Widespread haemorrhages in infants post-shunting (WHIPS): clinical features, risk factors and neuroimaging characteristics of a rare and under-recognised phenomenon.

PURPOSE: Infants undergoing CSF shunting procedures face a rare complication which we propose to rename "Widespread Haemorrhages in Infants Post-Shunting" (WHIPS) to better capture this unique phenomenon specific to infants undergoing CSF diversion. Our objective is to analyse the risk factors for WHIPS development and provide a detailed neuroradiological description of these haemorrhages. MATERIALS AND METHODS: A radiology information system (RIS) was searched using the search terms "shunt" and/or "catheter" and/or "drain" and/or "ventriculoperitoneal" and/or "VP" between September 2008 to January 2021 for patients < 12 months of age. Clinical data was compiled for each patient meeting the inclusion criteria. Included cases were reviewed by three radiologists for the presence of WHIPS with calculation of the bifrontal ratio and documenting haemorrhage number, morphology, location and lobar distribution. RESULTS: 51 patients met inclusion criteria, 8 WHIPS patients and 43 controls. There was a statistically significant correlation between a larger post-op head circumference and WHIPS (p = 0.04). WHIPS was associated with post-haemorrhagic hydrocephalus and post-infectious hydrocephalus (p = 0.009). WHIPS were identified in the cortico-subcortical regions, periventricular white matter, and deep white matter. Haemorrhages were either punctate, ovoid or confluent. Haemorrhages ranged from single to innumerable. CONCLUSIONS: WHIPS represent a rare and under-recognised complication of CSF shunting unique to the infantile population. We postulate deep and superficial medullary venous haemorrhage as an underlying mechanism related to disordered intracranial hydrodynamics which are exacerbated in the infantile population due to underdeveloped arachnoid granulations and a compliant skull.

Shunting for idiopathic normal pressure hydrocephalus.

BACKGROUND: Normal pressure hydrocephalus (NPH) occurs when the brain ventricles expand, causing a triad of gait, cognitive, and urinary impairment. It can occur after a clear brain injury such as trauma, but can also occur without a clear cause (termed idiopathic, or iNPH). Non-randomised studies have shown a benefit from surgically diverting ventricular fluid to an area of lower pressure by cerebrospinal fluid (CSF)-shunting in iNPH, but historically there have been limited randomised controlled trial (RCT) data to confirm this. OBJECTIVES: To determine the effect of CSF-shunting versus no CSF-shunting in people with iNPH and the frequency of adverse effects of CSF-shunting in iNPH. SEARCH METHODS: We searched the Cochrane Dementia and Cognitive Improvement Group's register, Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (Ovid SP), Embase (Ovid SP), PsycINFO (Ovid SP), CINAHL (EBSCOhost), Web of Science Core Collection (Clarivate), LILACS (BIREME), ClinicalTrials.gov, and the World Health Organization International Clinical Trials Registry Platform on 15 February 2023. SELECTION CRITERIA: We included only RCTs of people who had symptoms of gait, cognitive, or urinary impairment with communicating hydrocephalus (Evans index of > 0.3) and normal CSF pressure. Control groups included those with no CSF shunts or those with CSF shunts that were in 'inactive' mode. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methodological procedures. Where necessary, we contacted study authors requesting data not provided in the papers. We assessed the overall certainty of the evidence using GRADE. MAIN RESULTS: We included four RCTs, of which three were combined in a meta-analysis. The four RCTs included 140 participants (73 with immediate CSF-shunting and 67 controls who had delayed CSF-shunting) with an average age of 75 years. Risk of bias was low in all parallel-group outcomes evaluated apart from gait speed, cognitive function (general cognition and Symbol Digit Test) (some concerns) and adverse events, which were not blind-assessed. CSF-shunting probably improves gait speed at less than six months post-surgery (standardised mean difference (SMD) 0.62, 95% confidence interval (CI) 0.24 to 0.99; 3 studies, 116 participants; moderate-certainty evidence). CSF-shunting may improve qualitative gait function at less than six months post-surgery by an uncertain amount (1 study, 88 participants; low-certainty evidence). CSF-shunting probably results in a large reduction of disability at less than six months post-surgery (risk ratio 2.08, 95% CI 1.31 to 3.31; 3 studies, 118 participants; moderate-certainty evidence). The evidence is very uncertain about the effect of CSF-shunting on cognitive function at less than six months post-CSF-shunt surgery (SMD 0.35, 95% CI -0.04 to 0.74; 2 studies, 104 participants; very low-certainty evidence). The evidence is also very uncertain about the effect of CSF-shunt surgery on adverse events (1 study, 88 participants; very low-certainty evidence). There were no data regarding the effect of CSF-shunting on quality of life. AUTHORS' CONCLUSIONS: We found moderate-certainty evidence that CSF-shunting likely improves gait speed and disability in iNPH in the relative short term. The evidence is very uncertain regarding cognition and adverse events. There were no longer-term RCT data for any of our prespecified outcomes. More studies are required to improve the certainty of these findings. In addition, more information is required regarding patient ethnicity and the effect of CSF-shunting on quality of life.

