 17-75 years). The mean duration of the postoperative CSF leak was 3.3 years (range, 3 months to 21 years). MRI showed superficial siderosis in 6 patients. DSM showed the exact location of the CSF leak in 19 (90%) of the 21 patients. These 19 patients all underwent surgery to repair the CSF leak, and the location of the CSF leak could be confirmed intraoperatively in all 19 patients. In 4 (19%) of the 21 patients, DSM also showed a CSF-venous fistula at the same location as the postoperative dural tear. CONCLUSION: In this study, DSM had a 90% detection rate of visualizing the exact site of the dural breach in patients with extensive postoperative spinal CSF leaks. The coexistence of a CSF-venous fistula in addition to the primary dural tear was present in about one-fifth of patients. The presence of a CSF-venous fistula should be considered if CSF leak symptoms persist in spite of successful repair of a durotomy.

Spontaneous intracranial hypotension: from cerebral venous thrombosis to neurosurgical treatment: a case series experience from a Brazilian tertiary health care center.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a secondary cause of headache and an underdiagnosed disease. The clinical presentation can be highly variable. It typically presents with isolated classic orthostatic headache complaints, but patients can develop significant complications such as cerebral venous thrombosis (CVT). OBJECTIVE: To report 3 cases of SIH diagnosis admitted and treated in a tertiary-level neurology ward. METHODS: Review of the medical files of three patients and description of clinical and surgical outcomes. RESULTS: Three female patients with SIH with a mean age of 25.6 ± 10.0 years old. The patients had orthostatic headache, and one of them presented with somnolence and diplopia because of a CVT. Brain magnetic resonance imaging (MRI) ranges from normal findings to classic findings of SIH as pachymeningeal enhancement and downward displacement of the cerebellar tonsils. Spine MRI showed abnormal epidural fluid collections in all cases, and computed tomography (CT) myelography could determine an identifiable cerebrospinal fluid (CSF) leak in only one patient. One patient received a conservative approach, and the other two were submitted to open surgery with laminoplasty. Both of them had uneventful recovery and remission in surgery follow-up. CONCLUSION: The diagnosis and management of SIH are still a challenge in neurology practice. We highlight in the present study severe cases of incapacitating SIH, complication with CVT, and good outcomes with neurosurgical treatment.

ANTECEDENTES: Hipotensão intracraniana espontânea (HIE) é uma causa secundária de cefaleia e uma doença subdiagnosticada. A apresentação clínica pode ser muito variável. Tipicamente, se apresenta com queixas isoladas de cefaleia ortostática clássica, porém pode evoluir com complicações significativas como trombose venosa cerebral (TVC). OBJETIVO: Relatar 3 casos de diagnóstico de hipotensão intracraniana espontânea manejados em uma enfermaria de neurologia de nível terciário. MéTODOS: Revisão dos prontuários de três pacientes e descrição dos resultados clínicos e cirúrgicos. RESULTADOS: Três pacientes do sexo feminino com média de idade de 25.6 ± 10.0 anos. As pacientes apresentavam cefaleia ortostática e uma delas apresentou sonolência e diplopia devido a TVC. A ressonância magnética (RM) do encéfalo varia de achados normais até achados clássicos de HIE como realce paquimeníngeo e deslocamento inferior das tonsilas cerebelares. A RM da coluna mostrou coleções anormais de líquido epidural em todos os casos e a mielografia por tomografia computadorizada (TC) foi capaz de determinar fístula liquórica identificável em apenas uma paciente. Uma paciente recebeu abordagem conservadora e as outras duas foram submetidas a cirurgia aberta com laminoplastia. Ambas tiveram recuperação e remissão sem intercorrências no seguimento cirúrgico. CONCLUSãO: O diagnóstico e manejo da hipotensão intracraniana ainda são desafios na prática neurológica. Destacamos no presente estudo casos graves, complicação com TVC e bons resultados com tratamento neurocirúrgico.

Minimally invasive surgery for spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension.

OBJECTIVE: Spinal CSF leaks cause spontaneous intracranial hypotension (SIH). Surgical closure of spinal CSF leaks is the treatment of choice for persisting leaks. Surgical approaches vary, and there are no studies in which minimally invasive techniques were used. In this study, the authors aimed to detail the safety and feasibility of minimally invasive microsurgical sealing of spinal CSF leaks using nonexpandable tubular retractors. METHODS: Consecutive patients with SIH and a confirmed spinal CSF leak treated at a single institution between April 2019 and December 2020 were included in the study. Surgery was performed via a dorsal 2.5-cm skin incision using nonexpandable tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach. The primary outcome was successful sealing of the dura, and the secondary outcome was the occurrence of complications. RESULTS: Fifty-eight patients, 65.5% of whom were female (median age 46 years [IQR 36-55 years]), with 38 ventral leaks, 17 lateral leaks, and 2 CSF venous fistulas were included. In 56 (96.6%) patients, the leak could be closed, and in 2 (3.4%) patients the leak was missed because of misinterpretation of the imaging studies. One of these patients underwent successful reoperation, and the other patient decided to undergo surgery at another institution. Two other patients had to undergo reoperation because of insufficient closure and a persisting leak. The rate of permanent neurological deficit was 1.7%, the revision rate for a persisting or recurring leak was 3.4%, and the overall revision rate was 10.3%. The rate of successful sealing during the primary closure attempt was 96.6% and 3.4% patients needed a secondary attempt. Clinical short-term outcome at discharge was unchanged in 14 patients and improved in 25 patients, and 19 patients had signs of rebound intracranial hypertension. CONCLUSIONS: Minimally invasive surgery with tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach is safe and effective for the treatment of spinal CSF leaks. The authors suggest performing a minimally invasive closure of spinal CSF leaks in specialized centers.

