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Background: Ventriculoperitoneal (VP) shunting is widely accepted as the gold-standard treatment for idiopathic normal pressure hydrocephalus (iNPH). However, a restricted group of patients experience only minimal or no improvement after the operation. In such cases, the question whether the diagnosis was incorrect or the shunt is malfunctioning remains unanswered., Methods: We retrospectively collected data on a 10-year series of VP-shunted patients with iNPH showing transient or minimal improvement of symptoms within 3 weeks from surgery. A full workup (including noninvasive diagnostic, cognitive, and invasive tests) was performed. After ruling out mechanical malfunction, we performed a tap test followed by a Katzman test 2 weeks later. The confirmed persistence of disturbance of cerebrospinal fluid dynamics was treated by shunt revision and, if found working, by its replacement into the atrial cavity., Results: Twenty patients were diagnosed with shunt insufficiency. At surgery, the distal end of the shunt was easily extruded and found working in all cases. It was then repositioned into the right atrium (the first 8 patients of the series also underwent failed contralateral abdominal replacement). Early postoperative clinical improvement was always confirmed. In 1 case, shunt overdrainage was corrected by valve upregulation., Conclusions: According to our experience, inadequate distal end placement of a shunt might be one of the reasons needing investigation in patients with iNPH failing improvement after surgery. In such situations, the conversion to a ventriculoatrial shunt proved to be a low-cost and successful treatment option., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Idiopathic normal pressure hydrocephalus (iNPH) is a condition characterized by gait disturbance, dementia and/or urinary incontinence and enlarged ventricular system due to disturbance of cerebrospinal fluid (CSF). This study aims to describe the long-term experience with patients with iNPH submitted to ventriculoperitoneal shunt (VPS) with the programmable valve STRATA® (Medtronic). We prospectively selected a cohort of patients with a diagnosis of iNPH from January 2010 to April 2013 in a Brazilian tertiary hospital. All patients underwent clinical evaluation, which consists of the Mini-Mental State Examination and Time Up and Go tests and the application of Japanese Scale for Idiopathic Normal Pressure Hydrocephalus in three stages: prior to the TT, 3 h after the TT and 72 h after the TT. Fifty patients were submitted to VPS and followed. There were 32 men and 18 women. Mean age was 77.1 with standard deviation of 10.9. Follow-up time ranged from 96 to 120 months, with mean of 106 months. After 1 year of follow-up, 42 (83%) patients presented with clinical improvement, decreasing to 62% of patients at mid-term follow-up and 38% of patients at late follow-up. Complications occurred in 18% of subjects, needing reoperation in 16%. Our results show relevant clinical impact of shunting in iNPH patients, decreasing over time. Complications should not be underestimated, reaching up to 18% and demanding reoperation in 16% of cases. Thus, although much has been improved with current shunt technology, it is still important to consider the drawbacks of treatment., (© 2020. Belgian Neurological Society.)

Background/objectives: Prenatal myelomeningocele (MMC) repair has been shown to improve neurological outcomes. It has been suggested that decreases in the hysterotomy diameter during surgery can improve perinatal outcomes without altering neurologic outcomes. The objective of this study is to describe and compare the main maternal and fetal outcomes of fetuses undergoing open surgery for MMC repair, through the different modifications (standard-classical, mini-hysterotomy, and microneurosurgery)., Data Source: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Ovid, SciELO, LILACS, PROSPERO., Results: From a total of 112 studies, seven case series were selected including 399 fetuses with open fetal surgery, five studies using the classical technique (n = 181), one with mini-hysterotomy (n = 176), and one with the microneurosurgery technique (n = 42). The mini-hysterotomy and microneurosurgery techniques presented a lower risk of preterm delivery (21.4% and 30%, respectively) compared to the classic technique (47.3%), premature rupture of membranes (78%, 62%, and 72.5 %, respectively), oligohydramnios (0% and 72.5%, respectively), dehiscence of hysterotomy, maintaining the same frequency of Chiari reversion (78%, 62%, and 72.5%, respectively), postnatal correction requirement (0%, 4.8%, and 5.8%, respectively), and lower frequency of requirement for a ventriculoperitoneal shunt placement (13.0%, 7.5%, and 29.1%, respectively)., Conclusion: The least invasive techniques (minihysterotomy-microneurosurgery) are possible and reproduceable, as they are associated with better maternal and perinatal outcomes., (© 2021 John Wiley & Sons Ltd.)

Objective: For idiopathic normal pressure hydrocephalus (iNPH), risk stratifying patients and identifying those who are likely to fare well after ventriculoperitoneal shunt (VP) surgery may help improve quality of care and reduce unplanned readmissions. The aim of this study was to investigate the drivers of 30- and 90-day readmissions after VP shunt surgery for iNPH in elderly patients., Methods: The Nationwide Readmission Database, years 2013 to 2015, was queried. Elderly patients (≥65 years old) undergoing VP shunt surgery were identified using the International Classification of Diseases, Ninth Revision, Clinical Modification coding system. Unique patient linkage numbers were used to follow patients and identify 30- and 31- to 90-day readmission rates. Patients were grouped by no readmission (Non-R), readmission within 30 days (30-R), and readmission within 31 to 90 days (90-R)., Results: We identified 7199 elderly patients undergoing VP shunt surgery for iNPH. A total of 1413 (19.6%) patients were readmitted (30-R: n = 812 [11.3%] vs. 90-R: n = 601 [8.3%] vs. Non-R: n = 5786). The most prevalent 30- and 90-day complications seen among the readmitted cohort were mechanical complication of nervous system device implant (30-R: 16.1%, 90-R: 12.4%), extracranial postoperative infection (30-R: 10.4%, 90-R: 7.0%), and subdural hemorrhage (30-R: 6.0%, 90-R: 16.4%). On multivariate regression analysis, age, diabetes, and renal failure were independently associated with 30-day readmission; female sex, and 26th to 50th household income percentile were independently associated with reduced likelihood of 90-day readmission. Having any complication during the index admission independently associated with both 30- and 90-day readmission., Conclusions: In this study, we identify the most common drivers for readmission for elderly patients with iNPH undergoing VP shunt surgery., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Implantation of ventricular catheters (VCs) to drain cerebrospinal fluid (CSF) is a standard approach to treat hydrocephalus. VCs fail frequently due to tissue obstructing the lumen via the drainage holes. Mechanisms driving obstruction are poorly understood. This study aimed to characterize the histological features of VC obstructions and identify links to clinical factors., Methods: 343 VCs with relevant clinical data were collected from five centers. Each hole on the VCs was classified by degree of tissue obstruction after macroscopic analysis. A subgroup of 54 samples was analyzed using immunofluorescent labelling, histology and immunohistochemistry., Results: 61.5% of the 343 VCs analyzed had tissue aggregates occluding at least one hole (n = 211) however the vast majority of the holes (70%) showed no tissue aggregates. Mean age at which patients with occluded VCs had their first surgeries (3.25 yrs) was lower than in patients with non-occluded VCs (5.29 yrs, p < 0.02). Mean length of time of implantation of occluded VCs, 33.22 months was greater than for non-occluded VCs, 23.8 months (p = 0.02). Patients with myelomeningocele had a greater probability of having an occluded VC (p = 0.0426). VCs with occlusions had greater numbers of macrophages and astrocytes in comparison to non-occluded VCs (p < 0.01). Microglia comprised only 2-6% of the VC-obstructing tissue aggregates. Histologic analysis showed choroid plexus occlusion in 24%, vascularized glial tissue occlusion in 24%, prevalent lymphocytic inflammation in 29%, and foreign body giant cell reactions in 5% and no ependyma., Conclusion: Our data show that age of the first surgery and length of time a VC is implanted are factors that influence the degree of VC obstruction. The tissue aggregates obstructing VCs are composed predominantly of astrocytes and macrophages; microglia have a relatively small presence., (© 2021. The Author(s).)