Antibiotic impregnated catheters and intrathecal antibiotics for CSF shunt infection prevention in children undergoing low-risk CSF shunt surgery.

BACKGROUND: Cerebrospinal fluid (CSF) shunts allow children with hydrocephalus to survive and avoid brain injury (J Neurosurg 107:345-57, 2007; Childs Nerv Syst 12:192-9, 1996). The Hydrocephalus Clinical Research Network implemented non-randomized quality improvement protocols that were shown to decrease infection rates compared to pre-operative prophylactic intravenous antibiotics alone (standard care): initially with intrathecal (IT) antibiotics between 2007-2009 (J Neurosurg Pediatr 8:22-9, 2011), followed by antibiotic impregnated catheters (AIC) in 2012-2013 (J Neurosurg Pediatr 17:391-6, 2016). No large scale studies have compared infection prevention between the techniques in children. Our objectives were to compare the risk of infection following the use of IT antibiotics, AIC, and standard care during low-risk CSF shunt surgery (i.e., initial CSF shunt placement and revisions) in children. METHODS: A retrospective observational cohort study at 6 tertiary care children's hospitals was conducted using Pediatric Health Information System + (PHIS +) data augmented with manual chart review. The study population included children ≤ 18 years who underwent initial shunt placement between 01/2007 and 12/2012. Infection and subsequent CSF shunt surgery data were collected through 12/2015. Propensity score adjustment for regression analysis was developed based on site, procedure type, and year; surgeon was treated as a random effect. RESULTS: A total of 1723 children underwent initial shunt placement between 2007-2012, with 1371 subsequent shunt revisions and 138 shunt infections. Propensity adjusted regression demonstrated no statistically significant difference in odds of shunt infection between IT antibiotics (OR 1.22, 95% CI 0.82-1.81, p = 0.3) and AICs (OR 0.91, 95% CI 0.56-1.49, p = 0.7) compared to standard care. CONCLUSION: In a large, observational multicenter cohort, IT antibiotics and AICs do not confer a statistically significant risk reduction compared to standard care for pediatric patients undergoing low-risk (i.e., initial or revision) shunt surgeries.

Hydrocephalus in prematurity: does valve choice make a difference?

PURPOSE: Extremely premature neonates diagnosed with post-haemorrhagic hydrocephalus (PHH) are recognised to have particularly poor outcomes. This study assessed the impact of a number of variables on outcomes in this cohort, in particular the choice of shunt valve mechanism. METHODS: Electronic case notes were retrospectively reviewed of all premature neonates admitted to our centre for management of hydrocephalus between 2012 and 2021. Data included (i) gestational age, (ii) birth weight, (iii) hydrocephalus aetiology, (iv) surgical intervention, (v) shunt system, (vi) 'surgical burden' and (vii) wound failure and infection rate. Data was handled in Microsoft Excel and statistical analysis performed in SPSS v27.0 RESULTS: N = 53 premature hydrocephalic patients were identified (n = 28 (52.8%) female). Median gestational age at birth was 27 weeks (range: 23-36 + 6 weeks), with n = 35 extremely preterm patients and median birth weight of 1.9 kg (range: 0.8-3.6 kg). Total n = 99 programmable valves were implanted (n = 28 (28.3%) de novo, n = 71 (71.2%) revisions); n = 28 (28.3%) underwent n ≥ 1 pressure alterations, after which n = 21 (75%) patients had symptoms improve. In n = 8 patients exchanged from fixed to programmable valves, a mean reduction of 1.9 revisions per patient after exchange was observed (95%CI: 0.36-3.39, p = 0.02). Mean overall shunt survival was 39.5 weeks (95%CI: 30.6-48.5); 33.2 weeks (95%CI: 25.2-41.1) in programmable valves and 35.1 weeks (95%CI: 19.5-50.6) in fixed pressure (p = 0.22) with 12-month survival rates of 25.7% and 24.7%, respectively (p = 0.22). Shorter de novo shunt survival was associated with higher operation count overall (Pearson's R: - 0.54, 95%CI: - 0.72 to - 0.29, p < 0.01). Wound failure, gestational age and birth weight were significantly associated with shorter de novo shunt survival in a Cox regression proportional hazards model; gestational age had the greatest impact on shunt survival (Exp(B): 0.71, 95%CI: 0.63-0.81, p < 0.01). CONCLUSION: Hydrocephalus is especially challenging in extreme prematurity, with a shorter de novo shunt survival associated with higher number of future revisions. Programmable valves provide flexibility with regard to pressure setting, with the potential for fewer shunt revisions in this complex cohort.

