Cerebrospinal Fluid Diversion Research Articles

Chronic Pediatric Headache as a Manifestation of Shunt Over-Drainage and Slit Ventricle Syndrome in Patients Harboring a Cerebrospinal Fluid Diversion System: A Narrative Literature Review

The main subject of the current review is a specific subtype of headache, which is related to shunt over-drainage and slit ventricle syndrome, in pediatric patients harboring an implanted shunt device for the management of hydrocephalus. This clinical entity, along with its impairment regarding the quality of life of the affected individuals, is generally underestimated. This is partly due to the absence of universally agreed-upon diagnostic criteria, as well as due to a misunderstanding of the interactions among the implicated pathophysiological mechanisms. A lot of attempts have been performed to propose an integrative model, aiming at the determination of all the offending mechanisms of the shunt over-drainage syndrome, as well as the determination of all the clinical characteristics and related symptomatology that accompany these secondary headaches. This subcategory of headache, named postural dependent headache, can be associated with nausea, vomiting, and/or radiological signs of slim ventricles and/or subdural collections. The ultimate goal of our review is to draw clinicians’ attention, especially that of those that are managing pediatric patients with permanent, long-standing, ventriculoperitoneal, or, less commonly, ventriculoatrial shunts. We attempted to elucidate all clinical and neurological characteristics that are inherently related to this type of headache, as well as to highlight the current management options. This specific subgroup of patients may eventually suffer from severe, intractable headaches, which may negatively impair their quality of daily living. In the absence of any other clinical condition that could be incriminated as the cause of the headache, shunt over-drainage should not be overlooked. On the contrary, it should be seriously taken into consideration, and its management should be added to the therapeutic armamentarium of such cases, which are difficult to be handled.

Endoscopic Transventricular Transaqueductal Excision of Fourth Ventricular Neurocysticercosis: 2-Dimensional Endoscopic Video: 2-Dimensional Operative Video.

Ventricular neurocysticercosis is a challenging disease that may cause obstructive hydrocephalus and require surgical intervention.1,2 Cysts of the lateral and third ventricle can be addressed by neuroendoscopy and removal of the cyst, when feasible. Surgical excision of cysts from the fourth ventricle can be achieved through endoscopic or microsurgical techniques, depending on the experience of the surgeon and characteristics of the cyst. Unresectable cysts causing hydrocephalus require cerebrospinal fluid diversion through a ventriculoperitoneal shunt.1-8 Medical therapy consisting of antiparasitic and anti-inflammatory agents should be limited to patients where surgery is a poor option or in cases, where there are residual cysts in the body. Medical therapy can cause an inflammatory response resulting in adherence of the cyst to the ventricular wall, which increases the surgical complication rate. Therefore, surgical cyst excision should always precede medical therapy.2 The endoscopic transventricular transaqueductal approach has been described in the literature as a safe route to resect nonadherent cysts when performed by an experienced neuroendoscopist.1-5 Although descriptions of the procedure exist in the literature, no video outlines its detailed operative steps and relevant anatomy. In this video, we present a case of a 42-year-old woman who presented with obstructive hydrocephalus caused by a third ventricular cestode that migrated into the fourth ventricle during her hospitalization. The patient underwent an endoscopic transventricular transaqueductal excision of a fourth ventricular cyst with complete neurological recovery and without requiring ventricular shunting. The patient consented to the procedure and to the publication of her video and images. Institutional Review Board review is not required for a single case report.

Standardized Criteria to Initiate External Ventricular Drain (EVD) Weaning in a Neurological Intensive Care Unit to Increase the Safety of EVD Discontinuation and Reduce the Need for a Shunt.

