Category: hydrocephalus

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Placebo-Controlled Effectiveness of Idiopathic Normal Pressure Hydrocephalus Shunting

Neurosurgery 92:481–489, 2023

Multiple prospective nonrandomized studies have shown 60% to 70% of patients with idiopathic normal pressure hydrocephalus (iNPH) improve with shunt surgery, but multicenter placebo-controlled trial data are necessary to determine its effectiveness.

OBJECTIVE: To evaluate the effectiveness of cerebrospinal fluid shunting in iNPH through comparison of open vs placebo shunting groups at 4 months using a pilot study.

METHODS: Patients were randomized to a Codman Certas Plus valve (Integra LifeSciences) set at 4 (open shunt group) or 8 (“virtual off”; placebo group). Patients and assessors were blinded to treatment group. The primary outcome measure was 10-m gait velocity. Secondary outcome measures included functional scales for bladder control, activities of daily living, depression, and quality of life. Immediately after 4-month evaluation, all shunts were adjusted in a blinded fashion to an active setting and followed to 12months after shunting.

RESULTS: A total of 18 patients were randomized. At the 4-month evaluation, gait velocity increased by 0.28 ± 0.28m/s in the open shunt group vs 0.04 ± 0.17m/s in the placebo group. The estimated treatment difference was 0.22 m/s ([P = .071], 95% CI 0.02 to 0.46). Overactive Bladder Short Form symptom bother questionnaire significantly improved in open shunt vs placebo (P = .007). The 4-month treatment delay did not reduce the subsequent response to active shunting, nor did it increase the adverse advents rate at 12 months.

CONCLUSION: This multicenter, randomized pilot study demonstrates the effectiveness, safety, and feasibility of a placebo-controlled trial in iNPH, and found a trend suggesting gait velocity improves more in the open shunt group than in the placebo group.

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First Experience With Postoperative Transcranial Ultrasound Through Sonolucent Burr Hole Covers in Adult Hydrocephalus Patients

Neurosurgery 92:382–390, 2023

Managing patients with hydrocephalus and cerebrospinal fluid (CSF) disorders requires repeated head imaging. In adults, it is typically computed tomography (CT) or less commonly magnetic resonance imaging (MRI). However, CT poses cumulative radiation risks and MRI is costly. Ultrasound is a radiation-free, relatively inexpensive, and optionally point-of-care alternative, but is prohibited by very limited windows through an intact skull.

OBJECTIVE: To describe our initial experience with transcutaneous transcranial ultrasound through sonolucent burr hole covers in postoperative hydrocephalus and CSF disorder patients.

METHODS: Using cohort study design, infection and revision rates were compared between patients who underwent sonolucent burr hole cover placement during new ventriculoperitoneal shunt placement and endoscopic third ventriculostomy over the 1-year study time period and controls from the period 1 year before. Postoperatively, trans-burr hole ultrasound was performed in the clinic, at bedside inpatient, and in the radiology suite to assess ventricular anatomy.

RESULTS: Thirty-seven patients with sonolucent burr hole cover were compared with 57 historical control patients. There was no statistically significant difference in infection rates between the sonolucent burr hole cover group (1/37, 2.7%) and the control group (0/57, P = .394). Revision rates were 13.5% vs 15.8% (P = 1.000), but no revisions were related to the burr hole or cranial hardware.

CONCLUSION: Trans-burr hole ultrasound is feasible for gross evaluation of ventricular caliber postoperatively in patients with sonolucent burr hole covers. There was no increase in infection rate or revision rate. This imaging technique may serve as an alternative to CT and MRI in the management of select patients with hydrocephalus and CSF disorders.

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Vascular risk profiles for predicting outcome and long-term mortality in patients with idiopathic normal pressure hydrocephalus: comparison of clinical decision support tools

J Neurosurg 138:476–482, 2023

Vascular risk factors (VRFs) may act synergistically, and clinical decision support tools (CDSTs) have been developed that present vascular risk as a summarized score. Because VRFs are a major issue in patients with idiopathic normal pressure hydrocephalus (INPH), a CDST may be useful in the diagnostic workup. The objective was to compare 4 CDSTs to determine which one most accurately predicts short-term outcome and 10-year mortality after CSF shunt surgery in INPH patients.