Positional shunt assist for slit ventricle syndrome.

PURPOSE: Hydrocephalus is one of the most common pathologies in pediatric neurosurgery. One of the causes of recurring events of headaches among shunted children is "slit ventricle syndrome" (SVS). Several potential treatments have been proposed, yet SVS often represents a treatment challenge. The goal of the current series is to present our experience with adding a positional shunt-assist (SA) (Miethke, Aesculap) for the treatment of SVS. METHODS: Clinical data was retrospectively collected from all consecutive children with SVS that were treated with SA (Miethke, Aesculap) at our center. Surgical and clinical outcomes as expressed by hospital visits, or need for additional surgery, were evaluated. RESULTS: Nine cases were included. Hydrocephalus etiology included IVH (6), postinfectious (1), and congenital syndromes (2). Average age at first shunt was 4 months. Primary shunt type was differential-pressure-valve in all. Average age at SVS onset was 4 years. Average age at SA placement was 5.5 years. There were no perioperative complications besides a single stich abscess. A 6-month follow-up period after SA was compared to a 6-month period prior to the SA: average hospital visits decreased from 2.4 to 0.6 per patient (p < 0.0002). 4/9 patients needed an LP or shunt revision before the SA surgery, while no procedure was indicated during the immediate 6-month follow-up. At the last follow-up, there was a significant reduction in the rate of ER visits compared to prior to surgery; however, the number of neurosurgical procedures did not significantly differ. CONCLUSION: Using a SA for SVS was associated with a short-term improvement of symptoms in the majority of cases, reduction in hospital visits, and reduced need for SVS-related procedures.

Experience of a reference center on ventriculo-gallbladder shunt as an alternative treatment for peritoneal failure in children.

PURPOSE: Ventriculo-peritoneal shunt is the gold standard for non-obstructive hydrocephalus. Despite advances in material, infection prevention, and valve technologies, failure can still occur. The aim of this article is to present a comprehensive study based on the experience of a reference center in pediatric neurosurgery in Rio de Janeiro with the use of the ventriculo-gallbladder shunt as an alternative to peritoneal failure. METHODS: A retrospective study was conducted from January 2018 to December 2023 of patients diagnosed with cerebrospinal fluid shunt dysfunction due to peritoneal failure and submitted to ventriculo-gallbladder shunt as an alternative in a reference center of Rio de Janeiro. RESULTS: From 2018 to 2023, 18 peritoneal failures were diagnosed. Among them, 10 patients (55.5%) were selected for ventriculo-gallbladder shunt (VGS). Different causes were responsible for the hydrocephalus in these patients. VGS was placed at a mean age of 35.4 months. Four patients had temporary complications: 2 self-limited diarrheas in the first month and 2 shunt infections. After the resolution of the infection, a new VGS was placed successfully. The average follow-up was 18.8 months (follow-up 9-68 months) without further issues. CONCLUSION: VGS is a viable option for patients facing peritoneal failure. This paper provides valuable insights into the surgical technique and outcomes associated with this alternative.

Comparative study between ventriculosubgaleal shunt and external ventricular drain for management of post infective hydrocephalus among pediatrics.

