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.

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.

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.

[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.

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.

Sonography of the optic nerve sheath diameter before and after microsurgical closure of a dural CSF fistula in patients with spontaneous intracranial hypotension - a consecutive cohort study.

OBJECTIVE: Spontaneous intracranial hypotension is caused by spinal cerebrospinal fluid leakage. Patients with orthostatic headaches and cerebrospinal fluid leakage show a decrease in optic nerve sheath diameter upon movement from supine to upright position. We hypothesized that the decrease in optic nerve sheath diameter upon gravitational challenge would cease after closure of the leak. METHODS: We included 29 patients with spontaneous intracranial hypotension and refractory symptoms admitted from 2013 to 2016. The systematic workup included: Optic nerve sheath diameter sonography, spinal MRI and dynamic myelography with subsequent CT. Microsurgical sealing of the cerebrospinal fluid leak was the aim in all cases. RESULTS: Of 29 patients with a proven cerebrospinal fluid leak, one declined surgery. A single patient was lost to follow-up. In 27 cases, the cerebrospinal fluid leak was successfully sealed by microsurgery. The width of the optic nerve sheath diameter in supine position increased from 5.08 ± 0.66 mm before to 5.36 ± 0.53 mm after surgery ( p = 0.03). Comparing the response of the optic nerve sheath diameter to gravitational challenge, there was a significant change from before (-0.36 ± 0.32 mm) to after surgery (0.00 ± 0.19 mm, p < 0.01). In parallel, spontaneous intracranial hypotension-related symptoms resolved in 26, decreased in one and persisted in a single patient despite recovery of gait. CONCLUSIONS: The sonographic assessment of the optic nerve sheath diameter with gravitational challenge can distinguish open from closed spinal cerebrospinal fluid fistulas in spontaneous intracranial hypotension patients. A response to the gravitational challenge, that is, no more collapse of the optic nerve sheath while standing up, can be seen after successful treatment and correlates with the resolution of clinical symptoms. Sonography of the optic nerve sheath diameter may be utilized for non-invasive follow-up in spontaneous intracranial hypotension.

Chiari I malformation and altered cerebrospinal fluid dynamics-the highs and the lows.

PURPOSE: This paper reviews the altered cerebrospinal fluid dynamics that can be associated with paediatric Chiari I malformation and we present our own institutional experience. METHODS: We conducted a thorough review of the literature and retrospectively analyzed all cases of operatively managed paediatric Chiari 1 malformation at our institution between February 2006 and February 2019. RESULTS: Acquired Chiari malformation (ACM) can radiologically mimic Chiari I and has been associated with both intracranial hypotension (either secondary to lumboperitoneal shunting or spontaneous CSF hypotension) and idiopathic intracranial hypertension (IIH). At our institution, 61 paediatric cases (range, 2-15 years) underwent foramen magnum decompression (FMD) for Chiari I malformation. Whilst 80% (50/61) of cases underwent FMD with no preceding or post-operative problems of CSF dynamics, 8% (5/61) of cases had hydrocephalus at initial presentation requiring CSF diversion followed by FMD for persistent Chiari, and 10% (6/61) developed hydrocephalus following FMD and required long-term CSF diversion. CONCLUSIONS: In paediatric ACM, the management of intracranial hypotension involves thorough radiological assessment and inclusion/adjustment of a valve in the case of lumboperitoneal shunting or epidural blood patch or interventional techniques in the case of spontaneous CSF leak. Thereby, unwarranted posterior fossa decompression surgery is avoided. In the case of IIH and Chiari I malformation, children who have recurrent symptoms despite adequate posterior fossa decompression surgery (failed Chiari), there is a strong role for intracranial pressure monitoring as raised intracranial pressure may indicate long-term CSF diversion.

Duret hemorrhage complicating a sinking skin flap syndrome.

Duret hemorrhage has always been reported during an episode of increased intracranial pressure with transtentorial herniation. We reported a Duret hemorrhage occurring during an episode of intracranial hypotension resulted in sinking skin flap syndrome which was responsible for acute paradoxal descending transtentorial herniation and Duret hemorrhage, 10 days after large hemicraniectomy which could indicate early cranioplasty.

Clinical effect of the proximity of epidural blood patch injection to the leakage site in spontaneous intracranial hypotension.

BACKGROUND: Epidural blood patch (EBP) has been shown to be an effective treatment option for spontaneous intracranial hypotension (SIH). We investigated whether response to the EBP was related to the distance of the injection site from the leakage site in patients with SIH. METHODS: We reviewed patients with SIH who underwent EBP at a single hospital. Patients were assigned to group R (response after EBP) or group N (no response after EBP). We then analyzed the demographics, clinical characteristics, leakage site, leakage length, EBP injection level and distance from leakage site, and injected EBP volume. RESULTS: Sixty-two patients were included in the analysis. The overall response rate to EBP was 59.7% (37 patients). The leakage length and injection distance from the leakage site did not differ between the two groups. Age, gender, body mass index, leakage site, and EBP volume did not differ significantly between the two groups. CONCLUSION: The clinical effect of EBP in SIH was not affected by leakage length or injection distance to leakage site. Further large studies must be conducted to investigate the efficacy of targeted EBP for SIH.

