Mortality in pediatric hydrocephalus.

AIM: To clarify the extent to which medical comorbidities and goals-of-care decisions influence death among individuals with childhood-onset hydrocephalus. METHOD: This was a retrospective cohort study of 1705 individuals (759 males, 946 females, mean age 11y 5mo, SD 6y 6mo, range 0-37y 7mo at last follow-up) with childhood-onset hydrocephalus, of whom 88 (5.2%) were deceased. Existing medical records, death records, and publicly available internet sources were analyzed. We estimated hazard ratios for putative risk factors through Cox regression based upon 10 529 person-years of data and quantitatively and qualitatively analyzed the circumstances surrounding each death. RESULTS: Mortality did not differ statistically by demographic factors, although higher proportions of non-White and Hispanic individuals were deceased. Most deaths were related to medical comorbidities rather than hydrocephalus itself. Of the 14 deaths directly related to hydrocephalus, seven were caused by shunt complications and four occurred after decisions to forgo treatment, apparently in response to poor outcomes predicted by the medical team. Half the deaths were preceded by shifts to comfort-based care; however, these decisions appeared to substantially change the patient's clinical trajectory only half the time. INTERPRETATION: Children are more likely to die with, rather than from, hydrocephalus. Our results emphasize the complexities of medical decision-making and the influence of clinicians in guiding these choices.

Severe fetal ventriculomegaly: Fetal morbidity and mortality, caesarean delivery rates and obstetrical challenges in a large prospective cohort.

INTRODUCTION: Severe fetal ventriculomegaly (VM) is defined as an enlargement of the atria of the lateral cerebral ventricles (Vp) of greater than 15 mm. While it is well established that it confers significant risk of morbidity and mortality to the neonate, there is limited information pertaining to the caesarean delivery rates and the obstetric management of these complex cases. The aim of this study was twofold: firstly, to determine survival rates in fetuses with severe VM, and secondly to determine the caesarean delivery rates in continuing pregnancies. We explore the obstetric challenges associated with these difficult cases. METHODS: This was a prospective observational study of patients with antenatal severe VM, attending the Department of Fetal Medicine, National Maternity Hospital, Dublin, Ireland, from 1st January 2011 to 31st July 2020. Data were obtained from the hospital database and those with severe VM (Vp > 15 mm) were identified. The rates of chromosomal abnormalities, the survival rates and the caesarean delivery (CD) rates for the overall group were then determined. The data were then further sub-divided into two groups: 1. Vp < 20 mm and 2. Vp > 20 mm, and the results compared. Statistical analysis was performed using the Chi-Square test. RESULTS: A total of N = 95 pregnancies with severe VM were included for analysis, of which additional structural abnormalities on ultrasound were apparent in 67/95 (70.5%) and 28/95 (29.5%) had isolated severe VM. Chromosomal abnormalities were diagnosed in 15/95 (15.8%) of cases, with (2/28) 7.1% in the isolated SVM group versus (13/67) 19.4% in the non-isolated SVM group. The overall survival rate (excluding TOP) was 53/74 (71.6%), with 20/23 (86.9%) in the isolated SVM group. The overall CD rate was 47/72 (65.3%), which was significantly higher than the CD for the hospital during the same time period of 25.4% (P < 0.01). The data were subdivided into Vp < 20 and Vp > 20 and those with a Vp > 20 had higher rates of additional intracranial findings on ultrasound (Vp < 20 13/41 (31.7%) versus Vp > 20 32/54 (59.3%) (P < 0.05)) and macrocrania (Vp < 20 14/41 (34.1%) versus Vp > 20 35/54 (64.8%) (P < 0.05)). No significant difference was observed in the overall survival or CD rates between the two groups. CONCLUSION: In conclusion this study reports significant fetal morbidity and mortality with severe VM with high CD rates observed in this cohort. Significant challenges exist in relation to the obstetric management and counseling of parents regarding an often uncertain neonatal prognosis. In continuing pregnancies with significant macrocrania delivery plans should be individualized to improve neonatal outcomes where possible and minimize harm to the mother.

Effectiveness of Palliative Cerebrospinal Fluid Shunting for Patients With Leptomeningeal Carcinomatosis-related Hydrocephalus.

BACKGROUND/AIM: Leptomeningeal carcinomatosis (LMC) with hydrocephalus is particularly difficult to treat, and its prognosis is extremely poor. The therapeutic outcomes of 14 patients with LMC-associated hydrocephalus who were treated with cerebrospinal fluid shunting are reported. PATIENTS AND METHODS: The study subjects were 14 LMC patients with solid primary cancer who had developed hydrocephalus. RESULTS: Postoperatively, both symptoms and Karnofsky performance status improved in 100% of patients. Postoperative therapy consisted of whole-brain radiotherapy in 4 cases and molecular targeted therapy in 4, with 6 patients not receiving any postoperative treatment. Median overall survival was 3.7 months, with no significant difference between those who underwent postoperative therapy and those who did not. However, two of those who received molecular targeted therapy survived for more than one year. CONCLUSION: Cerebrospinal fluid shunting for LMC-associated hydrocephalus is an effective therapeutic procedure from the palliative viewpoint. Patients for whom molecular targeted therapy is indicated may have better long-term survival.