PURPOSE: Post infective hydrocephalus (PIH) is a type of hydrocephalus which occurs after an infection of the brain or cerebrospinal fluid (CSF). Treatment of PIH requires temporary measures such as external ventricular drain (EVD) and ventriculosubgaleal shunt (VSGS) until CSF becomes clear and ready to implement VP shunt. Limited research has been done to explore the tradeoff between these approaches particularly in pediatric PIH patients. Our study compares the complications, mortality rates, and the cost of used resources of both procedures. METHODS: A prospective study was conducted for 18 months in which we compared between VSGS and EVD for management of PIH involving 42 randomized cases with 21 patients in group A operated by VSGS and 21 patients in group B operated by EVD. RESULTS: Our results show a statistically significant difference between both groups in the duration of implementation of VSGS/EVD until resolution of infection occurs. Additionally, a higher rate of pediatric intensive care unit (PICU) admission and a longer length of hospital stay (LOS) were recorded among the EVD group. No statistically significant difference between the number of complications that happened in both despite variations in their forms. Moreover, both groups showed nearly similar mortality rates. CONCLUSION: There is no significant difference in the rate of complications between VSGS and EVD for PIH. Based on that, VSGS emerges as a favorable and cost-effective option for the management of PIH which leads to less economic burden on patients and the country's health resources, especially in developing countries.

Seizures as presentation of shunt malfunction: tertiary paediatric neurosurgery experience.

AIM: Patients with a background of cerebrospinal fluid (CSF)-diverting shunts are frequently investigated for shunt malfunction when presenting with seizures. However, there is very limited evidence in the literature regarding the association of seizures and shunt malfunction. We sought to determine the incidence of shunt malfunction in our cohort of shunted paediatric patients presenting with seizures, and the utility of seizures as a marker of shunt malfunction. METHODS: We retrospectively identified all shunted patients presenting with seizures, as well as all patients undergoing shunt revision following a presentation with seizures from our hospital database over a 14-year period from 2009 to 2023. Data gathered included demographics, de novo seizures or change in pattern of seizures, the aetiology of hydrocephalus and the segment of shunt requiring revision. Exclusion criteria included infected cases requiring shunt externalisation. A literature review of all papers discussing seizures as a presentation of shunt malfunction was also carried out. RESULTS: Overall, over a 14-year period of study, 338 shunted patients presented with seizures and were referred as suspected shunt malfunction with 10 having confirmed shunt malfunction requiring revision (2.9%). This group represented 6.2% of 161 cases of shunt revision carried out during the 14-year period of study. Post-haemorrhagic hydrocephalus secondary to prematurity was the commonest aetiology of shunted hydrocephalus presenting with seizures. Out of 10 patients presenting with seizures with shunt malfunction, 4 presented with de novo seizures, while 6 presented with a change in seizure pattern or frequency in already known epileptic patients. Shunt revision surgeries included 5 distal catheter, 2 proximal catheter, 1 proximal catheter-valve, 1 valve only and 1 case of whole shunt change. CONCLUSION: Our data supports that seizures are rare manifestation of shunt malfunction and can present either de novo or with a change in seizure frequency in already-known epileptic patients.

Risk factors of shunt-dependent hydrocephalus after subarachnoid hemorrhage: a systematic review and meta-analysis based on observational cohort studies.

Shunt dependent hydrocephalus (SDHC) is a common sequel after aneurysmal subarachnoid hemorrhage (aSAH) and factors contributing to the development of SDHC remain obscure. The aim of this study was to identify predictors of SDHC following aSAH. We conducted a systematic review based on the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines. We searched electronic databases including Pubmed, Embase, and Cochrane databases from 1980 through August 2019 for studies on the risk factors of SDHC after aSAH. Inclusion criteria were: (1) SAH and hydrocephalus confirmed by CT or magnetic resonance imaging findings; (2) the odds ratios (ORs) or the relative risk (RR) with 95% confidence interval (95%CI; or crude data that allowed their calculation) were reported; and (3) languages were restricted to English and Chinese. Two independent authors collected the data including study design, characteristics of patients and potential risk factors. Random-effects models were used to estimate weighted mean differences (WMD), relative risks (RR) with corresponding 95% confidence intervals (CI). For analysis with significant heterogeneity, subgroup analyses stratified by study design and geographic area were performed. In all, 37 cohort studies met inclusion criteria. Several factors were associated with SDHC. Infection, acute hydrocephalus, placement of external ventricular drainage, older age, higher Hunt and Hess grade, intraventricular hemorrhage, rebleeding, and mechanical ventilation were associated with greater 2-fold increased risk of SDHC. Vasospasm, female gender, high Fisher grade, preexisting hypertension, aneurysm in posterior location and intracerebral hemorrhage were associated with less than 2-fold increased risk. Treatment modality and diabetes mellitus were not associated with SDHC. SDHC is a multi-factorial disease that is associated with patient and treatment factors. Acknowledgement of these potential factors could help prevent SDHC.

