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.

Innovations in the Treatment of Spinal Cerebrospinal Fluid Leaks.

Spontaneous spinal cerebrospinal fluid (CSF) leaks are uncommon but can be neurologically debilitating. When initial treatments fail, definitive repair or closure of the leak is indicated. Depending upon the type of leak present, innovative strategies for their treatment have been developed. Among them are open surgical techniques using a transdural approach for the closure of ventral CSF leaks, minimally invasive tubular techniques for the reduction and repair of lateral meningeal diverticula, and endovascular embolization of CSF-venous fistulas. Illustrative cases demonstrating the indications for and implementation of these techniques are provided.

Suturing and Closure of Diaphragma Sella to Augment Sellar Floor Repair after Endonasal Endoscopic Resection of Large Pituitary Adenoma.

Background Large pituitary adenoma often pushes the diaphragma sella and extends to the suprasellar compartment. The thinned out diaphragma may get opened during endonasal endoscopic surgery and pose high risk for cerebrospinal fluid (CSF) leak. Such larger defects are difficult to plug with fat graft that tends to slip in to the subarachnoid space. Here, we describe a unique technique of closure of diaphragma sella that augment repair of the skull base in such cases. Materials and Method The free edge of diaphragma sella was sutured with the anterior tuberculum sella dura in five cases of large pituitary adenoma that needed extra arachnoidal resection. Suturing was done with 6-0 prolene using endoscopic needle holder that converted a large diaphragm defect in to a smaller arachnoid rent and was easily plugged with fat graft. Result None of these patients had postoperative CSF leak. Conclusion Though technically difficult, direct repair of the diaphragma sella is possible. This augments the skull base reconstruct and effectively reduces the chances of postoperative CSF leak.

Management of recurrent cerebrospinal fluid rhinorrhea caused by sequential, anatomically separated skull base defects - A case-based systematic review.

OBJECTIVE: Recurrent cerebrospinal fluid (CSF) rhinorrhea caused by sequential, anatomically separated skull base defects are rarely reported in the literature. Neither management nor etiology are sufficiently investigated. We herein present an illustrative case and a systematic review of the literature regarding etiology, diagnostics, and management of this rare phenomenon. METHODS: A systematic literature search looking for articles reporting sequential CSF-leaks with multiple skull base defects was performed. Data from included articles was descriptively reported, the quality of the included studies was assessed with GRADE. RESULTS: A 71-year-old female patient with posttraumatic rhino- and left-sided otorrhea due to a left-sided longitudinal fracture of the petrous bone presented at our institution. After initial surgical repair and a ten-week symptom-free interval, CSF-rhinorrhea reoccurred. Imaging review revealed a pre-existing contralateral meningoencephalocele of the lateral sphenoid recess causing recurrent CSF-rhinorrhea most likely after initial traumatic laceration. The defect was successfully treated. Literature search identified 366 reports, six of which were included in the systematic review with a total of ten cases. Quality was deemed good in 8/10 cases. The most common location for primary and sequential CSF-leaks was along the sphenoid bone (4/10 and 5/10 patients, respectively). All publications except one reported the presence of a meningo(encephalo)cele as cause of the sequential CSF-leak. CONCLUSION: Occurrence of recurrent CSF-rhinorrhea due to an anatomically separated sequential skull base lesion remains a rare yet described phenomenon. Reassessment of imaging studies and a structured diagnostic work-up to detect sequential CSF-leaks independent of the primary lesion should therefore be considered.

Peri-operative management of endoscopic endonasal cerebrospinal fluid leak repair: an international survey.

PURPOSE: Peri-operative management of nasal cerebrospinal fluid (CSF) leaks is not consensual due to limited evidence. The main aim of this study was to identify key factors in peri-operative management of endoscopic endonasal CSF leak repair among international experts. METHODS: A 60-item survey questionnaire collected opinions of members of international learned societies of ENT surgeons and neurosurgeons on nasal packing, post-operative instructions, antibiotic prophylaxis, and CSF volume depletion. RESULTS: The survey had 153 respondents (124 otorhinolaryngologists and 29 neurosurgeons). A resting position was recommended by 85% (130/151) of respondents for extended CSF leak of the anterior skull base, mainly in Fowler's position (72% (110/153)). Nasal packing was used by 85% (130/153) of respondents; 33.3% (51/153) used it to stabilize the reconstruction, and 22.2% (34/153) to prevent bleeding. It was usually removed after 48 h in 44.4% of cases (68/153). CSF depletion was considered by 47.1% (72/153) of respondents in case of CSF leak recurrence and by 34.6% (53/153) in cases of increased intracranial pressure. All respondents gave specific postoperative instructions to patients including driving, running, swimming, diving restrictions and flighting restrictions. In subgroup analysis, ENT surgeons more often recommended a resting position than neurosurgeons (71% vs. 37.9% ; p = 0.0008) and prescribed more antibiotics (82.3% vs. 21.4% ; p < 0.0001). CONCLUSION: Although postoperative management after CSF closure remains challenging and not codified, this international survey revealed some points of consensus concerning resting position and restriction of post-operative activities. Prospective clinical studies must be undertaken to evaluate their efficiency.