METHODS One-hundred forty INPH patients who underwent CSF shunt surgery were included. For each patient, 4 CDST scores (Systematic Coronary Risk Evaluation–Older Persons [SCORE-OP], Framingham Risk Score [FRS], Revised Framingham Stroke Risk Profile, and Kiefer’s Comorbidity Index [KCI]) were estimated. Short-term outcome (3 months after CSF shunt surgery) was defined on the basis of improvements in gait, Mini-Mental State Examination score, and modified Rankin Scale score. The 10-year mortality rate after surgery was noted. The CDSTs were compared by using Cox regression analysis, receiver operating characteristic curve analysis, and the chi-square test.

RESULTS For 3 CDSTs, increased score was associated with increased risk of 10-year mortality. A 1-point increase in the FRS indicated a 2% higher risk of death within 10 years (HR 1.02, 95% CI 1.003–1.035, p = 0.021); SCORE-OP, 5% (HR 1.05, 95% CI 1.019–1.087, p = 0.002); and KCI, 12% (HR 1.12, 95% CI 1.03–1.219, p = 0.008). FRS predicted short-term outcome of surgery (p = 0.024). When the cutoff value was set to 32.5%, the positive predictive value was 80% and the negative predictive value was 48% (p = 0.012).

CONCLUSIONS The authors recommend using FRS to predict short-term outcome and 10-year risk of mortality in INPH patients. The study indicated that extensive treatment of the risk factors of INPH may decrease risk of mortality.

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The positive impact of cisternostomy with cisternal drainage on delayed hydrocephalus after aneurysmal subarachnoid hemorrhage

Acta Neurochirurgica (2023) 165:187–195

Hydrocephalus is one of the major complications of aneurysmal subarachnoid haemorrhage (aSAH). In the acute setting, an external ventricular drain (EVD) is used for early management. A cisternal drain (CD) coupled with the micro-surgical opening of basal cisterns can be an alternative when the aneurysm is clipped. Chronic hydrocephalus after aSAH is managed with ventriculo-peritoneal (VP) shunt, a procedure associated with a wide range of complications. The aim of this study is to analyse the impact of microsurgical opening of basal cisterns coupled with CD on the incidence of VP shunt, compared to patients treated with EVD.

Methods The authors conducted a retrospective review of 89 consecutive cases of patients with aSAH treated surgically and endovascularly with either EVD or CD between January 2009 and September 2021. Patients were stratified into two groups: Group 1 included patients with EVD, Group 2 included patients with CD. Subgroup analysis with only patients treated surgically was also performed. We compared their baseline characteristics, clinical outcomes and shunting rates.

Results There were no statistically significant differences between the two groups in terms of epidemiological characteristics, WFNS score, Fisher scale, presence of intraventricular hemorrhage (IVH), acute hydrocephalus, postoperative meningitis or of clinical outcomes at last follow-up. Cisternostomy with CD (Group 2) was associated with a statistically significant reduction in VP-shunt compared with the use of an EVD (Group 1) (9.09% vs 53.78%; p < 0.001). This finding was confirmed in our subgroup analysis, as among patients with a surgical clipping, the rate of VP shunt was 43.7% for the EVD group and 9.5% for the CD group (p = 0.02).

Conclusions Cisternostomy with CD may reduce the rate of shunt-dependent hydrocephalus. Cisternostomy allows the removal of subarachnoid blood, thereby reducing arachnoid inflammation and fibrosis. CD may enhance this effect, thus resulting in lower rates of chronic hydrocephalus.

Keywords Cisternostomy · Cisternal drain · External ventricular drain · Aneurysmal subarachnoid haemorrhage · Hydrocephalus · Ventriculo-peritoneal shunt

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Failure of Internal Cerebrospinal Fluid Shunt: A Systematic Review and Meta-Analysis of the Overall Prevalence in Adults

World Neurosurg. (2023) 169:20-30

Reported rates of failures of internal cerebrospinal fluid shunt (ICSFS) vary greatly from less than 5% to more than 50% and no meta-analysis to assess the overall prevalence has been performed. We estimated the failure rate after ICSFS insertion and searched for associated factors.

METHODS: Six databases were searched from January 1990 to February 2022. Only original articles reporting the rate of adult shunt failure were included. Random-effects meta-analysis with a generalized linear mixed model method and logit transformation was used to compute the overall failure prevalence. Subgroup analysis and meta-regression were implemented to search for associated factors.