Predicting intraventricular hemorrhage growth with a machine learning-based, radiomics-clinical model.

We constructed a radiomics-clinical model to predict intraventricular hemorrhage (IVH) growth after spontaneous intracerebral hematoma. The model was developed using a training cohort (N=626) and validated with an independent testing cohort (N=270). Radiomics features and clinical predictors were selected using the least absolute shrinkage and selection operator (LASSO) method and multivariate analysis. The radiomics score (Rad-score) was calculated through linear combination of selected features multiplied by their respective LASSO coefficients. The support vector machine (SVM) method was used to construct the model. IVH growth was experienced by 13.4% and 13.7% of patients in the training and testing cohorts, respectively. The Rad-score was associated with severe IVH and poor outcome. Independent predictors of IVH growth included hypercholesterolemia (odds ratio [OR], 0.12 [95%CI, 0.02-0.90]; p=0.039), baseline Graeb score (OR, 1.26 [95%CI, 1.16-1.36]; p<0.001), time to initial CT (OR, 0.70 [95%CI, 0.58-0.86]; p<0.001), international normalized ratio (OR, 4.27 [95%CI, 1.40, 13.0]; p=0.011), and Rad-score (OR, 2.3 [95%CI, 1.6-3.3]; p<0.001). In the training cohort, the model achieved an AUC of 0.78, sensitivity of 0.83, and specificity of 0.66. In the testing cohort, AUC, sensitivity, and specificity were 0.71, 0.81, and 0.64, respectively. This radiomics-clinical model thus has the potential to predict IVH growth.

Management of Obstructive Hydrocephalus Associated with Pineal Region Cysts and Tumors and Its Implication in Long-Term Outcome.

BACKGROUND: Different treatment options have been proposed for obstructive hydrocephalus associated with pineal lesions. We discuss the obstructive hydrocephalus management associated with pineal region tumors and cysts in Helsinki Neurosurgery. METHODS: In this article, hydrocephalus treatment by tumor-cyst removal (n = 40), shunt surgery (n = 25), and endoscopic ventriculostomies (n = 3) is evaluated in 68 patients with obstructive hydrocephalus among 136 patients with pineal region tumor and cyst. Multivariate statistical analysis was followed by univariate and multivariate regression models of last functional status, last tumor-free imaging, and disease-specific mortality of the study population. RESULTS: Preoperative hydrocephalus was linked to higher World Health Organization tumor grades, poor functional status, higher mortality, and incomplete resection of pineal region cysts and tumors. Preoperative hydrocephalus remained a predictor of poor last functional status after multivariate regression. Pineal lesion removal with the posterior third ventricle opening as primary hydrocephalus treatment resulted in better last functional status, fewer postoperative shunts, fewer hydrocephalus-related procedures, and fewer postoperative infections than in the shunt-treatment group. Multivariate regression analysis linked higher World Health Organization tumor grade, poor immediate functional status, postoperative complications, and incomplete surgical resection as independent predictors of disease mortality in patients with hydrocephalus. Same variables (except immediate modified Rankin Scale score) and higher number of shunt surgeries became independent predictors of poor last functional status at multivariate analysis. Incomplete resection was the only independent predictor of tumor-free magnetic resonance imaging at the last evaluation. CONCLUSIONS: Direct removal of pineal lesions with the opening of the posterior third ventricle could represent effective and reliable management of the associated obstructive hydrocephalus. Further research is required to generalize our inferences.

Neurocritical care complications and interventions influence the outcome in aneurysmal subarachnoid hemorrhage.

BACKGROUND: This observational study was performed to show the impact of complications and interventions during neurocritical care on the outcome after aneurysmal subarachnoid hemorrhage (SAH). METHODS: We analyzed 203 cases treated for ruptured intracranial aneurysms, which were classified regarding clinical outcome after one year according to the modified Rankin Scale (mRS). We reviewed the data with reference to the occurrence of typical complications and interventions in neurocritical care units. RESULTS: Decompressive craniectomy (odds ratio 21.77 / 6.17 ; p < 0.0001 / p = 0.013), sepsis (odds ratio 14.67 / 6.08 ; p = 0.037 / 0.033) and hydrocephalus (odds ratio 3.71 / 6.46 ; p = 0.010 / 0.00095) were significant predictors for poor outcome and death after one year beside "World Federation of Neurosurgical Societies" (WFNS) grade (odds ratio 3.86 / 4.67 ; p < 0.0001 / p < 0.0001) and age (odds ratio 1.06 / 1.10 ; p = 0.0030 / p < 0.0001) in our multivariate analysis (binary logistic regression model). CONCLUSIONS: In summary, decompressive craniectomy, sepsis and hydrocephalus significantly influence the outcome and occurrence of death after aneurysmal SAH.

Long-term Outcomes and Risk Factors Related to Hydrocephalus After Intracerebral Hemorrhage.