The impact of obesity on rates of post-operative CSF leak following endoscopic skull base surgery: results from a prospective international multi-centre cohort study.

Aims: Post-operative CSF leak is the major source of morbidity following transsphenoidal approaches (TSA) and expanded endonasal approaches (EEA) to lesions of the sella turcica and the ventral skull base. There are conflicting reports in the literature as to whether obesity (BMI ≥30) is a risk factor for this complication. We aimed to evaluate data collected as part of prospective multi-centre cohort study to address this question. Methods: The CRANIAL (CSF Rhinorrhoea After Endonasal Intervention to the Skull Base) study database was reviewed and patients were divided into obese and non-obese cohorts. Data on patient demographics, underlying pathology, intra-operative findings and skull base repair techniques were analysed. Results: TSA were performed on 726 patients, of whom 210 were obese and 516 were non-obese. The rate of post-operative CSF leak in the obese cohort was 11/210 (5%), compared to 17/516 (3%) in the non-obese cohort, which was not statistically significant (χ2 = 1.520, p=0.217). EEA were performed on 140 patients, of whom 28 were obese and 112 were non-obese. The rate of post-operative CSF leak in the obese cohort was 2/28 (7%), which was identical to the rate observed in the non-obese cohort 8/112 (7%) Fisher's Exact Test, p=1.000). These results persisted following adjustment for inter-institutional variation and baseline risk of post-operative CSF leak. Conclusion: CSF leak rates following TSA and EEA, in association with modern skull base repair techniques, were found to be low in both obese and non-obese patients. However, due to the low rate of post-operative CSF leak, we were unable to fully exclude a small contributory effect of obesity to the risk of this complication.

Pediatric cerebrospinal fluid rhinorrhea caused by low flow vascular anomaly of the temporal bone: A case series.

OBJECTIVE: Atraumatic cerebrospinal fluid (CSF) rhinorrhea is uncommon in children and necessitates a multi-disciplinary evaluation for an etiology. Underlying osseous abnormality due to extensive or multifocal low flow vascular anomaly should be considered as a potential cause of spontaneous CSF leak. Treatment of multifocal low flow vascular anomalies may include medical and surgical approaches. In this series, we seek to determine the presenting signs and symptoms and medical and surgical treatment options for multifocal or extensive low flow vascular anomalies. METHODS: A retrospective case series at a quaternary care children's hospital was compiled. All children with CSF rhinorrhea diagnosed and treated for multifocal low flow vascular anomalies at our institution were included. A total of four patients were identified. RESULTS: All four patients had delay in initial diagnosis of underlying cause of meningitis and CSF rhinorrhea. Average age at diagnosis of multifocal low flow vascular anomaly was 7 years. This was on average 4 years after initial presentation for medical attention. Treatment approach was multidisciplinary and included medical management with sirolimus and bisphosphonates as well as surgical approaches to the skull base (lateral and anterior) to prevent CSF egress. CONCLUSION: Consideration of multifocal low flow vascular anomaly should be included in any pediatric patient presenting with CSF rhinorrhea.

Multimodal diagnosis of cerebrospinal fluid rhinorrhea: State of the art review and emerging concepts.

Objective: Currently, diagnosis of cerebrospinal fluid (CSF) rhinorrhea relies on a multimodal approach, increasing costs and ultimately delaying diagnosis. In the United States and internationally, the crux of such a diagnosis relies on confirmation testing (via biomarkers) and localization (e.g., imaging). Biomarker testing may require analysis at an outside facility, resulting in delays diagnosis and treatment. In addition, specialized imaging may be nonspecific and often requires an active leak for diagnosis. There remains a clear need for innovative new technology. Methods: A comprehensive review was conducted on both foundational and innovative scholarly articles regarding current and emerging diagnosis modalities for CSF. Results: Current modalities in CSF rhinorrhea diagnosis and localization include laboratory tests (namely, B2T immunofixation), imaging (CT and/or MRI) with or without intrathecal administration, and surgical exploration. Each of these modalities carry flaws, risks, and benefits, ultimately contributing to delays in diagnosis and morbidity. Promising emerging technologies include lateral flow immunoassays (LFI) and biologically functionalized field-effect transistors (BioFET). Nevertheless, these carry some drawbacks of their own, and require further validation. Conclusion: CSF rhinorrhea remains a challenging diagnosis, requiring a multimodal approach to differentiate from nonpathologic causes of rhinorrhea. Current methods in diagnosis are imperfect, as the ideal test would be a readily accessible, inexpensive, rapid, highly accurate point-of-care test without the need for excess fluid or specialized processing. Critical work is being done to develop promising, new, improved tests, though a clear successor has not yet emerged. Level of Evidence: N/A.

