Measuring CSF shunt flow with MRI using flow enhancement of signal intensity (FENSI).

PURPOSE: To develop and validate a noninvasive imaging technique for accurately assessing very slow CSF flow within shunt tubes in pediatric patients with hydrocephalus, aiming to identify obstructions that might impede CSF drainage. THEORY AND METHODS: A simulation of shunt flow enhancement of signal intensity (shunt-FENSI) signal is used to establish the relationship between signal change and flow rate. The quantification of flow enhancement of signal intensity data involves normalization, curve fitting, and calibration to match simulated data. Additionally, a phase sweep method is introduced to accommodate the impact of magnetic field inhomogeneity on the flow measurement. The method is tested in flow phantoms, healthy adults, intensive care unit patients with external ventricular drains (EVD), and shunt patients. EVDs enable shunt-flow measurements to be acquired with a ground truth measure of CSF drainage. RESULTS: The flow-rate-to-signal simulation establishes signal-flow relationships and takes into account the T1 of draining fluid. The phase sweep method accurately accounts for phase accumulation due to frequency offsets at the shunt. Results in phantom and healthy human participants reveal reliable quantification of flow rates using controlled flows and agreement with the flow simulation. EVD patients display reliable measures of flow rates. Shunt patient results demonstrate feasibility of the method and consistent flow rates for functional shunts. CONCLUSION: The results demonstrate the technique's applicability, accuracy, and potential for diagnosing and noninvasively monitoring hydrocephalus. Limitations of the current approach include a high sensitivity to motion and strict requirement of imaging slice prescription.

Clinical presentation and outcomes of neonates born with neural tube defects- an experience from a level III B NICU in Western India.

PURPOSE: Neural tube defects (NTDs) are one of the most common congenital anomalies and a cause of chronic disability. The study was done to study outcomes of neural tube defects admitted at a tertiary level neonatal intensive care unit (NICU) from 2018 to 2022, a period of 4 years that also coincided with the COVID pandemic. The secondary outcome was to study the clinical presentation, associated anomalies and epidemiological features. METHODS: It was a retrospective observational study; data of infants was obtained from medical records and analysis was done. RESULTS: Thirty-four neonates were enrolled, of which there were 16 (47%) males and 18 (53%) females. History of pre-pregnancy maternal folate intake was present in 4 (11.7%) cases. 33 (97%) babies were diagnosed with meningomyelocele (MMC) and one each had anencephaly, iniencephaly and encephalocele, of which one had frontal and two had occipital encephalocele. The median age of surgery was 16 days of life with primary repair being the most common procedure followed by MMC repair with VP shunt. Twenty babies (58.8%) were discharged successfully, while 9 (26.5%) expired and 5 (14.7%) were discharged against medical advice; which can be attributed to the financial problems of the patients in a developing country. The overall deaths in our series were four (26.5%) which is slightly higher than other studies which may be due to the fact that this study was conducted during the COVID era with lesser rates of folate supplementation, reduced access to prenatal diagnosis coupled with poor follow-up and compliance of patients post-surgical repair. CONCLUSION: This study emphasizes the importance of periconceptional folic acid supplementation, prenatal diagnosis, early surgery and meticulous follow-up as being pivotal to improving outcomes in children with NTDs.

Noninvasive CSF shunt patency evaluation by superb microvascular imaging.

Occlusion of a ventriculoperitoneal shunt system is a potentially life-threatening complication in patients suffering from hydrocephalus. However, there is no imaging established as standard approach in this acute setting. In the present study, we evaluate the use of superb microvascular imaging for investigation of the patency of ventriculoperitoneal shunt systems. Simulation of low flow rates of cerebrospinal fluid through a small diameter CSF shunt system was performed in a dedicated phantom in order to proof the principle of superb microvascular imaging (SMI) being feasible for the measurement of slow CSF flow through the dedicated CSF shunt system. SMI is able to detect low flow rates in CSF shunt systems effectively and fast. Visualization of a Duplex ultrasound flow and Doppler wave pattern in the VP shunt system after the reservoir has been pressed confirms patency. SMI is an effective method for evaluating CSF shunt patency and diagnosing shunt obstruction. This bears the potential to facilitate evaluation of clinically symptomatic VP shunt patients in an acute setting. Further evaluation of ultrasound flow patterns is granted.