The Lost and Variable Cause: A Systematic Review of Shunt-Related Craniosynostosis Occurrence.

OBJECTIVE: Shunt-related craniosynostosis (SRC) is the premature fusion of cranial sutures possibly due to a loss of tension across dura and suture lines after placement of a shunt for hydrocephalus. As modifications in approaches toward shunting represent a modifiable risk factor, prior literature has investigated the determinants and outcomes. However, the data remain highly variable and are limited by single-institution studies. METHODS: A systematic search of PubMed, Embase, and Web of Science from inception to February 2022 was conducted. Studies were screened by 2 reviewers for eligibility based on predefined inclusion/exclusion criteria. RESULTS: In the 9 included articles, the average follow-up time for the entire cohort ranged from 1.5 to 4.2 years. The pooled incidence of SRC across all 9 studies was 6.5% (140/2142), with an individual range of 0.53% (1/188) to 48.8% (61/125). The average time from shunt placement to SRC diagnosis ranged from 0.25 years to 4.6 years. 61% (65/110) of cases included only one suture, 88% (25/28) of these involved the sagittal suture, and those cases with multiple fusions also had 98% involvement of the sagittal suture (45/46). Overall, 94% (1783/1888) of patients had a fixed shunt placed. CONCLUSIONS: Shunt-related craniosynostosis is likely an underreported complication in the treatment of hydrocephalus. Older age at shunt placement, increased number of shunt revision procedures, and lower valve pressure settings may be risk factors for SRC development. Results also indicate that craniosynostosis can develop months to years after shunting. Future quality studies with standardization of data reporting processes are warranted to investigate this clinical problem.

Migration of atrial catheter of ventriculo-cysto-atrial shunt into heart and pulmonary artery - case report and literature review.

Ventriculoatrial shunts are the alternative treatments when it is impossible to use ventriculoperitoneal shunts. Limited indication for ventriculoatrial shunt is due to the possibility of very serious complications inherent with this procedure. We present a case report of a young patient who suffered from disconnection of an atrial catheter from the valve after an accidental blow to his neck. The atrial catheter was dislocated to the heart and pulmonary artery and it was extracted through the femoral vein in the groin area using an endovascular technique. The procedure went without complications. A new atrial catheter was introduced under ultrasonic guidance during surgical revision.

Postoperative course of cerebrospinal fluid diversion in the setting of leptomeningeal disease: a systematic review, meta-analysis, and meta-regression with an illustrative case.

