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Background Hydrocephalus is a neurological disease with higher prevalence in the pediatric population, often managed by placing a shunt. This hollow tube drains excess cerebrospinal fluid from the brain to other body parts, resulting in several complications, including neurological and psychometric manifestations and a compromised quality of life (QoL). This study aimed to evaluate QoL in patients with hydrocephalus shunt placement within the pediatric population. Methods This prospective observational study was conducted in two major Pakistani tertiary care hospitals. A total of 100 subjects were enrolled, of which 52 were found eligible. A validated questionnaire, Hydrocephalus Outcome Questionnaire, was used to evaluate patients' QoL. Results This study included pediatric patients with a mean age of 6.54 years and a standard deviation of ± 2.64. The male-to-female ratio was 27:25; 2% of patients had congenital or tumor-induced hydrocephalus, while cases of meningitis, encephalocele, and encephalitis accounted for 8, 4, and 2%, respectively. Myelomeningocele had the highest prevalence at 16%. The overall health scores range from 0.39 to 0.51. Social, cognitive, and physical health scores have mean values of 0.54, 0.50, and 0.48, respectively. The minimum physical health score is 0.17, indicating the most significant impact of hydrocephalus on physical function. Conclusion This study highlights variations in hydrocephalus severity among pediatric patients, impacting their overall QoL, primarily physical and behavioral functioning. Worse health outcomes were associated with frequent seizures, prolonged hospital stays for diagnosis and treatment, shunt infections, increased number of shunt catheters, and longer travel distances to medical facilities. [ABSTRACT FROM AUTHOR]

Introduction: Three-dimensional measurements of intracranial volume (ICV) can guide clinical management of brain and skull disorders. However, widespread clinical access is limited by the scarcity of software methods for analyzing CT scans, which are more available than MRI images, and the inaccessibility of proprietary and expensive commercial software. The presented method can calculate ICV from CT scans, using an open-source software, 3D Slicer. Methods: The open-source workflow was optimized with a data-driven approach to find the optimal parameters for ICV accuracy. The accuracy of the open-source method was determined by comparing it to commercial and proprietary software with CT scans of pediatric hydrocephalic macrocephaly patients with craniosynostosis undergoing total vault reconstruction (N = 5 patients,15 scans). Results: An open-source pipeline that combines an initial semi-automatic segmentation of a coronal CT reconstruction with a fully automatic segmentation minimizes the ICV error. The open-source method shows excellent agreement with both the commercial and proprietary software methods (R2 = 0.998 and 95% confidence interval of best-fit line slope: [0.986; 1.047], [0.985;1.066] respectively). The mean percent difference of ICV measurements of the open-source software from the commercial software was −0.56% [95% CI: −1.08%, −0.028%] and from the proprietary software was −0.07% [95% CI: −1.26%, 1.1%]. The mean percent difference of ICV measurements of the commercial software from the proprietary software was 0.36% [95% Confidence Interval: −0.61%, 1.32%]. Conclusions: This is the first study comparing an open-source method for measuring ICV with commercial and proprietary options. A high degree of fidelity was found, confirming this open-source method as a viable option for clinicians who are looking to incorporate ICV measurements into their practice. [ABSTRACT FROM AUTHOR]

Aim: Hydrocephalus is a common condition in neurosurgical practice in patients of all ages. Despite significant advances in the technology and design of ventriculoperitoneal (VP) shunt systems, shunt failure remains a major problem in neurosurgical clinics. Antibiotic prophylaxis is an important step in protocols to prevent VP shunt infections. In this study, we investigated VP shunt infection incidence and VP shunt infection prophylaxis used in pediatric hydrocephalus cases at our center and the parameters that may influence shunt infection. Materials and Methods: Between 01/01/2018 and 01/01/2023, cases younger than 18 years of age who underwent VP shunt surgery in our hospital were included in the study. Clinical data and demographic information were retrospectively obtained from medical records. The primary results of our study were the frequency of VP shunt infection in the first 3 months after surgery and whether there was a difference in shunt infection between antibiotic applications used for prophylaxis. Other factors that may influence VP shunt infection were evaluated as secondary outcomes. Results: Within a 5-year period, 28 cases were identified that met our study criteria. The overall rate of VP shunt infections was 21.4%. In 85.7% of patients, 3rdgeneration cephalosporins were used for prophylaxis, and in the others (14.3%), 1stgeneration cephalosporins were used. VP Shunt infections did not occur in patients receiving 1st generation cephalosporins. However, possibly due to the limited number of cases, no statistical significance (p=0.549) was detected between the groups. There was no statistical difference in other demographic data between the groups with and without VP shunt infection. Conclusion: In our patient group, which was predominantly infants, prophylactic use of 1st-generation cephalosporins to prevent VP shunt infection showed similar efficacy to the use of 3rd-generation cephalosporins. 1st-generation cephalosporins should be preferred, especially in terms of reducing costs and resistant infections. [ABSTRACT FROM AUTHOR]

Background: Infratentorial choroid plexus tumors are rare lesions in the pediatric population. At diagnosis, they most often present with signs of intracranial hypertension and cranial nerve deficits. The main objective of the surgery is total resection and the prognosis is favorable in most cases. Case Report: A 16-year-old boy with gait disturbance, headaches and left side hypoacusia had a diagnosis of posterior fossa tumor with hypertensive hydrocephalus. He underwent urgent placement of a ventriculoperitoneal shunt and subsequently microsurgery with gross total resection. At follow up, the patient was asymptomatic and without deficits. Conclusion: Choroid plexus tumors should be included in the differential diagnosis of posterior fossa tumors in childhood. Preoperative planning, choosing the best surgical approach and interruption of the lesion's vascular supply as the initial step of surgery are fundamental strategies for successful treatment. [ABSTRACT FROM AUTHOR]

Displacement of the catheter outside the intrabdominal area after ventriculoperitoneal (VP) shunt is an extremely rare complication. In a 4-month-old male patient with a history of VP shunt due to hydrocephalus in the neonatal period, abdominal X-ray showed that the distal end of the shunt catheter was in the scrotum. The catheter tip was reduced to the abdomen via left inguinal herniotomy, and hernia repair was performed with high ligation. Migration of the VP shunt catheter into the scrotal area is a complication that is easy to treat, but has not yet been clarified in terms of prevention. The recognition of this complication and its correction with early surgical intervention are important in terms of preventing possible visceral organ damage and clinical worsening due to shunt obstruction. [ABSTRACT FROM AUTHOR]

Background: Third ventricle morphological changes reflect changes in the ventricular system in pediatric hydrocephalus, so visual inspection of the third ventricle shape is standard practice. However, normal pediatric reference data are not available. Objective: To investigate both the normal development of the third ventricle in the 0–18-year age group and changes in its biometry due to hydrocephalus. Materials and methods: For this retrospective study, we selected individuals ages 0–18 years who had magnetic resonance imaging (MRI) from 2012 to 2020. We included 700 children (331 girls) who had three-dimensional (3-D) T1-weighted sequences without and 25 with hydrocephalus (11 girls). We measured the distances between the anatomical structures limiting the third ventricle by dividing the third ventricle into anterior and posterior regions. We made seven linear measurements and three index calculations using 3DSlicer and MRICloud pipeline, and we analyzed the results of 23 age groups in normal and hydrocephalic patients using SPSS (v. 23). Results: Salient findings are: (1) The posterior part of the third ventricle is more affected by both developmental and hydrocephalus-related changes. (2) For third ventricle measurements, gender was insignificant while age was significant. (3) Normal third ventricular volumetric development showed a segmental increase in the 0–18 age range. The hydrocephalic third ventricle volume cut-off value in this age group was 3 cm3. Conclusion: This study describes third ventricle morphometry using a linear measurement method. The ratios defined in the midsagittal plane were clinically useful for diagnosing the hydrocephalic third ventricle. The linear and volumetric reference data and ratios are expected to help increase diagnostic accuracy in distinguishing normal and hydrocephalic third ventricles. [ABSTRACT FROM AUTHOR]