RESULTS: Of 1763 identified articles, 46 were selected, comprising 70,859 ICSFS implantations and 13,603 shunt failures, suggesting an accumulated incidence of 19.2%. However, the calculated pooled prevalence value and its 95% confidence interval (CI) were 22.7% (95% CI, 19.8e5.8). The CI of the different estimates did not overlap, indicating a strong heterogeneity confirmed by a high I 2 of 97.5% (95% CI, 97.1e97.8; P < 0.001; s 2 [ 0.3). Ninety-five percent prediction interval of shunt failure prevalence ranged from 8.75% to 47.36%. A meta-regression of prevalence of publication found a barely significant decreasing failure rate of about 2% per year (e2.11; 95% CI, e4.02 to e0.2; P [ 0.031).

CONCLUSIONS: Despite being a simple neurosurgical procedure, ICSFS insertion has one of the highest risk of complications, with failure prevalence involving more than 1 patient of 5. Nonetheless, all efforts to lower this high level of shunt failure seem to be effective.

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Cerebrospinal fluid and venous biomarkers of shunt‐responsive idiopathic normal pressure hydrocephalus: a systematic review and meta‐analysis

Acta Neurochirurgica (2022) 164:1719–1746

Idiopathic normal pressure hydrocephalus (iNPH) is a neurodegenerative disease and dementia subtype involving disturbed cerebrospinal fluid (CSF) homeostasis. Patients with iNPH may improve clinically following CSF diversion through shunt surgery, but it remains a challenge to predict which patients respond to shunting. It has been proposed that CSF and blood biomarkers may be used to predict shunt response in iNPH.

Objective To conduct a systematic review and meta-analysis to identify which CSF and venous biomarkers predict shunt- responsive iNPH most accurately.

Methods Original studies that investigate the use of CSF and venous biomarkers to predict shunt response were searched using the following databases: Embase, MEDLINE, Scopus, PubMed, Google Scholar, and JSTOR. Included studies were assessed using the ROBINS-I tool, and eligible studies were evaluated utilising univariate meta-analyses.

Results The study included 13 studies; seven addressed lumbar CSF levels of amyloid-β 1–42, nine studies CSF levels of Total-Tau, six studies CSF levels of Phosphorylated-Tau, and seven studies miscellaneous biomarkers, proteomics, and genotyping. A meta-analysis of six eligible studies conducted for amyloid-β 1–42, Total-Tau, and Phosphorylated-Tau demonstrated significantly increased lumbar CSF Phosphorylated-Tau (− 0.55 SMD, p = 0.04) and Total-Tau (− 0.50 SMD, p = 0.02) in shunt-non-responsive iNPH, though no differences were seen between shunt responders and non-responders for amyloid-β 1–42 (− 0.26 SMD, p = 0.55) or the other included biomarkers.

Conclusion This meta-analysis found that lumbar CSF levels of Phosphorylated-Tau and Total-Tau are significantly increased in shunt non-responsive iNPH compared to shunt-responsive iNPH. The other biomarkers, including amyloid-β 1–42, did not significantly differentiate shunt-responsive from shunt-non-responsive iNPH. More studies on the Tau proteins examining sensitivity and specificity at different cut-off levels are needed for a robust analysis of the diagnostic efficiency of the Tau proteins.

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Effect of topical and intraventricular antibiotics used during ventriculoperitoneal shunt insertion on the rate of shunt infection—a meta‐analysis

Acta Neurochirurgica (2022) 164:1793–1803

The ventriculoperitoneal shunt is one of the most commonly performed neurosurgical procedures. One of the avoidable complications of shunt surgery is shunt infection. This PRISMA-compliant meta-analysis analysed the effectiveness of topical and/or intraventricular antibiotics in preventing shunt infections in patients undergoing shunt surgery.

Methods Four databases were searched from inception to 30th June 2021. Only original articles comparing the rate of shunt infection with and without antibiotics were included. Random-effects meta-analysis was used to compare the effect of the use of antibiotics in preventing infection and subgroup analysis for finding differences in various antibiotics.

Results The rate of shunt infection was 2.24% (53 out of 2362) in the topical antibiotic group in comparison to 5.24% (145 out of 2764) in the control group (p = 0.008). Subgroup analysis revealed that there is no significant difference between the antibiotics used.

Conclusions Our meta-analysis found that the risk of shunt infection is significantly reduced with the use of topical and intraventricular antibiotics without any serious adverse effect. No side effects of topical or intraventricular antibiotics were reported in the included studies. Further prospective studies are required to establish the safety and optimal dosage of topical antibiotics for them to be used routinely in neurosurgical practice. They can be used in patients at high risk of developing shunt infections till such studies are available.

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Is external hydrocephalus a possible differential diagnosis when child abuse is suspected?

