Management of Cerebrospinal Fluid Leak After Intrathecal Baclofen Pump Procedures in Adolescents With Cerebral Palsy: A Retrospective Study.

STUDY OBJECTIVE:  Epidural blood patches (EBPs) are frequently performed in children with cerebral palsy (CP) to manage post-dural puncture headache (PDPH) due to cerebrospinal fluid (CSF) leak after intrathecal baclofen pump (ITBP) placement or replacement procedures. The purpose of our study was to review the incidence and management of CSF leak following ITBP placement or replacement procedures in children with CP. The study was a retrospective review of 245 patients representing 310 surgical cases of baclofen pump insertion (n=141) or reinsertion (n=169) conducted at a 125-bed children's hospital with prominent specialty orthopedics surgical cases. MEASUREMENTS:  Demographic and clinical information was obtained from the anesthesia pain service database on all new ITBP placement and subsequent replacements over an eight-year period. MAIN RESULTS:  The overall incidence of CSF leak in our population was 16% (50 of 310) and 18% (25 of 141) with a new ITBP placement. Children with diplegia were associated with a threefold risk of developing CSF leak. Of patients who developed CSF leak (n=50), 68% (n=34) were successfully treated conservatively, while 32% (n=16) required EBPs. EBPs were successful in 87.5% (14 of 16) of patients at relieving PDPH on the first attempt.  Conclusions: CSF leak is a known problem after ITBP placement and replacement. Most patients were successfully treated with conservative management and EBPs were successful in patients failing conservative therapy. Diagnosing PDPH in non-verbal patients can be challenging.

Management of cervical CSF-venous fistula causing acute cognitive impairment and coma.

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cognitive decline progressing to coma. Brain imaging showed features of spontaneous intracranial hypotension and a right C7 CVF was identified at digital subtraction and CT myelography. Initial treatment with CT-guided injection of fibrin sealant produced temporary improvement in symptoms before surgical treatment resulted in total clinical remission and radiological resolution.

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.

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.

Prone Dynamic CT Myelography in Spontaneous Intracranial Hypotension : Diagnostic Need and Radiation Doses.

BACKGROUND AND PURPOSE: The diagnostic work-up in patients with spontaneous intracranial hypotension (SIH) and spinal longitudinal extradural CSF collection (SLEC) on magnetic resonance imaging (MRI) comprises dynamic digital subtraction myelography (dDSM) in prone position for leak detection. Dynamic computed tomography (CT) myelography (dCT-M) in prone position follows if the leak is not unequivocally located. A drawback of dCT­M is a high radiation dose. This study evaluates the diagnostic needs of dCT-M examinations and measures to reduce radiation doses. METHODS: Frequency, leak sites, length and number of spiral acquisitions, DLP and effective doses of dCT­M were retrospectively recorded in patients with ventral dural tears. RESULTS: Of 42 patients with ventral dural tears, 8 underwent 11 dCT­M when the leak was not unequivocally shown on digital subtraction myelography. The median number of spiral acquisitions was 4 (range 3-7) and the mean effective radiation dose 30.6 mSv (range 13.1-62.16 mSv) mSv. Five of eight leaks were located in the upper thoracic spine (range C7/Th1-Th2/3). Bolus tracking of intrathecal contrast agent in dCT­M was used to limit the number and length of spiral acquisitions. DISCUSSION: A dCT­M in prone position to localize a ventral dural tear is needed in every fifth patient with a SLEC on MRI. It is typically needed when the leak is located in the upper thoracic spine and when patients have broad shoulders. Measures to reduce the radiation dose include bolus tracking or to repeat the DSM with adjusted positioning of patient.