Introduction Patients with subarachnoid hemorrhages (SAH) with external ventricular drains (EVD) candevelop chronic hydrocephalus (HCP), requiring permanent cerebrospinal fluid (CSF) diversion via an external shunt. Two different strategies have been used to assess for dependence on EVD: 1) prompt closure, and 2) gradual weaning.Gradual weaning of EVDs is performed by increasing drainage resistance to outflow over days.However, when to start one strategy or the other is up to the physician. No uniform guidelines exist raising a question: Are standardized criteria necessary to initiate the EVD weaning process for SAH patients to increase the safety of EVD discontinuation and reduce the need for a shunt? This study shares criteria used to initiate EVD weaning that displayed increased safety of EVD discontinuation for patients with subarachnoid hemorrhage requiring EVD, particularly with regards to length of hospital stay (LOS), hospital-acquired infection rates, and ventriculoperitoneal shunt/endoscopic third ventriculostomy (VPS/ETV) placement. Methods One hundred and fifty-one SAHpatients from January 2016 to January 2019 were analyzed. 60 aneurysmal SAH (aSAH) and 18 non-aneurysmal nontraumatic SAH (naSAH) patients required EVD placement.A gradual EVD weaning protocol was initiated if patients met the following criteria: 1. The reason for EVD placement has resolved or is resolving, 2. The quantity of CSF output is <250mL over 24 hours, 3. Quality of CSF is nonbloody, 4. Intracranial Pressure (ICP) must be within normal limits, and 5. The patient must be neurologically stable. It was acceptable to initiate the weaning process when the patient had mild cerebral vasospasm, but not moderate to severe cerebral vasospasm. EVD weaning was performed by increasing the drain (chamber) height by 5 millimeters of mercury every 24 hours if the criteria were met. Charts were reviewed for LOS, infection rates, and rate of VPS/ETV. Gender, age, race, wean failure incidence, Hunt-Hess scores, modified Fisher scores, and syndrome of inappropriate antidiuretic hormone/cerebral salt wasting (SIADH/CSW) rates were obtained. Results The average LOS for aSAH patients with EVD was 20.35 days. The incidence of VPS/ETV was 11%. A chi-square analysis revealed that aSAH patients had higher rates of VPS/ETV placement (p<0.001) and EVD wean failures (p<0.001) than naSAH patients. aSAH patients had a lower incidence of VPS/ETV placement of 11% compared to 21% nationally. Conclusions Standardized criteria to initiate EVD weaning provided a reduction in VPS/ETV placement among aSAH patients compared to national averages and provided a uniform approach to EVD management. Comparable infection rates and LOS for SAH patients requiring EVDs compared to national averages were found.

Comparative Analysis of Surgical Site Infections in Pediatric Brain Tumor Patients: Hygiene Practices, Risk Factors, and Implications for Healthcare Costs and Mortality

Surgical site infections (SSIs) significantly impact pediatric central nervous system tumor outcomes. We present our data of SSIs and their influence on outcomes of pediatric brain tumor patients treated between January 2011 till December 2022. This study utilized retrospective data from patients' medical records. Chi-squared test was used for correlational analysis. Independent sample t-test was used for equality of means. Linear and logistic regression was done to review impact of independent variables on dependent variable. Survival analysis was done using Kaplan–Meier curves. Between 2011 and 2022, 336 pediatric patients (202 males, 134 females) were diagnosed with brain tumors. Majority patients (279; 83%) underwent surgery (91% elective). Commonest tumor site was cerebellum (84/279; 30%). Tumor resection status was gross total resection (29/279; 46%), subtotal resection (59/279; 21%), near total resection (48/279; 17%), and partial resection (20/279; 7%); while 32/279 patients (11%) had a biopsy only. Hydrocephalus was present in 166/279 patients (59%); while majority (160/166; 96%) underwent a cerebrospinal fluid (CSF) diversion procedure. SSI developed in 23/279 patients (8%), leading to delayed postoperative management in majority (15/23; 65%). SSIs were significantly associated with lower age of presentation (p = 0.01), less duration between symptoms and diagnosis (p = 0.00), performance of CSF diversion procedure (p = 0.04), increase in hospital stay (p = 0.00), delay in postoperative management (15/23; 65%) (p = 0.01), decline in treatment completion (p = 0.01), and poor survival (p = 0.01). Majority (171/279) of patients (61%) completed treatment. The overall survival of our cohort was 84.9% with a median follow-up time of 11 (interquartile range [IQR]: 36, 1) months. Survival was significantly lower (56.5%) in patients with SSI (p = 0.01). Median time to death was 18 months. Progression-free survival was 77.4% with a median progression-free time of 8 (IQR: 28, 1) months. Median time to progression was 9 (IQR: 24, 4.5) months. The incidence of SSIs in our cohort closely resembled that of high-income countries. Risk factors for SSIs included younger age, a shorter time between symptom onset and surgery, undertaking of CSF diversion procedures. Adverse effects of SSIs included increased costs, delays in postoperative management, incomplete treatment, and higher mortality rates. This study emphasizes the substantial impact of SSIs on healthcare resources and patient well-being.

The impact of a biocellulose-based repair of fetal open spina bifida on the need to untether the cord: is it time to unify techniques for prenatal repair?

