Temporal trends in paediatric hydrocephalus - Rising prematurity and persistent ophthalmological challenges.

AIM: To study changes in aetiology, prematurity, comorbidity and ophthalmological outcomes in children with surgically treated hydrocephalus to provide information needed to maintain the best possible healthcare for a fragile and changing population. METHODS: Two population-based cohorts, born two decades apart in Region Västra Götaland Sweden, surgically treated for hydrocephalus at Sahlgrenska University Hospital in Gothenburg were recruited at approximately 10 years of age. The participants were examined according to an ophthalmological protocol, including history taking regarding perceptual visual dysfunction (PVD). Gestational age, aetiology and comorbidities were registered. RESULTS: The 1989-1993 group, comprised 52 children (48% girls; mean age 10, range 7.7-12.8 years), was compared with 24 children, born in 2007-2012 (29% girls; mean age 10, range 7.0-13.8 years). Extreme prematurity (gestational age ≤ 28 weeks) increased over time (p = 0.001). The vast majority of the children showed ophthalmological abnormalities, although motility defects and nystagmus decreased in the latter population. Subnormal visual acuity was associated with extreme prematurity (RR = 4.69; p = 0.030), and PVD with learning disability (RR = 2.44; p = 0.032). CONCLUSION: Paediatric hydrocephalus populations may change with improved healthcare. Since a high percentage shows ophthalmological abnormalities and more children are born extremely preterm, the entirety needs consideration both neurologically and ophthalmologically.

Clinical characteristics and perinatal outcome of fetuses with ventriculomegaly.

PURPOSE: To assess the incidence of associated structural anomalies, chromosomal/genetic abnormalities, infections, and perinatal outcomes of fetuses with ventriculomegaly (VM), also to evaluate the role of fetal magnetic resonance imaging (MRI) in detecting associated intracranial anomalies. METHODS: Retrospective cohort study of 149 prenatally diagnosed pregnancies with fetal VM. VM was classified as mild (Vp = 10-12 mm), moderate (Vp = 12.1-15 mm), and severe (Vp > 15 mm). Fetal MRI was performed to 97 pregnancies. RESULTS: The incidences of an associated CNS, non-CNS, chromosomal anomaly, genetic abnormality and fetal infection were 42.3%, 11.4%, 6.1%, 2.1% and 1.3%, respectively. Fetal MRI identified additional CNS anomalies in 6.7% of cases, particularly in severe VM. The incidences of perinatal outcomes were 18.8% termination of pregnancy, 4% intrauterine and 8.1% neonatal or infant death. The rates of fetuses alive at > 12 months of age with neurological morbidity were 2.6%, 11.1% and 76.9% for mild, moderate and severe isolated VM, respectively. CONCLUSION: The prognosis of fetuses with VM mostly depends on the severity and the associated anomalies. Mild to moderate isolated VM generally have favorable outcomes. Fetal MRI is particularly valuable in fetuses with isolated severe VM.

Incidence of Ventriculomegaly in Patients With Craniosynostosis.

Hydrocephalus is variously associated with syndromic craniosynostosis (CS), while it is randomly encountered in nonsyndromic CS. But actually, the ventriculomegaly in CS is less described. In this study, the authors aim to establish whether ventriculomegaly is common in patients with CS, in both syndromic and nonsyndromic. Retrospective measurements of Evans index (EI) were taken from thin-section computed tomography scans of 169 preoperative CS patients to assess cerebral ventricular volume. EI >0.3 indicates ventricular enlargement. A total of 169 CS patients who underwent computed tomography scan from February 2018 to December 2021 were retrospectively evaluated, including 114 males and 55 females. The average age at diagnosis was 16 months (range: 1-103 mo). Among them, 37 with syndromic CS, including 17 ventricular megaly patients, had an EI >0.3 (46.0%), and 4 of them had intracranial hypertension and needed ventriculoperitoneal shunt treatment before cranial vault remolding. One hundred and thirty-two had nonsyndromic CS (100 single-suture CS, 32 multisuture CS), and 26 of them had an EI of 0.3 or greater (19.7%). Ventrocular megaly is common among patients with CS. Early craniotomy may stabilize ventricular dilation.

Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study.