Background: Hydrocephalus is frequent in sub-Saharan African countries. The postinfectious hydrocephalus tends to decrease. The objective of this study was to identify the etiologies and outcomes of hydrocephalus. Methods: This was a retrospective study of hydrocephalus cases (0-15 years old) treated in the neurosurgery unit of the Sylvanus Olympio Hospital in Lomé over 10 years (2012-2021). At 1 year, the evolution distinguished in two categories: (1) Good psychomotor development: no delay in the acquisition of walking, language, and school. (2) Psychomotor delay: delay in the acquisition of walking, language, and school. Results: We reported 305 children treated for hydrocephalus representing 1.8% of all neurosurgery unit patients and 34.2% of pediatric pathologies. There was a male predominance (60.6%). We noted second degree consanguinity in 8.5%. The positive maternal serologies were HIV (12.4%), syphilis (8.2%), and toxoplasmosis (2.6%). A malaria episode had been treated during the first trimester in 36.7% of the mothers. The main clinical sign of hydrocephalus was 91.5% of Macrocephalus. Congenital Malformafions were the most common etiologies of hydrocephalus (68.5%). Ventriculoperitoneal shunt was the main surgical method used and 16 deaths were recorded. The mediumterm evolution (1 year) was evaluated in 36.1% and noted 61.8% of psychomotor retardation. Conclusion: This study confirms the trend of the predominance of congenital causes of hydrocephalus in Africa, even if maternal infections can be involved in the development of some of them. The morbimortality of this pathology remains important, especially concerning neurocognitive outcomes. [ABSTRACT FROM AUTHOR]

Two thirds of pediatric gliomas are classified as low-grade (LGG), while in adults only around 20% of gliomas are low-grade. However, these tumors do not only differ in their incidence but also in their location, behavior and, subsequently, treatment. Pediatric LGG constitute 65% of pilocytic astrocytomas, while in adults the most commonly found histology is diffuse low-grade glioma (WHO II), which mostly occurs in eloquent regions of the brain, while its pediatric counterpart is frequently found in the infratentorial compartment. The different tumor locations require different skillsets from neurosurgeons. In adult LGG, a common practice is awake surgery, which is rarely performed on children. On the other hand, pediatric neurosurgeons are more commonly confronted with infratentorial tumors causing hydrocephalus, which more often require endoscopic or shunt procedures to restore the cerebrospinal fluid flow. In adult and pediatric LGG surgery, gross total excision is the primary treatment strategy. Only tumor recurrences or progression warrant adjuvant therapy with either chemo- or radiotherapy. In pediatric LGG, MEK inhibitors have shown promising initial results in treating recurrent LGG and several ongoing trials are investigating their role and safety. Moreover, predisposition syndromes, such as neurofibromatosis or tuberous sclerosis complex, can increase the risk of developing LGG in children, while in adults, usually no tumor growth in these syndromes is observed. In this review, we discuss and compare the differences between pediatric and adult LGG, emphasizing that pediatric LGG should not be approached and managed in the same way as adult LCG. [ABSTRACT FROM AUTHOR]

A 3, 5-years-old girl was being followed for prenatal intraventricular hemorrhage and hydrocephalus. A ventriculoperitoneal shunt had been placed to treat progressive hydrocephalus 1 year after cesarean delivery. The shunt was revised 2 years later due to shunt dysfunction. Six months later, wound revision was performed at our hospital due to the presence of discharge from the cranial tip wound. The patient wasplaced on antibiotic therapy. One week later she was admitted with intermittent vomiting. Physicalexamination revealed right lower quadrant tenderness. Standing abdominal x-ray and abdominal ultrasound showed free fluid in the abdomen and intestinal wall thickening. Preliminary diagnosis was ileus and the patient underwent emergency surgery by a pediatric surgeon. During surgery, the catheter was observed to revolve around the jejunal segment like a cuff, disrupting intestinal nutrition and causing necrosis. The necrotic intestine was resected and end-to-end anastomosis was performed. Recovery was uneventful and she began oral feeding 5 days postoperatively. Abdominal complications of a ventriculoperitoneal shunt should be considered in the differential diagnosis in a child with a VPS who presents to the hospital with abdominal pathology. [ABSTRACT FROM AUTHOR]

Introduction: The management of pediatric airway is of great challenge, especially when associated with congenital anomalies or acquired conditions like aspiration or tracheal stenosis. Cases: We present a case series of various conditions associated with anticipated difficult airway in pediatrics: An infant with Dandy-Walker syndrome and hydrocephalus, a toddler with history suggestive of foreign body aspiration 15 days back, and another infant presenting with post-intubation subglottic stenosis. Discussion: This case series describes the implications of various airway related challenges in pediatric patients whether congenital or acquired. Providing anesthetic care to infants adds to the risks as there are wide variations in anatomy, physiology and pharmacology when compared to older children. Therefore thorough pre-anesthetic evaluation of such patients is mandatory with special focus on airway examination. Conclusion: Considering the complexity of pediatric airway and associated anomalies, difficult airway should be anticipated in pediatric patients. Preparedness for the same along with optimal positioning increases the chances of successful intubation and reduces procedural complications. [ABSTRACT FROM AUTHOR]

Purpose: We report five rare cases of programmable valve breakage (Codman Hakim-Medos valve) in shunt systems of children with posthemorrhagic hydrocephalus. Only four similar studies have been published in the current literature. Methods: Between 2013 and 2018, five children with posthemorrhagic hydrocephalus were admitted to the pediatric department. All patients had a history of slight blows to the head in a minor trauma and follow up MRI scans. After initial clinical examination, cranial computed tomography (CT) and X-ray were conducted. Results: In all cases, pumping the reservoir resulted in very slow refilling. The cranial CT in one patient showed slit ventricles confirming the suspicion of overdrainage, the other cases a slight enhancement of the hydrocephalus. In lateral X-rays of the skull in comparison to the first X-ray control of the shunt valve, the pressure control chamber could be seen dislocated in the inferior part of the reservoir in all cases. Surgery revealed that the shunt valve was broken. The pressure control chamber had dropped to the bottom of the reservoir. After implantation of a new shunt valve, the symptoms resolved completely in all five children. Overall this complication occurred in 4.3% (5 of 85 implanted Codman Hakim-Medos valve) of all children necessitating ventriculoperitoneal shunt implantation between January 2013 and December 2018. Conclusion: The well-accepted Codman Hakim-Medos programmable valve is part of a tube-system, which is designed to offer the possibility of a reliable and precise treatment of hydrocephalus. Various mechanical and non-mechanical complications of shunt systems have been reported. Valve breakage is a very rare condition, often missed, and must be kept in mind when trauma and prior MRI scan are reported. [ABSTRACT FROM AUTHOR]