Background: Idiopathic normal pressure hydrocephalus (iNPH) is a reversible CNS disease characterized by disturbed cerebrospinal fluid (CSF) dynamics. Changes in the extracellular matrix (ECM) composition might be involved in the pathophysiology of iNPH. The aim of this study was to explore possible differences between lumbar and ventricular CSF concentrations of the ECM markers brevican and neurocan, matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinase-1 (TIMP-1) and their relation to clinical symptoms in iNPH patients before and after shunt surgery., Methods: Paired lumbar and ventricular CSF was collected from 31 iNPH patients, before and four months after shunt surgery. CSF was analysed for concentrations of tryptic peptides originating from brevican and neurocan using a mass spectrometry-based panel, and for MMP-1, -2, -9, -10 and TIMP-1 using fluorescent or electrochemiluminescent immunoassays., Results: Brevican and neurocan peptide levels were not influenced by CSF origin, but MMP-1, -2, -10 and TIMP-1 were increased (p ≤ 0.0005), and MMP-9 decreased (p ≤ 0.0003) in lumbar CSF compared with ventricular CSF. There was a general trend of ECM proteins to increase following shunt surgery. Ventricular TIMP-1 was inversely correlated with overall symptoms (rho = - 0.62, p < 0.0001). CSF concentrations of the majority of brevican and neurocan peptides were increased in iNPH patients with a history of cardiovascular disease (p ≤ 0.001, AUC = 0.84-0.94) compared with those without., Conclusion: Levels of the CNS-specific proteins brevican and neurocan did not differ between the lumbar and ventricular CSF, whereas the increase of several CNS-unspecific MMPs and TIMP-1 in lumbar CSF suggests contribution from peripheral tissues. The increase of ECM proteins in CSF following shunt surgery could indicate disturbed ECM dynamics in iNPH that are restored by restitution of CSF dynamics.

Hydrocephalus (HC) can be associated with vestibular schwannoma (VS) at presentation. Although spontaneous resolution of HC after VS removal is reported, first-line treatment is varied including preoperative ventriculoperitoneal (VP) shunt, external ventricular drainage (EVD), or lumbar drainage (LD). We performed a systematic review to clarify optimal management of HC associated with VS at presentation, as well as characteristics of patients with initial and persistent HC after VS removal, and prevalence of HC associated with VS. Fourteen studies were included. Patients were grouped according to the timing of HC treatment. The overall rate of VP shunts was 19.4%. Among patients who received VS removal as first-line treatment, 6.9% underwent permanent shunts. In a subgroup of 132 patients (studies with no-aggregate data), t test analysis for mean tumor size (P = 0.02) and mean CSF protein level (P < 0.001) demonstrated statistically significant differences between patients with resolved HC (3.48 cm and 201 mg/dL) and patients with persistent HC (2.46 cm and 76.8 mg/dL) after VS resection. Transient treatment of HC using EVD or LD further resolved the HC in 87.5% and 82.9% of patients, respectively, before and after VS removal. The overall prevalence of HC associated with VS in a population of 2336 patients was 9.3%. Schwannoma removal as first-line treatment is justified by its low rate of persistent HC requiring VP shunt (roughly 7%). Patients with smaller VS and lower CSF proteins present higher risk of persistent HC after schwannoma removal. Temporary treatment of HC contributes to its resolution, both before and after VS removal.

Abdominal pseudocysts are common complications of ventriculoperitoneal shunt (VPS). We report the case of a 37-year-old woman treated by VPS for congenital obstructive hydrocephalus, who presented shunt dysfunction related to a voluminous abdominal cyst initially diagnosed as cerebrospinal fluid pseudocyst. The cyst was drained and the VPS was removed after endoscopic third ventriculocisternostomy (ETV). A few months later, a large new abdominal cyst appeared and was operated on. Diagnosis was rectified as massive ovarian mucinous cystadenoma. In any intra-abdominal cyst, differential diagnoses need considering to avoid mis-diagnosis in shunted patients, especially if the cyst is very large. The etiology of the hydrocephalus should also be investigated in case of shunt dysfunction. Even in case of longstanding shunt, ETV can be an alternative to shunt revision surgery in obstructive hydrocephalus, enabling VPS withdrawal and treatment of the hydrocephalus., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background: Ventriculoperitoneal shunts (VPS) are placed for a variety of etiologies. It is common for general surgery to assist with insertion of the distal portion in the peritoneum., Objective: To determine if there is a difference in revision rates in patients undergoing VPS placement with general surgery as well as those undergoing laparoscopic insertion., Methods: A retrospective review of all consecutive patients undergoing VPS placements was performed in a three-year period (2017-2019). Those that underwent placement with general surgery were compared to those without general surgery. Additionally, patients undergoing distal placement via mini-laparotomy versus laparoscopy were compared. Multivariable logistic regression was used to examine risk factors for distal VPS failure., Results: 331 patients were included. 202 (61.0 %) underwent VPS placement with general surgery. 121 (36.6 %) patients underwent insertion via laparoscopic technique. General surgery involvement reduced operative times, decreased length of stay, and lowered overall revision rates with distal revision rates being most significant (1.5 % vs 8.5 %; p = 0.0034). Patients undergoing VPS placement via laparoscopic technique had decreased operative time, length of stay, in-hospital complications and revision rates, with significant decrease in shunt infection (1.7 % vs 7.1 %; p = 0.0366). A history of prior shunt or abdominal surgery (OR 3.826; p = 0.0282) and lack of general surgery involvement (OR 20.98; p = 0.0314) are independent risk factors for distal shunt revision in our cohort., Conclusion: The use of general surgeons in VPS insertion can be of benefit by decreasing operative time, length of stay, total revisions, and distal revision rates. Further prospective studies are warranted to determine true benefit., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Many patients undergoing decompressive craniectomy will develop persistent hydrocephalus before cranioplasty. Therefore, surgeons must decide whether to perform ventriculoperitoneal shunt (VPS) placement and cranioplasty simultaneously or in staged procedures. With limited, conflicting data reported, this decision has often been made by personal preference. The objective of the present study was to compare the surgical outcomes between patients undergoing concurrent or staged VPS placement and cranioplasty., Methods: We performed a 10-year retrospective comparative analysis of patients who had undergone either simultaneous or staged VPS placement and cranioplasty at a tertiary academic medical center., Results: Of the 40 patients, 18 had undergone concurrent procedures and 22 had undergone VPS placement before a separate cranioplasty procedure. The concurrent group was significantly older, had more often had the VPS placed in the external ventricular drain site, and had had more patients taking aspirin at surgery. The rates of infection, resorption, and reoperation did not differ significantly, although reoperation showed a trend toward occurring less frequently in the concurrent group. Hospital-acquired infection occurred significantly less frequently in the concurrent patients. The rate of VPS-associated outcomes did not differ significantly between the 2 groups., Conclusions: Because of the trend toward a reduced reoperation rate, the significantly reduced rate of hospital-acquired infection, and the reduction in the number of surgeries, we recommend that patients awaiting cranioplasty in the setting of persistent hydrocephalus undergo concurrent VPS placement and cranioplasty rather than staged procedures., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Idiopathic normal pressure hydrocephalus (iNPH) is the only form of dementia that can be cured by surgery. Its diagnosis relies on clinical and radiological criteria. Identifying patients who can benefit from surgery is challenging, as other neurological diseases can be concomitant or mimic iNPH. We performed a systematic review on the role of positron emission tomography (PET) in iNPH. We retrieved 35 papers evaluating four main functional aspects with different PET radiotracers: (1) PET with amyloid tracers, revealing Alzheimer's disease (AD) pathology in 20-57% of suspected iNPH patients, could be useful in predictions of surgical outcome. (2) PET with radiolabeled water as perfusion tracer showed a global decreased cerebral blood flow (CBF) and regional reduction of CBF in basal ganglia in iNPH; preoperative perfusion parameters could predict surgical outcome. (3) PET with 2-Deoxy-2-[ 18 F]fluoroglucose ([ 18 F]FDG ) showed a global reduction of glucose metabolism without a specific cortical pattern and a hypometabolism in basal ganglia; [ 18 F]FDG PET may identify a coexisting neurodegenerative disease, helping in patient selection for surgery; postsurgery increase in glucose metabolism was associated with clinical improvement. (4) Dopaminergic PET imaging showed a postsynaptic D2 receptor reduction and striatal upregulation of D2 receptor after treatment, associated with clinical improvement. Overall, PET imaging could be a useful tool in iNPH diagnoses and treatment response.