The aim of this study was to explore the association between sex and cerebellar mutism syndrome and to examine other potential risk factors. This ambispective cohort study examined 218 pediatric patients (132 boys) with a posterior fossa tumor who underwent tumor resection from July 2013 to March 2021. The patients' demographics and tumor characteristics were examined and statistically analyzed to explore the associations among the variables. Multivariable and subgroup analyses were conducted to validate the independent risk factors for cerebellar mutism syndrome (CMS). The male and female patients did not differ significantly in terms of age, tumor size, tumor location, tumor consistency, VP shunt placement before resection, extent of resection, or surgeon, as well as with respect to the presence of hydrocephalus or paraventricular edema. The overall incidence of CMS was 32.6%. The incidence of CMS was significantly higher in male patients than that in female patients (41.7% vs. 18.6%; P = 0.001). In the multivariable analysis, male sex (adjusted odds ratio [OR], 3.27; P = 0.001), solid tumor consistency (adjusted OR, 5.61; P = 0.001), midline location (adjusted OR, 3.78; P = 0.004), and hydrocephalus (adjusted OR, 2.56; P = 0.047) were independent risk factors for the CMS. Chi-square analysis revealed that solid tumor consistency and midline location were associated with medulloblastoma (P < 0.001). Male patients had a higher risk of developing CMS after a posterior fossa tumor resection. Midline location, solid tumor consistency, and hydrocephalus were independent risk factors for CMS.

Treatment of hydrocephalus following posterior fossa tumor resection: a multicenter collaboration from the Hydrocephalus Clinical Research Network.

OBJECTIVE: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT. METHODS: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF). RESULTS: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort. CONCLUSIONS: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion.

COVID-19- Experiences and support needs of children and young people with Hydrocephalus and parents in the United Kingdom.

PURPOSE: Little is known about the impact of COVID-19 on children and young people (CYP) with hydrocephalus and their families. This study explored the experiences and support needs of CYP with hydrocephalus and parents who have a child with hydrocephalus during the COVID-19 pandemic. METHODS: CYP with hydrocephalus and parents of CYP with hydrocephalus in the United Kingdom completed an online survey with open and closed questions exploring experiences, information, support needs and decision making processes. Qualitative thematic content analysis and descriptive quantitative analyses were undertaken. RESULTS: CYP aged 12-32 years (n=25) and parents of CYP aged 0-20 years (n=69) responded. Parents (63.5%) and CYP (40.9%) worried about the virus, and both were vigilant for virus symptoms (86.5% and 57.1%). Parents (71.2%) and CYP (59.1%) worried about their child/feeling more isolated during the virus outbreak. Parents felt concerned about having to take their child to hospital with a suspected shunt problem during the virus outbreak (64.0%). Qualitative findings reported the following themes: (1) Healthcare and treatment provision: delays and challenges to access and availability of care (2) Impact of COVID-19/lockdown on daily lives and routines, and (3) Provision of information and support for parents and CYP with hydrocephalus. CONCLUSION: The impact of COVID-19 and national measures to control the spread of the virus- no contact with anyone outside the household significantly impacted the daily lives and routines of CYP with hydrocephalus and parents. Social engagements were missed, families faced challenges to their work life, education and access to health care and support, which subsequently contributed negatively to their mental wellbeing. CYP and parents highlighted a need for clear, timely and targeted information to address their concerns.

Pre and postnatal care characteristics and management features of children born with myelomeningocele in the post-folate fortification era of staple foods in Costa Rica (2004-2022).