To report the need for cord untethering after prenatal repair of open spina bifida using a unique biocellulose-based technique performed at a later gestational age. An observational cohort study was conducted to determine the incidence of tethered cord syndrome. Between May 2013 and May 2022, we performed 172 procedures using the percutaneous fetoscopic approach in fetuses at 26-28 weeks of gestation. After placode dissection, a biocellulose patch was placed to cover the placode, a myofascial flap (when possible) was dissected, and the skin was closed. Owing to death or loss to follow-up, 23 cases were excluded. Cord tethering syndrome was defined as symptoms of medullary stretching, and the infants were evaluated and operated on by local neurosurgeons after an magnetic resonance imaging examination. Infants over 30-month had ambulation and neurodevelopment evaluations (PEDI scale). Among 172 cases operated at a median gestational age of 26.7 weeks and delivered at 33.2 weeks, 149 cases were available for postnatal follow-up, and cord untethering was needed in 4.4% of cases (6/136; excluding 13 cases younger than 12 months). Cerebrospinal fluid diversion and bladder catheterization were needed in 38% and 36% of cases, respectively. Of the 78 cases evaluated at 30 months, 49% were ambulating independently, and 94% had normal social function. The biocellulose-based technique was associated with a low rate of cord tethering, wich may be attributed to the lack of the duramater suture during prenatal repair, the formation of a neoduramater and/or later gestational age of surgery.

Utility of intracranial pressure monitoring as a diagnostic tool in pediatric ventriculomegaly

ObjectiveIntracranial pressure (ICP) monitoring is commonly utilized for identifying pathologic ICP in cases of traumatic brain injury; however, its utility in hydrocephalic children has not been elucidated. Although patients with typical (pressure-active) hydrocephalus present with clear signs and/or symptoms and the need for cerebrospinal fluid (CSF) diversion is often clear, others may have arrested or pressure-compensated hydrocephalus with pathologic ICP elevation masked by ambiguous signs or are completely asymptomatic. Without treatment these pathologic ICP elevations may affect neurologic development or crescendo over time leading to neurological decline. The purpose of this study is to investigate the utility of ICP monitoring as a diagnostic tool in this relatively common patient population and identify ventriculomegaly patients with and without pathologic ICP, thus improving accuracy of identifying those with and without surgical needs. Methods36 patients (≤ 17 years old) underwent 41 inpatient ICP recording sessions between 2016 and 2022 and were retrospectively reviewed. This included patients with a history of severe, nonprogressive ventriculomegaly and normal fundoscopic examinations lacking traditional signs and symptoms concerning for elevated ICP. Nighttime pathological plateau waves were defined as sustained elevations of ICP ≥ 2x baseline for a duration of ≥ 5 minutes. ResultsThe mean age of patients was 5.5 years old (range 0–17 years old). 46.3% of patients had prior endoscopic third ventriculostomy (ETV), 14.6% had prior ventriculoperitoneal shunt (VPS), and 39% were without prior surgical intervention. Roughly half (51.2%) of patients had congenital ventriculomegaly while other patients had ventriculomegaly due to other pathologies such as germinal matrix hemorrhage/intraventricular hemorrhage (GMH/IVH) (29.3%), stroke (4.9%), cerebral infections/meningitis (2.4%), or unknown etiology (12.2%). The average procedure time was 19.1 ± 10.5 minutes, and mean length of stay was 2.8 ± 0.7 days. Pathologic ICP was demonstrated in 12 cases (29.3%), 4 (33.3%) of which were asymptomatic. Pathologic ICP was found in 7 of 19 (36.8%) in the prior ETV group, 1 of 6 (16.7%) in prior shunt group, and 4 of 16 (25%) in the non-surgical group (p = 0.649). Among those with pathologic ICP, 6 (50%) cases received an ETV, 5 (41.7%) cases underwent VPS placement, and 1 (8.3%) case underwent a VPS revision. There were no infectious complications or cases of hemorrhage. 4 patients required repositioning of the ICP monitor due to dislodgement. ConclusionInpatient ICP monitoring is a safe and effective diagnostic tool for evaluating the presence of pathologic ICP in severe, persistent non-progressive ventriculomegaly. The use of ICP monitoring may aid in identifying patients with pressure-compensated hydrocephalus who demonstrate pathologic ICP where surgical intervention may be warranted, while preventing unnecessary CSF diversion in those without pathology.