Cerebrospinal Fluid-Venous Fistulas.

Cerebrospinal fluid-venous fistulas (CSFVFs) were first described in 2014 and have since become an increasingly diagnosed cause of spontaneous intracranial hypotension due to increased clinical recognition and advancements in diagnostic modalities. In this review, the authors discuss CSFVF epidemiology, the variety of clinical presentations, the authors' preferred diagnostic approach, recent advancements in diagnostic methods, treatment options, current challenges, and directions of future research.

Telovelar vs. Transvermian approach for the fourth ventricle tumors: A systematic review and meta-analysis.

BACKGROUND: Tumors in the fourth ventricle can be critical due to the small size of the fourth ventricle, which causes symptoms to be detected even in the presence of lesser mass effects. A proper surgical approach to the fourth ventricle poses challenges due to its deep location and proximity to vital compartments within the brainstem. The two commonly used approaches to these tumors are the transvermian and telovelar approaches. METHODS: A comprehensive systematic study was conducted based on a literature search of the databases. All case controls, cohorts, and case series including patients with fourth ventricle tumors, who were operated on with either telovelar or transvermian approaches were considered eligible. The evaluated outcomes were comparative postoperative complications of the telovelar vs. transvermian approach. After screening and data extraction, a meta-analysis was performed whenever adequate quantitative data were available. RESULTS: Seven studies with a total number of 848 patients, discussed both telovelar and transvermian approaches, with comparative reporting of outcomes in each group. Postoperative outcomes including cranial nerve deficit, mutism, diplopia, CSF leak, need for CSF diversion, and postoperative gait disturbance were not significantly different between telovelar and transvermian approaches. CONCLUSION: Postoperative complications were not significantly different between telovelar and transvermian approaches. Moreover, it could be proposed that such complications would be more likely to be a multifactorial matter concerning the patient's clinical condition, tumor characteristics, and surgeon's experience, rather than the surgical approach alone.

Full endoscopic resection of ventral thoracic osteophyte and repair of spontaneous CSF leak.

Spontaneous CSF leaks frequently cause headaches, meningismus, and nausea due to intracranial hypotension. When conservative treatment fails, surgical repair is indicated. Especially ventral leaks necessitate invasive approaches with substantial blood loss and tissue trauma. Full endoscopic spine surgery (FESS) enables circumferential access via the transforaminal approach. Here, the authors show the successful repair of a ventral CSF leak in the thoracic spine after removal of bony osteophytes utilizing FESS with placement of a dural substitute and sealant. Lasting symptom relief was reported. These results suggest that FESS is safe and efficient for the repair of spontaneous and incidental CSF leaks. The video can be found here: https://stream.cadmore.media/r10.3171/2024.1.FOCVID23209.

Unmasking the Masquerade: Lessons from a Case of Spontaneous Mid-Clival CSF Leak Mimicking an Ethmoidal Leak.

Spontaneous cerebrospinal fluid (CSF) leaks are an uncommon clinical entity, often challenging to diagnose accurately. We present a unique case of a patient with symptoms suggestive of an ethmoidal CSF leak, initially supported by radiological findings, but ultimately revealed to be a mid-clival CSF leak (from the posterior wall of the sphenoid sinus). This case underscores the complexities of diagnosing CSF leaks and highlights the importance of surgical exploration in cases where radiological evidence appears contradictory. The patient's Computed Tomography scan indicated a CSF leak in the cribriform plate, prompting a surgical approach to address this region. However, intraoperative findings surprisingly revealed no evidence of leak in the cribriform plate but instead a posterior wall of the sphenoid defect as the culprit. This report emphasizes the critical role of interdisciplinary collaboration, meticulous preoperative and intraoperative assessment, and adaptability in managing challenging cases of CSF leaks, ultimately leading to successful surgical repair and improved patient outcomes. It serves as a valuable reminder for clinicians to consider the possibility of a masquerading CSF leak when clinical and radiological findings do not align, thereby facilitating more precise diagnosis and targeted treatment.