Hydrocephalus after intracerebral hemorrhage (ICH) is a common and treatable complication. However, the long-term outcomes and factors for predicting hydrocephalus have seldom been studied. The goal of this study was to determine the long-term outcomes and analyze the risk factors of hydrocephalus after ICH. A consecutive series of 1342 patients with ICH were reviewed from 2010 to 2016 to identify significant risk factors for hydrocephalus. Patients with a first-ever ICH without any prior diagnosis of hydrocephalus after ICH were followed up for survival status and cause of death. Risk factors for hydrocephalus were evaluated by using logistic regression analysis. Out of a total of 1342 ICH patients, 120 patients (8.9%) had hydrocephalus. The risk factors for hydrocephalus (≤ 3 days) were infratentorial hemorrhage (p = 0.000), extension to ventricles (p = 0.000), greater ICH volume (p = 0.09), and hematoma expansion (p = 0.01). Extension to ventricles (p = 0.022) was the only independent risk factor for hydrocephalus (4-13 days), while extension to ventricles (p = 0.028), decompressive craniotomy (p = 0.032), and intracranial infection (p = 0.001) were independent predictors of hydrocephalus (≥ 14 days). Patients were followed up for a median of 5.2 years (IQR 3.3-7.3 years). Estimated all-cause mortality was significantly higher in the ICH patients with hydrocephalus than that without hydrocephalus (HR 3.22, 95% CI 2.42-4.28; p = 0.000). Fifty-nine (49.2%) died and 40 (33.3%) had a favorable outcome in patients with hydrocephalus. Of all deaths, 30.5% were from ICH and 64.4% from infection. Hydrocephalus is a frequent complication of ICH and most commonly occurs at the onset of ICH. Patients with hydrocephalus show relatively higher mortality. ClinicalTrials.gov Identifier: NCT02135783 (May 7, 2014).

Pediatric hydrocephalus outcomes in Lusaka, Zambia.

OBJECTIVE: Hydrocephalus is a global disease that disproportionally impacts low- and middle-income countries. Limited data are available from sub-Saharan Africa. This study aims to be the first to describe pediatric hydrocephalus epidemiology and outcomes in Lusaka, Zambia. METHODS: This retrospective cohort study included patients < 18 years of age who underwent surgical treatment for hydrocephalus at Beit-CURE Hospital and the University Teaching Hospital in Lusaka, Zambia, from August 2017 to May 2019. Surgeries included ventriculoperitoneal shunt insertions, revisions, and endoscopic third ventriculostomies (ETVs) with or without choroid plexus cauterization (CPC). A descriptive analysis of patient demographics, clinical presentation, and etiologies was summarized, followed by a multivariable analysis of mortality and 90-day complications. RESULTS: A total of 378 patients met the inclusion criteria. The median age at first surgery was 5.5 (IQR 3.1, 12.7) months, and 51% of patients were female (n = 193). The most common presenting symptom was irritability (65%, n = 247), followed by oculomotor abnormalities (54%, n = 204). Postinfectious hydrocephalus was the predominant etiology (65%, n = 226/347), and 9% had a myelomeningocele (n = 32/347). It was the first hydrocephalus surgery for 87% (n = 309) and, of that group, 15% underwent ETV/CPC (n = 45). Severe hydrocephalus was common, with 42% of head circumferences more than 6 cm above the 97th percentile (n = 111). The median follow-up duration was 33 (IQR 4, 117) days. The complication rate was 20% (n = 76), with infection being most common (n = 29). Overall, 7% of the patients died (n = 26). Postoperative complication was significantly associated with mortality (χ2 = 81.2, p < 0.001) with infections and CSF leaks showing the strongest association (χ2 = 14.6 and 15.2, respectively, p < 0.001). On adjusted multivariable analysis, shunt revisions were more likely to have a complication than ETV/CPC or primary shunt insertions (OR 2.45 [95% CI 1.26-4.76], p = 0.008), and the presence of any postoperative complication was the only significant predictor of mortality (OR 42.9 [95% CI 12.3-149.1], p < 0.001). CONCLUSIONS: Pediatric postinfectious hydrocephalus is the most common etiology of hydrocephalus in Lusaka, Zambia, which is similar to other countries in sub-Saharan Africa. Most children present late with neglected hydrocephalus. Shunt revision procedures are more prone to complication than ETV/CPC or primary shunt insertion, and postoperative complications represent a significant predictor of mortality in this population.

Ventriculo-peritoneal shunting devices for hydrocephalus.