T2-Sampling Perfection With Application-Optimized Contrasts by Using Flip Angle Evolution (SPACE) Protocol MRI: A Safe, Minimally Invasive Screening Tool for Spinal CSF Leak Causing Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension (SIH) due to a spinal cerebrospinal fluid (CSF) leak is secondary cause of headache with potentially devastating consequences. Its diagnosis is complicated owing to the lack of a reasonable, minimally invasive screening test. This results in many patients remaining undiagnosed for years after the headache onset. Current testing approaches are either overly invasive, namely the CSF infusion protocol or both invasive and insensitive viz. lumbar puncture (LP) with an opening pressure (OP) or computed tomography myelogram (CTM). These diagnostic methods are frequently employed in a clinical setting since they require access to the thecal space; they unfortunately have a dearth of sensitivity. CTM will not document a leak if it is intermittent or very slow and in the setting of a spinal CSF leak, the OP on LP may be high, low, or normal. A potential remedy for this state is the T2-sampling perfection with application-optimized contrasts by using flip angle evolution (SPACE) protocol spinal magnetic resonance imaging (MRI). We present two cases that demonstrate its potential value as a screening tool. It is well known for its high sensitivity for identifying spinal pathology and is minimally invasive, making it a good choice for a screening modality when diagnosing possible SIH cases.

Intraoperative localization and "snowman" muscle pledget repair for ventral dural defect in a case of spontaneous intracranial hypotension.

BACKGROUND: This image report with technical notes is the first to illustrate and describe the technique used to treat spinal cerebrospinal fluid (CSF) leaks with the "snowman" muscle pledget. A 49-year-old male presented with orthostatic headaches as well as the left abducens nerve palsy. Patient's workup including findings of diffuse meningeal enhancement on magnetic resonance imaging, lumbar puncture opening pressure of 4 cm H2O, and CT myelogram demonstrating evidence of ventral spinal thoracic CSF leak. CASE DESCRIPTION: Procedure took place in a hybrid biplane operating room so that simultaneous digital subtraction myelogram may also be performed for intraoperative localization. Dural defect was identified intraoperatively and repaired with thoracic laminectomy and "snowman" muscle pledget technique. Postoperatively, the patient did well with resolution of his symptoms. CONCLUSION: The authors have proposed a grading scale to aid in the work up and management of intracranial hypotension. The use of a hybrid biplane operating room and "snowman" muscle pledget technique is a safe and effective technique to treat spontaneous spinal CSF leaks resulting from dural defects.

CSF-Venous Fistulas: Anatomy and Diagnostic Imaging.

CSF-venous fistulas (CVFs), first described in 2014, are an important cause of spontaneous intracranial hypotension. CVFs can be challenging to detect on conventional anatomic imaging because, unlike other types of spinal CSF leak, they do not typically result in pooling of fluid in the epidural space, and imaging signs of CVF may be subtle. Specialized myelographic techniques have been developed to help with CVF identification, but these techniques are not yet widely disseminated. This article reviews the current understanding of CVFs, emphasizing correlations between venous anatomy and imaging findings as well as potential mechanisms for pathogenesis, and describes current imaging techniques used for CVF diagnosis and localization. These techniques are broadly classified into fluoroscopy-based methods, including digital subtraction myelography and dynamic myelography, and cross-sectional methods, including decubitus CT myelography and MR myelography with intrathecal injection of gadolinium. Knowledge of these various options, including their relative advantages and disadvantages, is critical in the care of patients with spontaneous intracranial hypotension. Investigation is ongoing, and continued advances in knowledge about CVFs as well as in optimal imaging detection are anticipated.

Cerebrospinal Fluid-Venous Fistulas: A Systematic Review and Examination of Individual Patient Data.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is usually caused by a spinal cerebrospinal fluid (CSF) leak. CSF-venous fistula is an underdiagnosed cause of spinal CSF leak, as it is challenging to identify on myelography. OBJECTIVE: To review existing literature to summarize common presentations, diagnostic imaging modalities, and current treatment strategies for CSF-venous fistulas. METHODS: We conducted a systematic review using PubMed, Embase, Scopus, and Web of Science databases to identify studies discussing CSF-venous fistulas. Titles and abstracts were screened. Studies meeting prespecified inclusion criteria were reviewed in full. RESULTS: Of 180 articles identified, 16 articles met inclusion criteria. Individual patient data was acquired from 7 studies reporting on 18 patients. CSF-venous fistula most frequently presented as positional headache. Digital subtraction myelography provided greatest detection of CSF-venous fistula in the lateral decubitus position and detected CSF-venous fistula in all individual patient cases. Dynamic computed tomography (CT) myelogram enabled detection and differentiation of CSF-venous fistulas from low-flow epidural leaks. The majority of fistulas were in the thoracic spine and slightly more common on the right. Epidural blood patch (EBP) provided temporary or no relief in all individual patients. Resolution or improvement of clinical symptoms and radiologic normalization were observed in all surgically treated patients. CONCLUSION: Although rare, CSF-venous fistula is an important cause of spinal CSF leak contributing to SIH. Dynamic CT myelogram and digital subtraction myelography, particularly in the lateral decubitus position, are the most accurate and effective diagnostic imaging modalities. EBPs often provide only transient relief, while surgical management is preferred.