Surgical management of spontaneous intracranial hypotension syndrome: a literature review.

INTRODUCTION: 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. OBJECTIVE: The clinical outcomes of patients diagnosed with SIH treated with either only surgery or with surgery as the primary method of treatment were analysed. MATERIAL AND METHODS: The PubMed, Scopus and Google Scholar databases were searched according to the established criteria. RESULTS: 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. CONCLUSIONS: 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.

Full Endoscopic Repair of Spontaneous Ventral Cerebrospinal Fluid Leaks in the Spine: Systematic Review of Surgical Treatment Options and Illustrative Case.

BACKGROUND: Spontaneous spinal cerebrospinal fluid (CSF) leaks are a rare entity that can lead to intracranial hypotension and associated headaches, meningismus, and patient debility. Surgical treatment may be necessary for patients who do not respond to conservative management. Surgical repair of CSF leaks located in the ventral thoracic spine traditionally require an invasive, open approach. METHODS: We describe the case of a patient with a ventral thoracic spontaneous spinal CSF leak associated with a ventral bony osteophyte successfully treated with spinal endoscopy. We also provide a systematic review of the literature to better understand outcomes of this approach. RESULTS: A total of 55 patients were included in the systematic review. The study designs found in the literature review included case reports (66.7%), retrospective cohorts (22.2%), and prospective cohorts (11.1%). Of the studies reporting data, 50% of studies stated they used an open posterior approach to the dural defect, while 37.5% reported using an open anterior approach to the pathology. Only 1 (12.5%) study reported using an endoscope. Most studies (62.5%) used primary closure of the dura in their technique, while 37.5% reported using a local tissue graft (fat or muscle) or a dural sealant for their closure technique, and 25% of studies reported using a dural substitute for their closure technique. Overall mean clinical follow-up was 19.8 months. CONCLUSIONS: The endoscopic approach described here for treatment of this rare entity allows for removal of bony spicules/osteophytes and dural repair without the morbidity associated with traditional open dorsolateral or ventrolateral approaches.

[Spinal CSF-venous fistula: case report and literature review]. / Spinal'naya likvoro-venoznaya fistula kak prichina sindroma spontannoi vnutricherepnoi gipotenzii: sluchai iz praktiki i obzor literatury.

Syndrome of spontaneous intracranial hypotension is increasingly described in the literature as a multifactorial disease with impairment of the quality of life and risk of mortality. CSF-venous fistula as a cause of intracranial hypotension syndrome is extremely rare and requires complex diagnosis and timely surgical treatment. OBJECTIVE: We present a 55-year-old patient with acute spontaneous intracranial hypotension and spinal CSF-venous fistula. Literature data are also analyzed. RESULTS: Algorithm for diagnosis and efficacy of microsurgical resection of CSF-venous fistula is demonstrated. CONCLUSION: Intracranial hypotension following spinal fistula requires careful examination. Accurate understanding of pathophysiological and anatomical features of this disease is necessary to select an effective treatment method.

Brain Spontaneous Intracranial Hypotension Score for Treatment Monitoring After Surgical Closure of the Underlying Spinal Dural Leak.

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.

Outcome after surgical treatment of cerebrospinal fluid leaks in spontaneous intracranial hypotension-a matter of time.

OBJECTIVE: Spinal 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. METHODS: We 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. RESULTS: Sixty-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. CONCLUSION: Shorter 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.

How I do it: the trans-laminar, facet-joint sparing minimal invasive approach for ventral dural repair in spontaneous intracranial hypotension-a 2-dimensional operative video.

BACKGROUND: We describe the minimally invasive, facet-sparing postero-lateral approach to the thoracic spine for a ventral dural repair in a patient with intracranial hypotension secondary to a spontaneous dural breach. METHODS: We performed a minimally invasive approach using a short paramedian posterior skin incision followed by a 10 × 10 mm targeted trans-laminar approach, to achieve a microsurgical repair of a symptomatic ventral dural defect causing severe disability. CONCLUSION: The facet-sparing postero-lateral approach is safe and effective in the surgical management of thoracic dural tears, even in the most anterior ones, and avoids the traditional costotransversectomy.

Intradural extraarachnoid sutureless technique combined with laminoplasty for indirect repair of ventral dural defects in spontaneous intracranial hypotension: technical note and case series.

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.

Monro-Kellie Hypothesis: Increase of Ventricular CSF Volume after Surgical Closure of a Spinal Dural Leak in Patients with Spontaneous Intracranial Hypotension.