BACKGROUND: Hydrocephalus is a common neurological disorder, caused by a progressive accumulation of cerebrospinal fluid (CSF) within the intracranial space that can lead to increased intracranial pressure, enlargement of the ventricles (ventriculomegaly) and, consequently, to brain damage. Ventriculo-peritoneal shunt systems are the mainstay therapy for this condition, however there are different types of shunt systems. OBJECTIVES: To compare the effectiveness and adverse effects of conventional and complex shunt devices for CSF diversion in people with hydrocephalus. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (2020 Issue 2); Ovid MEDLINE (1946 to February 2020); Embase (Elsevier) (1974 to February 2020); Latin American and Caribbean Health Science Information Database (LILACS) (1980 to February 2020); ClinicalTrials.gov; and World Health Organization International Clinical Trials Registry Platform. SELECTION CRITERIA: We selected randomised controlled trials or quasi-randomised trials of different types of ventriculo-peritoneal shunting devices for people with hydrocephalus. Primary outcomes included: treatment failure, adverse events and mortality. DATA COLLECTION AND ANALYSIS: Two review authors screened studies for selection, assessed risk of bias and extracted data. Due to the scarcity of data, we performed a Synthesis Without Meta-analysis (SWiM) incorporating GRADE for the quality of the evidence. MAIN RESULTS: We included six studies with 962 participants assessing the effects of standard valves compared to anti-syphon valves, other types of standard valves, self-adjusting CSF flow-regulating valves and external differential programmable pressure valves. All included studies started in a hospital setting and offered ambulatory follow-up. Most studies were conducted in infants or children with hydrocephalus from diverse causes. The certainty of the evidence for most comparisons was low to very low. 1. Standard valve versus anti-syphon valve Three studies with 296 randomised participants were included under this comparison. We are uncertain about the incidence of treatment failure in participants with standard valve and anti-syphon valves (very low certainty of the evidence). The incidence of adverse events may be similar in those with standard valves (range 0 to 1.9%) and anti-syphon valves (range 0 to 2.9%) (low certainty of the evidence). Mortality may be similar in those with standard valves (0%) and anti-syphon valves (0.9%) (RD 0.01%, 95% CI -0.02% to 0.03%, low certainty of the evidence). Ventricular size and head circumference may be similar in those with standard valves and anti-syphon valves (low certainty of the evidence). None of the included studies reported the quality of life of participants. 2. Comparison between different types of standard valves Two studies with 174 randomised participants were included under this comparison. We are uncertain about the incidence of treatment failure in participants with different types of standard valves (early postoperative period: RR 0.41, 95% CI 0.13 to 1.27; at 12 months follow-up: RR 1.17, 95% CI 0.72 to 1.92, very low certainty of the evidence). None of the included studies reported adverse events beyond those included under "treatment failure". We are uncertain about the effects of different types of standard valves on mortality (range 2% to 17%, very low certainty of the evidence). The included studies did not report the effects of these interventions on quality of life, ventricular size reduction or head circumference. 3. Standard valve versus self-adjusting CSF flow-regulating valve One study with 229 randomised participants addressed this comparison. The incidence of treatment failure may be similar in those with standard valves (42.98%) and self-adjusting CSF flow-regulating valves (39.13%) (low certainty of the evidence). The incidence of adverse events may be similar in those with standard valves (range 0 to 1.9%) and those with self-adjusting CSF flow-regulating valves (range 0 to 7.2%) (low certainty of the evidence). The included study reported no deaths in either group in the postoperative period. Beyond the early postoperative period, the authors stated that nine patients died (no disaggregated data by each type of intervention was available, low certainty of the evidence). The included studies did not report the effects of these interventions on quality of life, ventricular size reduction or head circumference. 4. External differential programmable pressure valve versus non-programmable valve One study with 377 randomised participants addressed this comparison. The incidence of treatment failure may be similar in those with programmable valves (52%) and non-programmable valves (52%)  (RR 1.02, 95% CI 0.84 to 1.24, low certainty of the evidence). The incidence of adverse events may be similar in those with programmable valves (6.19%) and non-programmable valves (6.01%) (RR 0.97, 95% CI 0.44 to 2.15, low certainty of the evidence). The included study did not report the effect of these interventions on mortality, quality of life or head circumference. Ventricular size reduction may be similar in those with programmable valves and non-programmable valves (low certainty of the evidence). AUTHORS' CONCLUSIONS: Standard shunt valves for hydrocephalus compared to anti-syphon or self-adjusting CSF flow-regulating valves may cause little to no difference on the main outcomes of this review, however we are very uncertain due to the low to very low certainty of evidence. Similarly, different types of standard valves and external differential programmable pressure valves versus non-programmable valves may be associated with similar outcomes. Nevertheless, this review did not include valves with the latest technology, for which we need high-quality randomised controlled trials focusing on patient-important outcomes including costs.

Trends in Perinatal Practices and Neonatal Outcomes of Very Low Birth Weight Infants during a 16-year Period at NEOCOSUR Centers.

OBJECTIVE: To describe trends in mortality, major morbidity, and perinatal care practices of very low birth weight infants born at NEOCOSUR Neonatal Network centers from January 1, 2001, through December 31, 2016. STUDY DESIGN: A retrospective analysis of prospectively collected data from all inborn infants with a birthweight of 500-1500 g and 23-35 weeks of gestation. RESULTS: We examined data for 13 987 very low birth weight infants with a mean birth weight of 1081 ± 281 g and a gestational age of 28.8 ± 2.9 weeks. Overall mortality was 26.8% without significant changes throughout the study period. Decreases in early onset sepsis from 6.3% to 2.8% (P <.001), late onset sepsis from 21.1% to 19.5% (P = .002), retinopathy of prematurity from 21.3% to 13.8% (P <.001), and hydrocephalus from 3.8% to 2.4% (P <.001), were observed. The incidence for bronchopulmonary dysplasia decreased from 17.3% to 16% (P = .043), incidence of severe intraventricular hemorrhage was 10.4%, necrotizing enterocolitis 11.1%, and periventricular leukomalacia 3.8%, and did not change over the study period. Administration of antenatal corticosteroids increased from 70.2% to 82.3% and cesarean delivery from 65.9% to 75.4% (P <.001). The use of conventional mechanical ventilation decreased from 67.7% to 63.9% (P <.001) and continuous positive airway pressure use increased from 41.3% to 64.3% (P <.001). Survival without major morbidity increased from 37.4% to 44.5% over the study period (P <.001). CONCLUSIONS: Progress in perinatal and neonatal care at network centers was associated with an improvement in survival without major morbidity of very low birth weight infants during a 16-year period. However, overall mortality remained unchanged.