Background: Incontrovertible predictors of shunt malfunction remain elusive., Objective: To determine predictors of shunt failure within 30 d of index surgery., Methods: This was a single-center retrospective cohort study from January 2010 through November 2016. Using a ventricular shunt surgery research database, clinical and procedural variables were procured. An "index surgery" was defined as implantation of a new shunt or revision or augmentation of an existing shunt system. The primary outcome was shunt failure of any kind within the first 30 days of index surgery. Bivariate models were created, followed by a final multivariable logistic regression model using a backward-forward selection procedure., Results: Our dataset contained 655 unique patients with a total of 1206 operations. The median age for the cohort at the time of first shunt surgery was 4.6 yr (range, 0-28; first and third quartile, .37 and 11.8, respectively). The 30-day failure rates were 12.4% when analyzing the first-index operation only (81/655), and 15.7% when analyzing all-index operations (189/1206). Small or slit ventricles at the time of index surgery and prior ventricular shunt operations were found to be significant covariates in both the "first-index" (P < .01 and P = .05, respectively) and "all-index" (P = .02 and P < .01, respectively) multivariable models. Intraventricular hemorrhage at the time of index surgery was an additional predictor in the all-index model (P = .01)., Conclusion: This study demonstrates that only 3 variables are predictive of 30-day shunt failure when following established variable selection procedures, 2 of which are potentially under direct control of the surgeon., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Although ventriculoperitoneal shunts (VPS) remain the first-line option in most instances of pediatric hydrocephalus, the long-term efficacy of ventriculoatrial shunts (VAS) remains unknown., Objective: To characterize the long-term outcomes and adverse occurrences associated with both VPS and VAS at our institution., Methods: The authors retrospectively analyzed all cerebrospinal fluid (CSF) shunting procedures performed over a 13-yr period at a single institution. A total of 544 pediatric shunt patients were followed for at least 90 d (VPS: 5.9 yr; VAS: 5.3 yr)., Results: A total of 54% of VPS and 60% of VAS required at least 1 revision. VPS demonstrated superior survival overall; however, if electively scheduled VAS lengthening procedures are not considered true "failures," no statistical difference is noted in overall survival (P = .08). VPS demonstrated significantly greater survival in patients less than 7 yr of age (P = .001), but showed no difference in older children (P = .4). VAS had a significantly lower rate of infection (P < .05) and proximal failure (P < .001)., Conclusion: VAS can be a useful alternative to VPS when the abdomen is unsuitable, particularly in older children. Although VPS demonstrates superior overall survival, it should be understood that elective VAS lengthening procedures are often necessary, especially in younger patients. If elective lengthening procedures are not considered true failures, then the devices show similar survival., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Research on age-related complications secondary to shunts in normal pressure hydrocephalus (NPH) is primarily limited to single-center studies and small cohorts., Objective: To determine the rates of hospital readmission and surgical complications, and factors that predict them, following shunt surgery for NPH in a large healthcare network., Methods: Surgical procedures, complications, and readmissions for adults undergoing ventricular shunting for NPH were determined using de-identified claims from a privately insured United States healthcare network in years 2007-2014. Univariate and multivariate statistics were used to determine factors that predict poor surgical outcomes. The primary outcome variable was surgical complications or readmissions (composite variable for any major perioperative complication or 30-d readmission)., Results: The 30-d readmission rate for 974 patients with NPH who underwent ventricular shunting was 7.29%; the most common reasons for readmission were shunt-related complications, infection, hemorrhage, altered mental status, and cardiopulmonary and musculoskeletal problems. The perioperative complication rate was 21.15%, including intraparenchymal hemorrhage (5.85%) and extra-axial (subdural or epidural) hematoma (5.54%). The overall rate of having a surgical complication or 30-d readmission was 25.15%. Age did not predict surgical complication or 30-d readmission. Preoperative comorbidities independently associated with poor outcome were myocardial infarction within 1 yr (OR = 3.984, 95% CI = 1.105-14.368); existing cerebrovascular disease (odds ratio [OR] = 2.206, 95% CI = 1.544-3.152); and moderate/severe renal disease (OR = 2.000, 95% CI = 1.155-3.464)., Conclusion: The rate of complications or readmission within 30 d of ventricular shunting for NPH is 25.15%. Preoperative comorbidities of myocardial infarction within 1 yr, cerebrovascular disease, and moderate/severe renal disease are independent risk factors for poor outcome., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Hydrocephalus with the need for shunt placement is a common sequela after aneurysmal subarachnoid hemorrhage (aSAH). In 2009 Chan et al. published a formula to predict shunt dependency in SAH patients, the failure risk index (FRI). We reevaluated the FRI within the aSAH population in our hospital and wanted to identify easier measurements forecast shunt dependency. We retrospectively analyzed data from patients with aSAH treated in our neuro-intensive care unit and calculated the FRI according to the paper by Chan et al. 2013 and data were compared to the results of Chan et al. 38 patients were included in this study, 24 female and 14 male. 38% suffered a SAH WFNS I, 19% WFNS II, 24% WFNS III, 5% WFNS IV and 14% WFNS V. 17 patients underwent a shunt implantation (group 1), 21 patients did not (group 2). The calculated FRI Index did not correlate with the expectancy of shunt implantation in 22% of the cases (group 1). In group 2 the FRI index and the prediction of shunt dependency did not match in 33% of the cases. Furthermore, we found the increase of the third ventricle diameter to be predictive in 67% for failed EVD challenge and the decrease of the third ventricle diameter predictive in 67% for successful EVD challenge. In this study, we were not able to confirm the results of the FRI designed by Chan et al within our patient population. Furthermore, we consider the increase of the third ventricle diameter to be a simpler and more reliable predictor of shunt dependency., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Idiopathic intracranial hypertension results in increased intracranial pressure leading to headache and visual loss. This disease frequently requires surgical intervention through lumboperitoneal (LP) or ventriculoperitoneal (VP) shunting., Objective: To compare postoperative outcomes between LP and VP shunts, including failure and complication rates., Methods: A retrospective analysis was conducted using a national administrative database (MarketScan) to identify idiopathic intracranial hypertension (IIH) patients who underwent LP or VP shunting from 2007 to 2014. Multivariate logistic and Cox regressions were performed to compare rates of shunt failure and time to shunt failure between LP and VP shunts while controlling for demographics and comorbidities., Results: The analytic cohort included 1082 IIH patients, 347 of whom underwent LP shunt placement at index hospitalization and 735 of whom underwent VP shunt placement. Rates of shunt failure were similar among patients with LP and VP shunt (34.6% vs 31.7%; P = .382). Among patients who experienced shunt failure, the mean number of shunt failures was 2.1 ± 1.6 and was similar between LP and VP cohorts. Ninety-day readmission rates, complication rates, and costs did not differ significantly between LP and VP shunts. Patients who experienced more than two shunt failures tended to have an earlier time to first shunt failure (hazard ratio 1.41; 95% confidence interval 1.08-1.85; P = .013)., Conclusion: These findings suggest that LP and VP shunts may have comparable rates of shunt failure and complication. Regardless of shunt type, earlier time to first shunt failure may be associated with multiple shunt failures., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Aim: We aimed to study the rate and pattern of ventriculoperitoneal shunt (VPS) infections at the Jordan University Hospital (JUH) over 10 years and investigate the yield of ventricular catheter tip culture in the evaluation of shunt infection., Patients and Methods: All patients operated on at our institution for ventriculoperitoneal shunt (VPS) insertion, reinsertion, and revision between 2009 and 2018 were included. Data (age, gender, pathology, and history of shunt infection) were collected from patients' files. Rates of infection and results of culture (cerebrospinal fluid and ventricular catheter tip) were obtained from laboratory records., Results: According to our hospital database, 310 patients were eligible for this study. The rate of infection was 11.1% per ventriculoperitoneal procedure, and the most common -organism was coagulase-negative Staphylococcus (CoNS) which was the cause of infection in 51.4% of the cases. Twenty-one percent of proved shunt infection cases showed a positive ventricular catheter tip culture., Conclusion: The rate and pattern of shunt infections at our institution are comparable with the international figures and data. Methods of catheter tip culturing should be improved to increase the yield of this practice., (© 2020 S. Karger AG, Basel.)