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Objective: The goal of this study was to determine the relative frequencies of benign external hydrocephalus (BEH), hydrocephalus and other conditions in a large series of imaging studies performed for macrocephaly and identify additional risk factors for patients with macrocephaly that would most likely benefit from neuroimaging evaluation. Material and Methods: Medical records at our center were searched for the term macrocephaly retrospectively. The search extended from 1th January 2014 to 1th June 2019. Patients older than 36 months of age were excluded. Information about age, gender, symptoms and clinical signs (seizures, neurologic abnormalities on exam such as hypotonia), neuroimaging findings, developmental delay, family history of macrocephaly and head circumference (HC) were collected for each patient. Results: A total of 103 patients were included in the analysis. The mean age at the time of imaging was 9 months (±5.5 months). Twenty-one (20.3%) of the subjects were female and 82 (79.6%) were male. Twenty-nine of the imaging studies were magnetic resonance imaging, 26 were computed tomography and 65 were head ultrasounds. Patients with abnormal neuroimaging results had significantly higher rates of developmental delay or abnormal neurologic exam than patients with normal neuroimaging results or BEH (p=0.003 and p<0.0001). There was no significant difference between the neuroimaging results of patients with and without positive family history of macrocephaly. Conclusion: This study suggests that neuroimaging for macrocephaly has almost negligible diagnostic yield unless having developmental delay or abnormal neurological examination. [ABSTRACT FROM AUTHOR]

Background: Kleeblattschädel skull, also known as the cloverleaf is a complex synostosis which presents with an enlarged tri-lobar skull resulting temporal bulging and a flat posterior skull, a rare find in the population. This pansynostosis is associated with other congenital syndromes which include Crouzon, Pfeifer, and Carpenter's. The genetic disorder Pfeiffer syndrome results from skull bones' premature fusion characterized by deformities of the hand and feet. The author reports a 10-month-old girl who presented with a tri-lobar skull and wide toes which is a significant finding of Pfeiffer syndrome. Case presentation: A 10-month-old girl presents with congenital obstructive hydrocephalus due to cloverleaf skull with horizontally enlarged head and large fontanelles. The child also had mid-facial hypoplasia and pre-axial bilateral lower limb polydactyly. Wide toes were also observed, an indicator of Pfeiffer's Syndrome. Computed tomography (CAT) scans grossly abnormal craniofacial appearances, with premature closure of the sagittal suture giving a cloverleaf skull appearance. There was also a significant thinning of the brain parenchyma. Conclusion: Premature closure of sutures leads to a forced growth in a plane perpendicular to the closed suture. Cloverleaf deformity can present with multiple syndromes such as Pfeiffer's. This leads to gross alteration of the skull with potential underlying morbidity. [ABSTRACT FROM AUTHOR]

The purpose of this study was to investigate the presence of an apraxic condition in children with meningomyelocele. Subjects were grouped according to the presence or absence of hydrocephalus as diagnosed by a computerized tomography scan. Subjects performed tasks of simple movements and motor sequences first to verbal command and then to imitation. For simple movements there was a significant Group by Cue interaction. The hydrocephalic group, significantly inferior on the verbal cue, were noticeably better on imitation. For those subjects without hydrocephalus, such was unlikely due to a ceiling effect to the verbal cue. For motor sequences, there was a significant Cue and Group effect. Both groups improved to the visual cue with the nonhydrocephalus group being consistently superior. The results are discussed in terms of patterns of response, and a cognitive or brainstem/cerebellar-based disorder. [ABSTRACT FROM AUTHOR]

Objective: To explore the clinical characteristics of and analyze the risk factors for hydrocephalus in children with bacterial meningitis. Methods: Retrospective study of a sample of children with bacterial meningitis seen on the pediatric service of Shengjing Hospital of China Medical University between January 1, 2010, and December 31, 2016. Results: Overall, 9.36% (25/267) of patients presented with hydrocephalus. Among patients with hydrocephalus, the age at onset of bacterial meningitis was usually <6 months, 15 patients had confirmed bacterial etiology, and 1 patient died. The most significant results of multivariate analysis for hydrocephalus were a rural living situation, altered level of consciousness, previous treatment with antibiotics, initial cerebrospinal fluid protein >2 g/L, C-reactive protein >100 mg/L, and dexamethasone use. Conclusions: A severe clinical manifestation and significant laboratory index at admission are the most important predictors of hydrocephalus in children with bacterial meningitis. [ABSTRACT FROM AUTHOR]

Objective: The treatment of infectious CSF condition with ventriculitis and hydrocephalus in children is an interdisciplinary challenge. Conventional surgical treatment includes external ventricular drain (EVD) and systemic antibiotic therapy. However, infectious contamination of large ventricles combined with CSF protein overload often requires long treatment regimens. We retrospectively investigated neuroendoscopic lavage as a new option for clearance of CSF in children with hydrocephalus and active CSF infection.Patients and methods: A database review identified 50 consecutive patients treated for CSF infection with hydrocephalus at our institution. Twenty-seven patients (control group, CG) were treated conventionally between 2004 and 2010, while 23 patients (neuroendoscopic group, NEG) underwent neuroendoscopic lavage for removal of intraventricular debris between 2010 and 2015. Clinical data, microbiology, laboratory measures, shunt dependency, and shunt revision rate were evaluated retrospectively.Results: The patient groups did not differ regarding basic clinical characteristics. Patients in NEG received neuroendoscopic lavage at mean of 1.6 ± 1times (1-4). No immediate postoperative complications were observed in NEG patients. Shunt rate in NEG patients was 91% as compared 100% in CG patients (p = 0.109). Within 24 months after shunt implantation, incidence of shunt revision was higher in CG (23/27) compared to NEG (5/23; p < 0.001). Reinfection was observed more often in CG (n = 17) compared to one patient in NEG (p < 0.001).Conclusions: We experienced that neuroendoscopic lavage is a safe and effective treatment for hydrocephalus in children with infectious conditions. Neuroendoscopic lavage resulted in a decreased number of overall shunt revisions in shunt-depended patients as well as a lower number of recurrent infections. [ABSTRACT FROM AUTHOR]

The treatment of hydrocephalus has changed in recent years with better imaging and introduction of endoscopic procedures as well as enhanced shunts. Indications of endoscopic third ventriculostomy (ETV) are now more refined with better quantification of outcome. This article reviews the current state of neuroendoscopy for infective hydrocephalus in children. The roles of third ventriculostomy as a primary procedure or after shunt malfunction, endoscopic interventions in multiloculated hydrocephalus and introduction of intraventricular lavage to salvage severely infected children are evaluated. [ABSTRACT FROM AUTHOR]