Increased Survival and Symptom Relief Following Palliative Cerebrospinal Fluid Diversion for Neoplastic Leptomeningeal Disease: A Case Series and Systematic Review

INTRODUCTION: Leptomeningeal disease (LMD) indicates widespread or advanced cancer and can lead to symptomatic hydrocephalus, for which palliative cerebrospinal fluid (CSF) diversion may be indicated. METHODS: We analyzed survival and symptom relief outcomes from a 10-year period at a single institution of patients with LMD secondary to cancer, symptoms of obstructive hydrocephalus, and documented date of death. We also searched PubMed, Embase, and Web of Science from database inception until 8/20/2022, using search terms including ‘cancer’, ‘hydrocephalus’, and ‘shunt’. Preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines were followed. RESULTS: In our series of 50 patients, 30 (60%) underwent CSF diversion surgery after LMD diagnosis with comparable median age in both surgery (58.4 ± 14.4 years) and non-surgery (57.8 ± 14.5 years) groups. Twenty-three patients (76.7%) achieved symptom relief. The surgery group lived longer after LMD diagnosis than patients in the non-surgery group (6.6 ± 6.0 vs. 1.3 ± 4.3 months, p &lt; 0.001) and had higher likelihood of survival (hazard ratio 2.49, 95% confidence interval 1.37-4.52, p = 0.002). Median survival after surgery was 2.8 ± 3.8 months. Out of 23 articles reporting outcomes for 2895 patients, 995 (34.3%) presented with LMD. Postoperative complication rates were 0-37.7% with no cases of extraneural metastases. Symptom relief rates were 50-100%. Three studies reported median survival after surgery for LMD (2-3.3 months). CONCLUSIONS: A rare entity with dismal prognosis, LMD can present as symptomatic hydrocephalus in patients with cancer. Symptom relief can be achieved with palliative surgery for permanent CSF diversion with relatively low complication rates. Prospective studies are needed to further assess outcomes and needs of this patient population.

104 Use of the eShunt Implant in Treatment of Aneurysmal Subarachnoid Hemorrhage Communicating Hydrocephalus

INTRODUCTION: Aneurysmal subarachnoid hemorrhage (aSAH) may cause communicating hydrocephalus and elevated intracranial pressure (ICP) not amenable to weaning of cerebrospinal fluid (CSF) external ventricular drainage (EVD), often requiring surgical ventriculoperitoneal shunting (VPS) with risk of hemorrhage and infection. METHODS: Patients having intractable elevated ICP with EVD clamping and favorable inferior petrosal sinus and bony anatomy were included. ICP was monitored before and after eShunt deployment for 36-48 hours. Primary endpoint was reached if ICP remained &lt;20 cmH2O enabling EVD removal. CT imaging of the brain was obtained immediately post-eShunt placement to evaluate eShunt placement and assess for any presence of new procedural hemorrhage. RESULTS: Seven out of eight patients (5 female; age 64 ± 12 years) underwent successful transfemoral transvenous eShunt procedure at 25.3 days (Range 14-38) post aSAH. Primary endpoint was achieved in all with EVD removal by 36-48 hours without procedural or delayed hemorrhage. Mean ICP rapidly decreased from 33.4 to 13 cmH2O at 1 hour (p &lt; 0.0001) and to 9 cmH2O (p &lt; 0.0001) at 36 hours post eShunt placement. CONCLUSIONS: These early encouraging results of the eShunt implant suggest clinical role and utility in the management of subarachnoid hemorrhage associated hydrocephalus possibly eschewing the need for VP shunt surgery and enabling minimally invasive CSF diversion in patients requiring dual anti-platelet or anticoagulant therapy.

Immunocompetent isolated cerebral mucormycosis presenting with obstructive hydrocephalus: illustrative case.

Isolated cerebral mucormycosis is rare in immunocompetent adults and is only sparsely reported to be associated with obstructive hydrocephalus. Here, the authors report a case of obstructive hydrocephalus secondary to central nervous system mucormycosis without other systems or rhino-orbital involvement and its technical surgical management. A 23-year-old, incarcerated, immunocompetent patient with history of intravenous (IV) drug use presented with syncope. Although clinical and radiographic findings failed to elucidate an infectious pathology, endoscopy revealed an obstructive mass lesion at the level of the third ventricle, which, on microbiological testing, was confirmed to be Rhizopus fungal ventriculitis. Perioperative cerebrospinal fluid diversion, endoscopic third ventriculostomy, endoscopic biopsy technique, patient outcomes, and the literature are reviewed here. The patient received intrathecal and IV amphotericin B followed by a course of oral antifungal treatment and currently remains in remission. The patient's unique presentation and diagnosis of isolated cerebral mucormycosis reveal this pathogen as a cause of ventriculitis and obstructive hydrocephalus in immunocompetent adult patients, even in the absence of infectious sequelae on neuroimaging.

Microcirculatory Impairment and Cerebral Injury in Hydrocephalus and the Effects of Cerebrospinal Fluid Diversion.