Objectives: Miyazaki syndrome is a cervical myelopathy or radiculopathy caused by cervical epidural venous congestion, due to cerebrospinal fluid over-drainage by an implanted ventricular shunt. The complex pathophysiology includes CSF pressure-changes consistent with the Monro-Kellie doctrine and a non-functional Starling resistor, leading to spinal epidural venous plexus enlargement and dilation. This venous congestion may be significant enough to exert compression on the spinal cord or nerve roots. The typical clinical and imaging findings together with a history of ventricular CSF shunting may establish the diagnosis, proven by a successful treatment. The aim of treatment is the abrogation of CSF over-drainage. The eligible interventions may be the followings: the increase of the opening-pressure of the valve system by the insertion of a new programmable valve if necessary, closing or removing the shunt., Aim: We want to call attention to this rare iatrogenic condition with potentially severe consequences., Patients and Methods: We perform a systematic literature-review and present our five cases., Results: Once recognized in time, Miyazaki syndrome can be well taken care of., Conclusions: Patients with chronic ventricular shunt need monitoring for CSF over-drainage to recognise potential complications such as cervical myelopathy or radiculopathy., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: There are currently no published data directly comparing postoperative seizure incidence following endoscopic third ventriculostomy (ETV), with/without choroid plexus cauterization (CPC), to that for ventriculoperitoneal shunt (VPS) placement., Objective: To compare postoperative epilepsy incidence for ETV/CPC and VPS in Ugandan infants treated for postinfectious hydrocephalus (PIH)., Methods: We performed an exploratory post hoc analysis of a randomized trial comparing VPS and ETV/CPC in 100 infants (<6 mo old) presenting with PIH. Minimum follow-up was 2 yr. Variables associated with and the incidence of postoperative epilepsy were compared (intention-to-treat) using a bivariate analysis. Time to first seizure was compared using the Kaplan-Meier method, and the relative risk for the 2 treatments was determined using Mantel-Haenszel hazard ratios., Results: Seizure incidence was not related to age (P = .075), weight (P = .768), sex (P = .151), head circumference (P = .281), time from illness to hydrocephalus onset (P = .973), or hydrocephalus onset to treatment (P = .074). Irritability (P = .027) and vision deficit (P = .04) were preoperative symptoms associated with postoperative seizures. Ten (10%) patients died, and 20 (20%) developed seizures over the follow-up period. Overall seizure incidence was 9.4 per 100 person-years (9.4 and 9.5 for ETV/CPC and VPS, respectively; P = .483), with no significant difference in seizure risk between groups (hazard ratio, 1.02; 95% CI: 0.42, 2.45; P = .966). Mean time to seizure onset was 8.5 mo for ETV/CPC and 11.2 mo for VPS (P = .464). As-treated, per-protocol, and attributable-intervention analyses yielded similar results., Conclusion: Postoperative seizure incidence following treatment of PIH was 20% within 2 yr, regardless of treatment modality., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Though it is well known that normal pressure hydrocephalus (NPH) patients can cognitively improve after ventriculoperitoneal shunting (VPS), one of the major dilemmas in NPH is the ability to prospectively predict which patients will improve., Objective: To prospectively assess preoperative predictors of postshunt cognitive improvement., Methods: This was a prospective observational cohort including 52 consecutive patients with approximately 1-yr follow-up. Patients underwent neuropsychological testing at baseline, postlumbar drainage, and postshunt. Cerebrospinal fluid (CSF) biomarkers and cortical biopsies were also collected to examine their relationship with postshunt cognitive improvement., Results: Rey Auditory Verbal Learning Test-L (RAVLT-L) was the only neuropsychological test to demonstrate statistically significant improvement both postlumbar drain and postshunt. Improvement on the RAVLT-L postlumbar drain predicted improvement on the RAVLT-L postshunt. Patients with biopsies demonstrating Aβ+ Tau+ had lower ventricular CSF Aβ42 and higher lumbar CSF pTau compared to Aβ- Tau- patients. A receiver operating curve analysis using lumbar pTau predicted Aβ+ Tau+ biopsy status but was not related to neuropsychological test outcome., Conclusion: The RAVLT can be a useful preoperative predictor of postoperative cognitive improvement, and thus, we recommend using the RAVLT to evaluate NPH patients. CSF biomarkers could not be related to neuropsychological test outcome. Future research in a larger patient sample will help determine the prospective utility of CSF biomarkers in the evaluation of NPH patients., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Ventriculoperitoneal shunt (VPS) placement is one of the most common neurosurgical procedures. VPSs are associated with high costs, which predominantly arise from relatively high complication and revision rates. It is reasonable to assume that revision rates in developing countries would be higher. In this study we report the single-institution revision rates and costs from a high-volume department in sub-Saharan Africa., Methods: A pediatric neurosurgical database was studied in the extent of 5 years. The 30-day shunt failure rate, overall revision rate, and costs were calculated, and results were compared with previously published studies from developed countries., Results: In the selected time period 1840 VPS surgeries were performed, of which 592 were shunt revisions (32.14%). The majority of revision surgeries was performed in the first year- 501 (representing 84.63%); second year, 64; third year, 21; fourth year, 6; and fifth year, 2. The overall shunt revision rate was 28.94% with a 30-day revision rate of 14.58%. During the course of the study, costs of VPS surgery, the shunt, and daily ward charges did not change significantly. The average total charge for VPS insertion was 60,000 KES (586 USD), VPS removal 30,000 KES (293 USD), and VPS revision 50,000 KES (489 USD)., Conclusions: This retrospective study proves that VPSs, with their known complication risks, can be performed in a sub-Saharan missionary hospital with acceptable costs and results that are comparable with those achieved in some Western hospitals. Keys to those outcomes include high volume and a highly experienced team., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Image guidance for shunt surgery results in more accurate proximal catheter placement. However, reduction in shunt failure remains unclear in the literature. There have been no prior studies evaluating the cost effectiveness of neuronavigation for shunt surgery., Objective: To perform a cost analysis using available hospital charges of hypothetical shunt surgery performed with/without electromagnetic neuronavigation (EMN)., Methods: Hospital charges were collected for physician fees, radiology, operating room (OR) time and supplies, postanesthesia care unit, hospitalization days, laboratory, and medications. Index shunt surgery charges (de novo or revision) were totaled and the difference calculated. This difference was compared with hospital charges for shunt revision surgery performed under 2 clinical scenarios: (1) same hospital stay as the index surgery; and (2) readmission through the emergency department., Results: Costs for freehand de novo and revision shunt surgery were $23 946.22 and $23 359.22, respectively. For stealth-guided de novo and revision surgery, the costs were $33 646.94 and $33 059.94, a difference of $9700.72. The largest charge increase was due to additional OR time (34 min; $4794), followed by disposable EMN equipment ($2672). Total effective charges to revise the shunt for scenarios 1 and 2 were $34 622.94 and $35 934.94, respectively. The cost ratios between the total revision charges for both scenarios and the difference in freehand vs EMN-assisted shunt surgery ($9700.72) were 3.57 and 3.70, respectively., Conclusion: From an economic standpoint and within the limitations of our models, the number needed to prevent must be 4 or less for the use of neuronavigation to be considered cost effective., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Hydrocephalus is a common complication following decompressive craniectomy. Ventriculoperitoneal shunt (VPS) is required for some patients before receiving a cranioplasty (CP). The presence of a VPS is regarded as a risk factor for overall CP complications., Methods: A retrospective survey was conducted on 176 patients with traumatic brain injury who underwent late (>3 months) titanium CP (Ti-CP) in our hospital from April 2014 to July 2018. Thirteen patients (7.4%) had preoperative VPS. Propensity score matching was performed for these 13 patients with a ratio of 1:5. A total of 78 patients were selected. Preoperative clinical parameters and postoperative complications were analyzed. The period of postoperative follow-up ranged from 3 to 63 months (mean 21.3 ± 17.0 months)., Results: The overall complication rate was greater in the VPS group (P = 0.010). These patients were more likely to develop a sunken skin flap (P < 0.001). The rate of postoperative cerebral hemorrhage was greater in the VPS group. Logistic analysis showed that preoperative VPS was an independent risk factor for postoperative extradural collection (odds ratio 17.714, P < 0.001). VPS was not related to postoperative infection and seizure. Postoperative drainage duration longer than 2.5 days significantly increased the risk of postoperative infection (odds ratio 7.715, P = 0.023)., Conclusions: The presence of a VPS significantly increased the risk of extradural collection in patients with traumatic brain injury who underwent late Ti-CP. It also was related to postoperative hemorrhage. The sunken skin flap in patients with VPS increased surgical difficulty and the likelihood of extradural accumulation. Preoperative VPS was not related to postoperative infection and seizure in Ti-CP., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Dissemination of diffuse intrinsic pontine glioma (DIPG) outside the central nervous system is exceptional. Here, we present a child diagnosed with DIPG who developed seeding along the track of the ventriculoperitoneal shunt and review the literature on this unusual occurrence.