Rebound Intracranial Hypertension.

PURPOSE OF REVIEW: Rebound intracranial hypertension (RIH) is a post-procedural treatment complication in patients with spontaneous intracranial hypotension (SIH) characterized by transient high-pressure headache symptoms. This article reviews the epidemiology, clinical features, risk factors, and treatment options for RIH. RECENT FINDINGS: This article discusses how changes in underlying venous pressure and craniospinal elastance can explain symptoms of RIH, idiopathic intracranial hypertension (IIH), and SIH. The pathophysiology of RIH provides a clue for how high and low intracranial pressure disorders, such as IIH and SIH, are connected on a shared spectrum.

American Rhinologic Society expert practice statement part 1: Skull base reconstruction following endoscopic skull base surgery.

The goal of this American Rhinologic Society expert practice statement (EPS) is to summarize the best available evidence for technical factors that optimize outcomes in skull base reconstruction following endoscopic skull base surgery for intradural pathologies. These topics include the use of free mucosal grafts versus vascularized pedicled nasoseptal flaps; the use of autologous versus synthetic grafts; and the roles of lumbar drains, dural sealants, and nasal packing. This EPS was developed following the recommended methodology and approval process as previously outlined. As there are a myriad of techniques and limited agreement on the accepted principles of skull base reconstruction, this EPS aims to summarize the existing evidence and provide clinically meaningful guidance on these divergent practices. Following a modified Delphi approach, five statements were developed, four of which reached consensus and one of which reached near consensus. These statements and the accompanying evidence are summarized along with an assessment of future needs.

Endoscopic Sellar Defect Reconstruction with Avascular Modified Gasket Seal Technique for Sellar Tumors.

Watertight repair of the skull base defect is necessary during endonasal skull base surgery to avoid postoperative CSF leak (poCSFl) and consequent intracranial complications. Various techniques have been described for reconstructing sphenoid-sellar defects with varying success rates. We have described the immediate and long-term outcomes following the reconstruction of sphenoid-sellar defects with our technique. A retrospective analysis of the patients following transsphenoidal sellar surgery underwent barrier restoring reconstruction by multi-layered (inlay-overlay) with autologous thigh fat, fascia lata, fibrin glue, knitted collagen, and absorbable gelatin sponge (modified gasket seal technique). A total of 44 patients were included in the study (n = 44). Reconstruction with modified gasket seal technique was done for all patients. 26 (59.1%) had intraoperative CSF leak (ioCSFl), and 9 (20.4%) patients had grade 3 Esposito-Kelly ioCSFl requiring adjunct short-term pressure reducing procedure (Lumbar drain) intraoperatively. 11/44 (25%) had poCSFl, 7/11 patients with poCSFl were managed conservatively, and 4/11 patients required rescue second surgery and ventriculoperitoneal shunting. 1 (2.3%) had severe meningitis and succumbed to it. Pneumocephalus was seen in 6 (13.6%). Multivariate analysis showed that revision surgery, GH-secreting tumors, and defects extending to the suprasellar region had higher chances of poCSFl (p < 0.001). All 43 alive patients had no CSF leak on long-term follow-up. The modified gasket seal technique is a viable technique for endoscopic sellar reconstruction for ioCSFl with an immediate success rate of 79.6% and 97.72% in the long term in preventing the postoperative CSF leak with a 13.6% rate of meningitis.

Successful Management of Multiple Giant Anterior Sacral Meningoceles Through an Anterior Approach in a Patient With Marfan Syndrome: A Case Report and Literature Review.

Meningoceles refer to the protrusion of meninges filled with cerebrospinal fluid (CSF) through a bone defect. There is scarce literature on the management of multiple giant anterior sacral meningoceles (ASMs). We report the case of a patient with Marfan syndrome presenting with gait disturbances and dizziness triggered by posture changes due to multiple giant ASMs. The patient was managed through an anterior approach involving a multidisciplinary team of surgeons. Care was taken to limit the persistence of CSF leak using an omental pedicled flap. This technique has only been mentioned twice in the literature for such cases. A literature review was conducted focusing on the evolution course and surgical strategy of meningoceles.

Sphenoid Sinus Mucosal Flap after Transsphenoidal Surgery-A Systematic Review.