Ventriculoperitoneal shunt insertion in human immunodeficiency virus infected adults: a systematic review and meta-analysis.

BACKGROUND: Hydrocephalus is a common, life threatening complication of human immunodeficiency virus (HIV)-related central nervous system opportunistic infection which can be treated by insertion of a ventriculoperitoneal shunt (VPS). In HIV-infected patients there is concern that VPS might be associated with unacceptably high mortality. To identify prognostic indicators, we aimed to compare survival and clinical outcome following VPS placement between all studied causes of hydrocephalus in HIV infected patients. METHODS: The following electronic databases were searched: The Cochrane Central Register of Controlled Trials, MEDLINE (PubMed), EMBASE, CINAHL Plus, LILACS, Research Registry, the metaRegister of Controlled Trials, ClinicalTrials.gov, African Journals Online, and the OpenGrey database. We included observational studies of HIV-infected patients treated with VPS which reported of survival or clinical outcome. Data was extracted using standardised proformas. Risk of bias was assessed using validated domain-based tools. RESULTS: Seven Hunderd twenty-three unique study records were screened. Nine observational studies were included. Three included a total of 75 patients with tuberculous meningitis (TBM) and six included a total of 49 patients with cryptococcal meningitis (CM). All of the CM and two of the TBM studies were of weak quality. One of the TBM studies was of moderate quality. One-month mortality ranged from 62.5-100% for CM and 33.3-61.9% for TBM. These pooled data were of low to very-low quality and was inadequate to support meta-analysis between aetiologies. Pooling of results from two studies with a total of 77 participants indicated that HIV-infected patients with TBM had higher risk of one-month mortality compared with HIV non-infected controls (odds ratio 3.03; 95% confidence-interval 1.13-8.12; p = 0.03). CONCLUSIONS: The evidence base is currently inadequate to inform prognostication in VPS insertion in HIV-infected patients. A population-based prospective cohort study is required to address this, in the first instance.

Urologic Disorders are Still the Leading Cause of In-hospital Death in Patients With Spina Bifida.

OBJECTIVE: To assess and analyze the contemporary causes of in-hospital deaths of spina bifida patients. METHODS: It was a cross-sectional observational study of the longitudinal national cohort of all patients hospitalized in French public and private hospitals. We analyzed the data from the French hospital discharge database (Programme de Médicalisation des Systemes d'Information, PMSI) from 2009 to 2014. The number of in-hospital deaths was extracted using the combination of the ICD-10 codes "Q05" or "Q760" and a discharge code = 9. RESULTS: There were 138 in-hospital deaths of spina bifida patients over the 6-year study period. The median age at death was 41 years (IQR: 25-52). The median age at death was significantly lower in patients with vs without hydrocephalus (26.6 vs 45.5 years; P <.0001). The leading cause of in-hospital death was urologic disorders (n = 24; 17.3%). Other main causes of death were pulmonary disorders (n = 23; 16.7%), neurologic disorders (n = 19; 13.8%), and bowel disorders (n = 15; 10.9%). Upper urinary tract damage accounted for most of the urologic causes of death: 8 patients died from urinary tract infections (33.3%), 7 patients died from renal failure (29.2%), 4 died from bladder cancer (16.7%), and 5 from other urologic causes. The only variable significantly associated with a death from urologic causes was the absence of hydrocephalus (OR = 0.26; P = .009). CONCLUSION: Urologic disorders remain the leading cause of in-hospital death in spina bifida patients in France. The present study highlights that efforts to improve the urologic management of the spina bifida population are still greatly needed.

Effectiveness of Combined External Ventricular Drainage with Intraventricular Fibrinolysis for the Treatment of Intraventricular Haemorrhage with Acute Obstructive Hydrocephalus.

BACKGROUND: Intraventricular haemorrhage (IVH) patients with acute obstructive hydrocephalus (AOH) who require external ventricular drainage (EVD) are at high risk for poor outcomes. Intraventricular fibrinolysis (IVF) with low-dose recombinant tissue plasminogen activator (rtPA) can be used to improve patient outcomes. Here, we evaluated the impact of IVF on the risk of death and the functional outcomes in IVH patients with AOH. METHODS: This prospective cohort study included IVH patients with hypertensive intracranial haemorrhage complicated by AOH who required EVD. We evaluated the risk of death and the functional outcomes at 1 and 3 months, with a specific focus on the impact of combined EVD with IVF by low-dose rtPA. RESULTS: Between November 30, 2011 and December 30, 2014, 80 patients were included. Forty-five patients were treated with EVD alone (EVD group) and 35 received IVF (EVD+IVF group). The 30- and 90-day mortality rates were lower in the EVD+IVF group than in the EVD group (42.2 vs. 11.4%, p = 0.003, and 62.2 vs. 20%, p < 0.001, respectively). The Graeb scores were significantly lower in the EVD+IVF group than in the EVD group (p ≤ 0.001) during the first 3 days and on day 7 after assignment. The 30-day good functional outcome (modified Rankin Scale [mRS] score 0-3) was also higher in the EVD+IVF group than in the EVD group (6.7 vs. 28.6%, p = 0.008). However, the 90-day good functional outcome (mRS score 0-3) did not significantly increase in the EVD+IVF group (30.8% in the EVD group vs. 51.6% in the EVD+IVF group, p = 0.112). CONCLUSIONS: In our prospective observational study, EVD+IVF was associated with a lower risk of death in IVH patients. EVD+IVF improved the chance of having a good functional outcome at 1 month; however, this result was no longer observed at 3 months.