Functional herniated choroid plexus in brain parenchyma following VP shunt removal.

We report a 4-year-old male who presented with a blocked ventriculoperitoneal (VP) shunt inserted post excision of a WHO Grade 1 cerebellar pilocytic astrocytoma complicated post-operatively by pseudo meningocoele formation. Imaging revealed choroid plexus that had herniated along the shunt tract. Subsequent MRI showed development of cystic changes around the tract. The ectopic choroid plexus was still in continuity with the ventricular ependyma and was producing CSF in the left parietal lobe.

Risk of early failure of VP shunts implanted for hydrocephalus after craniotomies for brain tumors in adults.

Risks and survival times of ventriculoperitoneal (VP) shunts implanted due to hydrocephalus after craniotomies for brain tumors are largely unknown. The purpose of this study was to determine the overall timing of VP shunting and its failure after craniotomy for brain tumors in adults. The authors also wished to explore risk factors for early VP shunt failure (within 90 days). A population-based consecutive patient cohort of all craniotomies for intracranial tumors leading to VP shunt dependency in adults (> 18 years) from 2004 to 2013 was studied. Patients with pre-existing VP shunts prior to craniotomy were excluded. The survival time of VP shunts, i.e., the shunt longevity, was calculated from the day of shunt insertion post-craniotomy for a brain tumor until the day of shunt revision requiring replacement or removal of the shunt system. Out of 4774 craniotomies, 85 patients became VP shunt-dependent (1.8% of craniotomies). Median time from craniotomy to VP shunting was 1.9 months. Patients with hydrocephalus prior to tumor resection (N = 39) had significantly shorter time to shunt insertion than those without (N = 46) (p < 0.001), but there was no significant difference with respect to early shunt failure. Median time from shunt insertion to shunt failure was 20 days (range 1-35). At 90 days, 17 patients (20%) had confirmed shunt failure. Patient age, sex, tumor location, primary/secondary craniotomy, extra-axial/intra-axial tumor, ventricular entry, post-craniotomy bleeding, and infection did not show statistical significance. The risk of early shunt failure (within 90 days) of shunts after craniotomies for brain tumors was 20%. This study can serve as benchmark for future studies.

Long-term risk of shunt failure after brain tumor surgery.

Long-term risks and survival times of ventriculoperitoneal (VP) shunts implanted due to hydrocephalus (HC) after craniotomy for brain tumors are largely unknown. The aim of this study was to establish the overall VP shunt survival rates during a decade after shunt insertion and to determine risks of shunt failure after brain tumor surgery in the long-term period. In this population-based cohort from a well-defined geographical region, all adult patients (> 18 years) from 2004 to 2013 who underwent craniotomies for intracranial tumors leading to VP shunt dependency were included. Our brain tumor database was cross-linked to procedure codes for shunt surgery (codes AAF) to extract brain tumor patients who became VP shunt dependent after craniotomy. The VP shunt survival time, i.e. the shunt longevity, was calculated from the day of shunt insertion after brain tumor surgery until the day of its failure. A total of 4174 patients underwent craniotomies, of whom 85 became VP shunt dependent (2%) afterwards. Twenty-eight patients (33%) had one or more shunt failures during their long-term follow-up, yielding 1-, 5-, and 10-year shunt success rates of 77%, 71%, and 67%, respectively. Patient age, sex, tumor location, primary/repeat craniotomy, placement of external ventricular drainage (EVD), ventricular entry, post-craniotomy hemorrhage, post-shunting meningitis/infection, and multiple shunt revisions were not statistically significant risk factors for shunt failure. Median shunt longevity was 457.5 days and 21.5 days for those with and without pre-craniotomy HC, respectively (p < 0.01). This study can serve as benchmark for future studies.