Purpose: The aim of the study was to evaluate established risk factors and define new inflammation-associated factors associated with postoperative ventriculoperitoneal shunt placement., Methods: The electronic medical records of children who underwent surgery for a tumor in the posterior fossa between January 2009 and January 2018 were retrospectively analyzed. Factors evaluated include age, clinical symptoms, tumor type, extent of surgical tumor resection, treatment with EVD and/or ETV, radiological findings, postoperative serum CRP, and leucocyte levels. Tumor tissue was stained immunohistochemically with antibodies against CD3, and leucocyte counts were performed. Patients with pre- or postoperative signs of infection or confirmation of a concurrent infection were excluded from some analyses., Results: Seventy patients ages 0.4-20.8 years (median, 8.2) were included. Forty-five of 70 (65.3%) presented postoperative radiological signs of hydrocephalus. Fifteen of 70 (21.4%) patients required shunt placement postoperatively. Shunt placement was significantly associated with age < 3 years at diagnosis (p = 0.013), perioperative EVD placement (p < 0.001), signs of hydrocephalus in postoperative imaging (p = 0.047), a frontooccipital horn ratio (FOHR) > 0.46 within the first 72 h postoperatively (p < 0.001), and the presence of intraventricular blood postoperatively (p = 0.007). Six patients who underwent shunting had serum CRP levels > 40 mg/l (p = 0.030) within the first 48 h postoperatively. Tumor type or extent of resection did not correlate with shunt placement., Conclusions: Several established and new factors associated with shunt placement after posterior fossa tumor surgery could be identified. Additional studies are needed to explore the aseptic inflammation pathways involved with increased CRP levels and shunt placement.

Migrating intracranial tumors are extremely rare occurrences in the neurosurgery literature. Introduction of any factor causing disequilibrium in cerebrospinal fluid circulation and pressure can potentially precipitate transventricular migration of pedunculated intraventricular lesions. The identification of such factors, prior to excision of intraventricular pedunculated tumors, is imperative to avoid intraoperative mismanagement. We report an extremely rare case of transventricular migration of a choroid plexus carcinoma in an infant, possibly precipitated by a ventriculoperitoneal (VP) shunt on the opposite side. This resulted in intraoperative confusion and a subsequent re-exploration of the opposite side for excision of the tumor. The literature provided only two similar occurrences in the past; however, in both cases, the migration was within the same ventricle and was documented prior to definitive resection. We report the first instance of transventricular migration of a tumor to the opposite ventricle following VP shunt which resulted in a negative intraoperative finding requiring a subsequent re-intervention on the opposite side. We believe that for any pedunculated intraventricular lesion, where an emergency management of hydrocephalus takes priority, a repeat neuroimaging is a must prior to definitive resection., (© 2019 S. Karger AG, Basel.)

Objectives: Distal obstruction is a common cause of shunt failure and need for revision in patients undergoing ventriculoperitoneal shunting (VPS) for idiopathic normal pressure hydrocephalus (iNPH)., Patients and Methods: Records of patients with iNPH treated with VPS between 2001 and 2017 were reviewed. Patients undergoing initial shunt placement at our institution were included for analysis and the incidence of revision surgery due to distal obstruction was noted. Risk factors for distal obstruction were identified using a stepwise Cox proportional hazards model., Results: There were 341 patients included for analysis. Assistance from a general surgeon in placement of the peritoneal catheter was provided in 54 patients (15.8%). Shunt revision was necessary in 69 patients (20.2%), with 17 patients (5.0%) found to have a distal malfunction. On univariate analysis, increasing age was associated with reduced risk of distal malfunction (Unit RR 0.92, 95% CI 0.89-0.96; p < 0.001). BMI ≥ 38.9 (RR 6.60, 95% CI 1.84-19.00), prior abdominal surgery (RR 2.95, 95% CI 1.11-7.70; p = 0.032), and fixed-setting valve (RR 6.24, 95% CI 1.27-112.72; p = 0.020) were associated with increased likelihood of distal malfunction. General surgery involvement had no effect on distal malfunction rates (OR 1.30, 95% CI 0.25-3.21; p = 0.693). On multivariate analysis, increasing age (Unit RR 0.92, 95% CI 0.89-0.95; p < 0.001) and prior abdominal surgery (RR 3.30, 95% CI 1.23-8.71; p = 0.019) were independently associated with decreased and increased risk of distal obstruction, respectively., Conclusions: We identify multiple factors associated with distal shunt obstruction, and found that general surgery assistance was not protective against distal malfunction. These data may aid in the risk-stratification of patients undergoing VPS for iNPH., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Shunt infections remain a significant challenge in pediatric neurosurgery. Numerous surgical checklists have been introduced to reduce infection rates., Objective: To introduce an evidence-based shunt surgery checklist and its impact on our shunt infection rate., Methods: Between January 1, 2008 and December 31, 2015, pediatric patients who underwent shunt surgery at our institution were indexed in a prospectively maintained database. All definitive shunt procedures were included. Shunt infection was defined according to the Center for Disease Control and Prevention's National Hospital Safety Network surveillance definition for surgical site infection. Clinical and procedural variables were abstracted per procedure. Infection data were compared for the 4 year before and 4 year after protocol implementation. Compliance was calculated from retrospective review of our checklists., Results: Over the 8-year study period, 1813 procedures met inclusion criteria with a total of 37 shunt infections (2%). Prechecklist (2008-2011) infection rate was 3.03% (28/924) and decreased to 1.01% (9/889; P = .003) postchecklist (2012-2015), representing an absolute risk reduction of 2.02% and relative risk reduction of 66.6%. One shunt infection was prevented for every 50 times the checklist was used. Those patients who developed an infection after protocol implementation were younger (0.95 years vs 3.40 years (P = .027)), but there were no other clinical or procedural variables, including time to infection, that were significantly different between the cohorts. Average compliance rate among required checklist components was 97% (range 85%-100%)., Conclusion: Shunt surgery checklist implementation correlated with lower infection rates that persisted in the 4 years after implementation.

Purpose: To develop a better understanding of pediatric intracranial aneurysms (PIAs)., Methods: All PIAs treated in our center from January 2012 to April 2017 were retrospectively included. Clinical data, treatment summaries, and follow-up outcomes were retrieved and analyzed., Results: A total of 66 PIAs were found in 64 patients with a mean age of 11.4 ± 5.7 years, 68.8% of whom were male. The most common symptoms were seizure (n = 7, 63.6%) for the 0-5 age group and headache (n = 38, 71.7%) for the 6-18 age group. Fifty-one PIAs (77.3%) were located in the anterior circulation, with the middle cerebral artery (MCA) being the most common site (n = 28, 42.4%). Fifteen patients (23.4%) had PIAs that were pseudoaneurysms, and nine of them (60%) had a combined history of head trauma. Thirty-five patients (54.7%) had distal arterial aneurysms, and 21 of them (60%) presented with seizure. During a mean follow-up time of 1.6 ± 1.2 years, 79.7% of patients (n = 51) had favorable outcomes, 5 patients (7.8%) died, and the remaining 8 patients (12.5%) had unfavorable outcomes with severe neurological deficits., Conclusion: Apart from characteristics consistent with previous studies, several new findings regarding PIAs were reported, including a difference in the most common symptoms in different age groups, the MCA as the predominant location of PIAs, the high ratio of pseudoaneurysms and their head trauma etiology, and the incidence of distal arterial aneurysms and their relationship with the risk of seizures.