Clinical outcome of cerebrospinal fluid shunts in patients with leptomeningeal carcinomatosis.

BACKGROUND: Leptomeningeal carcinomatosis (LMC) is frequently associated with hydrocephalus, which quickly devastates the performance of the patient. Cerebrospinal fluid (CSF) shunt is a widely accepted treatment of choice, but the clinical outcomes in patients with LMC are not well studied. This study aimed to examine the efficacy of a CSF shunt in patients with LMC. METHODS: Seventy patients with LMC confirmed by cytology or magnetic resonance imaging (MRI) underwent ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt surgery. We retrospectively analyzed the clinical characteristics of patients, symptom improvement after the shunt, rate of complications associated with the surgery, and overall survival. RESULTS: Fifty-five patients had systemic cancer as a preceding disease, including lung cancer (45), breast cancer (6), and others (4). Primary brain tumors were mainly glioma (7) and medulloblastoma (5). Fifty-one patients had VP shunt, and 19 had LP shunt. After surgery, preoperative symptoms "improved" in 35 patients (50%) and were "normalized" in 24 of those patients (34%). Shunt malfunction occurred in eight patients, and infection occurred in eight patients. Seventeen patients underwent revision due to infection, shunt malfunction, or over-drainage. There were no complications associated with peritoneal seeding during a median follow-up of 3.3 months after surgery. The median overall survival was 8.7 months (95% confidence interval, 6.0-11.4) from LMC diagnosis and 4.1 months from shunt surgery. CONCLUSION: VP or LP shunt is effective for patients with hydrocephalus from LMC in terms of symptom improvement and prolonging of overall survival with an acceptable rate of procedure-related complications. TRIAL REGISTRATION: This study was approved by the Institutional Review Board (IRB) of the National Cancer Center (retrospectively registered, NCC2018-0051 ).

Healthcare Economics of Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage in the United States.

Hydrocephalus is one of the most common sequelae after aneurysmal subarachnoid hemorrhage (aSAH), and it is a large contributor to the condition's high rates of readmission and mortality. Our objective was to quantify the healthcare resource utilization (HCRU) and health economic burden incurred by the US health system due to post-aSAH hydrocephalus. The Truven Health MarketScan® Research database was used to retrospectively quantify the prevalence and HCRU associated with hydrocephalus in aSAH patients undergoing surgical clipping or endovascular coiling from 2008 to 2015. Multivariable longitudinal analysis was conducted to model the relationship between annual cost and hydrocephalus status. In total, 2374 patients were included; hydrocephalus was diagnosed in 959 (40.4%). Those with hydrocephalus had significantly longer initial lengths of stay (median 19.0 days vs. 12.0 days, p < .001) and higher 30-day readmission rates (20.5% vs. 10.4%, p < .001). With other covariates held fixed, in the first 90 days after aSAH diagnosis, the average cost multiplier relative to annual baseline for hydrocephalus patients was 24.60 (95% CI, 20.13 to 30.06; p < .001) whereas for non-hydrocephalus patients, it was 11.52 (95% CI, 9.89 to 13.41; p < .001). The 5-year cumulative median total cost for the hydrocephalus group was $230,282.38 (IQR, 166,023.65 to 318,962.35) versus $174,897.72 (IQR, 110,474.24 to 271,404.80) for those without hydrocephalus. We characterize one of the largest cohorts of post-aSAH hydrocephalus patients in the USA. Importantly, the substantial health economic impact and long-term morbidity and costs from this condition are quantified and reviewed.

Prediction of Outcomes for Ruptured Aneurysm Surgery.