Acta Neurochirurgica (2022) 164:1161–1172

Criteria for diagnosing abusive head trauma (AHT) or “shaken baby syndrome” are not well defined; consequently, these conditions might be diagnosed on failing premises.

Methods The authors have collected a total of 28 infants, from the US (20) and Norway (8), suspected of having been violently shaken, and their caregivers had been suspected, investigated, prosecuted or convicted of having performed this action. Among 26 symptomatic infants, there were 18 boys (69%) and 8 girls (31%)—mean age 5.1 month, without age difference between genders.

Results Twenty-one of 26 symptomatic children (81%) had a head circumference at or above the 90 percentile, and 18 had a head circumference at or above the 97 percentile. After macrocephaly, seizure was the most frequent initial symptom in 13 (50%) of the symptomatic infants. Seventeen (65%) of the symptomatic infants had bilateral retinal haemorrhages, and two had unilateral retinal haemorrhages. All infants had neuroimaging compatible with chronic subdural haematomas/hygromas as well as radiological characteristics compatible with benign external hydrocephalus (BEH).

Conclusions BEH with subdural haematomas/hygromas in infants may sometimes be misdiagnosed as abusive head trauma. Based on the authors’ experience and findings of the study, the following measures are suggested to avoid this diagnostic pitfall: medical experts in infant abuse cases should be trained in recognising clinical and radiological BEH features, clinicians with neuro-paediatric experience should always be included in the expert teams and reliable information about the head circumference development from birth should always be available.

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Prediction of Shunt Responsiveness in Suspected Patients With Normal Pressure Hydrocephalus Using the Lumbar Infusion Test: A Machine Learning Approach

Neurosurgery 90:407–418, 2022

Machine learning (ML) approaches can significantly improve the classical Rout -based evaluation of the lumbar infusion test (LIT) and the clinical management of the normal pressure hydrocephalus.

OBJECTIVE: To develop a ML model that accurately identifies patients as candidates for permanent cerebral spinal fluid shunt implantation using only intracranial pressure and electrocardiogram signals recorded throughout LIT.

METHODS: This was a single-center cohort study of prospectively collected data of 96 patients who underwent LIT and 5-day external lumbar cerebral spinal fluid drainage (external lumbar drainage) as a reference diagnostic method. A set of selected 48 intracranial pressure/ electrocardiogram complex signal waveform features describing nonlinear behavior, wavelet transform spectral signatures, or recurrent map patterns were calculated for each patient. After applying a leave-one-out cross-validation training–testing split of the data set, we trained and evaluated the performance of various state-of-the-art ML algorithms.

RESULTS: The highest performing ML algorithm was the eXtreme Gradient Boosting. This model showed a good calibration and discrimination on the testing data, with an area under the receiver operating characteristic curve of 0.891 (accuracy: 82.3%, sensitivity: 86.1%, and specificity: 73.9%) obtained for 8 selected features. Our ML model clearly outperforms the classical Rout based manual classification commonly used in clinical practice with an accuracy of 62.5%.

CONCLUSION: This study successfully used the ML approach to predict the outcome of a 5-day external lumbar drainage and hence which patients are likely to benefit from permanent shunt implantation. Our automated ML model thus enhances the diagnostic utility ofLIT in management.

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Reducing the risks of proximal and distal shunt failure in adult hydrocephalus

J Neurosurg 136:877–886, 2022

Patient outcomes of ventriculoperitoneal (VP) shunt surgery, the mainstay treatment for hydrocephalus in adults, are poor because of high shunt failure rates. The use of neuronavigation or laparoscopy can reduce the risks of proximal or distal shunt catheter failure, respectively, but has less independent effect on overall shunt failures. No adult studies to date have combined both approaches in the setting of a shunt infection prevention protocol to reduce shunt failure. The goal of this study was to determine whether combining neuronavigation and laparoscopy with a shunt infection prevention strategy would reduce the incidence of shunt failures in adult hydrocephalic patients.

METHODS Adult patients (age ≥ 18 years) undergoing VP shunt surgery at a tertiary care institution prior to (pre–Shunt Outcomes [ShOut]) and after (post-ShOut) the start of a prospective continuous quality improvement (QI) study were compared. Pre-ShOut patients had their proximal and distal catheters placed under conventional freehand approaches. Post-ShOut patients had their shunts inserted with neuronavigational and laparoscopy assistance in placing the distal catheter in the perihepatic space (falciform technique). A shunt infection reduction protocol had been instituted 1.5 years prior to the start of the QI initiative. The primary outcome of interest was the incidence of shunt failure (including infection) confirmed by standardized criteria indicating shunt revision surgery.