BACKGROUND: Management of hydrocephalus symptoms in the setting of leptomeningeal disease (LMD) includes cerebrospinal fluid (CSF) diversion, which can in the form of ventriculoperitoneal shunting (VPS) and lumboperitoneal shunting (LPS). However, the quantifiable postoperative course following this intervention is poorly defined. Correspondingly the aim of our study was to quantitatively define and analyze the pooled metadata regarding this topic. METHODS: Multiple electronic databases from inception to March 2023 were searched following PRISMA guidelines. Respective cohort-level outcomes were then abstracted and pooled by means of meta-analyses and analyzed by means meta-regression, both utilizing random-effects modeling. Post-hoc bias evaluation was then performed for all outcomes. RESULTS: A total of 12 studies were identified for inclusion, describing 503 LMD patients managed by CSF diversion - 442 (88%) by VPS and 61 (12%) by LPS. Median male percentage and age at diversion were 32% and 58 years respectively, with lung and breast cancer the most common primary diagnoses. Meta-analysis demonstrated pooled incidence of symptom resolution in 79% (95% CI 68-88%) of patients after index shunt surgery, and shunt revision required in 10% (95% CI 6-15%) of cases. Pooled overall survival from index shunt surgery was 3.8 mo (95% CI 2.9-4.6 mo) across all studies. Meta-regression demonstrated that studies published later trended towards significantly shorter overall survival from index shunt surgery (co-efficient=-0.38, P = 0.023), whereas the proportion of VPS to LPS in each study did not impact survival (P = 0.89). When accounting for these biases, overall survival from index shunt surgery was re-estimated to be shorter 3.1 mo (95% CI 1.7-4.4 mo). We present an illustrative case demonstrating the course of symptom improvement, shunt revision and an overall survival of 2 weeks from index CSF diversion. CONCLUSION: Although CSF diversion in the setting of LMD can improve hydrocephalus symptoms in the majority of patients, there is a non-negligible proportion that will require shunt revision. Postoperatively, the prognosis of LMD remains poor irrespective of shunt type, and despite possible biases within the current literature, the expected median overall survival after index surgery is a matter of months. These findings support CSF diversion as an effective palliative procedure when considering symptoms and quality of life. Further research is required to understand how postoperative expectations can be managed to respect the best wishes of patients, their family, and the treating clinical team.

Role of Endoscopy in Treatment of Complex Hydrocephalus in Children.

Complex hydrocephalus or loculated hydrocephalus is a challenging problem in the field of pediatric neurosurgery. Early diagnosis and treatment are paramount in order to ensure success of treatment. Therefore, alertness is required among pediatricians who are dealing with premature children and children having meningitis and/or intraventricular hemorrhage. Disproportionate hydrocephalic changes in CT scan of the brain are suspicious, whereas gadolinium-enhanced multiplanar MR imaging (axial, sagittal, and coronal) is the best diagnostic modality. The definitive treatment is surgical, yet the approach remains a matter of debate. Cyst fenestration, communicating the isolated compartments together and with the ventricular system, is the main strategy of treatment. Cyst fenestration can be performed through either microsurgery or endoscopy, in order to improve the hydrocephalus, decrease number of shunts, and reduce shunt revision rates. However, the endoscopic procedure has an advantage over microsurgery of being simple and minimally invasive. It is evident that uniloculated hydrocephalus carries better prognosis than multiloculated hydrocephalus; this can be attributed to the initial pathological disease contributing to the ventricular compartmentalization. Because of the bad prognosis in multiloculated hydrocephalus, and because there are few numbers of patients available in any given center, a multicentric prospective study with long-term follow-up evaluating the results of outcome and quality of life is warranted.

Cerebrospinal fluid shunt malfunctions: A reflective review.

PURPOSE: Pediatric hydrocephalus is a common and challenging condition. To date, the ventriculoperitoneal shunt (VPS) is still the main lifesaving treatment option. Nonetheless, it remains imperfect and is associated with multiple short- and long-term complications. This paper is a reflective review of the current state of the VPS, our knowledge gaps, and the future state of shunts in neurosurgical practice. METHODS AND RESULTS: The authors' reflections are based on a review of shunts and shunt-related literature. CONCLUSION: Overall, there is still an urgent need for the neurosurgical community to actively improve current strategies for shunt failures and shunt-related morbidity. The authors emphasize the role of collaborative efforts amongst like-minded clinicians to establish pragmatic approaches to avoid shunt complications.

Development of shunt valves used for treating hydrocephalus: comparison with endoscopy treatment.

The pathophysiology of hydrocephalus is not clearly defined. Thus, treatment will remain empirical until a fuller understanding of the various forms of hydrocephalus is achieved. Valve-controlled shunting has been the mainstay of therapy since the late 1950s. Initially, shunting occurred from the ventricular system to the atrium. In the 1970s, VA shunts were replaced by ventriculoperitoneal shunts as the primary location for the distal end. Multiple types of one-way valve systems have been developed in the pursuit of draining the appropriate amount of CSF that avoids either overdrainage or underdrainage while preserving normal brain development and cognition. These valves are reviewed and compared as to their function. Other locations for the distal end of the shunting system are reviewed to include pleural space and gallbladder. The lumbar subarachnoid space as the proximal location for a shunt is also reviewed. The only other surgical alternative for treating hydrocephalus is endoscopic third ventriculostomy. Since 2000, approximately 50% of children with hydrocephalus have been shown to be candidates for ETV. The benefits are the lack of need for an artificial shunt system and thus lower rates of infection and over time fewer reoperations. Future progress is dependent on improved shunt valve systems that are affordable worldwide and ready availability of ETV in developing countries. Anatomic and molecular causes of hydrocephalus need to be defined so that medications or genetic modifications become available for potential cure of hydrocephalus.