Spontaneous intracranial hypotension complicated by diffuse cerebral edema and episodes of severely elevated intracranial pressure: illustrative case.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a well-documented condition that typically follows a defined clinical course. Previously published studies describing the pathophysiology of SIH have demonstrated extensive evidence of low intracranial pressure (ICP) driving the clinical features of the condition. Through lumbar puncture and use of intracranial monitoring devices, however, both low and normal cerebrospinal fluid (CSF) pressures have been documented. This report outlined and discussed the unique finding of elevated ICP associated with clinical features of SIH. OBSERVATIONS: Here, the authors presented a case of a patient with spontaneous spinal CSF leak who developed tonsillar herniation, cerebral edema, and subsequent episodes of elevated ICP. Although more diverse presentations of SIH are being reported, the authors believed the case to be unique because SIH was accompanied by elevated ICP. LESSONS: This case adds to the growing body of literature surrounding SIH by demonstrating that patients can develop elevated CSF pressures associated with acute encephalopathy.

Surgical Management of Symptomatic Boxing-Induced Spinal Cerebrospinal Fluid Leak After Failed Epidural Blood Patch.

BACKGROUND: Spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized condition responsible for spontaneous intracranial hypotension. A host of connective tissue disorders and mechanical factors may precipitate the condition. Conservative treatment has limited efficacy, and many patients with persistent symptoms ultimately require epidural blood patch (EBP). However, about 23%-44% of patients experience recurrence of symptoms after EBP. Unidentified or multiple sites of CSF leaks are thought to be responsible for the failure of EBP. Using our previously published technique, we treated a patient who developed a large spontaneous CSF leak in her cervicothoracic spine after a boxing class, offering further evidence of the utility of the technique for select patients. CASE DESCRIPTION: A 28-year-old woman was referred to our center with recurrent, severe headaches and associated nausea and vomiting. She underwent EBP 3 times with no resolution of her symptoms. A right-side partial C7-T1 hemilaminotomy was performed to identify a large meningocele filled with CSF. After dissection, dural sealant was applied using an angled needle with a syringe to buttress the meningocele to allow for normalization of the hydrostatic pressure. At 5-month follow-up, her symptoms have resolved with no headaches and paresthesias in upper limbs. CONCLUSIONS: Commonplace events, such as a boxing/sparring class, may precipitate a spontaneous spinal CSF leak. Our minimally invasive surgical approach can be safely used in patients with spontaneous intracranial hypotension with recurrent symptoms, no identified leak site, and prior failed attempts of EBP.

Spinal cerebrospinal fluid leak in the context of pars interarticularis fracture.

BACKGROUND: Spinal cerebrospinal fluid (CSF) leak can lead to intracranial hypotension and is an important differential diagnosis to consider in patients with sudden-onset chronic daily headaches. Pars interarticularis (PI) fracture is a potential rare cause of suspected spinal CSF leak. METHODS: This is a retrospective case series of 6 patients with suspected spinal CSF leak evaluated between January 2016 and September 2019. All patients received a magnetic resonance imaging (MRI) of the brain with and without gadolinium, MRI whole spine and full spine computed tomography (CT) myelogram. Targeted epidural patches with fibrin sealant were performed. Treatment response at return visit (3 months post-patch) was documented. RESULTS: Six patients (4 females, 2 males) were diagnosed with a suspected spinal CSF leak and PI fracture. Mean age at the time of headache onset was 39 years old, and a range from 32 to 50 years old. Mean time to targeted epidural patches with fibrin sealant was 4.5 years. All 6 patients had PI fractures identified on CT myelogram and received targeted epidural patches with fibrin sealant at the site of the PI fracture. All patients had significant improvement in their headache intensity. CONCLUSION: Our study highlights: 1) the importance of PI fracture as a possible culprit of suspected spinal CSF leak in patients with intracranial hypotension; 2) the added benefit of CT imaging for detecting bony abnormalities such as fractures in patients with intracranial hypotension; and 3) the successful treatment of suspected spinal CSF leak when targeting the fracture site.