Hydrocephalus is characterized by progressive enlargement of cerebral ventricles, resulting in impaired microvasculature and cerebral hypoperfusion. This study aimed to demonstrate the microvascular changes in hydrocephalic rats and the effects of cerebrospinal fluid (CSF) release on cerebral blood flow (CBF). On postnatal day 21 (P21), male Wistar rats were intracisternally injected with either a kaolin suspension or saline. On P47, Evan's ratio (ER) was measured using MRI. On P49, the arteriolar diameter and vascular density of the pia were quantified using a capillary video microscope. The CBF was measured using laser Doppler flowmetry. The expressions of NeuN and glial fibrillary acidic protein determined by immunochemical staining were correlated with the ER. The CBF and rotarod test performance were recorded before and after CSF release. The expressions of 4-hydroxynonenal (4-HNE) and c-caspase-3 were studied on P56. Ventriculomegaly was induced to varying degrees, resulting in the stretching and abnormal narrowing of pial arterioles, which regressed with increasing ER. Quantitative analysis revealed significant decreases in the arteriolar diameter and vascular density in the hydrocephalic group compared with those in the control group. In addition, the CBF in the hydrocephalic group decreased to 30%-50% of that in the control group. In hydrocephalus, the neurons appear distorted, and the expression of 4-HNE and reactive astrogliosis increase in the cortex. After CSF was released, improvements in the CBF and rotarod test performance were inversely associated with the ER. In addition, the levels of 4-HNE and c-caspase-3 were further elevated. Rapid ventricular dilatation is associated with severe microvascular distortion, vascular regression, cortical hypoperfusion, and cellular changes that impair the recovery of CBF and motor function after CSF release. Moreover, CSF release may induce reperfusion injury. This pathophysiology should be taken into account when treating hydrocephalus.

Endoscopic third ventriculostomy in hydrocephalus patients with functioning ventriculoperitoneal shunts: challenging the dictum that shunts treat all types of hydrocephalus. Patient series.

Although ventriculoperitoneal shunting is standard for hydrocephalus, shunting may not be ideal for aqueductal stenosis. A cohort of patients with aqueductal stenosis displayed symptoms of over- and underdrainage, despite a patent ventriculoperitoneal shunt (VPS) and optimized valve settings. Endoscopic third ventriculostomies (ETVs) were performed in a subset of these patients with successful treatment of their underlying hydrocephalus, despite a functioning shunt. All patients who had undergone ETV with a history of ventriculoperitoneal shunting were retrospectively reviewed. Patients experiencing over- or underdrainage symptoms despite a patent shunt were included. Cerebral aqueduct anatomy and third ventricle bowing were reviewed on preoperative imaging. Seven patients met the study criteria. All showed cerebral aqueductal stenosis and third ventricle bowing. After ETV, all patients demonstrated decreased third ventricle bowing and clinical improvement without the need for secondary cerebrospinal fluid (CSF) diversion. Despite a functioning VPS, patients with aqueductal stenosis may not be adequately treated. The underlying reasons are not clearly understood but suggest abnormal CSF dynamics due to aberrant parenchymal compliance. The authors theorize that ETV can more effectively treat these patients. ETV can be considered a viable treatment option in aqueductal stenosis despite a patent VPS, challenging the traditional teaching that shunts ideally treat all types of hydrocephalus.

Cerebrospinal Fluid Diversion from the Cisterna Magna in Patients with Idiopathic Intracranial Hypertension and Slit Ventricles: Long-Term Effectiveness, Revision Rates, and Clinical Outcomes

ObjectiveIdiopathic intracranial hypertension (IIH) is a cerebrospinal fluid (CSF) disorder defect that is frequently treated with CSF shunts. Shunts utilizing the cisterna magna as a proximal reservoir have been described in literature, however, long-term outcomes are unknown. The present study aims to describe the long-term effectiveness, revision rates, and clinical outcomes of this shunt in 14 patients with IIH and slit ventricles. MethodsA single-center retrospective review of 14 IIH patients treated by cisterna magna shunts was performed. Shunt histories, including revision rate and time until first shunt failure for ventricular, lumbar, and cisterna magna shunts were recorded. “Revision rate” was calculated as the total number of shunt revisions over years of total shunt placement. The average follow-up time was 12.08 years. ResultsThe mean age at first cisterna magna shunt placement was 18.1 years (6.6 – 43.3 years) and all patients had radiological evidence of slit ventricles. Cisterna magna shunts improved or resolved clinical symptoms for all 14 patients and had a lower rate of revisions (0.42 revision/year) compared to ventricular (0.72 revision/year) and lumbar (1.30 revision/year) shunts. Of the 11 patients still requiring CSF diversion at the end of the study, eight had functioning shunts that utilized the cisterna magna. ConclusionsThe cisterna magna shunt may be a suitable option for patients with IIH and slit ventricles. Further study is needed to understand the clinical utility of this shunt for the population in which it is indicated.