Ventriculosubgaleal shunt: an institutional experience.

BACKGROUND AND OBJECTIVE: Ventriculosubgaleal shunt (VSGS) was first described in 1896 by Von Mickulicz. It allows CSF to flow from the dilated ventricle to the subgaleal pouch developed in the scalp through a small silicon tube. The possibility that the hydrocephalic state in infants caused by hemorrhage/ + infection will only last a short while prompts the search for temporary management options, which must be a closed system in order to be more physiological given that these infants are frequently preterm (LBW) and immunologically immature. VSGS is one such option. To the best of our knowledge, most of the studies done on VSGS are retrospective, and there are very few prospective studies (none on Indian subjects till now). We aim to study the efficacy of VSGS in preventing the placement of VP Shunt in infants with post-hemorrhagic/post-infectious hydrocephalus. METHOD: Our study (prospective observational) comprises 30 infants in whom VSGS was inserted at Sir Gangaram Hospital between August 2017 and October 2020 and who were followed up for 6 months after VSGS insertion. RESULT: Most of the infants had a birth weight between 1501 and 2000 g with a mean gestational age of 31.2 weeks. Thirteen out of 30 patients did not require the placement of VP shunt. The rates were higher in the post-infectious group (50%) in contrary to the post hemorrhagic group where it was 38.8%. Complications encountered with VSGS include CSF leak (13.3%) and wound dehiscence (6.6%). None had VSGS blockage, migration, and infection. CONCLUSION: We conclude that VSGS is a reliable, safe, easy to perform, and temporary treatment option in infants with post-hemorrhagic and post-infectious hydrocephalus and helps in avoiding VP shunt dependency in nearly half of them.

Spontaneous shunt independence in paediatric tumour-related hydrocephalus: case series and review of the literature.

PURPOSE: Hydrocephalus is commonly associated with paediatric posterior fossa tumours and their resection. This is commonly managed by ventriculoperitoneal shunt insertion, which is associated with a lifelong risk of malfunction, necessitating revisional surgery. Few opportunities ever arise for the patient to be free of the shunt and this risk. We describe three patients shunted for tumour-related hydrocephalus who subsequently developed spontaneous shunt independence. We discuss this in the context of the literature. METHODS: A single-centre retrospective case series analysis was performed using a departmental database. Case notes were retrieved from a local electronic records database, and images were reviewed using national Picture Archiving and Communication Systems. RESULTS: Over a 10-year period, 28 patients underwent ventriculoperitoneal shunt insertion for tumour-related hydrocephalus. Of these, 3 patients (10.7%) went on to have their shunts successfully removed. Age at presentation varied from 1 to 16 years. In all cases, the patient required shunt externalization due to shunt or intra-abdominal infection. This was used as an opportunity to challenge the need for ongoing cerebrospinal fluid (CSF) diversion. In one case, this occurred only several months after a shunt blockage with intracranial pressure monitoring that proved her shunt dependence. All three patients tolerated this challenge, their shunt systems were removed without complication, and they remain free of hydrocephalus at last follow-up. CONCLUSION: These cases reflect our poor understanding of the heterogenous physiology of patients with shunted hydrocephalus and underline the importance of challenging the need for CSF diversion at any appropriate opportunity.

Ventriculopleural shunt outcomes for pediatric hydrocephalus: a single-institution experience.