Predictors and incidence of orthostatic headache associated with lumbar drain placement following endoscopic endonasal skull base surgery.

BACKGROUND: Orthostatic headache (OH) is a potential complication of lumbar drainage (LD) usage. The incidence and risk factors for OH with the use of lumbar drainage during endoscopic endonasal procedures have not been documented. OBJECTIVE: To investigate the incidence of post-procedure OHs associated with placement of LD in patients undergoing endoscopic endonasal procedures. METHODS: We prospectively noted the placement of LDs in a consecutive series of endoscopic endonasal skull base surgeries. Charts were retrospectively reviewed, and patients were divided into two groups: those with OH and those without. The patient demographics, drain durations, imaging findings of intracranial hypotension, pathologies and need for a blood patch were compared between the two groups. RESULTS: Two hundred forty-nine patients were included in the study. Seven patients (2.8%) suffered post-dural puncture OH, which was mild to moderate and disappeared 2-8 days (median 3 days) after treatment. Blood patches were used in four patients. Significant predisposing factors were age (33.0 vs. 53.5, P = 0.014) and a strong trend for female gender (85.7% vs. 47.9%, P = 0.062). BMI and drain duration were not significant. Postoperative intracranial hypotension was diagnosed radiographically in 43% of OH patients and in 5.4% of those without OH (P = 0.003). Four (1.6%) patients required treatment with an epidural blood patch. CONCLUSION: OH associated with intracranial hypotension in patients undergoing endoscopic endonasal procedures with LDs is an infrequent complication seen more commonly in young female patients. Radiographic signs of intracranial hypotension are a specific but not sensitive test for OH.

Vanishing calcification associated with a spontaneous ventral spinal cerebrospinal fluid leak.

BACKGROUND: Some patients with spontaneous intracranial hypotension have a ventral spinal cerebrospinal fluid (CSF) leak and these CSF leaks may be associated with calcified disk herniations. Identifying these calcifications is helpful in directing treatment. We report here the unusual case of a patient with a ventral CSF leak in whom the associated calcification absorbed over a five-month period. CASE REPORT: A 42-year-old woman developed orthostatic headaches and bilateral abducens nerve palsies. Magnetic resonance imaging of her brain showed typical findings of spontaneous intracranial hypotension. Magnetic resonance imaging of her spine showed an extensive cervicothoracic CSF leak. Computed tomographic myelography showed calcification at the Th1-2 disk space. Three epidural blood patches were performed, but her symptoms persisted. Digital subtraction myelography performed five months later showed an upper thoracic ventral CSF, but the calcification was no longer present. A dural tear, found at surgery at the Th1-2 level, was repaired and the patient made an uneventful recovery. DISCUSSION: The resorption of calcifications at the level of a ventral spinal CSF leak could explain the absence of any calcifications in at least some patients with such leaks and demonstrates the usefulness of reviewing previous imaging in patients with ventral CSF leaks if the exact site of the leak remains unknown.

Treatment strategy for cerebral hypotension caused by spontaneous cerebrospinal fluid leaks.

OBJECTIVE: Spontaneous spinal cerebrospinal fluid (CSF) leaks are rare (5 per 100,000 per year). Treatment generally consists of conservative therapy or interventional therapy with epidural blood patching. Surgical treatment is conducted rarely, usually in cases when conservative or interventional treatment has failed. The aim of our case series was to assess the clinical outcome after surgery. METHODS: Our clinical database was reviewed for patients with spontaneous spinal CSF leaks who underwent surgical exploration between 2010 and 2013. Etiology, symptoms, preoperative imaging, type of required surgical method, intraoperative findings, and clinical outcome were reported. RESULTS: We identified five patients with a mean age of 62 years with spontaneous spinal CSF leaks who were treated surgically. Two patients received surgery after failure of interventional treatment. The origin of the CSF leak could be identified intraoperatively in three cases. Surgical technique in all cases consisted of an interlaminar fenestration or hemilaminectomy and a complete foraminotomy to explore the thecal sack and the exiting nerve roots and identify the CSF leak. After surgery, the preoperative symptoms improved in all patients. In one case, there was a relapse after 4 weeks. CONCLUSIONS: Preoperative identification of a CSF leak with MRI was positive in only one case. In all other cases, a post-myelography CT had to be performed. In all cases, the preoperative symptoms improved after surgery. Surgical treatment is an effective treatment of spontaneous cerebrospinal fluid leaks in cases of refractory symptoms after failed conservative or interventional treatment.

[A Case of Spontaneous Cerebrospinal Fluid Leak Associated with Cervical Spondylosis].