PURPOSE: To describe the clinical characteristics of pre- and postnatal care of children born with myelomeningocele in Costa Rica from 2004 to 2022 after the introduction of mandatory fortification of four major staple foods, describing the clinical features of this cohort including the size of the meningomyelocele, neurological level, presence of symptomatic Chiari II at birth, kyphosis, and the severity of hydrocephalus requiring cerebrospinal fluid (CSF) shunting. These results were compared against the pre-fortification historical data to determine favorable outcomes from this health policy. METHODS: We performed a retrospective review of the clinical records of patients with myelomeningocele at the spina bifida clinic from the National Children's Hospital of Costa Rica who were born between 2004 and 2022, a period when staple food fortification was implemented in the country for four food staples (wheat and corn flour, rice, and dairy products). Pre and postnatal care data pertaining to the number of obstetric ultrasound studies, the trimester in which they were performed, the detection of myelomeningocele and associated hydrocephalus, gestational age and route of delivery, neurological level, myelomeningocele defect size, associated kyphosis and symptomatic Chiari II malformation at birth, time periods of myelomeningocele repair, and CSF shunting were examined. A descriptive comparative frequency analysis between myelomeningocele (MMC) defect size, CSF shunt insertion, symptomatic Chiari II, and kyphosis between the different functional levels was performed with the estimation of the Fisher's exact chi-square test by contingency tables, and 0.05 was set as significance level. Additionally, the postnatal features of this cohort were compared against pre-fortification historical data obtained from 100 live-born patients between 1995 and 1996. RESULTS: A total of 215 patients that were live born between 2004 and 2022 were eligible for analysis with a follow up ranging from 1 to 19 years (median follow up: 7.9 years). Among 99.1% of the mothers of patients who had prenatal consultations, 95.8% had an average of 3.8 obstetric ultrasound studies which led to a 59% prenatal detection rate of myelomeningocele. The pre and post fortification features showed a male/female ratio that changed from 0.92 to 1.25 respectively. Among these newborns, there was an increase from 54 to 64% cesarean sections as method for delivery. Only 26% of the pre fortification patients had the MMC defect repaired in the first 24 h, 32% from 24 to 72 h, 20% from 72 h to 1 week, and 22% later than 10 days respectively which deeply contrasted with the post fortification cohort where 7.5% had the MMC defect repaired in less than 8 h, 12.2% from 8 to 12 h, 66.5% 12-24 h, and 12.7% from 24 to 48 h and 1% later than 48 h, respectively (P < 0.01). Regarding the post fortification myelomeningocele anatomic and functional characteristics, defect size was measured as less than 3 cm in 7% of cases, 3 to 5 cm in 50% of cases, 5 to 7 cm in 42% of cases, and greater than 7 cm in 1% of cases. Thirteen percent of the cases had paraplegia due to a thoracic level, 10% had a high lumbar level, 58% had a middle lumbar level, 13% a lower lumbar level, and 6% only sacral compromise. When the data from the pre and post fortification cohorts were adjusted and compared, there was a reduction from thoracic/high lumbar cases from 26 to 23% (P < 0.56), with an increase of middle lumbar cases from 43 to 58% and a reduction from 25 to 13% of low lumbar cases (P < 0.01) while there was no change in the 6% percentage of sacral cases respectively. Lesions that were considered too extensive or larger than 7 cm decreased from 7 to 1% while associated kyphotic deformities decreased from 6 to 1.4% (P < 0.01); symptomatic Chiari II malformation at birth also decreased from 7 to 2% in the pre- and post-fortification cohorts respectively with all these changes being statistically significant (P < 0.01). Seventy-nine percent and 80% of the pre and post FAF cohorts required CSF VP shunting with a mean time for insertion of 10 days after spinal defect closure with no significant statistical change between the two groups. CONCLUSIONS: This study describes a four-staple folate fortified population of live-born patients with myelomeningocele lesions whose neurological level, defect size, and associated deformities such as spinal kyphosis and symptomatic Chiari II at birth suggest that folate fortification could have diminished the severity of this congenital disease.

Molecular subgroup of medulloblastoma: evaluation of contribution to CSF diversion following tumour resection.

INTRODUCTION: Medulloblastoma is the commonest malignant brain tumour in children. Pre-operative hydrocephalus is present in up to 90% of these patients at presentation. Following posterior fossa surgery, despite resolution of fourth ventricular obstruction, a proportion of these children will still require cerebrospinal fluid (CSF) diversion for management of persistent or new hydrocephalus. Various scoring systems have been developed to predict the risk for CSF diversion following posterior fossa surgery. However, no accurate tool exists regarding which pathological subset or group of medulloblastoma patients will require a shunt post-operatively. In this study we investigated the impact of molecular subgroup of medulloblastoma on shunt dependency post-operatively in paediatric patients. METHODS: We undertook a retrospective multi-centre study of children with medulloblastoma who underwent tumour resection. Those with available molecular subgroup were identified. Demographic data and clinical parameters including age, sex, presence of pre-operative hydrocephalus, extent of surgical resection, evidence of metastasis/leptomeningeal disease and need for CSF diversion post-operatively were further analysed. RESULTS: Sixty-nine children with medulloblastoma with available molecular data were identified during the study period with male to female ratio of 1.5:1 (42M:27F). Twelve patients (17.4%) belonged to SHH, 10 (14.5%) Wnt, 19 (27.5%) Group 3 and 15 (21.7%) Group 4; 13 (18.8%) were non-specified Group 3 or 4. A total of 18 (26%) patients had evidence of leptomeningeal disease at presentation (20% of Wnt, 42% of Group 3, 33% of group 4, 23% of group 3/4, and 0% of SHH). Fifteen patients (22%) underwent post-operative ventriculoperitoneal (VP) shunt insertion. No patient in the Wnt group required ventriculoperitoneal (VP) shunt post-operatively in this cohort. Need for shunt was associated with pre-operative hydrocephalus, leptomeningeal disease, with molecular group 3 or 4 demonstrating higher rate of leptomeningeal disease, and pre-operative hydrocephalus. Age, extent of resection and pre-operative EVD were not associated with need for shunt in this cohort. Regression analysis identified only pre-operative hydrocephalus and leptomeningeal disease as independent predictors of need for shunt post-resection in this cohort. CONCLUSION: All patients requiring permanent post-operative VP shunt belonged to non-Wnt groups, particularly group 3 and 4. Although medulloblastoma subgroup does not independently predict need for post-operative shunt, presence of leptomeningeal disease and pre-operative hydrocephalus, and their higher prevalence in group 3 and 4, likely account for observed higher rate of shunting in these groups.