Motor skills, cognitive impairment, and quality of life in normal pressure hydrocephalus: early effects of shunt placement.

BACKGROUND: Traditionally, clinical findings of normal pressure hydrocephalus are mainly characterized by the Hakim triad. The aim of this study is to evaluate the performance of patients suffering from idiopathic normal pressure hydrocephalus (iNPH) in a more holistic manner regarding motor skills, cognitive impairment, and quality of life. METHODS: In total, 30 individuals diagnosed with iNPH as well as a reference group with another 30 individuals were included. The iNPH patients and the reference group were age, educational, and morbidity matched. A standardized test battery for psychomotor skills, gait, neuropsychological abilities as well as questionnaires for quality of life was applied. The iNPH group was tested prior to surgery, at 6 weeks, and 3 months postoperatively. The reference group was tested once. RESULTS: Patients showed a significant improved performance in various items of the test battery during the first 3 months postoperatively. This included neuropsychological evaluation, motor skills including gait and upper motor function as well as the quality of life of the patients. Compared to reference individuals, neuropsychological aspects and quality of life of iNPH patients improved in some parts nearly to normal values. CONCLUSION: Our findings underline that shunt surgery does not only improve the symptoms in iNPH patients but also ameliorates the quality of life to a great extent close to those of age and comorbidity matched reference individuals. This data enables an optimized counseling of iNPH patients regarding the expectable outcome after shunt surgery especially regarding cognitive performance, motor skills as well as life quality.

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

INTRODUCTION: 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.

Fully percutaneous fetoscopic repair of myelomeningocele: 30-month follow-up data.

OBJECTIVE: This observational study reports on the postnatal mortality and 30-month outcome of children who underwent fully percutaneous fetoscopic repair of myelomeningocele (MMC) at a single center in Giessen, Germany. METHODS: Between October 2010 and August 2014, a total of 72 patients underwent fully percutaneous fetoscopic MMC closure at 21 + 0 to 29 + 1 (mean, 23 + 5) weeks' gestation. Of these, 52 (72%) participated in this study; however, 30-month mortality data are available for all 72 children. Children were examined at four timepoints: shortly after birth and at 3 months, 12 months and 30 months of corrected age. The patients underwent age-specific standardized neurological examinations and assessment of leg movements and ambulation at all timepoints. Cognitive and motor development were assessed using the Bayley Scales of Infant Development, second edition (BSID-II), at 30 months. RESULTS: All 72 children survived the intrauterine procedure, however, four (5.6%) infants died postnatally (including two of the 52 comprising the study cohort). Of the 52 patients included in the study, 11.5% were delivered before the 30th week of gestation (mean, 33 + 1 weeks) and, of the survivors, 48.1% had ventriculoperitoneal shunt placement. Of the 50 infants that were alive at 30 months, independent ambulation, without orthosis, was feasible for 46%. At 30 months of follow-up, 46% of children presented with a functional level that was at least two segments better than the anatomical level of the lesion. At 30 months, 70% of the children presented with BSID-II psychomotor development index score of ≥ 70 and 80% with BSID-II mental development index score of ≥ 70. CONCLUSION: Intrauterine repair of MMC by percutaneous fetoscopy shows largely similar outcomes to those reported for open repair, with respect to mortality, prematurity, shunt-placement rates, motor and mental development and free ambulation. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

A CSF pseudocyst.

A 51-year-old female with prior history of ventriculoperitoneal shunt presented with worsening abdominal distension. Her abdomen was diffusely tender and firm with a slight fluid wave. CT imaging with IV contrast was notable for a large cystic lesion in the abdominal cavity with the differential of CSF pseudocyst versus ovarian mass. She underwent paracentesis of the cyst with interventional radiology and required a revision of her ventriculoperitoneal shunt. CSF pseudocysts are a rare complication of ventriculoperitoneal shunts, however, are an important consideration in patients presenting with abdominal complaints and require specialized intervention and assessment.