Introduction Hydrocephalus can be progressive or spontaneously arrested. In arrested hydrocephalus, the balance between production and absorption of the cerebrospinal fluid is restored. Patients are mostly asymptomatic, and no surgical treatment is necessary for them. Methods We performed a two-center consecutive case series study, aimed at investigating the safety of nonsurgical management of hydrocephalus in selected pediatric patients. We retrospectively selected all consecutive patients, suspected to suffer from arrested hydrocephalus and referred to our two institutions between January 2011 and December 2013. Data on clinical and radiological follow-up were collected until June 2017. Results Five children diagnosed with arrested hydrocephalus were included in the study. All patients presented macrocephaly as the main presenting sign. Associated mild-to-moderate stable motor disorders were assessed in four out of five cases. Typical symptoms and signs associated with acute raised intracranial pressure were absent in all patients. Magnetic resonance imaging studies showed ventriculomegaly in all patients. A diagnosis of arrested hydrocephalus was made in all five cases based on stable clinical and radiological findings during the initial observation. Conservative management based on active surveillance was, therefore, proposed. During the followup period, we observed stable or improved conditions in four out of five patients, while the remaining patient presented progressive hydrocephalus. Discussion Making a distinction between arrested and progressive hydrocephalus is fundamental, because of the opposed appropriate management. Any newly discovered case of hydrocephalus, not characterized by clear signs of progressive hydrocephalus, should benefit from active surveillance before any definitive decision is taken. [ABSTRACT FROM AUTHOR]

Background: Endoscopic third ventriculostomy (ETV) and ventriculo-peritoneal shunt (VPS) although recognized surgical options for non-communicating hydrocephalus have debatable applications.Objective: We analysed a prospective cohort of age-matched children with non-tumor, non-communicating hydrocephalus treated with the two surgical modalities using clinically measurable parameters.Methods: A single institution analysis of age-matched patients with non-communicating hydrocephalus treated with VPS or ETV over a 3-year period. Occipitofrontal circumference (OFC), milestone, shunt independence as well as complication profiles of patients were recorded and analysed. Mean follow-up period was 1.27 ± 0.19 years 95%CI). Data analysis were performed using SPSS version 15, Chicago, IL. Statistical tests were set at 95% significance level.Results: Fifty-five patients were enrolled, 25 patients had ETV, while 30 had VPS. Mean age was 2.3 ± 0.7 years (95% CI) with a range of 3 months to 4.5 years. Aqueductal stenosis was the most common indication. OFC profile decline was significant among the VPS group when compared with ETV group at 3 months follow-up (χ2 = 7.59, df = 1, p < 0.05). There was no difference among the two treatment groups χ2 = 2.47, df = 1, p > 0.05) in milestone profile. Thirteen percent of VPS, compared to (4%) ETV patients, had sepsis (χ2 = 4.59, df = 1 p < 0.05). Ninety-two percent of ETV patients remained shunt free, while 80% of shunted patients achieved ETV independence. Two patients died among the VPS group compared to one patient in the ETV group.Conclusion: VPS compared to ETV is associated with an earlier milestone and OFC response. ETV is associated with lower rates of sepsis and mortality. [ABSTRACT FROM AUTHOR]

Background: Juvenile pilocytic astrocytomas represent the largest group of pediatric brain tumors. The ideal management for these tumors is early, total surgical resection. To detect and track treatment response, a screening tool is needed to identify patients for surgical evaluation and assess the quality of treatment. The identification of aberrant miRNA profiles in the sera of juvenile pilocytic astrocytoma patients could provide such a screening tool.Methods: The authors reviewed the serum profiles of 84 oncologically relevant miRNAs in pediatric juvenile pilocytic astrocytoma patients via qPCR screening.Results: miR-21, miR-15b, miR-23a, and miR-146b were significantly elevated in the sera of JPA patients as compared to non-oncologic controls, oncologic controls, and post-JPA resection samples (p < 0.001, 0.022, 0.034, 0.044). miR-21 had the highest AUC on ROC analysis (AUC > 0.99, sensitivity 75%, specificity 100%). All four miRNAs also correlated well with tumor mural nodule size, though they only poorly correlated with total tumor size, including cystic components (Spearman’s R2: miR-21 91.7 vs 6.9%, miR-15b 86.3 vs 23.1%, miR-23a 85.8 vs 23.0%, miR-146b 59.8 vs 11.9%).Conclusion: In this small pilot study, pediatric juvenile pilocytic astrocytoma patients had significant elevations in serum miR-21, miR-15b, miR-23a, and miR-146b levels that do not appear to be driven by hydrocephalus or local distortion of the intracranial contents. These alterations correlate with solid tumor component volume and reverse with complete tumor resection, suggesting that this serum miRNA profile may delineate biomarkers for screening and tracking juvenile pilocytic astrocytoma patients. Additional studies, with a larger cohort, are needed to verify these results. [ABSTRACT FROM AUTHOR]

We studied the intellectual profiles of 13 Italian children diagnosed with spina bifida myelomeningocele and shunted hydrocephalus (MMC HC) against a control group of children. The results showed that MMC HC group had lower performance in all subtests, four indices, and the FSIQ of the Wechsler Intelligence Scale for Children, forth-edition. The MMC HC group showed flat cognitive profiles between subtests within each index and between four core indices. However, the cognitive abilities that were mostly impaired seemed to be related to visual selective and focused attention, to visual acuity, to visuo-perceptive organization and visuomotor integration and to visual short-term memory. [ABSTRACT FROM AUTHOR]

Although ventriculoperitoneal shunt (VPS) is the most common procedure performed by pediatric neurosurgeons, it is still associated with frustrating complications, most common of which are obstruction and infection. Traumatic occipital artery pseudoaneurysm is a very rare complication of VPS procedure. To the best of our knowledge, there is no similar case reported in the English language literature. A 12-month-old patient suffered posthemorrhagic hydrocephalus of prematurity, and multiple other complications due to extreme prematurity, including immature lung disease, retinopathy of prematurity, necrotizing enterocolitis, bowel perforation, short bowel syndrome resulting in total parenteral nutrition-dependence, and hydrocephalus which was treated by insertion of VPS. Four weeks after the shunt, a slowly enlarging pulsatile swelling distal to the valve under the catheter altering the shunt function was noted. The swelling was diagnosed as a pseudoaneurysm of the occipital artery and treated by excision of the aneurysm. The child developed isolated dilated fourth ventricle, which was treated by endoscopic fourth ventriculostomy. Traumatic occipital artery pseudoaneurysm as a result of tunneling of VPS catheter is a very rare complication of VPS. Clinical and radiological imaging is diagnostic of the aneurysm. Surgical repair should be considered in such cases. This case report is aimed to raise the awareness among physicians about this rare complication. [ABSTRACT FROM AUTHOR]

Objective: Depending on the etiology of hydrocephalus in childhood, the shunt therapy still remains challenging due to frequent shunt complications leading to possible revisions such as shunt infection or shunt malfunction. In myelomeningocele (MMC) patients who often require shunt therapy, higher revisions rates were reported. In a single-center retrospective study, experiences on shunt regimen on hydrocephalus associated with MMC are presented.Methods: Data of 160 infant hydrocephalus cases younger than 1 year of age at the time of implantation were retrospectively reviewed from the hospital database. These patients received an adjustable differential pressure valve with gravitational unit and antibiotic impregnated catheters as a primary or secondary implant during the time period of April 2007 to July 2015. The subgroup of infants cases with MMC (n = 44; age 50.6 ± 80.6 days) were compared to the remaining cohort of other hydrocephalus etiology (control group). The shunt and valve revision free survival rates were recorded until July 2017.Results: During the mean follow-up of 48.7 ± 19.2 (7-114) months, the shunt revision free survival was 87% at 1 year and 49% at 60 months in the MMC cohort. The control group showed a shunt survival rate of 68% at 1 year and 39% at 60 months. Similarly, the valve revision free survival rate showed a significant higher rate of 92% at 1 year and 69% at 60 months in the MMC group compared to the control group (75% at 1 year and 51% at 60 months; p < 0.05). During the entire follow-up period, 37% of the MMC infants underwent a revision operation in contrast to the control group of 40%.Conclusion: The presented shunt strategy showed improved revision free survival rates in infants with a MMC-related hydrocephalus in comparison to other etiologies of hydrocephalus in infants, which might relate to infection prophylaxis and high drainage resistance integrated in the shunt system. [ABSTRACT FROM AUTHOR]