Spontaneous cerebrospinal fluid leak and intracranial hypotension associated with cervical spondylosis have rarely been observed, and only a few cases are reported. A 69-year-old woman, previously treated for rectal and thyroid cancer, complained of a non-postural persistent headache. The patient regularly practiced aerobic exercise, but a month earlier she had started experiencing headache and neck pain while exercising. Computed tomography(CT)showed bilateral chronic subdural hematomas, and magnetic resonance imaging(MRI)revealed diffuse dural enhancement and tonsillar herniation. We drained the subdural hematomas and replaced the ventricular reservoir to safely access the cerebrospinal fluid space. After surgery, the persistent headache disappeared for several days, but a postural headache emerged. CT myelogram showed extradural accumulation of the contrast medium at the C2-5 level with cervical spondylosis. The patient was treated with conservative therapy of bed rest and intravenous fluid hydration for two weeks, and the headache improved. CT myelogram after treatment showed no extradural accumulation of the contrast medium. Spontaneous cerebrospinal fluid leak associated with cervical spondylosis could be induced by the repeated minor mechanical stress caused by physical exercise. Therefore, the possibility that non-postural persistent headache may be caused by spontaneous cerebrospinal fluid leak should not be underestimated.

Flow-related noise in patients with ventriculoperitoneal shunt using gravitational adjustable valves.

BACKGROUND: Noise disturbance arising from the valve is a rare event of ventriculoperitoneal shunts. We queried and investigated shunt patients for occurrence and evaluated the possible factors related to noise development. METHODS: Fifty ambulatory patients with implanted proGAV valve were investigated consecutively. Patients were asked for any noise arising from the shunt. In all cases, the valve was auscultated in sitting and upright position. The position of the gravitational unit (GU) was determined in respect to the Frankfurt horizontal plane (FHP) and in head reclination. Ten valves were perfused in vitro at different settings. One valve was opened for video documentation, and a frequency analysis of the noise was performed in nine valves. RESULTS: Eight percent (4/50) of the patients reported a noise arising from the valve only in upright position in combination with maximum head reclination, and immediately stopped when performing Vasalva's maneuver. In three out of four of these patients, the noise was also audible for the investigator (FS) with a prepared stethoscope. Patients complaining about a noise had a larger GU deviation from vertical during head reclination (median: -80 vs -43°, p = 0.0007, t-test). A deviations threshold of less than -58.4° excluding audible noise by a negative predictive value of 1 (95 % confidence interval [CI] 0.9 to 1.0). In an experimental setting, the noise came from vibrations of the ball in the cone of the adjustable unit and was restricted to a flow of at least 220 ml/h. The noise frequencies tended to be higher at higher opening pressures. CONCLUSIONS: Valve-related noise development may occur in patients with proGAV valves. This event could be prevented during shunt placement by avoiding posterior tilt of the gravitational unit, especially in patients with a good cervical mobility. The noise might indicate transient peak flows and was not associated with clinical or radiological signs of overdrainage.

Spontaneous intracranial hypotension: improving recognition and treatment strategies in the local setting.

We report a case of spontaneous intracranial hypotension with classic symptoms of orthostatic headache and acute presentation of subdural haematoma on computed tomographic scan. Conventional approach with conservative treatment was initially adopted. The patient's condition, however, deteriorated after 2 weeks, requiring surgical evacuation of the intracranial haemorrhage. We reviewed the clinical features of this disease and the correlated magnetic resonance imaging findings with the pathophysiological mechanisms, and described treatment strategies in the local setting. Subtle findings on initial computed tomographic scan are also reported which might improve pathology recognition. Spontaneous intracranial hypotension is not uncommonly encountered in Hong Kong, and physicians must adopt a high level of clinical suspicion to facilitate early diagnosis and appropriate management. In addition, novel therapeutic approaches may be required in those with recurrent symptoms or who are refractory to current treatment strategies.

[A case of bilateral chronic subdural hematoma due to spontaneous intracranial hypotension without orthostatic headache].

We herein present a case of bilateral chronic subdural hematoma(bilateral CSDH)caused by spontaneous intracranial hypotension(SIH)without orthostatic headache. A 67-year-old male patient with mild head injury suffered from a chronic, non-postural headache. Computed tomography of the brain showed bilateral CSDH. The hematomas were surgically evacuated using a closed drainage method. His headache immediately disappeared, but it recurred after 2 weeks. We further performed hematoma irrigation;however, his neurological status deteriorated with the development of pneumocephalus. Magnetic resonance imaging with gadolinium-DTPA showed diffuse pachymeningeal enhancement. His cerebrospinal fluid pressure was 3cmH2O, and radioisotope cisternography revealed cerebrospinal fluid leakage at the level of the cervicothoracic transition. No recurrence was found after 6 weeks of horizontal bed rest. The possibility of SIH should be considered in patients with CSDH, especially bilateral CSDH, even in elderly patients with a history of mild head trauma, and absence of orthostatic headache. Closed drainage method may be preferable in cases where surgical treatment is used.

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.