Case report of mesenteric strangulation secondary to longstanding ventriculoperitoneal shunt catheter.

BACKGROUND: Ventriculoperitoneal (VP) shunts are the most common treatment for hydrocephalus in both pediatric and adult patients. Complications resulting from the abdominal portion of shunts include tube disconnection, obstruction of the shunt tip, catheter migration, infection, abdominal pseudocysts, and bowel perforation. However, other less common complications can occur. The authors present a unique case of a patient with a longstanding VP shunt presenting with an acute abdomen secondary to knotting of the peritoneal portion of the catheter tubing. CASE DESCRIPTION: A 13-year-old male with past medical history significant for myelomeningocele, requiring ventriculoperitoneal shunt placement at 18 months of age, presented to an outside hospital with chief complaint of abdominal pain. Cross-sectional imaging revealed spontaneous knot formation within the shunt tubing around the base of the small bowel mesentery. He was then transferred to our facility for general and neurosurgical evaluation. His abdominal exam was notable for diffuse distension in addition to tenderness to palpation with guarding and rebound. Given his tenuous clinical status and peritonitis, he was emergently booked for abdominal exploration. He underwent bowel resection, externalization of his shunt, with later re-anastomosis and shunt internalization. He eventually made a full recovery. DISCUSSION: Given the potential for significant bowel loss with this and other shunt-related complications, this case serves as a reminder that even longstanding VP shunts should be considered in the differential diagnosis of abdominal pain in any patient with a shunt.

"Shunt Pumping Test": role of practice on an experimental model.

PURPOSE: To ascertain the benefits of practicing shunt pumping test on a validated experimental model. METHODS: A validated experimental model of shunt was used and 25 medical professionals were asked to assess the block in the model where artificial blocks were created. The assessment was repeated after the participants had practiced on the same model. The performance of participants before and after practice was compared and statistically evaluated. RESULTS: The ability to predict the status of shunt showed an improvement in all scenarios after practice. The odds ratio for predicting a blocked shunt before and after practice was 7.25 (95% credible interval: 1.50-21.01). The odds ratio for predicting a functional shunt before and after practice was 8.81 (95% credible interval of 1.64 to 13.65). CONCLUSION: Practicing on the experimental model significantly improves the ability to predict the status of shunt. Training of primary caregivers on similar shunt models based on the shunts used in respective centers can improve an early detection of shunt block and reduce reliance on more invasive and expensive evaluation modalities.

"Shunt pumping test": detecting its efficacy through an experimental model.

PURPOSE: Shunt pumping test has often been used clinically to detect functional status of ventriculoperitoneal (VP) shunt. Its ability to correctly predict the status is not reliably known. Ethical dilemmas make it difficult to perform any studies in patients with blocked shunts, and hence, a requirement of devising an experimental model was felt. METHOD: An experimental model was devised using a Chhabra Slit N Spring shunt. The pressures were maintained in the proximal and distal chamber by real-time monitoring and maintained similar to intra-ventricular and intra-abdominal pressures. Three such models with scenarios of proximal block (PB), distal block (DB), and a functional shunt (BO) were created. Twenty-five participants were tested using these models to assess the efficacy of shunt pumping test. RESULTS: The experimental model could be used successfully to perform the test. The sensitivity of the test to detect a shunt with block on any side (AB) was found to be 0.79 (95% confidence interval 0.72-0.85) and specificity to be 0.69 (95% confidence interval 0.59-0.80). Its ability to detect the side of block was also evaluated. Absolute correctness value, odds ratio, and interpersonal heterogeneity were also evaluated. Pressure changes in proximal and distal catheter on compressing the chamber in various scenarios were recorded. CONCLUSION: The shunt pumping test has moderate ability to predict a blocked shunt and can aid clinical assessment of shunt block. It has only limited ability to detect the side of block. Pressure changes in the proximal and distal catheters on chamber compression are commensurate with the rationale of "shunt pumping test."

Megalencephaly-capillary malformation syndrome and associated hydrocephalus: treatment options and revision of the literature.