Objective To build and validate journal-type digital and printed educational material for informal caregivers (i.e., the parents who provide care) of children with hydrocephalus. Methods This is a methodologic study conducted in the children's neurosurgery outpatient clinic of a university hospital. The content validity index with a concordance of ≥0.78 was used for validation. Content was validated with the participation of 5 nurses and 3 neurosurgeons, and appearance was validated with the participation of 9 informal caregivers of children with hydrocephalus. Results A 21-page journal was created to address hydrocephalus and its treatment. The content and semantics of this material were validated with a universal content validity index of 0.90 and 0.98, respectively. Conclusions Content and appearance of the educational material for informal caregivers of children with hydrocephalus were successfully validated and considered suitable and user-friendly for health education. [ABSTRACT FROM AUTHOR]

The article discusses the research papers presented at 26th Congress of the European Society for Pediatric Neurosurgery (ESPN) at Bonn, Germany from May 6-9, 2018. Topics discusses include Pattern of closure of the metopic suture and Improvement of sleep apnea after FrontoFacial MonoBloc Advancement and Infant external hydrocephalus is a cerebro-venous disease related to outflow impairment.

Introduction: Recognizing patients with ventriculomegaly who are at risk of developing acute hydrocephalus presents a challenge for the clinician. The association between disturbed cerebrospinal fluid flow (CSF) and impaired brain compliance may play a role in the pathogenesis of hydrocephalus. Phase contrast MRI is a noninvasive technique which can be used to assess CSF parameters. The aim of the work is to evaluate the effectiveness of phase contrast MRI in recognizing patients at risk of acute hydrocephalus, based on measuring the pulsatile CSF flow parameters in the Sylvian aqueduct and prepontine cistern in children with ventriculomegaly.Aim: The aim of the work is to characterize the parameters of cerebrospinal fluid (CSF) flow in the Sylvian aqueduct and prepontine cistern in children with ventriculomegaly with regard to patient age and symptoms. We hypothesize that the relationship between CSF flow parameters in these two regions will vary according to analyzed factors and it will allow to recognize children at risk of hydrocephalus.Materials and methods: A group of 26 children with ventriculomegaly (five girls and 21 boys) underwent phase contrast MRI examinations (Philips 3T Achieva with Q-flow integral application). Amplitudes of average and peak velocities of the CSF flow through the Sylvian aqueduct and prepontine cistern were used to calculate ratios of oscillation and peak velocities, respectively. The relationship between the oscillation coefficient, the peak velocity coefficient, and stroke volume was then assessed in accordance with age and clinical symptoms.Results: The peak velocity coefficient was significantly higher in patients with hyper-oscillating flow through the Sylvian aqueduct (3.04 ± 3.37 vs. 0.54 ± 0.28; p = 0.0094). Moreover, these patients tended to develop symptoms more often (p = 0.0612). No significant age-related changes were observed in CSF flow parameters.Conclusion: Phase contrast MRI is a useful tool for noninvasive assessment of CSF flow parameters. The application of coefficients instead of direct values seems to better represent hemodynamic conditions in the ventricular system. However, further studies are required to evaluate their clinical significance and normal limits. [ABSTRACT FROM AUTHOR]

Objective: Endoscopic third ventriculostomy (ETV) provides a shunt-free treatment for obstructive hydrocephalus children. With rapidly evolving technology, the semi-rigid fiber optic neuroendoscopy shows a potential application in ETV by blunt fenestration. A retrospective analysis of our experience is reviewed.Methods: The authors review infants and children who underwent ETV using this technique from June 2004 to June 2016 with radiological and clinical follow-up done by a single surgeon. Patients who underwent ETV with channel scope were excluded. Demographic variables and operative reports were collected. Improvement of preoperative symptoms and avoidance of additional cerebrospinal fluid (CSF) diversion procedures were considered a success. The ETV success score (ETVSS) was used to correlate with clinical outcomes.Results: A total of 79 patients were included with a mean age of 8.3 ± 5.5 years, and 40.5% were female. The mean clinical and radiographic follow-up was 38.6 ± 40.9 months. The overall complication rate was 6.3%, while 73.4% were considered successful. The ETV failure cases received conversion to ventriculoperitoneal shunt or redo of ETV with a median time of 2 months. The mean ETV success score was 74.3 ± 11.8 with positive correlation between success rate (P < 0.05). Kaplan-Meier failure-free survival rates of 30-day, 90-day, 6-month, 1-year, and 2-year were 89.9, 83.5, 78.5, 75.9, and 74.6%. Eight patients required redo ETV, and five of these patients required eventual shunt placements. Approximately 61.9% of failure occurred within 3 months. Patients with post-intraventricular hemorrhage (IVH) /infection, and age younger than 12 months had the poorest outcome (P < 0.05).Conclusions: Blunt dissection of the third ventricle floor under endoscopic vision with the stylet tip of a fiber optic neuroendoscopy is safe and requires less equipment in the pediatric population. This technique is successful with an optimistic long-term outcome except for infants and the post-IVH and infectious subgroups. [ABSTRACT FROM AUTHOR]

Purpose: Evaluate the management of hydrocephalus in pediatric patients in the Eastern Democratic Republic of Congo by a general surgeon.Methods: Retrospective review of a single institution in the province of North Kivu. Patient charts and surgical notes were reviewed from 2003 to 2016.Results: 116 procedures were performed for an average of 8.9 per year. 51.7% of surgeries were on female patients with an average age of 13.6 ± 22.7. The average distance traveled from home to hospital was 153.7 km but ranged from 5 to 1420 km. The majority of hydrocephalus was due to neonatal sepsis (57%); 33.6% were classified as congenital; 9.5% of cases followed myelomeningocele closure. 97.4% had a ventriculoperitoneal (VP) shunt placed. Endoscopic third ventriculostomy combined choroid plexus cauterization (ETV/CPC) was performed in 2.5% of patients. Shunt infection occurred in 9.5% of patients, shunt dysfunction or obstruction in 5.2% and shunt exteriorization in 1.7%; no complications occurred in patients who underwent ETV/CPC.Conclusion: VP shunt is the predominant management for hydrocephalus in this environment with increasing use of ETV/CPC. Further research is needed to evaluate variability by etiology, short and long-term outcomes of procedures performed by neurosurgeons and general surgeons, and regional epidemiologic variability. [ABSTRACT FROM AUTHOR]

We report a case of a 12-year-old boy with previously shunted congenital hydrocephalus, presenting with a progressive headache, nausea, vomiting, and lethargy. In the brain magnetic resonance imaging, a large cyst was seen in the superior recess of the fourth ventricle extending through the cerebral aqueduct toward the third ventricle. Endoscopic dual fenestration of the cyst was performed successfully using the posterior suboccipital approach through the foramen of Magendie, which resulted in the relief of symptoms without any complications, and the patient was symptom-free in the subsequent follow-up visits for 4 years. [ABSTRACT FROM AUTHOR]