Background and Purpose- Predicting long-term functional outcomes after intracranial aneurysmal rupture can be challenging. We developed and validated a scoring system-the Southwestern Aneurysm Severity Index-that would predict functional outcomes at 1 year after clipping of ruptured aneurysms. Methods- Ruptured aneurysms treated microsurgically between 2000 and 2014 were included. Outcome was defined as Glasgow Outcome Score (ranging from 1, death, to 5, good recovery) at 1 year. The Southwestern Aneurysm Severity Index is composed of multiple prospectively recorded patient demographic, clinical, radiographic, and aneurysm-specific variables. Multivariable analyses were used to construct the best predictive models for patient outcomes in a random 50% of the cohort and validated in the remaining 50%. A scoring system was created using the best model. Results- We identified 527 eligible patients. The Glasgow Outcome Score at 1 year was 4 to 5 in 375 patients (71.2%). In the multivariable logistic regression, the best predictive model for unfavorable outcome included intracerebral hemorrhage (odds ratio [OR], 2.53; 95% CI, 1.55-4.13), aneurysmal size ≥20 mm (OR, 6.07; 95% CI, 1.92-19.2), intraventricular hemorrhage (OR, 2.56; 95% CI, 1.15-5.67), age >64 (OR, 3.53; 95% CI, 1.70-7.35), location (OR, 1.82; 95% CI, 1.10-3.03), and hydrocephalus (OR, 2.39; 95% CI, 1.07-5.35). The Southwestern Aneurysm Severity Index predicts Glasgow Outcome Score at 1 year with good discrimination (area under the receiver operating characteristic curve, derivation: 0.816, 95% CI, 0.759-0.873; validation: 0.803, 95% CI, 0.746-0.861) and accurate calibration ( R2=0.939). Conclusions- The Southwestern Aneurysm Severity Index has been internally validated to predict 1 year Glasgow Outcome Scores at initial presentation, thus optimizing patient or family counseling and possibly guiding therapeutic efforts.

Extent of resection predicts risk of progression in adult pilocytic astrocytoma.

Object: Pilocytic astrocytomas are rare tumours in adults. Presentation, management and prognostic factors are poorly characterised. Methods: Retrospective single centre study from 2000 to 2016. Results: 50 cases were identified (median age 29 years; range 16-76). Symptoms at presentation were neurological deficit (n = 21), headache (n = 18) and seizures (n = 6). Five were incidental findings. Five patients had hydrocephalus at presentation and required emergent management, two by endoscopic third ventriculostomy and three by external ventricular drain. Symptoms were present for a median of 16 weeks (range 1 week to 34 years). Surgery consisted of gross total resection (n = 23), subtotal resection (n = 21) or biopsy (n = 6). Progression occurred in 20 patients at a median time of 7 years following surgery and was asymptomatic in just over half of these cases. A greater degree of resection (complete vs. subtotal) was associated with longer time to progression (Kaplan-Meier analysis, log rank test = 3.58, p = 0.059). At their first progression 12 patients underwent re-resective surgery and the remainder received radiotherapy. The median 5-year survival was 80%. Conclusions: In adult patients with a pilocytic astrocytoma, a macroscopic resection should be the aim at the first resective operation. Emergency management of hydrocephalus may be required in the first instance.

Neurodevelopmental disorder in children believed to have isolated mild ventriculomegaly prenatally.

OBJECTIVE: To estimate the prevalence of specific neurodevelopmental disorders in children believed to have isolated mild ventriculomegaly (IMV) prenatally in the second trimester of pregnancy, in order to optimize the counseling process. METHODS: This was a nationwide registry-based study including all singleton pregnancies that had first- and second-trimester ultrasound scans in the period 1st January 2008 to 1st October 2014, identified in the Danish Fetal Medicine Database and local clinical databases in Denmark. All fetuses diagnosed prenatally with IMV (measurement of the atrium of the lateral ventricles, 10.0-15.0 mm) between 18 and 22 weeks' gestation were followed up in national patient registers until the age of 2-7 years. Information was obtained on the diagnoses of intellectual disability, cerebral palsy, autism spectrum disorder, epilepsy and impaired psychomotor development. Neurodevelopmental disorders were compared between those with postnatally confirmed IMV and a reference population of children in the same age range. RESULTS: Of a cohort of 292 046 fetuses, 133 were found to have apparent IMV on the second-trimester scan for fetal malformations. In 11 cases, long-term follow-up was not possible owing to termination of pregnancy, spontaneous miscarriage, neonatal death or loss to follow-up. Of the 122 liveborn children followed up until 2-7 years, 15 were identified as having an additional abnormality while 107 were confirmed postnatally to have IMV. Of these 107 children, the diagnosis of a neurodevelopmental disorder was registered in six (5.6%), corresponding to an odds ratio of 2.64 (95% CI, 1.16-6.02), as compared with the reference population. The diagnoses were autism spectrum disorder, epilepsy and impaired psychomotor development. None of these 107 children was diagnosed with intellectual disability or cerebral palsy. CONCLUSIONS: Our results show that a confirmed diagnosis of IMV was associated with an increased risk of a neurodevelopmental disorder, as compared with the reference population, but the absolute risk was low and there were no cases of intellectual disability or cerebral palsy. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Trastorno del desarrollo neurológico en fetos con sospecha de ventriculomegalia leve aislada prenatal OBJETIVO: Estimar la prevalencia de trastornos específicos del desarrollo neurológico en fetos con sospecha de ventriculomegalia leve aislada (IMV, por sus siglas en inglés) prenatal en el segundo trimestre del embarazo, a fin de optimizar el proceso de asesoramiento. MÉTODOS: Este estudio estuvo basado en un registro nacional que incluyó todos los embarazos con feto único a los que se les hizo ecografías en el primer y segundo trimestre entre el 1 de enero de 2008 y el 1 de octubre de 2014, identificados en la Base de Datos Danesa de Medicina Fetal y en las bases de datos clínicas locales en Dinamarca. Todos los fetos diagnosticados prenatalmente con IMV (por medición de la aurícula de los ventrículos laterales, 10,0-15,0 mm) entre las semanas de gestación 18 y 22 fueron monitoreados en los registros nacionales de pacientes hasta la edad de 2-7 años. Se obtuvo información sobre los diagnósticos de discapacidad intelectual, parálisis cerebral, trastornos del espectro autista, epilepsia y trastornos del desarrollo psicomotor. Se compararon los trastornos del desarrollo neurológico entre aquellos con IMV confirmada después del nacimiento y una población de referencia de niños en el mismo rango de edad. RESULTADOS: De una cohorte de 292 046 fetos, se encontró que 133 tenían IMV aparente en la ecografía del segundo trimestre realizada para detectar malformaciones fetales. El seguimiento a largo plazo no fue posible en 11 casos debido a la interrupción del embarazo, el aborto espontáneo, la muerte del recién nacido o el abandono del monitoreo. De los 122 niños nacidos vivos a los que se les dio seguimiento hasta los 2-7 años, se identificó a 15 con una anomalía adicional, mientras que a 107 se les confirmó postnatalmente que tenían IMV. De estos 107 niños, se registró el diagnóstico de un trastorno del desarrollo neurológico en seis (5,6%), lo que corresponde a una razón de momios de 2,64 (IC 95%: 1,16-6,02), en comparación con la población de referencia. Los diagnósticos fueron trastornos del espectro autista, epilepsia y trastornos del desarrollo psicomotor. Ninguno de estos 107 niños fue diagnosticado con discapacidad intelectual o parálisis cerebral. CONCLUSIONES: Nuestros resultados muestran que un diagnóstico confirmado de IMV se asoció con un mayor riesgo de trastorno del desarrollo neurológico, en comparación con la población de referencia, pero que el riesgo absoluto fue bajo y no hubo casos de discapacidad intelectual o parálisis cerebral.