Pregnancy in spina bifida patients: a comparative analysis of peripartum procedures and complications.

PURPOSE: Spina bifida (SB) is caused by a failure in neural tube closure that can present with lower extremity sensory deficits, paralysis, and hydrocephalus. Medical advances have allowed increased pregnancies among SB patients, but management and pregnancy-associated complications have not been thoroughly investigated. The objective is to delineate peripartum procedures and complications in patients with SB. METHODS: A national de-identified database, TriNetX, was retrospectively queried to evaluate pregnant SB patients and the general population. Procedures and complications were investigated using corresponding ICD-10 and CPT codes within 1 year of pregnancy diagnosis. RESULTS: 11,405 SB patients were identified and compared to 9,269,084 non-SB patients. SB patients were significantly more likely to undergo cesarean delivery (1.200; 95% CI [1.133-1.271]) and less likely to receive neuraxial analgesia (0.406; 95% CI [0.383-0.431]). Additionally, patients with SB had an increased risk of seizures (3.922; 95% CI [3.529-4.360]) and venous thromboembolism (VTE) (3.490; 95% CI [3.070-3.969]). Risks of preeclampsia and hemorrhage were comparable. SB patients with hydrocephalus and Chiari malformation type 1 (CM-1) or type 2 (CM-2) were compared to patients without these comorbid conditions. This sub-group analysis showed a significantly increased risk of having cesarean deliveries (SB with hydrocephalus: 12.55%, S.B. with CM-1 or CM-2: 12.81% vs. SB without hydrocephalus or CM, 6.16%) and VTE (3.74%, 2.43% vs. 0.81%). There were also increased risks of hemorrhage and seizures and decreased use of neuraxial analgesia, but the sample size was insufficient. CONCLUSION: SB patients were more likely to undergo cesarean section and exhibit peripartum complications compared to those without SB.

The Predictors of Seizures in Patients with Encephalocele: An 11-Year Experience from a Tertiary Hospital.

INTRODUCTION: The aim of the study was to investigate and identify the predictors associated with the incidence of seizures in patients with encephalocele (EC). METHODS: A retrospective analysis was undertaken of patients treated for EC at a tertiary medical center in Tehran between 2010 and 2021. Data including age at presentation, gender, location, size, and content of EC, ventriculomegaly, hydrocephalus, associated anomalies, and neurodevelopmental delay (NDD) were evaluated for their prognostic value. In addition, univariate and multivariate analyses were performed to identify the correlation between independent predictors and seizure incidence. RESULTS: One hundred and two cases of EC were identified. Seventy-one ECs (69.6%) were posterior ECs, while 31 (30.4%) were anterior. Neural tissue was found in 43 (42.2%) of the ECs. Thirty-three patients (32.4%) had ventriculomegaly, of which 90.9% underwent shunt placement for progressive or symptomatic hydrocephalus. Seizure was found in 26 (25.5%) patients. On univariate analysis, presence of other anomalies, postoperative infections, and NDD were associated with seizures (p < 0.05). When the anomalies were categorized into intracranial and extracranial groups in univariate analysis, none was associated with statistically significant increase in seizure (p values of 0.09 and 0.61, respectively). Although according to multivariate analysis, only the association between other associated anomalies and seizure was near significant (OR: 2.0, 95% CI: 0.95-4.2, p = 0.049). Children with NDD and postoperative infection were, respectively, 3.04 and 1.3 times more at risk to experience seizures compared to other patients. CONCLUSION: We found a rate of 25.5% risk of seizure in patients with EC. This study could not find any significant predictors of seizure in children with EC. However, pediatric patients with postoperative infections including sepsis, wound infection, and NDD require more consideration to reduce the risk of seizure.