Introduction: Porencephalic cysts and cerebrospinal fluid (CSF) edema around the intracranial shuntcatheter are rare complications of ventriculoperitoneal shunt (VPS) surgery. Possible mechanisms leading to a porencephalic cyst formation in a patient with a VPS include taut ventricle, dysfunction of distalcatheters, and irreversible damage to the brain parenchyma caused by shunt insertion, chemotherapy, or radiation. Most of the previous reports were due to shunt malfunction and treatment consisted of shunt revision or removal.Case Report: We present a case of porencephalic cyst formation in a 6-year-old female as a result ofcerebrospinal fluid under-drainage that was promptly improved with shunt valve adjustment.Coclusions: A heightened index of suspicion is required to prevent misdiagnosis of porencephalic cysts astumors or abscesses that may lead to unnecessary surgical explorations. Further research is needed toelucidate the pathophysiological mechanism that causes a porencephalic cyst formation. [ABSTRACT FROM AUTHOR]

Introduction: Children with myelomeningocele (MMC) often develop hydrocephalus, and the combination of these conditions can lead to psychosocial, cognitive, and physical health issues that decrease their health-related quality of life (HRQOL). The goal of the present study was to understand the QOL in patients with MMC and shunted hydrocephalus.Methods: Data from the Toronto Hydrocephalus QOL Database was acquired between 2004 and 2009 using parent-completed questionnaires and, when appropriate, child-completed questionnaires: Hydrocephalus Outcome Questionnaire (HOQ) and the Health Utilities Index. We identified several medical and socioeconomic factors potentially relevant to the outcome measure (e.g., level of myelomeningocele, length of stay in hospital, and shunt-related hospital admissions; family functioning, income, parental education, employment status, etc.). Linear regression models were used to examine associations between potential predictor variables and HRQOL, with P < 0.05 in the multivariate model suggesting significance.Results: The analysis consisted of 131 patients (mean age = 12.6, SD = 3.7). The mean HUI score was 0.51, and the mean HOQ overall health score was 0.67. There was a significant association between lower age and higher HOQ social-emotional health (P = 0.03) and HUI scores (P = 0.03), lower anatomical level of myelomeningocele and higher HUI scores (P = 0.01), better family functioning and higher HOQ overall health scores (P = 0.004), and higher family income and higher HOQ overall health, physical health, and HUI scores (P = 0.001, P = 0.003, and P = 0.02, respectively).Conclusion: Myelomeningocele patients with shunted hydrocephalus can have a poor health-related quality of life. Our results indicate a strong association of income and family functioning on quality of life, along with younger age and lower level of myelomeningocele. [ABSTRACT FROM AUTHOR]

Non-syndromic congenital hydrocephalus is aetiologically diverse and while a genetic cause is frequently suspected, it often cannot be confirmed. The most common genetic cause is L1CAM- related X-linked hydrocephalus and that explains only 5%–10% of all male cases. This underlines a current limitation in our understanding of the genetic burden of non-syndromic congenital hydrocephalus, especially for those cases with likely autosomal recessive inheritance. Additionally, the prognosis for most cases of severe congenital hydrocephalus is poor, with most of the surviving infants displaying significant intellectual impairment despite surgical intervention. It is for this reason that couples with an antenatal diagnosis of severe hydrocephalus are given the option, and may opt, for termination of the pregnancy. We present two families with CCDC88C -related recessive congenital hydrocephalus with children who had severe hydrocephalus. Those individuals who were shunted within the first few weeks of life, who did not require multiple surgical revisions, and who had a more distal truncating variant of the CCDC88C gene met their early childhood developmental milestones in some cases. This suggests that children with CCDC88C -related autosomal recessive hydrocephalus can have normal developmental outcomes under certain circumstances. We recommend CCDC88C analysis in cases of severe non-syndromic congenital hydrocephalus, especially when aqueduct stenosis with or without a medial diverticulum is seen, in order to aid prognosis discussion. [ABSTRACT FROM AUTHOR]

Hydrocephalus is a common condition in the pediatric population known to have many causes and presentation patterns. We report from the analysis of 2 cases the existence of a new complication of pediatric hydrocephalus. Naming this entity "dissecting intraparenchymal cerebrospinal fluid collection", we advance a hypothesis regarding its pathophysiology and discuss its clinical implications and management. [ABSTRACT FROM AUTHOR]

Purpose: Ventriculo-peritoneal (VP) shunts are effective for treatment of hydrocephalus in all age groups; however, they are associated with complications, a common one being ventricular catheter (VC) obstruction. VC position is likely to influence VC survival; however, most VCs are positioned freehand without guidance. This paper describes the accuracy of ultrasound guidance for VC placement and the impact of tip location on VC occlusion rate.Methods: This is a retrospective cohort study of hydrocephalic children with first-time VP shunt and ultrasound-guided VC placement. Data recorded were age, sex, cause of hydrocephalus, side (left or right) and location (frontal or occipital) of VC, and exact postoperative position within the ventricle on first postoperative imaging: middle of ventricle (optimal position), near or touching the medial or lateral ventricle wall, within the third ventricle, and at the contralateral side.Results: Of the 128 screened patients, 85 had a first postoperative imaging that clearly defined the VC position and were included. The follow-up was at least 12 months. Seventy-three percent of VCs were placed on the right and 71% via a frontal burhole. Eighty-three of 85 VC tips (95%) were in the intended ventricle, 61% at optimal position. Nine of 85 VCs (10%) obstructed within the first 12 months. Seven of nine (78%) obstructed VCs were located in a nonoptimal position (p = 0.016). Two of nine (22%) obstructed VCs entered through a frontal and seven of nine (78%) through an occipital burrhole (p = 0.016).Conclusion: Ultrasound-guided VC placement is as precise as frameless navigated placement. The optimal VC position was associated to a significant lower VC obstruction rate. The frontal position was superior to the occipital. Intraoperative US guidance is fast with almost no extra time and no extra cost. US-guided VC placement should become standard of care in VP shunt surgery. [ABSTRACT FROM AUTHOR]