RESULTS There were 244 (115 pre-ShOut and 129 post-ShOut) patients observed over 7 years. With a background of shunt infection prophylaxis, combined neuronavigation and laparoscopy was associated with a reduction in overall shunt failure rates from 37% to 14%, 45% to 22%, and 51% to 29% at 1, 2, and 3 years, respectively (HR 0.44, p < 0.001). Shunt infection rates decreased from 8% in the pre-ShOut group to 0% in the post-ShOut group. There were no proximal catheter failures in the post-ShOut group. The 2-year rates of distal catheter failure were 42% versus 20% in the pre- and post-ShOut groups, respectively (p < 0.001).

CONCLUSIONS Introducing a shunt infection prevention protocol, placing the proximal catheter under neuronavigation, and placing the peritoneal catheter in the perihepatic space by using the falciform technique led to decreased rates of infection, distal shunt failure, and overall shunt failure.

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Ventriculoatrial Shunts: Review of Technical Aspects and Complications

World Neurosurg. (2022) 158:158-164.

Diversion of cerebrospinal fluid is required in many neurosurgical conditions. When a standard ventriculoperitoneal shunt and endoscopic third ventriculostomy are not appropriate options, placement of a ventriculoatrial shunt is a safe, relatively familiar second-line shunting procedure.

Herein we reviewed the technical aspects of ventriculoatrial shunt placement using an illustrative case. We focused on the different modalities for inserting and confirming the location of the distal catheter tip. We discussed how to overcome typical difficulties and significant concerns, such as cardiac arrhythmias and venous thrombosis. In addition, we reviewed the current literature for the different complications associated with ventriculoatrial shunt placement

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Ventriculoperitoneal Shunt Drainage Increases With Gravity and Cerebrospinal Fluid Pressure Pulsations: Benchtop Model

Neurosurgery 89:1141–1147, 2021

There have been few improvements in cerebrospinal fluid (CSF) shunt technology since John Holter introduced the silicon valve, with overdrainage remaining a major source of complications.

OBJECTIVE: To better understand why valves are afflicted by supra-normal CSF flow rates. We present in Vitro benchtop analyses of flow through a differential pressure valve under simulated physiological conditions.

METHODS: The pseudo-ventricle benchtop valve testing platform that comprises a rigid pseudo-ventricle, compliance chamber, pulsation generator, and pressure sensors was used to measure flow rates through a differential pressure shunt valve under the following simulated physiological conditions: orientation (horizontal/vertical), compliance (low/medium/high), and pulsation generator force (low/medium/high).

RESULTS: Our data show that pulse pressures are faithfully transmitted from the ventricle to the valve, that lower compliance and higher pulse generator forces lead to higher pulse pressures in the pseudo-ventricle, and that both gravity and higher pulse pressure lead to higher flow rates. The presence of a valve mitigates but does not eliminate these higher flow rates.

CONCLUSION: Shunt valves are prone to gravity-dependent overdrainage, which has motivated the development of gravitational valves and antisiphon devices. This study shows that overdrainage is not limited to the vertical position but that pulse pressures that simulate rhythmic (eg, cardiac) and provoked (eg, Valsalva) physiological CSF pulsations increase outflow in both the horizontal and vertical positions and are dependent on compliance. A deeper understanding of the physiological parameters that affect intracranial pressure and flow through shunt systems is prerequisite to the development of novel valves.

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Longstanding overt ventriculomegaly in adults (LOVA) with patent aqueduct

Acta Neurochirurgica (2021) 163:3343–3352

Longstanding overt ventriculomegaly in adults (LOVA) represents a form of chronic adulthood hydrocephalus with symptomatic manifestation in late adulthood. Based on the patency of the aqueduct, two different subcohorts of LOVA can be distinguished. Surgical treatments of this condition are also debated. Therefore, we analyzed preoperative characteristics and clinical outcome after different surgical treatments in a subgroup of LOVA patients with a patent aqueduct.

Methods Eighteen LOVA patients with a patent aqueduct consecutively treated at our institution between July 2013 and December 2019 were analyzed for this study. Median age was 70 years. Preoperative radiological and clinical features, surgical procedures (ventriculo-peritoneal shunt or endoscopic third ventriculostomy), and outcomes were collected. Successful outcome was qualitatively defined as an improvement or a halt of progression of the presenting symptoms at follow-up, and quantitatively by changes in mRS and iNPHGS scales.