Survival status and predictors of mortality among children who underwent ventriculoperitoneal shunt surgery at public hospitals in Addis Ababa, Ethiopia.

Background: Despite the increase in ventriculoperitoneal shunt surgeries performed for children with hydrocephalus, the potential complications and survival of patients after the procedure remains the major challenge for both clinical and public health aspects. This study intends to assess the survival status and scrutinize the predictive factors of mortality among children after a ventriculoperitoneal shunt.Methods: A retrospective cohort study was employed by reviewing charts of 337 systematically selected children who have undergone a ventriculoperitoneal shunt from 2016 to 2018 in Addis Ababa. The extraction tool was used for data collection, Epi-data version 4.4.2 for data entry, and Stata version-14 for cleaning and analysis. Kaplan-Meier curve, log-rank test, and life table were used to describe the data. Cox proportional hazard regression model was used for analysis. Any variable at p < 0.25 in the bi-variable analysis was fitted to multivariate analysis, and significance was declared at p ≤ 0.05. Then, AHR with 95% CI was used to report the association and test the statistical significance. Finally, texts and tables were used to present the results.Results and Conclusion: The incidence rate of mortality was 58.4 per 1000 child-months of observation with a median survival time of 12 months (95%CI: 9.04-14.96). Communicative hydrocephalus (AHR: 1.99, 95% CI: 1.18-3.36), post-traumatic brain injury (AHR: 7.43, 95% CI: 3.21-16.88), emergency surgery (AHR: 1.86, 95% CI: 1.17-3.13) as well as revised shunt procedure (AHR: 8.01, 95% CI: 6.12-13.43) were independent predictors of death. Besides, sunset eye (ARH: 2.01; 95% CI: 1.17-3.47), rapidly increased head size (ARH: 2.05, 95% CI: 1.14-3.37), prolonged antibiotics treatment (AHR: 2.46, 95% CI: 1.82-7.37), and gram-negative infections (AHR: 1.95, 95% CI: 1.60-12.64) were also significantly associated. Hence, health professionals ought to give special attention to patients with identified predictors.

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.

Assessment of the prevalence and associated risk factors of pediatric hydrocephalus in diagnostic centers in Addis Ababa, Ethiopia.

BACKGROUND: Hydrocephalus (HCP) is a common disorder of cerebral spinal fluid (CSF) physiology resulting in abnormal expansion of the cerebral ventricles. Infants commonly present with progressive macrocephaly whereas children older than 2 years generally present with signs and symptoms of intracranial hypertension. Neither qualitatively nor quantitatively are there adequate data to determine the prevalence and incidence of HCP in the developing world. HCP is a treatable condition that when left untreated, has fatal consequences. OBJECTIVE: The objective of this study was to assess the prevalence of pediatric HCP and associated risk factors in diagnostic centers in Addis Ababa, Ethiopia. METHODS: This study was conducted using a cross-sectional facility-based study design over a two-time period, i.e. a 2-year retrospective data collection from January 2018 to January 2020 included 1101 patients and a prospective data collection from May 2019 to February 2020 included 99 patients. Children aged 5 years and below who came to the selected diagnostic centers for MRI/CT examination were studied. The collected data were analyzed using binary logistic regression. RESULT: The retrospective study included 639(58%) males and 462 (42%) females. The mean age calculated was 22.3 months. Infants aged younger than 24 months 753 (68.4%) were significantly associated with HCP development (P < 0.05). In the retrospective study, HCP etiologies; Aqueductal stenosis (17.9%), Neural Tube defects (NTDs) (35.7%), post-infectious (10.1%) were identified. In the prospective study, the gender and age distribution was 57(57.6%) males, 42 (42.4%) females, 60.6% infants aged younger than 24 months with a mean age of 24.9 months. Inadequate consumption of folic acid and development of HCP was found to be statistically significant (P < 0.05). In the prospective study, HCP etiologies; Aqueductal stenosis (26.1%), Neural Tube defects (26.08%), and post-infectious (8.69%) were identified. The 3 years prevalence of HCP calculated in both studies was 22% (223 per 1000 live births). CONCLUSION: The results of this study suggest that the high prevalence of HCP was due to the high prevalence of aqueductal stenosis and neural tube defects; with a small contribution of post-infectious causes. The majority of infants who present with HCP were aged younger than 24 months.