Objective: Treatment monitoring and outcome evaluation in pediatric hydrocephalus require gapless documentation regarding surgical and clinical follow-up data beginning from day 1 of treatment in order to apply high quality of care. Endoscopic procedures, shunt insertion and revision surgeries, and individual modifications of valve hardware or pressure settings during follow-up as well as established outcome measurements are highly relevant for complete illustration of the patient’s hydrocephalus histories. A digital tool to capture, organize, and analyze comprehensive treatment-related data was estimated long overdue, consequentially developed, and implemented in daily pediatric neurosurgical routine.Methods: We established a self-contained, network-capable database application to supply and back up clinical information of complete surgical treatment history with implant status and follow up for all institutional pediatric hydrocephalus patients from 1995 to date. The application content has been prospectively complemented since 2012 during daily pediatric neurosurgical routine. Beside surgical data, neurological outcome and quality of life assessment were integrated according to validated scales to be recordable 2, 3, and 5 years after initial surgical intervention for prospective administration. The application is in continuous and problem-free use since implementation offering homogeneous and structured real-time information of surgical and corresponding neurological hydrocephalus-related data. By using an automatized data extraction tool, an exemplary surgical outcome evaluation reviewing institutional ventriculo-peritoneal shunt (VPS) treatment in infants over a period of more than 20 years was performed. To validate applicability, the Registry was successfully implemented in an external institution under identical conditions continuously serving for the same purpose until today.Results: Upon completion of the developing process, the application was successfully implemented into routine clinical workflow of our institution. In total, 579 pediatric hydrocephalus patients entered into the Registry with collectively 1874 corresponding hydrocephalus-related surgeries (9% neuro-endoscopic procedures, 18% temporary CSF-diversions, 73% shunt surgeries) so far. For exemplary surgical outcome analysis, the total volume of complex data sets could easily be reduced stepwise in regard to requested inclusion criteria. The selection process generated conclusive data of 256 institutional pediatric VPS patients providing a median follow-up of 8.5 years. Surgical outcome was evaluated in regard to hydrocephalus etiology, applied valve design, valve augmentation, cause of initial malfunction, time to initial shunt revision, and number of total revisions.Conclusion: The pediatric hydrocephalus registry application delivers easy access to contemporary and up-to-date clinical information during daily clinical routine and proves comprehensive value for various scientific purposes. Institutional hydrocephalus etiologies, treatment modalities, and surgical outcome could be reviewed for a selected pediatric patient collective during an interval of more than 20 years and confirmed initial shunt treatment within the first year of age, communicating hydrocephalus and a history of prematurity as significant variables for unfavorable shunt survival and long-term revision rate. At our institution, the Registry emerged to an essential and sustainable tool to capture, organize, and analyze patterns of care in pediatric hydrocephalus patients of all etiologies and treatment modalities. Because of its adaptable and reliable predicate, a prospective multi-center utilization is currently in preparation. [ABSTRACT FROM AUTHOR]

Ventriculoperitoneal shunt (VPS) is a modern and efficient treatment, but it still presents morbidity and mortality due to complications related to the procedure. The medical records of 48 patients were reviewed and an epidemiological analysis was carried out. Among the patients 42,2% were newborns, 16% were between 28 days and 6 months, 15.6% between 6 months and 2 years, 11.1% between 2 and 12 years and 16% over 12 years, predominantly males (69%). The main etiologies included malformations (21%), neoplasia (19%) and myelomeningocele (10%). The most common symptoms before surgery were vomiting (42%), lowered level of consciousness (35%), headache (27%) and macrocrania (51% of patients younger than 2 years). The technique that precedes the procedure consists of degerming the skin, followed by placing compresses around the surgical field with OPSITE dressing and then the sterile drapes are positioned. It is believed that the method positively influences the infection rate of surgeries, which was 14.6%, consistent with literary findings in other hospitals, of 15% (take into account that this is a university training hospital, that not all surgeries were performed in the first hour and among other limitations of the study). Among the main infectious symptoms, fever (33%) and valve malfunction (44%) were observed. In addition, 42% of the patients underwent PVD revision and 31% underwent device replacement. death rate found was 10.4%, also similar to the bibliography. Longer follow-up time would be necessary for better evaluation. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to present the clinical and surgical treatment of arachnoid cysts in pediatric patients. Method/materials: The research was carried out from the BVS, Scielo and Cochrane Library databases and the descriptors used were "Tratamento cisto aracnóideo" and "Manejo cisto aracnóideo", as well as their respective in English ("arachnoid cyst treatment" and "arachnoid cyst management"). The applied criteria included articles in Portuguese and English and published in the last 5 years. From the eligibility analysis by reading the title and their respective abstracts, 27 articles were selected, which were read in full. RESULTS: Arachnoid cysts (AC) are prevalent in children aged between 1 and 10 years. Although conservative management is the most applied, headache, vomiting and increased intracranial pressure (ICP) were the most frequent clinical presentations and the most indication for surgery. Obstructive hydrocephalus was also a common finding in the studies. Increased ICP was observed even during pregnancy in most cases of patients younger than 1 year old, with the diagnosis made in utero, and is related to developmental delay. Endoscopic fenestration was the most frequently performed procedure, with the best postoperative outcome for CA, leaving cyst bypass as the second option. The need for a revision surgery is strictly associated with 1 year after the initial operation, and in these cases, refenestration and/or placement of an additional shunt were performed. Midline interhemispheric cysts usually require a second intervention and cause higher rates of neurodevelopmental deficit. Discussion: AC in pediatric patients have different anatomical locations, which is why there are different clinical manifestations. The most affected sites are the middle cranial fossa (40.5%), posterior fossa (24.3%) and interhemispheric fossa (13.5%), leading to clinical alterations such as: increased intracranial pressure (34.2%), obstructive hydrocephalus (28.9%) and AC rupture (21.1%). Treatments consist of clinical and surgical approaches (craniotomies, endoscopic fenestrations, cystoperitoneal shunts, drainage operations with trepanning, and spinal CA excision). Conclusion: The therapeutic approach for pediatric patients points to an individualized approach due to the wide spectrum of anatomical locations and clinical manifestations associated with the condition. [ABSTRACT FROM AUTHOR]

Background: There is little knowledge about the factors influencing the long-term outcome after surgery for idiopathic normal pressure hydrocephalus (iNPH). Objective: To evaluate the effects of reoperation due to complications and of vascular comorbidity (hypertension, diabetes, stroke and heart disease) on the outcome in iNPH patients, 2-6 years after shunt surgery. Methods: We included 979 patients from the Swedish Hydrocephalus Quality Registry (SHQR), operated on for iNPH during 2004-2011. The patients were followed yearly by mailed questionnaires, including a self-assessed modified Rankin Scale (smRS) and a subjective comparison between their present and their preoperative health condition. The replies were grouped according to the length of follow-up after surgery. Data on clinical evaluations, vascular comorbidity, and reoperations were extracted from the SHQR. Results: On the smRS, 40% (38-41) of the patients were improved 2-6 years after surgery and around 60% reported their general health condition to be better than preoperatively. Reoperation did not influence the outcome after 2-6 years. The presence of vascular comorbidity had no negative impact on the outcome after 2-6 years, assessed as improvement on the smRS or subjective improvement of the health condition, except after 6 years when patients with hypertension and a history of stroke showed a less favorable development on the smRS. Conclusion: This registry-based study shows no negative impact of complications and only minor effects of vascular comorbidity on the long-term outcome in iNPH. [ABSTRACT FROM AUTHOR]

A young bilateral cochlear implant (CI) user required magnetic resonance imaging (MRI) to determine the cause of hydrocephalus. The images obtained with the CIs in place were not diagnostically useful due to large artefacts generated by the CI magnets. We obtained useful images by bilaterally explanting the CI-magnets and replacing them with non-magnetic placeholder dummies then conducted the imaging. The artefact in the new images was greatly reduced and the images were diagnostically useful. Lastly, we explanted the dummies and reimplanted the CI-magnets. This procedure should be useful to obtain useful images in CI users. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to determine whether drainage and revision are an effective treatment for abdominal pseudocyst associated ventriculoperitoneal (VP) shunt failure by estimating the total rate of secondary shunt failure. Methods: We performed a retrospective review of children with hydrocephalus diagnosed with and treated for an abdominal pseudocyst at the Children's Hospital, London Health Sciences Centre (LHSC) between January 1, 2000 and May 31, 2016 (ethics approval # 108136). Patients with a VP shunt were included if (i) the development of an abdominal pseudocyst at age 2 to 18 years was identified, (ii) treatment of the pseudocyst by either interventional radiology (IR) or surgical drainage, and (iii) revision of the VP shunt. Demographic data and details of pseudocyst formation/ treatment as well as subsequent failures were identified. Results: Twelve patients who had a VP shunt developed abdominal pseudocyst and met inclusion criteria. A 91% shunt failure rate after drainage and shunt revision was identified. Three patients had the pseudocyst drained in interventional radiology and then externalized due to shunt infection. Nine patients were treated by surgical revision. Ten patients experienced recurrent shunt failure following initial drainage of the pseudocyst: pseudocyst reoccurrence ( n = 3), distal obstruction from adhesions ( n = 1), and uncleared infection ( n = 6). Conclusion: The results suggest that pseudocyst drainage and shunt revision is ineffective in providing long-term resolution of shunt problems. [ABSTRACT FROM AUTHOR]