Background: Endoscopic third ventriculostomy (ETV) and ventriculo-peritoneal shunt (VPS) although recognized surgical options for non-communicating hydrocephalus have debatable applications., Objective: We analysed a prospective cohort of age-matched children with non-tumor, non-communicating hydrocephalus treated with the two surgical modalities using clinically measurable parameters., Methods: A single institution analysis of age-matched patients with non-communicating hydrocephalus treated with VPS or ETV over a 3-year period. Occipitofrontal circumference (OFC), milestone, shunt independence as well as complication profiles of patients were recorded and analysed. Mean follow-up period was 1.27 ± 0.19 years 95%CI). Data analysis were performed using SPSS version 15, Chicago, IL. Statistical tests were set at 95% significance level., Results: Fifty-five patients were enrolled, 25 patients had ETV, while 30 had VPS. Mean age was 2.3 ± 0.7 years (95% CI) with a range of 3 months to 4.5 years. Aqueductal stenosis was the most common indication. OFC profile decline was significant among the VPS group when compared with ETV group at 3 months follow-up (χ 2  = 7.59, df = 1, p < 0.05). There was no difference among the two treatment groups χ 2  = 2.47, df = 1, p > 0.05) in milestone profile. Thirteen percent of VPS, compared to (4%) ETV patients, had sepsis (χ 2  = 4.59, df = 1 p < 0.05). Ninety-two percent of ETV patients remained shunt free, while 80% of shunted patients achieved ETV independence. Two patients died among the VPS group compared to one patient in the ETV group., Conclusion: VPS compared to ETV is associated with an earlier milestone and OFC response. ETV is associated with lower rates of sepsis and mortality.

Background: The signs and pathomechanism of Miyazaki syndrome is presented through the case of a young female patient., Case Description: The 33-year-old patient had undergone placement of a ventriculoperitoneal shunt with a pressure-adjustable valve for communicating hydrocephalus years before presenting to our department with the complaints of constant headache and unsteady gait. On the basis of the clinical picture and her history, plain and contrast-enhanced cranial and whole spine magnetic resonance imaging and magnetic resonance angiography examinations were performed, with the scans revealing signs indicative of cerebrospinal fluid hypotension typical of Miyazaki syndrome., Conclusion: The article discusses the available literature suggesting the underlying cause in such cases to be the dysfunction of the Starling resistor mechanism due to an improperly adjusted ventriculoperitoneal shunt, which results in excessive cerebrospinal fluid loss accompanied by consequent cerebral venous overflow with vertebral venous engorgement and compressive cervical myelopathy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Pneumocephalus is usually seen in trauma or cranial surgery. It is rarely reported as a delayed complication of ventriculoperitoneal shunt placement for hydrocephalus secondary to trauma, tumor, or aqueduct stenosis. We describe a case of intraventricular pneumocephalus manifesting 10 months after placement of a shunt for normal-pressure hydrocephalus., Case Description: A pressure-regulated ventriculoperitoneal shunt was implanted in an 81-year-old patient after diagnosis of normal-pressure hydrocephalus. He showed postoperative clinical improvement. Routine computed tomography performed 2 months after the procedure showed no abnormalities. He presented 10 months after shunting with a subacute deterioration of gait. Imaging revealed major intraventricular pneumocephalus and a left-sided temporal porencephalic cyst with a small, bony defect in the left petrous bone. A middle fossa approach was performed, and the temporal defect was covered with fascia of the temporal muscle. This resulted in a gradual resolution of pneumocephalus., Conclusions: Pneumocephalus after shunting for NPH is rare and described as a complication only during the first 2 postoperative months. This case is unique, as the pneumocephalus developed 10 months after shunting. The combination of an occult, possibly congenital, skull base defect and the insertion of a shunt resulted in delayed intraventricular and intraparenchymal pneumocephalus., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background and Purpose- Pathological obstruction in arachnoid granulations after subarachnoid hemorrhage (SAH) can impede cerebrospinal fluid flow outward to the venous sinus and causing hydrocephalus. Because bone and arachnoid granulations share the same collagen type, we evaluated the possible relation between bone mineral density and shunt-dependent hydrocephalus after SAH. Methods- We measured Hounsfield units of the frontal skull on admission brain computed tomography in patients with SAH. Receiver operating characteristic curve analysis was performed to determine the optimal cutoff Hounsfield unit in skull to predict osteopenia and osteoporosis in a large sample registry. According to the optimal cutoff skull Hounsfield unit values, study patients were then categorized as hypothetical normal, osteopenia, and osteoporosis. Odds ratios were estimated using logistic regression to determine whether the osteoporotic conditions are independent predictive factors for the development of shunt-dependent hydrocephalus after clipping for SAH. Results- A total of 447 patients (alive ≥14 days) with ruptured aneurysm SAH who underwent surgical clipping were retrospectively enrolled in this study during a 9-year period from 2 hospitals. We found that hypothetical osteoporosis was an independent predictor for shunt-dependent hydrocephalus after aneurysmal clipping for SAH after full adjustment for other predictive factors, including age (odds ratio, 2.08; 95% confidence interval, 1.06-4.08; P=0.032). Conclusions- Our study demonstrates a possible relation between possible osteoporosis and hydrocephalus after SAH. Hounsfield unit measurement on admission brain computed tomography may be helpful for predicting hydrocephalus during the clinical course of SAH in patients with osteoporosis or suspected osteoporosis.