BACKGROUND AND PURPOSE: CSF loss in spontaneous intracranial hypotension disrupts a well-regulated equilibrium. We aimed to evaluate the volume shift between intracranial compartments in patients with spontaneous intracranial hypotension before and after surgical closure of the underlying spinal dural breach. MATERIALS AND METHODS: In total, 19 patients with spontaneous intracranial hypotension with a proved spinal CSF leak investigated at our institution between July 2014 and March 2017 (mean age, 41.8 years; 13 women) were included. Brain MR imaging-based volumetry at baseline and after surgery was performed with FreeSurfer. In addition, the spontaneous intracranial hypotension score, ranging from 0 to 9, with 0 indicating very low and 9 very high probability of spinal CSF loss, was calculated. RESULTS: Total mean ventricular CSF volume significantly increased from baseline (15.3 mL) to posttreatment MR imaging (18.0 mL), resulting in a mean absolute and relative difference, +2.7 mL and +18.8% (95% CI, +1.2 to +3.9 mL; P < .001). The change was apparent in the early follow-up (mean, 4 days). No significant change in mean total brain volume was observed (1136.9 versus 1133.1 mL, P = .58). The mean spontaneous intracranial hypotension score decreased from 6.9 ± 1.5 at baseline to 2.9 ± 1.5 postoperatively. CONCLUSIONS: Our study demonstrated a substantial increase in ventricular CSF volume in the early follow-up after surgical closure of the underlying spinal dural breach and may provide a causal link between spinal CSF loss and spontaneous intracranial hypotension. The concomitant decrease in the spontaneous intracranial hypotension score postoperatively implies the restoration of an equilibrium within the CSF compartment.

Superficial siderosis and intracranial hypotension syndrome following brachial plexus avulsion injury. A case of surgical treatment.

Superficial siderosis (SS) is a slowly progressive neurodegenerative disorder caused by persistent or intermittent bleeding into the subarachnoid space. It leads to characteristic clinical and radiographic findings. Dural pathology is believed to be the most common identifiable etiology of SS. It has been suggested that dural tear may be the common pathology of both SS and intracranial hypotension syndrome. We present a patient with SS caused by posttraumatic duropathy that was associated with cerebrospinal fluid (CSF) hypotension headache. Patient was treated surgically with stabilization of neurological deficit and orthostatic headache improvement. It supports the speculated link between both entities and may confirm surgery being a reasonable approach in patients with SS.

Clinical Outcomes Following Surgical Ligation of Cerebrospinal Fluid-Venous Fistula in Patients With Spontaneous Intracranial Hypotension: A Prospective Case Series.

BACKGROUND: Cerebrospinal fluid-venous fistula (CVF) is a recently described cause of spontaneous intracranial hypotension (SIH). Surgical ligation of CVF has been reported, but clinical outcomes are not well described. OBJECTIVE: To determine the clinical efficacy of surgical ligation for treatment of CVF. METHODS: Outcomes metrics were collected in this prospective, single-arm, cross-sectional investigation. Inclusion criteria were as follows: diagnosis of SIH, demonstration of CVF on myelography, and surgical treatment of CVF. Pre- and postoperative headache severity was assessed with the Headache Impact Test (HIT-6), a validated headache scale ranging from 36 (asymptomatic) to 78 (most severe). Patient satisfaction with treatment was measured with Patient Global Impression of Change (PGIC). RESULTS: Twenty subjects were enrolled, with mean postoperative follow-up at 16.0 ± 9.7 mo. All CVFs were located in the thoracic region (between T4 and T12). Pretreatment headache severity was high (mean HIT-6 scores 65 ± 6). Surgical treatment resulted in marked improvement in headache severity (mean HIT-6 change of -21 ± -9, mean postoperative HIT-6 of 44 ± 8). Of subjects with baseline headache scores in the most severe category, 83% showed a major improvement in severity (transition to the lowest 2 severity categories) after surgery. All subjects (100%) reported clinically significant levels of satisfaction with treatment (PGIC score 6 or 7); 90% reported the highest level of satisfaction. There were no short- or long-term complications or 30-d readmissions. CONCLUSION: Surgical ligation is highly effective for the treatment of SIH due to CVF. Larger controlled trials with longer follow-up period are indicated to better assess its long-term efficacy and safety profile.

Spontaneous spinal CSF-venous fistulas associated with venous/venolymphatic vascular malformations: report of 3 cases.

Spontaneous CSF-venous fistulas may be present in up to one-fourth of patients with spontaneous intracranial hypotension. This is a recently discovered type of CSF leak, and much remains unknown about these fistulas. Spinal CSF-venous fistulas are usually seen in coexistence with a spinal meningeal diverticulum, suggesting the presence of an underlying structural dural weakness at the proximal portion of the fistula. The authors now report the presence of soft-tissue venous/venolymphatic malformations associated with spontaneous spinal CSF-venous fistulas in 2 patients with spontaneous intracranial hypotension, suggesting a role for distal venous pathology. In a third patient with spontaneous intracranial hypotension and a venolymphatic malformation, such a CSF-venous fistula is strongly suspected.