Periventricular hypodensity is associated with the incidence of pre-shunt seizure in hydrocephalic children.

BACKGROUND: The seizure incidence in hydrocephalic children has been acknowledged in a lot of studies previously; nonetheless, seizure pathogenesis in these children remains unclear. Its high proportion of hydrocephalic children who underwent shunt surgery suggests that the seizure might be associated with the protocol of shunt placement and/or the shunt existence intracranially; however, this hypothesis could not explain the pre-shunt seizure incidence in hydrocephalic children. OBJECTIVE: This study aims to evaluate the patients' characteristics and CT findings in pre-shunt hydrocephalic children to identify the possible seizure etiology in these patients. METHODS: Three hundred and thirty-four children with hydrocephalus were included in this study, including 147 hydrocephalic children with the pre-shunt seizure history and 187 hydrocephalic children presented without the pre-shunt seizure history. The following information was retrieved from the patients' medical records: gender, age, pediatric Glasgow Coma Scale (pGCS) upon admission, and hydrocephalus diagnoses. CT findings were re-evaluated to assess the compression association of sulci and gyri, Sylvian fissures, cisterns, FH/ID ratio, Evan's ratio, and periventricular hypodensity with pre-shunt seizure. RESULTS: The results show that the pre-shunt seizure incidence is significantly higher in hydrocephalic children aged 1 to 5 years old (63/113 (55%), p = 0.0001), diagnosed with communicating hydrocephalus (97/163 (59%), p = 0.0001) or infectious hydrocephalus (80/109 (73%), p = 0.0001). The presence of periventricular hypodensity is significantly associated with the pre-shunt seizure incidence (132/205 (64.3%), p = 0.0001). Results from univariate analyses suggest significant association between periventricular hypodensity in every location and pre-shunt seizure (p < 0.0001). Multivariate analyses identify that temporal horn in the right lateral ventricle as the location of periventricular hypodensity has the strongest association with the pre-shunt seizure. CONCLUSION: The presence of periventricular hypodensity in head CT scan is significantly associated with the pre-shunt seizure incidence. Further investigation to confirm this finding and evaluate the possible roles of inflammation in the pre-shunt seizure in hydrocephalic children is important to seek its possible implication on the treatment of pre-shunt seizure in these children.

Characteristics and management of hydrocephalus in adult patients with cerebellar glioblastoma: lessons from a French nationwide series of 118 cases.

The characteristics of hydrocephalus associated with cerebellar glioblastoma (cGB) remain poorly known. The objectives were to describe the occurence of hydrocephalus in a French nationwide series of adult patients with cGB, to identify the characteristics associated with hydrocephalus and to analyze the outcomes associated with the different surgical strategies, in order to propose practical guidelines. Consecutive cases of adult cGB patients prospectively recorded into the French Brain Tumor Database between 2003 and 2017 were screened. Diagnosis was confirmed by a centralized neuropathological review. Among 118 patients with cGB (mean age 55.9 years), 49 patients (41.5%) presented with pre-operative hydrocephalus. Thirteen patients (11.0%) developed acute (n=7) or delayed (n=6) hydrocephalus postoperatively. Compared to patients without hydrocephalus at admission, patients with hydrocephalus were younger (52.0 years vs 58.6 years, p=0.03) and underwent more frequently tumor resection (93.9% vs 73.9%, p=0.006). A total of 40 cerebrospinal-fluid diversion procedures were performed, including 18 endoscopic third ventriculostomies, 12 ventriculoperitoneal shunts and 10 external ventricular drains. The different cerebrospinal-fluid diversion options had comparable functional results and complication rates. Among the 89 patients surgically managed for cGB without prior cerebrospinal-fluid diversion, 7 (7.9%) were long-term shunt-dependant. Hydrocephalus is frequent in patients with cGB and has to be carefully managed in order not to interfere with adjuvant oncological treatments. In case of symptomatic hydrocephalus, a cerebrospinal-fluid diversion is mandatory, especially if surgical resection is not feasible. In case of asymptomatic hydrocephalus, a cerebrospinal-fluid diversion has to be discussed only if surgical resection is not feasible.