Background: Diagnosing normal-pressure hydrocephalus (NPH) and selecting patients who will experience a sustained benefit from fluid diversion surgery remains challenging. This study seeks to evaluate the association between the callosal angle (CA) and the long-term postoperative response to ventriculoperitoneal shunt surgery in a different subgroup population than previously studied to assess its generalizability., Methods: We studied 72 patients with idiopathic NPH who underwent ventriculoperitoneal shunt surgery and had at least 18 months of follow-up between 2000 and 2016. We recorded their pre- and postoperative symptoms according to the NPH Eide scale and their comorbidities with the Kiefer index. Their CA, as well as Evans' Index, ventricular height, and transependymal signal were measured. Multivariable statistical models were used to determine which factors were associated with postoperative improvement while we controlled for the presence of the NPH triad., Results: Fifty-nine patients (82%) demonstrated a successful response to surgery at their first postoperative follow-up. However, this declined to 54 patients (75%) at 1 year and 45 (62.5%) patients at their last follow-up. When we controlled for the presence of the triad of symptoms, the CA significantly predicted a good, sustained response to surgery; for every degree decrease in the CA, a patient is 4% more likely to experience benefit from surgery., Conclusions: The CA is a useful preoperative prognostic tool for predicting which patients will experience a sustained benefit from surgery. Further studies are required to clarify this disease in the context of old age, comorbidity, and possible concomitant neurodegenerative diseases., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: Recent Normal Pressure Hydrocephalus (NPH) practice guidelines describe a serious adverse event (SAE) rate following surgery of 11%., Patients & Methods: We conducted a retrospective review of 162 consecutive patients who have undergone work-up at our center's multidisciplinary NPH clinic over a 47 month time period (2/2014-12/2017). Of these, 22 ultimately underwent neurosurgical ventricular shunt surgery as treatment for NPH. Clinical records were reviewed for SAEs categorized as possibly/probably/definitely related to NPH surgery., Results: In 10/22 (45.5%) operated subjects, there were 11 qualifying SAEs over this 3-year period: 1 central nervous system infections, 4 subdural hematomas, 2 seizures resulting in hospitalization, 1 catheter malfunction, 2 perioperative AEs, and 1 death of uncertain cause. Eight SAEs were coded as probably/definitely related. Six occurred >3 months from the time of surgery., Conclusions: SAEs following NPH surgery are common. Additional studies are needed to determine the long-term safety of NPH surgery in older adults., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Introduction: Hydrocephalus due to congenital aqueductal stenosis (CAS) has significant long-term clinical implications. Previous reports on outcomes after treatment of congenital hydrocephalus are heterogenous and lack specificity for the subgroup of patients with isolated aqueductal stenosis., Methods: An institutional surgical database was queried for the time period of 2005-2013 for patients with the diagnosis of isolated CAS and >2 years of clinical follow-up. Medical history and neurodevelopmental outcomes were recorded., Results: The institutional cohort consisted of 41 patients with isolated CAS who underwent cerebrospinal fluid diversion. At a mean follow-up of 5.9 years, 48.8% carried a diagnosis of epilepsy and 68% were developmentally delayed. Four patients were diagnosed with cerebral palsy (9.8%). In total, 78% of patients were shunt-dependent, and the remainder had patent third ventriculostomies. Only 32% of patients in our cohort were neurologically normal after long-term follow-up despite contemporary management., Conclusions: Regardless of the initial treatment strategy, the age at diagnosis, or the timing of cerebrospinal fluid diversion after birth, patients with aqueductal stenosis have high rates of epilepsy, neurodevelopmental delay, and educational difficulties, and few are neurologically normal despite contemporary management. Investigation into in utero identification and correction of hydrocephalus may result in improved outcomes and warrants further investigation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: Treatment monitoring and outcome evaluation in pediatric hydrocephalus require gapless documentation regarding surgical and clinical follow-up data beginning from day 1 of treatment in order to apply high quality of care. Endoscopic procedures, shunt insertion and revision surgeries, and individual modifications of valve hardware or pressure settings during follow-up as well as established outcome measurements are highly relevant for complete illustration of the patient's hydrocephalus histories. A digital tool to capture, organize, and analyze comprehensive treatment-related data was estimated long overdue, consequentially developed, and implemented in daily pediatric neurosurgical routine., Methods: We established a self-contained, network-capable database application to supply and back up clinical information of complete surgical treatment history with implant status and follow up for all institutional pediatric hydrocephalus patients from 1995 to date. The application content has been prospectively complemented since 2012 during daily pediatric neurosurgical routine. Beside surgical data, neurological outcome and quality of life assessment were integrated according to validated scales to be recordable 2, 3, and 5 years after initial surgical intervention for prospective administration. The application is in continuous and problem-free use since implementation offering homogeneous and structured real-time information of surgical and corresponding neurological hydrocephalus-related data. By using an automatized data extraction tool, an exemplary surgical outcome evaluation reviewing institutional ventriculo-peritoneal shunt (VPS) treatment in infants over a period of more than 20 years was performed. To validate applicability, the Registry was successfully implemented in an external institution under identical conditions continuously serving for the same purpose until today., Results: Upon completion of the developing process, the application was successfully implemented into routine clinical workflow of our institution. In total, 579 pediatric hydrocephalus patients entered into the Registry with collectively 1874 corresponding hydrocephalus-related surgeries (9% neuro-endoscopic procedures, 18% temporary CSF-diversions, 73% shunt surgeries) so far. For exemplary surgical outcome analysis, the total volume of complex data sets could easily be reduced stepwise in regard to requested inclusion criteria. The selection process generated conclusive data of 256 institutional pediatric VPS patients providing a median follow-up of 8.5 years. Surgical outcome was evaluated in regard to hydrocephalus etiology, applied valve design, valve augmentation, cause of initial malfunction, time to initial shunt revision, and number of total revisions., Conclusion: The pediatric hydrocephalus registry application delivers easy access to contemporary and up-to-date clinical information during daily clinical routine and proves comprehensive value for various scientific purposes. Institutional hydrocephalus etiologies, treatment modalities, and surgical outcome could be reviewed for a selected pediatric patient collective during an interval of more than 20 years and confirmed initial shunt treatment within the first year of age, communicating hydrocephalus and a history of prematurity as significant variables for unfavorable shunt survival and long-term revision rate. At our institution, the Registry emerged to an essential and sustainable tool to capture, organize, and analyze patterns of care in pediatric hydrocephalus patients of all etiologies and treatment modalities. Because of its adaptable and reliable predicate, a prospective multi-center utilization is currently in preparation.

Introduction: Chronic overdrainage affects shunted patients producing a variety of symptoms that may be misdiagnosed. The best known symptoms are so-called shunt-related headaches. There is mounting evidence that changes in cerebrospinal venous system dynamics are a key factor to the pathophysiology of chronic overdrainage syndrome., Clinical Presentation: We report the case of a 29-year-old woman with a shunt since the postnatal period suffering from chronic but the most severe intermittent headache attacks, despite an open shunt and with unchanged ventricular width during attacks. Intracranial pressure (ICP) recordings were performed during headache attacks and thereafter., Diagnosis and Managment: Massively increased ICPs, a continuous B wave "storm," and severely compromised intracranial compliance despite an open shunt were found, a scenario that was always self-limiting with the resolution of symptoms after several hours. When mobilized to the upright position, her ICPs dropped to - 17 mm Hg, proving shunt overdrainage., Outcome and Conclusions: Symptomatology can only be explained by sudden venous entrapment following chronic venous distention as a result of chronic overdrainage. Subsequent therapeutic management with an overdrainage preventing shunt and satisfying clinical outcome with complete ceasing of headache attacks adds insight into the pathophysiology of chronic overdrainage syndrome.

Background: Idiopathic normal pressure hydrocephalus (iNPH) is commonly treated by cerebrospinal fluid (CSF) shunting. However, the long-term efficacy of shunt intervention in the presence of comorbid Alzheimer's disease (AD) pathology is debated., Objective: To identify AD-associated CSF biomarkers predictive of shunting surgery outcomes in patients with iNPH., Methods: Preoperative levels of total and phosphorylated Tau (p-Tau) were measured in 40 patients with iNPH divided into low (<30 pg/mL) and high (≥30 pg/mL) p-Tau groups and followed up for three years after lumboperitoneal shunting. The modified Rankin Scale (mRS), Mini-Mental State Examination (MMSE), Frontal Assessment Battery, and iNPH Grading Scale scores were compared between the age-adjusted low (n = 24; mean age 75.7 years [SD 5.3]) and high (n = 11; mean age 76.0 years [SD 5.6]) p-Tau groups., Results: Cognitive function improved early in the low p-Tau group and was maintained thereafter (p = 0.005). In contrast, the high p-Tau group showed a gradual decline to baseline levels by the third postoperative year (p = 0.040). Although the p-Tau concentration did not correlate with the preoperative MMSE score, a negative correlation appeared and strengthened during follow-up (R2 = 0.352, p < 0.001). Furthermore, the low p-Tau group showed rapid and sustained mRS grade improvement, whereas mRS performance gradually declined in the high p-Tau group., Conclusions: Preoperative CSF p-Tau concentration predicted some aspects of cognitive function after shunt intervention in patients with iNPH. The therapeutic effects of shunt treatment were shorter-lasting in patients with coexisting AD pathology.

Background: The entity of low-pressure hydrocephalus remains poorly understood and thoroughly debated. Symptomatic improvement accompanied by decrease in ventricular size after prolonged subatmospheric drainage has been well documented, and this method has been considered the criterion standard of management. Few studies have examined alternative treatment options, either to avoid the risks associated with prolonged external ventricular drainage or because of the failure of traditional methods., Objectives: This study compiled and examined reported cases of low-pressure hydrocephalus in an attempt to provide an up-to-date summary of the condition., Methods: A literature search was conducted by use of Ovid Medline and PubMed filtered for the past 25 years with specific key terms, inclusion criteria, and exclusion criteria. Selected case studies and case series were then compared, and statistical analysis was performed where appropriate., Results: Over 25 years, 17 articles met our criteria. In addition to our case, 90 cases of LPH were reported. Magnetic resonance elastography (MRE) has proved to be an effective means of studying the viscoelastic properties of the brain. Endoscopic third ventriculostomy (ETV) appears to be a strong alternative, or additional, treatment., Conclusion: MRE may prove to be effective in studying LPH because of its ability to quantify viscoelastic properties in response to therapy. Additionally, ETV should be considered in cases of LPH, although there is little evidence in the current literature to support its use. There are suggestions that it may lead to fewer shunt-dependent patients. Future studies are needed because there are few documented examples., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to determine whether drainage and revision are an effective treatment for abdominal pseudocyst associated ventriculoperitoneal (VP) shunt failure by estimating the total rate of secondary shunt failure., Methods: We performed a retrospective review of children with hydrocephalus diagnosed with and treated for an abdominal pseudocyst at the Children's Hospital, London Health Sciences Centre (LHSC) between January 1, 2000 and May 31, 2016 (ethics approval # 108136). Patients with a VP shunt were included if (i) the development of an abdominal pseudocyst at age 2 to 18 years was identified, (ii) treatment of the pseudocyst by either interventional radiology (IR) or surgical drainage, and (iii) revision of the VP shunt. Demographic data and details of pseudocyst formation/ treatment as well as subsequent failures were identified., Results: Twelve patients who had a VP shunt developed abdominal pseudocyst and met inclusion criteria. A 91% shunt failure rate after drainage and shunt revision was identified. Three patients had the pseudocyst drained in interventional radiology and then externalized due to shunt infection. Nine patients were treated by surgical revision. Ten patients experienced recurrent shunt failure following initial drainage of the pseudocyst: pseudocyst reoccurrence (n = 3), distal obstruction from adhesions (n = 1), and uncleared infection (n = 6)., Conclusion: The results suggest that pseudocyst drainage and shunt revision is ineffective in providing long-term resolution of shunt problems.