Background and Objectives: Skull base reconstruction is a crucial step during transsphenoidal surgery. Sphenoid mucosa is a mucosal membrane located in the sphenoid sinus. Preservation and lateral shifting of sphenoid mucosa as sphenoid mucosal flap (SMF) during the transsphenoidal exposure of the sella may be important for later closure. This is the first systematic review to evaluate the utility of sphenoid mucosal flap for sellar reconstruction after transsphenoidal surgery. Materials and Methods: A systematic literature search was performed in January 2023: Cochrane, EMBASE, PubMed, Scopus, and Web of Science. The following keywords and their combinations were used: "sphenoid mucosa", "sphenoid sinus mucosa", "sphenoid mucosal flap", "sphenoid sinus mucosal flap". From a total number of 749 records, 10 articles involving 1671 patients were included in our systematic review. Results: Sphenoid sinus mucosa used to be applied for sellar reconstruction as either a vascularized pedicled flap or as a free flap. Three different types of mucosal flaps, an intersinus septal flap, a superiorly based flap and an inferiorly based flap, were described in the literature. Total SMF covering compared to partial or no SMF covering in sellar floor reconstruction resulted in fewer postoperative CSF leaks (p = 0.008) and a shorter duration of the postoperative lumbar drain (p = 0.003), if applied. Total or partial SMF resulted in fewer local complications (p = 0.012), such as fat graft necrosis, bone graft necrosis, sinusitis or fungal infection, in contrast to no SMF implementation. Conclusions: SMF seems to be an effective technique for skull base reconstruction after transsphenoidal surgery, as it can reduce the usage of avascular grafts such as fat along with the incidence of local complications, such as fat graft necrosis, bone graft necrosis, sinusitis and fungal infection, or it may improve the sinonasal quality of life by maintaining favorable wound healing through vascular flap and promote the normalization of the sphenoid sinus posterior wall. Further clinical studies evaluating sphenoid mucosal flap preservation and application in combination with other techniques, particularly for higher-grade CSF leaks, are required.

The Association Between Obesity and Spontaneous Temporal Bone CSF Leak Outcomes: A Systematic Review and Meta-Analysis.

OBJECTIVE: We undertook a systematic review of the literature with meta-analysis to identify the role of obesity (BMI ≥30) in the patient characteristics presenting with spontaneous cerebrospinal fluid (sCSF) leaks of the lateral skull base and the outcomes of their repair. DATA SOURCES: A Systematic Review of English Articles using MEDLINE, EMBASE, and Cochrane Library. REVIEW METHODS: The research algorithm included the following keywords: "spontaneous CSF leak," "lateral skull base," "temporal bone," "meningocele," "encephalocele," and "otorrhea." We also manually searched the references of included studies, to identify possible studies missed during our literature search. RESULTS: More than two-thirds of the patients were female (69.2%) and often were obese (mean BMI 36.5 kg/m2) with a mean age of 57. Most common presenting symptoms were otorrhea and hearing loss. Most authors did not report a routine use of a post-operative lumbar drain. Most patients had a single skull base defect and encephaloceles prolapsing through, across obese and non-obese groups. Median length of stay in hospital was 3.2 days, and the majority of patients did not have any recurrence during their follow-up (89.6%), which was not affected by obesity. CONCLUSION: Obesity does not affect length of hospital stay or recurrence rate following surgical repair of lateral skull base sCSF leaks. Surgical repair is a safe and viable approach in the management of obese patients with sCSF leaks in the temporal bone. LEVEL OF EVIDENCE: NA Laryngoscope, 134:2012-2018, 2024.

PEG hydrogel sealant versus fibrin glue in posterior fossa surgery: an economic comparison across five European countries.

Aim: Posterior cranial fossa (PCF) surgery is associated with complications, including cerebrospinal fluid (CSF) leakage. Dural sealants such as polyethylene glycol (PEG)-based hydrogels and fibrin glue can prevent CSF leaks, with evidence suggesting PEG hydrogels may outperform fibrin glue. However, the budget impact of using PEG hydrogels in PCF surgeries in Europe is unclear. Materials & methods: A decision tree was developed based on a previous US model, to assess the budget impact of switching from fibrin glue to PEG hydrogel in PCF surgery across five European countries. Input costs were derived from published sources for the financial year 2022/2023. Health outcomes, including CSF leaks, were considered. Results: The model predicted that using PEG hydrogel instead of fibrin glue in PCF surgery can lead to cost savings in five European countries. Cost savings per patient ranged from EUR 419 to EUR 1279, depending on the country. Sensitivity analysis showed that the incidence of CSF leaks and pseudomeningoceles had a substantial impact on the model's results. Conclusion: PEG hydrogels may be a cost-effective alternative to fibrin glue in PCF surgery. The model predicted that cost savings would be mainly driven by a reduction in the incidence of postoperative CSF leaks, resulting in reduced reliance on lumbar drains, reparative surgery and shortened hospital stays.

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.