Results Twelve patients underwent an ETV as a primary treatment, while 6 underwent VPS. A total of 22.2% of them were lost to follow-up. Median follow-up time was 38 months. Six patients (66.7%) in the ETV cohort achieved a successful outcome after treatment, with a complication rate of 11.1%. Two patients underwent rescue VPS after ETV failure with a good outcome. Four patients (100%) underwent primary VPS and achieved a satisfactory outcome after treatment, with a reported complications rate of 25%.

Conclusion LOVA with patent aqueduct represents, in our opinion, a distinct clinical form of chronic hydrocephalus. For this subgroup, as well as for other forms of LOVA, ETV remains an acceptable first-line treatment option considering the good results, and the low complication rate, obtained in those patients and the hypothesis that hydrocephalus is due to an “intracisternal” obstruction.

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Comparison Between Flow-Regulated and Gravitational Shunt Valves in the Treatment of Normal Pressure Hydrocephalus: Flow-Grav Study

Neurosurgery 89:413–419, 2021

Normal pressure hydrocephalus (NPH) is frequently treated with ventriculoperitoneal shunt (VPS) surgery. However, VPS implantation can lead to overdrainage and complications such as headaches, hygroma, and subdural hematoma due to a siphon effect in an upright position. Gravitational valves prevent overdrainage through positiondependent adjustment of valve resistance. Flow-regulated valves that increase resistance in presence of high cerebrospinal fluid flow may provide similar protection against overdrainage and present an alternative to gravitational valves.

OBJECTIVE: To compare gravitational and flow-regulated shunt valves in patients with symptomatic NPH.

METHODS: We performed a retrospective analysis of 97 patients suffering from NPH who underwent VPS implantation with a gravitational or a flow-regulated valve. The primary endpoint was the occurrence of hygroma or subdural hematoma. Secondary endpoints were neurological outcome (Kiefer score, Stein and Langfitt score, and NPH recovery rate), frequency of valve adjustments, and reoperations.

RESULTS: No significant differences in the occurrence of hygroma and subdural hematoma (11.4% for flow-regulated valves vs 5.7% for gravitational valves, P = .462) or response to treatment (77.3% vs 81.1%, P = .802) were found. Patients with flow-regulated valves required fewer valve adjustments (1.12 vs 2.02, P < .001) to reach their optimal neurological outcome and underwent fewer surgical revisions (11.4% vs 28.3%, P = .047).

CONCLUSION: Our data suggest that shunt therapy in NPH patients with a flow-regulated instead of a gravitational valve is safe and effective with a comparable clinical outcome and risk of overdrainage complications. Moreover, patients with flow-regulated valves may need fewer valve adjustments and reoperations.

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Acute low-pressure hydrocephalus: a case series and systematic review of 195 patients

J Neurosurg 135:300–308, 2021

Acute low-pressure hydrocephalus (ALPH) is characterized by clinical manifestations of an apparent raised intracranial pressure (ICP) and ventriculomegaly despite measured ICP that is below the expected range (i.e., typically ≤ 5 cm H2O). ALPH is often refractory to standard hydrocephalus intervention protocols and the ICP paradox commonly leads to delayed diagnosis. The aim of this study was to characterize ALPH and develop an algorithm to facilitate diagnosis and management for patients with ALPH.

METHODS EMBASE, MEDLINE, and Google Scholar databases were searched for ALPH cases from its first description in 1994 until 2019. Cases that met inclusion criteria were pooled with cases managed at the authors’ institution. Patient characteristics, presenting signs/symptoms, precipitating factors, temporizing interventions, definitive treatment, and patient outcomes were recorded.

RESULTS There were 195 patients identified, with 42 local and 153 from the literature review (53 pediatric patients and 142 adults). Decreased level of consciousness was the predominant clinical sign. The most common etiologies of hydrocephalus were neoplasm and hemorrhage. While the majority of ALPH occurred spontaneously, 39% of pediatric patients had previously undergone a lumbar puncture. Prior to ALPH diagnosis, 92% of pediatric and 39% of adult patients had a ventricular shunt in situ. The most common temporizing intervention was subatmospheric CSF drainage. The majority of patients underwent a shunt insertion/revision or endoscopic third ventriculostomy as definitive ALPH treatment. Although the mortality rate was 11%, 83% of pediatric and 49% of adult patients returned to their pre-ALPH neurological functional status after definitive treatment. Outcomes were related to both the severity of the underlying neurosurgical disease causing the hydrocephalus and the efficacy of ALPH treatment.