Endoscopic Third Ventriculostomy versus Ventriculo-Peritoneal Shunt for Infant Hydrocephalus

Objective: To compare the outcome of ventriculo-peritoneal shunt (VP shunt) versus endoscopic third ventriculostomy (ETV) as surgical intervention in management of Infant hydrocephalus. Methodology: A prospective, randomized controlled trial was conducted at the department of Neurosurgery Nishtar University Hospital between June 2023 and November 2023. Study was conducted on 62 pediatric patients with hydrocephalus, all fewer than two years of age, who underwent cerebrospinal fluid (CSF) diversion through either endoscopic third ventriculostomy (ETV) or ventriculoperitoneal (VP) shunt placement. Patients were randomly assigned to one of two treatment groups: ETV or VP shunt. Randomization was done using a computer-generated sequence to ensure unbiased allocation. The ETV group underwent endoscopic surgery to create an opening in the third ventricle to allow CSF to bypass the obstruction. The VP shunt group received a surgically implanted device to divert CSF from the ventricles to the peritoneal cavity. Results: Infection was higher in Group VP than Group ETV as 5 (16.1%) and 1 (3.2%), respectively, (p=0.086). Similarly, obstruction was higher in Group VP than Group ETV as 11 (35.5%) and 9 (29.0%), respectively, (p=0.587). Whereas, surgery was revised 16 (51.6%) in Group VP and 13 (41.9%) in Group Erate TV, (p=0.445). Conclusion: This suggests that ETV presents as a favorable alternative to VP shunt placement due to its reduced risk of postoperative complications and quicker operative duration, while still maintaining comparable efficacy in terms of preventing obstruction, and the necessity for revision surgeries.

Undiagnosed sagittal synostosis as cause of "idiopathic" intracranial hypertension.

Idiopathic intracranial hypertension (IIH) is a rare condition in children, but if diagnosed needs to be promptly treated to avoid clinical sequalae. The main purpose of this paper was to test our clinical experience with a cohort of normocephalic children with craniosynostosis who do not present in the routine way to craniofacial services, due to the normal head shape and age, diagnosed with IIH. We retrospectively reviewed all children who were referred to neurosurgery from 2012 to 2022 for management of IIH on our prospectively kept database. We determined what treatments were offered and if there was an associated craniosynostosis. In total, 19 children were identified with an average age at referral of 11.5years (st dev 4.0years) with 11 male and 8 female. The most common presenting symptoms and signs were papilloedema (18/19), headaches (15/19), visual deterioration (9/19), nausea and vomiting (7/19) and diplopia (4/19). Five out of 19 children (26.3%) had a sagittal suture fused that was not identified at the time of treatment and all children were normocephalic. There is a cohort of children with IIH who will have concomitant craniosynostosis and ideally would benefit from cranial vault expansion as primary surgery rather than cerebrospinal fluid (CSF) diversion. We suggest all children with IIH requiring neurosurgical intervention have cross-sectional imaging to look for occult craniosynostosis prior to intervention.

Detection and Management of Elevated Intracranial Pressure in the Treatment of Acute Community-Acquired Bacterial Meningitis: A Systematic Review.

Acute bacterial meningitis (ABM) is associated with severe morbidity and mortality. The most prevalent pathogens in community-acquired ABM are Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. Other pathogens may affect specific patient groups, such as newborns, older patients, or immunocompromised patients. It is well established that ABM is associated with elevated intracranial pressure (ICP). However, the role of ICP monitoring and management in the treatment of ABM has been poorly described.An electronic search was performed in four electronic databases: PubMed, Web of Science, Embase, and the Cochrane Library. The search strategy chosen for this review used the following terms: Intracranial Pressure AND (management OR monitoring) AND bacterial meningitis. The search yielded a total of 403 studies, of which 18 were selected for inclusion. Eighteen studies were finally included in this review. Only one study was a randomized controlled trial. All studies employed invasive ICP monitoring techniques, whereas some also relied on assessment of ICP-based on clinical and/or radiological observations. The most commonly used invasive tools were external ventricular drains, which were used both to monitor and treat elevated ICP. Results from the included studies revealed a clear association between elevated ICP and mortality, and possibly improved outcomes when invasive ICP monitoring and management were used. Finally, the review highlights the absence of clear standardized protocols for the monitoring and management of ICP in patients with ABM. This review provides an insight into the role of invasive ICP monitoring and ICP-based management in the treatment of ABM. Despite weak evidence certainty, the present literature points toward enhanced patient outcomes in ABM with the use of treatment strategies aiming to normalize ICP using continuous invasive monitoring and cerebrospinal fluid diversion techniques. Continued research is needed to define when and how to employ these strategies to best improve outcomes in ABM.