PURPOSE: The aim of this study is to analyze rates of ventriculopleural (VPL) shunt failure and complications among patients with pediatric hydrocephalus, and to analyze which factors may predict early (< 1 year) or late (> 1 year) VPL shunt failure in this sample. METHODS: A retrospective chart review was conducted of all consecutive VPL shunt placements from 2000 to 2019 at our institution. Data was collected on patient characteristics, shunt history, and shunt type. Primary endpoints include rates of VPL shunt survival and rates of symptomatic pleural effusion. The Kaplan-Meier method was used to calculate shunt survival, and Fisher's exact test and t-test were used to compare differences between categorical variables and means, respectively (p < 0.05). RESULTS: Thirty-one patients with pediatric hydrocephalus underwent VPL shunt placement (mean age 14.2 years). Of the 27 patients with long-term follow-up (mean 46 months), VPL shunt revision was required in 19, seven of which were due to pleural effusion. Overall shunt survival rates at 1, 3, 5, and 7 years were 76%, 62%, 55%, and 46%, respectively. Mean duration of shunt survival was 26.74 months. Overall pleural effusion rate was 26%. No patient-specific factors, including shunt valve type, were significantly associated with shunt survival, risk of early revision, or risk of pleural effusion. CONCLUSIONS: Our results are comparable to those reported in the literature and represent one of the largest case series on the topic. VPL shunts are a viable second-line option when ventriculoperitoneal (VP) shunt placement is not possible or desirable, though there are high rates of shunt revision and pleural effusion.

Retrospective comparison of long-term functionality and revision rate of two different shunt valves in pediatric and adult patients.

PURPOSE: The most frequent therapy of hydrocephalus is implantation of ventriculoperitoneal shunts for diverting cerebrospinal into the peritoneal cavity. We compared two adjustable valves, proGAV and proGAV 2.0, for complications resulting in revision surgery. METHODS: Four hundred patients undergoing primary shunt implantation between 2014 and 2020 were analyzed for overall revision rate, 1-year revision rate, and revision-free survival observing patient age, sex, etiology of hydrocephalus, implantation site, prior diversion of cerebrospinal fluid, and cause of revision. RESULTS: All data were available of all 400 patients (female/male 208/192). Overall, 99 patients underwent revision surgery after primary implantation. proGAV valve was implanted in 283 patients, and proGAV 2.0 valves were implanted in 117 patients. There was no significant difference between the two shunt valves concerning revision rate (p = 0.8069), 1-year revision rate (p = 0.9077), revision-free survival (p = 0.6921), and overall survival (p = 0.3232). Regarding 1-year revision rate, we observed no significant difference between the two shunt valves in pediatric patients (40.7% vs 27.6%; p = 0.2247). Revision operation had to be performed more frequently in pediatric patients (46.6% vs 24.8%; p = 0.0093) with a significant higher number of total revisions with proGAV than proGAV 2.0 (33 of 59 implanted shunts [55.9%] vs. 8 of 29 implanted shunts [27.6%]; p = 0.0110) most likely due to longer follow-up in the proGAV-group. For this reason, we clearly put emphasis on analyzing results regarding 1-year revision rate. CONCLUSION: According to the target variables we analyzed, aside from lifetime revision rate in pediatric patients, there is no significant difference between the two shunt valves.

How to define CSF overdrainage: a systematic literature review.

PURPOSE: Overdrainage (OD) is one of the most frequent complications related to drainage of the cerebrospinal fluid (CSF). It is mostly associated with valve-bearing shunt systems but should probably be considered as a risk factor in any type of CSF diversion procedure. There is extreme variation in the reported incidence of OD due to the lack of consensus on defining criteria and an unclear perception of the pathophysiology. Hence, OD is probably underreported and underestimated. The objective of this paper was to establish a definition of OD, based on a systematic review of the literature. METHODS: A systematic search was conducted in MEDLNE and EMBASE. Studies providing a definition or a description of diagnostic findings related to OD in ventriculoperitoneal shunt treated hydrocephalus were included. Non-English titles, abstracts and manuscripts were excluded. Extracted descriptions were graded into five groups (class I-V studies) based on how precise the terminology used to describe OD was. Class I studies were included for further analysis and characteristics of OD were extracted. The quality of included descriptions was assessed by a clinical expert panel. RESULTS: A total of 1309 studies were screened, 190 were graded into groups, and 22, which provided specific definitions or descriptions of OD, were graded as class I studies. We extracted 32 different characteristics consistent with OD (e.g., clinical symptoms, radiological signs, and syndromes). CONCLUSION: There was an overall agreement that CSF overdrainage following implantation of a ventriculoperitoneal shunt in a mixed pediatric and adult population is characterized as a persistent condition with clinically manifestations as postural dependent headache, nausea, and vomiting and/or radiological signs of slim ventricles and/or subdural collections.