CONCLUSIONS ALPH is an underrecognized variant phenotype of hydrocephalus that is associated with multiple etiologies and can be challenging to treat as it frequently does not initially respond to standard strategies of CSF shunting. With early recognition, ALPH can be effectively managed. A management algorithm is provided as a guide for this purpose.

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CSF shunt valve occlusion—does CSF protein and cell count matter?

Acta Neurochirurgica (2021) 163:1991–1996

Shunt obstruction is a common cause of shunt failure in the treatment of hydrocephalus. Valve occlusion is traditionally believed to originate from elevated CSF protein or cellular components, although detailed evidence is scarce and contradictory. Therefore, this study aimed to examine CSF protein and cell count as risk factors for valve obstruction.

Methods We retrospectively examined 274 patients who underwent shunt placement for hydrocephalus between 2009 and 2018 and had at least 1 year follow-up. Age, aetiology of hydrocephalus, valve type, occurrence of revision, reason for revision and CSF protein and cell count at the time of shunt insertion and revision surgery were analysed.

Results Thirty-two of 274 patients (11.7%) required revision surgery due to valve occlusion. Mean time to revision was 143 days. CSF white blood cell (WBC) count but not protein was associated with valve occlusion overall. Of all obstructed valve patients, 25% showed CSF protein level within the normal range, whereas 13.6% of the patients overall showed greatly elevated CSF protein level without evidence of valve obstruction. Persistently elevated CSF protein level at the time of shunt revision was significantly associated with valve obstruction within 90 days of initial insertion (early occlusion). Children with congenital malformations and post-haemorrhagic patients were significantly overrepresented in the occlusion group, particularly in the early occlusion group.

Conclusion Pathological CSF values such as WBC count and persistently elevated protein level serves as a risk factor for early valve obstruction. Late obstruction occurs independent of normal CSF values. Infants are particularly prone to early and late valve obstructions. CSF protein level at shunt insertion is not predictive of valve occlusion.

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Clinical significance of vitamin D in idiopathic normal pressure hydrocephalus

Acta Neurochirurgica (2021) 163:1969–1977

Although recent studies show vitamin D deficiency is associated with cognitive decline, urinary incontinence, and gait instability, there has been no study on the effect of vitamin D on idiopathic normal pressure hydrocephalus (iNPH) characterized by the classic symptom triad of cognitive decline, urinary incontinence, and gait instability. We investigated the clinical significance of vitamin D in patients with iNPH.

Methods Between 2017 and 2020, 44 patients who underwent ventriculoperitoneal shunt surgery were divided into low (< 15 ng/mL) and high (≥ 15 ng/mL) vitamin D groups according to the concentration of 25(OH)D, an effective indicator of vitamin D status. They were respectively evaluated according to clinical and radiological findings.

Results The low vitamin D group (n = 24) showed lower preoperative cognition compared to the high vitamin D group (n = 20) in terms of Korean-Mini Mental Status Examination (K-MMSE) and iNPH grading scale (iNPHGS) (K-MMSE: 20.5   5.4 versus 24.0   4.5, p = 0.041; iNPHGS cognitive score: 2   0.9 versus 1   0.6, p = 0.025). And the low vitamin D group showed pre- and postoperatively more severe urinary incontinence (preoperative iNPHGS urinary score: 1   1.0 versus 0   0.9, p = 0.012; postoperative iNPHGS urinary score:1   1.0 versus 0   0.9, p = 0.014). The score of narrow high-convexity sulci for the low vitamin D group was lower (low vitamin D group: 1   0.7 versus high vitamin D group: 2   0.4, p = 0.031).

Conclusion Lower concentration of vitamin D in iNPH may be related to lower preoperative cognition, pre- and postoperative urinary incontinence, and brain morphological change.

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Falls and Fear of Falling in Shunted Idiopathic Normal Pressure Hydrocephalus

Neurosurgery 89:122–128, 2021

Gait and balance impairment are typical symptoms of idiopathic normal pressure hydrocephalus (INPH), implicating that falls may afflict these patients.

OBJECTIVE: To investigate falls, related injuries, and associated psychological features, before and after shunt surgery for INPH and compared to the general population.

METHODS: The study included 176 patients shunted for INPH and 368 age- and sexmatched controls. Falls, fear of falling (FOF), fall-related injuries (mild-severe), confidence in avoiding falls (Swedish Falls Efficacy Scale (FES(S)), quality of life (QoL; EuroQoL 5-dimension 5 level instrument), and symptoms of depression (Geriatric Depression Scale 15) were investigated. Pre- and postoperative observational times were 12 mo before surgery and 21 mo after (mean). Recurrent fallers fell ≥2 times.