False localizing signs of spinal CSF-venous fistulas in spontaneous intracranial hypotension: report of 2 cases.

A spinal CSF-venous fistula is one of three specific types of spinal CSF leak that can be seen in patients with spontaneous intracranial hypotension (SIH). They are best demonstrated on specialized imaging, such as digital subtraction myelography (DSM) or dynamic myelography, but often they are diagnosed on the basis of increased contrast density in the draining veins (the so-called hyperdense paraspinal vein sign) on early postmyelography CT scans. The authors report on 2 patients who underwent directed treatment (surgery in one patient and glue injection in the other) based on the hyperdense paraspinal vein sign, in whom the actual site of the fistula did not correspond to the level or laterality of the hyperdense paraspinal vein sign. The authors suggest consideration of DSM or dynamic myelography prior to undertaking treatment directed at these fistulas.

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.

Updating superficial siderosis of the central nervous system: bleeding of a dorsal osteophyte into the subarachnoid space from a perforating artery.

Superficial siderosis of the central nervous system (SSCNS) is an uncommon and often unrecognized disorder that results from recurrent and persistent bleeding into the subarachnoid space. Currently, there is no effective treatment for SSCNS. The identification and surgical resolution of the cause of bleeding remains the most reliable method of treatment, but the cause of bleeding is often not apparent. The identified sources of recurrent bleeding have typically included neoplasms, vascular malformations, brachial plexus or nerve root injury or avulsion, and previous head and spinal surgery. An association between recurrent bleeding in the CNS and dural abnormalities in the spine has recently been suggested. Dural tears have been identified in relation to a protruding disc or osteophyte. Also in these patients, the exact mechanism of bleeding remains unknown because of a lack of objective surgical data, even in patients who undergo neurosurgical procedures.The present case concerns a 48-year-old man who presented with longstanding symptoms of mild hearing loss and mild gait ataxia. A diagnosis of SSCNS was made in light of the patient's history and the findings on physical examination, imaging, and laboratory testing. MRI and CT detected a small calcific osteophyte in the anterior epidural space of T8-9. The patient underwent surgical removal of the bone spur and dural tear repair. During the surgery, the authors detected a perforating artery, which was on the osteophyte, that was bleeding into the subarachnoid space. This case shows a possible mechanism of chronic bleeding from an osteophyte into the subarachnoid space. In the literature currently available, a perforating artery on an osteophyte bleeding into the subarachnoid space has never been described in SSCNS.

Spinal cerebrospinal fluid leak as the cause of chronic subdural hematomas in nongeriatric patients.

OBJECT: The etiology of chronic subdural hematoma (CSDH) in nongeriatric patients (≤ 60 years old) often remains unclear. The primary objective of this study was to identify spinal CSF leaks in young patients, after formulating the hypothesis that spinal CSF leaks are causally related to CSDH. METHODS: All consecutive patients 60 years of age or younger who underwent operations for CSDH between September 2009 and April 2011 at Bern University Hospital were included in this prospective cohort study. The patient workup included an extended search for a spinal CSF leak using a systematic algorithm: MRI of the spinal axis with or without intrathecal contrast application, myelography/fluoroscopy, and postmyelography CT. Spinal pathologies were classified according to direct proof of CSF outflow from the intrathecal to the extrathecal space, presence of extrathecal fluid accumulation, presence of spinal meningeal cysts, or no pathological findings. The primary outcome was proof of a CSF leak. RESULTS: Twenty-seven patients, with a mean age of 49.6 ± 9.2 years, underwent operations for CSDH. Hematomas were unilateral in 20 patients and bilateral in 7 patients. In 7 (25.9%) of 27 patients, spinal CSF leakage was proven, in 9 patients (33.3%) spinal meningeal cysts in the cervicothoracic region were found, and 3 patients (11.1%) had spinal cysts in the sacral region. The remaining 8 patients (29.6%) showed no pathological findings. CONCLUSIONS: The direct proof of spinal CSF leakage in 25.9% of patients suggests that spinal CSF leaks may be a frequent cause of nongeriatric CSDH.