Characterizing the association between CSF biomarkers and risk for ventriculoperitoneal shunt following posterior fossa tumor resection in a case-control study

BackgroundRecalcitrant hydrocephalus necessitating permanent cerebrospinal fluid (CSF) diversion is a known complication after resection of a posterior fossa tumor (PFT). Various CSF contents, such as protein and other markers, have been variably correlated with the need for permanent CSF diversion. This study aims to evaluate which CSF laboratory values are associated with permanent CSF diversion following PFT resection in adults. MethodsThis study queried our multi-institutional database (Central Nervous System Tumor Outcome Registry at Emory; CTORE) consisting of 617 adult patients with PFT resections from 2006 to 2021. Retrospective data was collected from the 89 patients of this cohort that required EVD placement. Patients were stratified into two groups: those that required a shunt following EVD removal (n = 30) and those that did not (n = 40). CSF variables collected included glucose, protein, nucleated cell count, and presence of infection. An unadjusted logistic regression was performed to assess exposures associated with shunt requirement and unadjusted odds ratios (ORs) and their associated 95 % confidence intervals (CIs) were obtained. ResultsImmediately following surgery, no CSF variables were significantly associated with shunt placement. Except for post-operative CSF being not-clear (OR: 4.15 (1.47–12.56), p = 0.009) and CSF glucose (OR: 0.97 (1.03–1.07), p = 0.031) all other variables were not significantly associated with shunt at time point 2. ConclusionIn our retrospective analysis, most routinely collected CSF values were not associated with permanent CSF diversion via a ventriculoperitoneal shunt following PFT resection. Further research is needed to identify other potential predictive markers.

Neurocognitive change over the course of a multiday external lumbar drain trial in patients with suspected normal pressure hydrocephalus

Objective: To characterize neurocognitive response to cerebrospinal fluid (CSF) diversion during a multiday external lumbar drainage (ELD) trial in patients with suspected normal pressure hydrocephalus (NPH). Methods: Inpatients (N = 70) undergoing an ELD trial as part of NPH evaluation participated. Cognition and balance were assessed using standardized measures before and after a three-day ELD trial. Cognitive change pre- to post-ELD trial was assessed in relation to change in balance, baseline neuroimaging findings, NPH symptoms, demographics, and other disease-relevant clinical parameters. Results: Multiday ELD resulted in significant cognitive improvement (particularly on measures of memory and language). This improvement was independent of demographics, test-retest interval, number of medical and psychiatric comorbidities, NPH symptom duration, estimated premorbid intelligence, baseline level of cognitive impairment, cerebrovascular disease burden, degree of ventriculomegaly, or other NPH-related morphological brain alterations. Balance scores evidenced a greater magnitude of improvement than cognitive scores and were weakly, but positively correlated with cognitive change scores. Conclusions: Findings suggest that cognitive improvement associated with a multiday ELD trial can be sufficiently captured with bedside neurocognitive testing. These findings support the utility of neuropsychological consultation, along with balance assessment, in informing clinical decision-making regarding responsiveness to temporary CSF diversion for patients undergoing elective NPH evaluation. Implications for the understanding of neuroanatomical and cognitive underpinnings of NPH are discussed.

Skull Base Repair following Resection of Vestibular Schwannoma: A Systematic Review (Part 2: The Translabyrinthine Approach)

Abstract Objectives Despite advances in skull base reconstruction techniques, cerebrospinal fluid (CSF) leaks remain a relatively common complication after translabyrinthine (TL) vestibular schwannoma (VS) surgery. We conducted a systematic review to synthesize the repair techniques and materials used in TL VS surgery to prevent CSF leaks. Design A systematic review of studies published since 2000 reporting techniques to prevent CSF leaks during adult TL VS surgery was conducted. A narrative synthesis of primary repair protocols was produced, and a taxonomy was established. Additionally, the advantages, disadvantages, and associated CSF leak rates of different repair protocols were extracted. Results All 43 studies were case series, and 39 were retrospective. Repair strategies included heterogeneous combinations of autografts, xenografts, and synthetic materials. A taxonomy was produced, classifying repairs into seven distinct stages, including approaches to the dura, middle ear cleft, air cells, TL bony defect, extra-cranial soft tissue, postoperative dressings, and CSF diversion. The median postoperative incidence of CSF leaks was 6% (interquartile range: 0–10%). Conclusions This systematic review reveals substantial inter-institutional heterogeneity in intraoperative strategies to prevent CSF leaks following TL VS surgery. However, comparing these techniques is challenging due to the multiple predictive factors for CSF leaks and their inconsistent reporting. We propose a taxonomy of seven stages to classify operative techniques and materials aimed at preventing CSF leaks. We recommend that future evaluations should adopt a prospective approach encompassing data collection strategies that considers all operative stages described by our taxonomy.