RESULTS: More INPH patients than controls were recurrent fallers (67% vs 11%; P < .001). They feared falling more often (FOF, mean   standard deviation: 3.3   1.1 vs 1.6   0.9; P < .001) and had lower confidence in avoiding falls (FES(S) 78   40 vs 126   14; P < .001). After surgery, INPH patients improved in all parameters but they did not reach the levels of the controls. Among fallers there was no difference between patients and controls in the severity of injuries suffered. Low QoL and symptoms of depression were more common among recurrent fallers than one-time or nonfallers in both shunted patients and controls (P ≤ .001).

CONCLUSION: Falls, FOF, and low confidence in avoiding falls are considerable problems in INPH that may be reduced by shunt surgery. We suggest that remaining risk of falling and preventative measures are routinely considered in postoperative follow-ups and rehabilitation planning.

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Freehand stereotactic ventricular catheter insertion for ventriculoperitoneal shunts based on individualized radio-anatomical landmarks

Acta Neurochirurgica (2021) 163:1103–1112

The accurate placement of the ventricular catheter (VC) is critical in reducing the incidence of proximal failure of ventriculoperitoneal shunts (VPSs). The standard freehand technique is based on validated external anatomical landmarks but remains associated with a relatively high rate of VC malposition. Already proposed alternative methods have all their specific limitations. Herein, we evaluate the accuracy of our adapted freehand technique based on an individualized radio-anatomical approach. Reproducing the preoperative imaging on the patient’s head using common anatomical landmarks allows to define stereotactic VC coordinates to be followed at surgery.

Material and methods Fifty-five consecutive patients treated with 56 VPS between 11/2005 and 02/2020 fulfilled the inclusion criteria of this retrospective study. Burr hole coordinates, VC trajectory, and length were determined in all cases on preoperative computed tomography (CT) scan and were accurately reported on patients’ head. The primary endpoint was to evaluate VC placement accuracy. The secondary endpoint was to evaluate the rate and nature of postoperative VC-related complications.

Results Our new technique was applicable in all patients and no VC-related complications were observed. Postoperative imaging showed VC optimally placed in 85.7% and sub-optimally placed in 14.3% of cases. In all procedures, all the holes on the VC tip were found in the ventricular system.

Conclusions This simple individualized technique improves the freehand VC placement in VPS surgery, making its accuracy comparable to that of more sophisticated and expensive techniques. Further randomized controlled studies are required to compare our results with those of the other available techniques.

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Readmission and reoperation for hydrocephalus: a population-based analysis across the spectrum of age

J Neurosurg 134:1210–1217, 2021

Hydrocephalus is a common, chronic illness that generally requires lifelong, longitudinal, neurosurgical care. Except at select research centers, surgical outcomes in the United States have not been well documented. Comparative outcomes across the spectrum of age have not been studied.

METHODS Data were derived for the year 2015 from the Nationwide Readmissions Database, a product of the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality. In this data set patients are assigned state-specific codes that link repeated discharges through the calendar year. Discharges with diagnostic codes for hydrocephalus were extracted, and for each patient the first discharge defined the index admission. The study event was readmission. Observations were censored at the end of the year. In a similar fashion the first definitive surgical procedure for hydrocephalus was defined as the index operation, and the study event was reoperation for hydrocephalus or complications. Survival without readmission and survival without reoperation were analyzed using life tables and Kaplan- Meier plots.

RESULTS Readmission rates at 30 days ranged between 15.6% and 16.8% by age group without significant differences. After the index admission the first readmission alone generated estimated hospital charges of $2.25 billion nationwide. Reoperation rates at 30 days were 34.9% for infants, 39.2% for children, 47.4% for adults, and 32.4% for elders. These differences were highly significant. More than 3 times as many index operations were captured for adults and elders as for infants and children. Estimated 1-year reoperation rates were 74.2% for shunt insertion, 63.9% for shunt revision, but only 34.5% for endoscopic third ventriculostomy. Univariate associations with survival without readmission and survival without reoperation are presented.

CONCLUSIONS In the United States hydrocephalus is predominantly a disease of adults. Surgical outcomes in this population-based study were substantially worse than outcomes reported from research centers. High reoperation rates after CSF shunt surgery accounted for this discrepancy.

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