Risk Factors Associated with the Outcome of Post-Traumatic Hydrocephalus.

BACKGROUND AND AIM: Post-traumatic hydrocephalus is a common complication that arises after head injury. However, risk factors associated with the outcome of post-traumatic hydrocephalus have seldom been addressed. Therefore, we performed this clinical study to analyze the risk factors affecting the outcome of post-traumatic hydrocephalus in patients with head injuries. METHODS: A total of 116 post-traumatic hydrocephalus patients, admitted in our hospital between March 2012 and October 2017 were reviewed. The related factors assessed were age, gender, Glasgow Coma Score on admission, platelet count, plasma fibrinogen levels, D-dimer concentration, subarachnoid hemorrhage, subdural hygroma, cerebral hernia, cisterna ambiens, decompressive craniectomy, cranioplasty, ventriculoperitoneal shunt implantation, intracranial infection, and duration of comatous state. The patient outcomes after 6 months of treatment were evaluated by the Glasgow Outcome Scale. Risk factors for the outcome of post-traumatic hydrocephalus were evaluated by applying logistic regression analysis. RESULTS: Poor outcome was observed in 66.4% of the patients (77/116). Univariate and multivariate analyses revealed that the disappearance of cisterna ambiens, the long duration of comatous state (>2 months), the high levels of plasma fibrinogen, and the ventriculoperitoneal shunt implantation were related to adverse outcomes (p < 0.05). CONCLUSION: The disappearance of cisterna ambiens, the prolonged duration of comatous state (>2 months), the high plasma fibrinogen levels are the most important factors affecting the outcome of post-traumatic hydrocephalus, and the ventriculoperitoneal shunt implantation is the most critical predictor of the outcome of post-traumatic hydrocephalus.

Outcome of Ventriculoperitoneal Shunt Surgery in Human Immunodeficiency Virus-Positive Patients on Combination Antiretroviral Therapy with Tuberculosis Meningitis and Hydrocephalus.

BACKGROUND: In 2000, we advised against insertion of a ventriculoperitoneal shunt (VPS) in human immunodeficiency virus (HIV)-positive patients with tuberculous meningitis (TBM) complicated by hydrocephalus. However, this was in the era when combination antiretroviral therapy (ART) was not freely available in South Africa. In this subsequent preliminary report, we describe the outcome of ventriculoperitoneal shunting in patients with TBM and hydrocephalus who are HIV positive and receiving ART. METHODS: We compared a group of 15 HIV-positive patients with TBM and hydrocephalus on ART with a retrospective control group of 15 patients (demographically and clinically matched) but not on ART. All patients were otherwise managed similarly and evaluated at 1 month after VPS insertion. RESULTS: In historical controls, 10 patients died (66.7%) and no patient showed any improvement 1 month after shunting. In contrast, in the current group on ART, 4 patients died (26.7%), with 11 patients (73.3%) having a good outcome. Eight of 12 patients with grade 3 TBM had a good outcome, whereas all 3 with grade 1 TBM made a good recovery. CONCLUSIONS: The outcome of VP shunting in HIV-positive patients with TBM and hydrocephalus is markedly improved in patients on ART. Based on limited data from this study, we recommend that better grades of TBM (1 and 2) undergo immediate VPS surgery. Patients with grade 4 TBM should undergo a trial of external ventricular drainage and those who improve should undergo a definitive procedure. Further research is required for patients with grade 3 TBM to identify characteristics associated with better outcomes to allow for effective use of limited resources.