PURPOSE: We describe our series of 4 patients with megalencephaly-capillary malformation syndrome (MCAP) and review the literature in order to assess the optimal treatment for the associated hydrocephalus. METHODS: We review our institutional series of hydrocephalus associated with MCAP and review the literature, analyzing the causes that could originate the hydrocephalus and the different types of treatments proposed for them. RESULTS: Of our patients treated with ventriculoperitoneal (VP) shunt, one suffered a surgical revision of the shunt and died due to a cranial trauma unrelated to her syndrome or the previous shunt surgery, and the other did not undergo surgical revisions until the end of her follow-up. Our patients treated with endoscopic third ventriculostomy (ETV) have improved their symptomatology and have not suffered of any complications related to the hydrocephalus after the ETV surgery. CONCLUSIONS: We update the treatment of MCAP-associated hydrocephalus and propose ETV as a valid treatment, as it seems a safe procedure with a low rate of complications.

Surgical management of raised intracranial pressure secondary to otogenic infection and venous sinus thrombosis.

PURPOSE: This study reviews paediatric patients with raised intracranial pressure as a result of venous sinus thrombosis secondary to otogenic mastoiditis, requiring admission to the paediatric neuroscience centre at the University Hospital Wales, Cardiff. The consensus regarding the management of otogenic hydrocephalus in the published literature is inconsistent, with a trend towards conservative over surgical management. We reviewed our management of this condition over a 9-year period especially with regard to ventriculo-peritoneal (VP) shunting. METHODS: Analysis of a prospectively collected database of paediatric surgical patients was analysed and patients diagnosed with otogenic hydrocephalus from November 2010 to August 2018 were identified. Our data was compared with the published literature on this condition. RESULTS: Eleven children, 7 males and 4 females, were diagnosed with otogenic hydrocephalus over the 9-year period. Five (45.5%) required VP shunt insertion to manage their intracranial pressure and protect their vision. The remaining six patients (54.5%) were managed medically. CONCLUSIONS: When children with mastoiditis and venous sinus thrombosis progress to having symptoms or signs of raised intracranial pressure, they should ideally be managed within a neuroscience centre. Of those children, almost half will need permanent cerebrospinal fluid diversion to protect their sight.

Revision surgery following CSF shunt insertion: how often could it be avoided?

BACKGROUND: Cerebrospinal fluid (CSF) shunt revision surgery represents a huge social and economic burden. Few studies, however, have evaluated shunt revision surgeries in the context of their avoidability, and existing data are from paediatric populations. Using ratings from an expert panel, we classified avoidable and unavoidable shunt revisions in a mixed cohort of CSF-shunt patients. METHODS: In a retrospective review of a prospectively maintained, single-centre database, we identified all shunt systems implanted for the first time over a 10-year period (2007-2016) and all subsequent revision surgeries with a follow-up of at least 1 year. A panel of five expert shunt surgeons classified each revision surgery as avoidable or unavoidable. Rates of each were calculated and correlated with clinical data. RESULTS: Of 210 revision surgeries (314 patients, mean age, 49.9 years; mean follow-up, 4.2 years), the panel judged 114 as unavoidable (54.3%) and 96 (45.7%) as avoidable. Level of surgeon education correlated with these rates, but even in the most experienced hands, 12.5% of revisions were classified as avoidable. Avoidable revisions occurred significantly earlier than unavoidable interventions (mean; 112 and 448 days, respectively) after the index surgery. CONCLUSION: Rates of avoidable shunt revision surgery are alarmingly high, even in experienced hands. Avoidable revisions occur significantly earlier, predominantly within the first 3 months after the index surgery.

Ventriculoperitoneal shunt in treating of idiopathic normal pressure hydrocephalus-single-center study.