Background: Hydrocephalus is a well-recognized complication after aneurysmal subarachnoid hemorrhage (aSAH). This study aimed to identify predictors for shunt-dependent hydrocephalus (SDHC) after aSAH via a systematic review and meta-analysis., Methods: A systematic search was conducted using the Embase, MEDLINE, and Web of Science databases for studies pertaining to aSAH and SDHC. Risk factors were assessed by meta-analysis when they were reported by at least 2 studies. The results were presented as odd ratios or risk ratios according to the study design with the corresponding 95% confidence intervals (CI)., Results: Twenty-five studies were included. In primary analysis of 14 potential risk factors, 12 were identified as predictors of SDHC after aSAH including age ≥50 years, female gender, high Hunt-Hess grade, Glasgow Coma Scale ≤8, Fisher grade ≥3, acute hydrocephalus, external ventricular drainage insertion, intraventricular hemorrhage, postcirculation aneurysm, anterior communicating artery aneurysm, meningitis, and rebleeding. The meta-analysis based on cohort studies found a significantly increased risk for SDHC in patients with aSAH treated by coiling (risk ratio, 1.16; 95% CI, 1.05-1.29), while the meta-analysis based on case-controlled studies failed to replicate this finding (odds ratio, 1.27; 95% CI, 0.95-1.71)., Conclusions: Several new predictors of SDHC after aSAH were identified that may assist with the early recognition and prevention of SDHC. The controversial evidence found in this study was insufficient to support the potential of neurosurgical clipping for reducing the risk of shunt dependency. Further well-designed studies are warranted to explore the effect of treatment modality on SDHC risk., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: Management of pregnancy and delivery of a patient with a history of myelomeningocele requires a multidisciplinary team approach., Case Report: We report a case of pregnancy and delivery by a patient who had a history of myelomeningocele surgical repair, ventriculoperitoneal (VP) shunt, and bladder augmentation enterocystoplasty. Regarding types of delivery style, anesthesiologists recommended a Cesarean section under general anesthesia. However, urologists recommended a vaginal delivery because they were concerned that she would require a nephrostomy because of severe adhesion between her uterus and the neobladder if she had a Cesarean section., Discussion: In a pregnant myelomeningocele patient with a VP shunt, neurosurgeons are expected to manage the VP shunt during pregnancy and delivery. The possible types of delivery style and the best options based on the neurological deficit should be discussed together with a medical team.

Background: Ventriculoperitoneal shunt (VPS) is a common treatment for patients with hydrocephalus (HC). VPS is associated with complications that may lead to shunt revisions. We studied the surgical outcome of pediatric patients with HC in a population-based setting., Methods: The medical charts and imaging findings of 80 patients ≤16 years old who required VPS secondary to HC were studied., Results: Mean age at time of initial shunt placement was 3.2 years (SD 4.5) and mean follow-up time was 3.3 years (SD 2.9); 57% of patients were male. Half of patients underwent shunt revision with mean time to first revision of 8 months. Patients ≤6 months old had a higher shunt revision rate compared with patients >6 months old (P < 0.001). The most common causes of HC requiring VPS were tumors (27.5%), congenital defects (22.5%), and intraventricular hemorrhage (19%). Revision rates in the intraventricular hemorrhage and congenital defects groups were 67% (P = 0.017) and 72% (P = 0.016) compared with 32% in the tumor group. Programmable valves (56%) were more common than nonprogrammable valves, but there was no significant difference in shunt survival (P = 0.632). The mean biparietal measurement change between preoperative and postoperative images was +0.9 mm in the no revision group and +6.6 mm in the revision group (P = 0.003)., Conclusions: Half of patients with shunts required revision. Age ≤6 months and intraventricular hemorrhage and congenital defects etiologies of HC were associated with increased risk for shunt revision. Most revisions were done during the first year after the initial VPS., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Ventriculoperitoneal (VP) shunt placement, the mainstay of treatment for hydrocephalus, can place a substantial burden on patients and health care systems because of high complication and revision rates. We aimed to identify factors associated with 30-day VP shunt failure in children undergoing either initial placement or revision., Methods: VP shunt placements performed on patients in the 2012-2013 American College of Surgeons National Surgical Quality Improvement Program (NSQIP) Pediatric were identified., Results: VP shunts were placed in 3,984 patients either as an initial placement (n = 1,093) or as a revision (n = 2,891). Compared to the initial-placement group, the revision group was significantly more likely to experience shunt failure (14 vs. 8%, p < 0.0001). In the initial-placement group, congenital hydrocephalus was independently associated with shunt failure (OR 1.83; 95% CI 1.01-3.31, p = 0.047). In the revision group, cardiac risk factors (OR 1.38; 95% CI 1.00-1.90, p = 0.047), a chronic history of seizures (OR 1.33; 95% CI 1.04-1.71, p = 0.022), and a history of neuromuscular disease (OR 0.61; 95% CI 0.41-0.90, p = 0.014) were independently associated with shunt failure., Conclusions: Identifying the factors associated with VP shunt failure may allow the development of interventions to decrease failures. Further refinement of the collected variables in the NSQIP Pediatric specific to neurosurgical procedures is necessary to identify modifiable risk factors., (© 2016 S. Karger AG, Basel.)

Objective/aim: The aim of this report was to investigate the effect of ventriculoperitoneal shunt insertion for the treatment of hydrocephalus on thyroid hormones in the first 3 months of life., Methods: Thyroid-stimulating hormone (TSH), free T3 (fT3), and free T4 (fT4) levels were compared at 7 days (preoperatively) and at 30 and 90 days (postoperatively) after birth between 25 ventriculoperitoneal shunt-inserted hydrocephalic newborns and 20 healthy newborns., Results: The TSH level at 7 days was higher in the hydrocephalic patient group (6.33 µIU) compared to the control group (3.76 µIU). This value was significantly decreased at 90 days in the ventriculoperitoneal shunt-inserted newborns (2.35 µIU) compared to the control group (3.33 µIU; p < 0.05). There were no significant differences between time points for fT4 and fT3 values in the patient group or for TSH, fT4, and fT3 values in the control group., Conclusion: We propose that a ventriculoperitoneal shunt inserted in the early period of life may have beneficial effects on thyroid hormones., (© 2016 S. Karger AG, Basel.)

OBJECTIVE The introduction of ventriculoperitoneal shunts changed the way hydrocephalus was treated. Whereas much is known about the causes of shunt failure in the first few years, there is a paucity of data in the literature regarding the cause of late shunt failures. The authors conducted a study to find out the different causes of late shunt failures in their institution. METHODS A 10-year retrospective study of all the patients who were treated in the authors' hospital between 2006 and 2015 was conducted. Late shunt failures included those in patients who had to undergo shunt revision more than 5 years after their initial shunt insertion. The patient's notes and scans were reviewed to obtain the age and sex of the patient, the time it took for the shunt to fail, the reason for failure, and the patient's follow-up. RESULTS Forty-six patients in the authors' institution experienced 48 late shunt failures in the last 10 years. Their ages ranged from 7 to 26 years (12.23 ± 4.459 years [mean ± SD]). The time it took for the shunts to fail was between 6 and 24 years (mean 10.25 ± 3.77 years). Reasons for failure resulting in shunt revision include shunt fracture in 24 patients (50%), shunt blockage in 14 patients (29.2%), tract fibrosis in 6 patients (12.5%), shunt dislodgement in 2 patients (4.2%), and shunt erosion in 2 patients (4.2%). Postoperative follow-up for the patients ranged from 6 to 138 months (mean 45.15 ± 33.26 months). CONCLUSIONS Late shunt failure is caused by the effects of aging on the shunt, and the complications are different from early shunt failure. A large proportion are complications associated with shunt calcification. The authors advocate a long follow-up for pediatric patients with shunts in situ to monitor them for various causes of late shunt failure.