Ophthalmological Evaluation in Children Presenting With a Primary Brain Tumor.

BACKGROUND: Children with a brain tumor are prone to develop visual impairment, which to date is often underestimated and unrecognized. Our aim was to assess the prevalence of ophthalmological evaluation and abnormal ophthalmological findings, and investigate whether demographic and tumor-related characteristics are associated with abnormal ophthalmological findings in children presenting with a primary brain tumor. METHODS: Medical records of all 90 children diagnosed with a primary brain tumor between June 2018 and May 2019 and treated at the Princess Máxima Center for Pediatric Oncology, a tertiary referral center in the Netherlands, were retrospectively reviewed. Univariate regression analysis was used to investigate associations between demographic, tumor-related and clinical characteristics, and abnormal ophthalmological findings. RESULTS: Sixty children (34 male [56.7%]; median [range] age, 9.3 [0-16.9] years) underwent ophthalmological evaluation within 6 weeks before or after diagnosis, 11 children (5 male [45.5%]; median [range] age, 5.7 [0.1-17.2] years) were seen more than 6 weeks before or after diagnosis, and 19 children (7 male [36.8%]; median [range] age, 7.2 [1.9-16.6] years) did not receive ophthalmological evaluation within at least 6 months from diagnosis. A total of 19 children (21.1%) presented with visual symptoms as first sign leading to the diagnosis of a brain tumor. Children who presented with visual symptoms (odds ratio [OR], 22.52; 95% confidence interval [CI], 4.90-103.60) and/or hydrocephalus (OR, 3.60; 95% CI, 1.38-9.36) at diagnosis were more often seen for ophthalmological evaluation. The most common abnormal ophthalmological findings were eye movement disorders (66.0%), papilledema (44.1%), and visual field defects (58.1%). Eye movement disorders occurred more frequently in patients with an infratentorial tumor (OR, 4.71; 95% CI, 1.03-21.65). The risk of papilledema was associated with older age (OR, 1.19; 95% CI, 1.05-1.34), hydrocephalus (OR, 9.63; 95% CI, 2.68-34.61), and infratentorial (OR, 9.11; 95% CI, 1.77-46.78) and supratentorial (OR, 13.13; 95% CI, 1.92-89.52) tumors. CONCLUSIONS: In this study, most children with a primary brain tumor underwent ophthalmological evaluation around diagnosis, 21% of the children were not evaluated. The high prevalence of abnormal ophthalmological findings stresses the importance of early standardized ophthalmological evaluation to detect visual impairment and provide timely treatment to potentially prevent permanent visual loss.

What Are the Risk Factors for Epilepsy Among Patients With Craniosynostosis?

PURPOSE: The purpose of this study was to determine the risk factors for epilepsy among patients with craniosynostosis. METHODS: This is a retrospective cohort study that was completed with the Kids' Inpatient Database. All patients diagnosed with craniosynostosis between the years 2000 and 2012 were included. The primary predictor variables were obstructive sleep apnea (OSA), hydrocephalus, brain compression (BC), cerebral edema, papilledema, dolichocephaly, and plagiocephaly. The outcome variable was epilepsy. Logistic regression analysis was used to determine odds ratios (ORs) for the outcome (epilepsy). RESULTS: Our final sample had 4,709 patients with craniosynostosis, of whom 244 patients exhibited epilepsy (5.2%). The mean age of the patients was 1.43 years (range: 0-20). Relative to Asian patients, Black patients were 4 times more likely to have epilepsy (P < .05). Relative to patients in fringe counties of metro areas with a population of at least 1 million, patients in metro areas of 50,000 to 249,999 population were almost 2 times more likely to have epilepsy (P < .01). Hydrocephalus (OR, 6.6; P < .001), BC (OR, 2.4; P < .01), and OSA (OR, 3.0; P < .001) were independent risk factors for epilepsy among our sample of patients with craniosynostosis. CONCLUSIONS: Hydrocephalus, OSA, and BC increase the risk of epilepsy in patients with craniosynostosis. Black patients with craniosynostosis are also at increased risk for epilepsy. With regard to location, patients in areas with a population of 50,000 to 250,000 were at increased risk for epilepsy.