OBJECT: Idiopathic normal pressure hydrocephalus (iNPH) is the only variant of dementia disorders possibly treatable by neurosurgical intervention. iNPH is a neurodegenerative condition clinically characterized by gait ataxia, urinary incontinence, and memory disturbance. We present one of the largest single-center studies, which was designed to prove efficacy of our low-pressure setting of gravitational valve at all three symptoms of iNPH and to find statistically significant cut-off time for best clinical improvement according to the duration of symptoms. METHODS: Sixty-one consecutive patients (mean age 74.9 ± 5.3) with iNPH were prospectively observed from the time of surgery with minimal 6 months follow-up. All patients underwent implantation of the same type of gravitational valve with the same setting-pro GAV with low opening pressure at 5 cm H2O-and were operated by the same team of 2 neurosurgeons. We statistically evaluated gait disturbance, psychological changes, and incontinence preoperatively and at 6 months after surgery and timing of the surgery according to the duration of symptoms and to the age. RESULTS: Paired t test showed a statistically significant increase in MMSE, a statistically significant decrease in 10-m walk test and 360 deg. rotation test (p < 0.0001). The correlation among the change of the MMSE, the walk test, and the rotation test, and the age and time of symptoms' duration was verified by Pearson's correlation coefficient. Pearson's correlation coefficient showed a medium strong correlation between the change of MMSE and the time of symptoms (r = - 0.580; p < 0.0001) and between the change of the number of steps and the time of symptoms (r = 0.517, p < 0.0001). There was a statistically significant weak (poor) correlation between the change of the walk test and the time of symptoms (r = 0.351, p = 0.006). All 3 ROC tests confirmed optimal cut-off for the best improvement of symptoms as 9.5 months of the symptom duration. CONCLUSIONS: We proved statistical significant optimal cut-off for the best improvement of the symptoms as 9.5 months of the symptom duration. This study also confirmed successful treatment of iNPH with VP shunting using low pressure setting of gravitational valve with overall improvement in 75% and low over drainage complications in 5% We proved statistically significant increase in MMSE, decrease in 10 m walk test and number of steps test, p < 0.0001.

Spreading of struma ovarii tumour via a lumbo-peritoneal shunt: first case report and short review of the literature.

Neurosurgical shunts occasionally act as a conduit for seeding of central nervous system tumours to the abdomen. Retrograde spread of extra-neural tumours to the central nervous system is exceedingly rare. We report the first case of an abdominal primary tumour spreading to the spinal cord via a lumbo-peritoneal shunt. This is also the first case report of a struma ovarii tumour metastasis to the central nervous system via any route.

Intraventricular Hemorrhage and Posthemorrhagic Ventricular Dilation: Current Approaches to Improve Outcomes.

Intraventricular hemorrhage (IVH) and posthemorrhagic ventricular dilation (PHVD) are important complications of prematurity with short- and long-term implications for the patient and for nursing care. Several approaches have been shown to reduce the incidence of IVH and, more recently, mitigate the impact of IVH on long-term neurodevelopment. This article discusses the pathophysiology of IVH, with a focus on prevention strategies. Posthemorrhagic ventricular dilation is a common complication of severe IVH and has implications for neurodevelopmental sequelae. Both surgical and nonsurgical interventions for PHVD are described.

Late-onset hydrocephalus in a child with Joubert syndrome: a case report.

INTRODUCTION: The ciliopathy "Joubert syndrome" was first described in 1969 by Dr. Marie Joubert and most subtypes follow an autosomal recessive inheritance. The complex disorder shows typical clinical features, such as hyperventilation, abnormal eye movements, and retardation. A pathognomonic midbrain-hindbrain malformation, the molar tooth sign, can be found on magnetic resonance imaging of the brainstem. There are a little more than 200 reports of Joubert syndrome in the literature. CASE PRESENTATION: We report a case of a 9-year-old boy who developed a progressive hydrocephalus starting from the age of 4. He underwent VP shunt placement at 8 years, which relieved hydrocephalus-related clinical symptoms and put development of the macrocephalus to a halt. CONCLUSION: Neonatal hydrocephalus due to the altered anatomy of the posterior fossa has been reported earlier, but to our knowledge, this is the first case of a delayed onset of hydrocephalus in a patient with Joubert syndrome.