Skull Base Repair following Resection of Vestibular Schwannoma: A Systematic Review (Part 1: The Retrosigmoid Approach)

Abstract Objective Despite advances in skull-base reconstruction techniques, cerebrospinal fluid (CSF) leaks remain a common complication following retrosigmoid (RS) vestibular schwannoma (VS) surgery. We aimed to review and classify the available strategies used to prevent CSF leaks following RS VS surgery. Methods A systematic review, including studies of adults undergoing RS VS surgery since 2000, was conducted. Repair protocols were synthesized into a narrative summary, and a taxonomic classification of techniques and materials was produced. Additionally, the advantages, disadvantages, and associated CSF leak rates of different repair protocols were described. Results All 42 studies were case series, of which 34 were retrospective, and eight were prospective. Repair strategies included heterogeneous combinations of autografts, xenografts, and synthetic materials. A repair taxonomy was produced considering seven distinct stages to CSF leak prevention, including intraoperative approaches to the dura, internal auditory canal (IAC), air cells, RS bony defect, extracranial soft tissue, postoperative dressings, and CSF diversion. Notably, there was significant heterogeneity among institutions, particularly in the dural and IAC stages. The median postoperative incidence of CSF leaks was 6.3% (IQR: 1.3–8.44%). Conclusions The intraoperative strategies used to prevent CSF leaks during RS VS surgery vary between and within institutions. As a result of this heterogeneity and inconsistent reporting of CSF leak predictive factors, a meaningful comparative analysis of repair protocols was not feasible. Instead, we propose the development of a prospective multicenter observational evaluation designed to accurately capture a comprehensive dataset of potential CSF risk factors, including all stages of the operative repair protocol.

MRI analysis of neurodevelopmental anatomy in myelomeningocele: prenatal vs postnatal repair.

Prenatal myelomeningocele (MMC) repair offers improved motor function and decreased rates of cerebrospinal fluid (CSF) diversion compared to than postnatal repair. However, comparative analysis of other associated neuroanatomical findings is lacking. The purpose of this study is to use magnetic resonance imaging (MRI) imaging to compare characteristic Chiari II malformation stigmata in patients who underwent fetal MMC repair vs. postnatal repair. A retrospective review was performed of neonates who underwent prenatal or postnatal MMC repair at our institution and had postnatal MRIs. We analyzed anatomical findings characteristically seen with Chiari II malformation on brain MRI in patients who underwent prenatal MMC repair vs. postnatal repair. CSF diversion was required in 24% of prenatal cohort vs. 67% of postnatal cohort (p=0.002), and syrinx was present in 12% of prenatal cohort compared to 42% in postnatal cohort (p=0.03). Corpus callosum (CC) morphology was abnormal in 52% of prenatal cohort vs. 53% of postnatal cohort (p=0.92), while falx morphology was normal in 92% of the prenatal cohort vs. 34% of the postnatal cohort (p = <0.001). Prenatal cohort patients had shorter tentorium to foramen magnum distance than postnatal cohort patients (18.4mm vs. 22.4mm, p=0.01), overall larger foramen magnum diameter (22.9mm vs. 18.9mm, p < 0.001), and a smaller mean degree of hindbrain herniation (1.5mm vs. 8.7mm, p < 0.001). Finally, the cerebral aqueduct was patent in 79% of prenatal cohort vs. 100% of postnatal cohort (p=0.007). There was no significant difference in presence of gray matter heterotopia, presence of septum pellucidum, or size of massa intermedia between the two cohorts. We report baseline variations in developmental neuroanatomy in patients with MMC including rates of CC dysgenesis, gray matter heterotopia and additional cranial and spinal MRI findings. We found that prenatal surgery results in changes to infratentorial anatomy, with minimal effect on supratentorial brain development. This information will be useful in myelomeningocele counseling and in understanding how prenatal repair of myelomeningocele affects brain development. This article is protected by copyright. All rights reserved.