Management and safety of intraoperative ventriculostomy during early surgery for ruptured intracranial aneurysms.

BACKGROUND: Brain edema and/or acute hydrocephalus are common features that limit working space during early surgery of aneurysmal subarachnoid hemorrhage (aSAH). Intraoperative ventriculostomy offers an immediate brain relaxation. However, management and complications related to the routine use of intraoperative external ventricular drainage (iEVD) are not well investigated. METHODS: We retrospectively reviewed all patients who were treated with pterional craniotomy and clipping for ruptured anterior circulation aneurysms in our center between 2012 and 2019. We included in this study all patients submitted to iEVD using the Paine's point on the side of craniotomy. Indication for positioning of an iEVD was given in all cases whenever initial cisternal dissection was hampered by the lack of cerebrospinal fluid (CSF) circulation due to SAH and/or hydrocephalus. RESULTS: In the study period, 162 patients with aSAH underwent surgical clipping. In 103 patients, an iEVD was used. The overall rate of iEVD-related complications was 6.7%, including 3 cases of catheter misplacement, one case of catheter obstruction, one case of related hemorrhage, and 2 cases of infection. The rate of shunt-dependent hydrocephalus was 16.5% (17/103 patients). CONCLUSION: In our experience, iEVD is a safe technique that facilitates dissection during early surgery for intracranial ruptured aneurysms, without requiring an additional burr hole procedure.

Post-traumatic hydrocephalus - incidence, risk factors, treatment, and clinical outcome.

OBJECTIVES: Post-traumatic hydrocephalus (PTH) is well-known after traumatic brain injury (TBI), but there is limited evidence regarding patient selection for ventriculo-peritoneal (VP)-shunt treatment. In this study, we investigated the incidence and risk factors for PTH and the indication for and outcome after shunt treatment. MATERIALS AND METHODS: In this retrospective study, 836 TBI patients, treated at our neurointensive care (NIC) unit at Uppsala university hospital, Sweden, between 2008 and 2018, were included. Demography, admission status, radiology, treatments, and outcome variables were evaluated. RESULTS: Post-traumatic ventriculomegaly occurred in 46% of all patients at NIC discharge. Twenty-nine (3.5%) patients received a VP-shunt. Lower GCS M at admission, greater amount of subarachnoid hemorrhage, meningitis, decompressive craniectomy (DC), and ventriculomegaly at NIC discharge were risk factors for receiving a VP-shunt. Fourteen of the PTH patients showed impeded recovery or low-pressure hydrocephalus symptoms, of whom 13 experienced subjective clinical improvement after shunt treatment. Five PTH patients showed deterioration in consciousness, of whom four improved following shunt treatment. Five DC patients received a shunt due to subdural hygromas (n =2) or external brain herniation (n = 3), of whom two patients improved following treatment. Five patients were vegetative with concurrent ventriculomegaly and these patients did not have any positive shunt response. Altogether, 19 (66%) PTH patients improved after shunt surgery. CONCLUSION: Post-traumatic ventriculomegaly was common, but few developed symptomatic PTH and received a VP-shunt. Patients with low-pressure hydrocephalus symptoms had the best shunt response, whereas patients with suspected vegetative state exhibited a minimal shunt response.

[Epidemiology of Idiopathic Normal Pressure Hydrocephalus and Hereditary Hydrocephalus].

Several cohort studies in Japan have revealed that the prevalence of idiopathic normal pressure hydrocephalus(iNPH)is around 1.6% among the elderly population(≧ 50 years old). The incidence of iNPH from the Yamagata(Takahata)cohort was 1.2/ 1,000 person-years in the elderly population. Although the Japanese guidelines for iNPH clearly describe the definition of "possible iNPH with MRI support," it is still difficult to find out not only patients with iNPH but also individuals in its preclinical stage with radiological findings of asymptomatic ventriculomegaly with features of iNPH on MRI(AVIM)or asymptomatic ventricular enlargement(AVE). It is assumed that only less than 10% of patients with iNPH were referred to hospitals in Japan. Several genes associated with congenital hydrocephalus have been found, including ciliopathy-related genes that directly affect the ependymal cilia in ventricles. Loss of the copy number of SFMBT1 was found to be a risk factor for iNPH. Knowledge about risk genes and their mechanisms in congenital and familial NPH may be a clue for the further understanding of the pathophysiology of iNPH.