Hydrocephalus can occur in children alone or in combination with other neurodevelopmental disorders that are often associated with brain overgrowth. Despite the severity of these disorders, the molecular and cellular mechanisms underlying these pathologies and their comorbidity are poorly understood. Here, we studied the consequences of genetically inactivating in mice dual-specificity phosphatase 16 (Dusp16), which is known to negatively regulate mitogen-activated protein kinases (MAPKs) and which has never previously been implicated in brain development and disorders. Mouse mutants lacking a functional Dusp16 gene (Dusp16 =) developed fully-penetrant congenital obstructive hydrocephalus together with brain overgrowth. The midbrain aqueduct in Dusp16 = mutants was obstructed during mid-gestation by an expansion of neural progenitors, and during later gestational stages by neurons resulting in a blockage of cerebrospinal fluid (CSF) outflow. In contrast, the roof plate and ependymal cells developed normally. We identified a delayed cell cycle exit of neural progenitors in Dusp16 = mutants as a cause of progenitor overproliferation during midgestation. At later gestational stages, this expanded neural progenitor pool generated an increased number of neurons associated with enlarged brain volume. Taken together, we found that Dusp16 plays a critical role in neurogenesis by balancing neural progenitor cell proliferation and neural differentiation. Moreover our results suggest that a lack of functional Dusp16 could play a central role in the molecular mechanisms linking brain overgrowth and hydrocephalus. [ABSTRACT FROM AUTHOR]

BACKGROUND: Hydrocephalus is a fatal disorder mostly affecting neonate sand infants. It is treatable in pediatric patients but lacks a grading system for these verity of the disease. This paper proposes a grading system for hydrocephalic pediatric patients based on linear measurements applied on CT scan. OBJECTIVES: This study aims to determine average linear parameters of the hydrocephalus brain among pediatric patients by using CT scan, to propose a new grading system for hydrocephalus based on these linear measurement sand to find the most common type and grade of hydrocephalus. METHODOLOGY: A cross sectional study was conducted on 37 pediatric hydrocephalus subjects in Abha. Five linear measurements FHR, FOHR, BFI, BCI and VI were applied on CT scan and generated a new grading system. Statistical analysis was done by SPSS (V-21) software. Also classifies the subjects by this system into grades of mild to severe and discovers the most common type and category of hydrocephalus. RESULTS: The highest frequency of hydrocephalus was found at two years. The most common type was communicated and most common grades were mild and moderate. The mean measurements (reference value) in mm were found for FOHR=0.63, FHR=0.52, BCI=0.34, BFI=0.60 and VI=0.55. There was significant difference of linear parameters in FOHR, FHR, BFI related to age groups. CONCLUSION: The grading system successfully divides pediatric patients in to mild to severe categories, effect of age on each linear measurement can also be evaluated in hydrocephalic patients and this grading can also be helpful to evaluate any mass effect like brain atrophy. [ABSTRACT FROM AUTHOR]

Background Endoscopic third ventriculostomy (ETV) is one of the important management options for hydrocephalus, especially obstructive hydrocephalus. The result of ETV in different age and etiology is quite variable according to the different authors. The study was carried out to assess the success rate of ETV as a treatment for this type of hydrocephalus in infant age group. Material and Methods The study was done prospectively on infants (age ≤ 12 months) admitted in the period from January 2009 to June 2013 suffering from obstructive hydrocephalus due to cerebral aqueductal stenosis (CAS). After making the diagnosis, we counseled the patient party about the probability of high failure rate of ETV in this age group and probable necessity of a second operation. With the informed consent, we performed ETV in these cases and followed up regularly in postoperative period. Control neuroimaging studies were done whenever needed (suspected failure of ETV and suspected stomal block). Results Total 17 infants were studied. Average follow-up was 18.7 months. Twelve infants were between the age group of six and under six months while five were above six months to one year old. Fourteen (82.35%) out of 17 patients showed overall clinical improvement. Clinical improvement was seen in two (66.6%) infants aged 2 months or younger, three (75%) aged 2+ to 3 months, five (100%) aged 3+ to 6 months, and four (80%) aged 6+ months to 1 year. Two patients had bleeding during surgery. Three developed CSF leak through the burr hole. In one case (the patient's age was 2 months), the stoma was found blocked, and hydrocephalus returned 9 weeks after ETV. In two patients, in whom VP shunt was needed, "failed ETV" was obvious in early postoperative period. In this series, average ETV success score (ETVSS) was 52.35 (range: 40-70) and overall success rate was 82.35%. This indicates that ETVSS does not correlate with the outcome of ETV in infants with CAS. Conclusion Outcome of ETV for hydrocephalus from CAS in infant is quite good and ETVSS does not correlate with the outcome. [ABSTRACT FROM AUTHOR]

Background/Aims: A broad spectrum of cranio-cervical decompression techniques (CCD) is employed for the treatment of Chiari malformation type I (CM1). The aim of this study was to compare the clinical and radiological outcome of 3 different CCD performed in a single paediatric centre. Methods: A retrospective analysis of children treated between 2008 and 2014 was performed. Three different surgical techniques were offered to the patients: an extradural osseo-ligamentous bony decompression (BD), or a BD plus opening of the dura either without duroplasty (DOWD) or with watertight expansile duroplasty (DOPD). The primary clinical outcome was measured by utilizing the Chicago Chiari Outcome Scale (CCOS). Results: Forty-six children underwent 51 CCD: 17 BD, 17 DOWD, and 17 DOPD. The median follow-up period was 46 months (16-98 months). Patients who underwent BD had a shorter length of hospital stay than those who underwent DPTC and DOWD. Clinical improvement, defined by the mean CCOS score, was comparable in patients receiving BD (n = 14.6) and DOPD (n = 14.5), but lower in patients with DOWD (n = 12). Post-operative complications were noted in 7 DOWD patients (41%) and 3 DOPD patients (17.6%). Conclusions: BD is a safe and effective procedure with comparable clinical and radiological outcomes to DOPD in children with CM1. The risk of post-operative complications and worse clinical outcome was noted to be higher when performing a DOWD. [ABSTRACT FROM AUTHOR]

Background: Pediatric hemangioblastomas are rare, and the clinical features, timing of surgical intervention, optimal treatment, and clinical outcomes are still unclear. Methods: We performed a retrospective study of all patients with CNS hemangioblastomas who were treated at West China Hospital from January 2003 to March 2015. Patients under the age of 16 years were included in the study. The medical records of these patients were reviewed and statistically analyzed. Results: Twenty-five children (15 females and ten males, [mean age 12.6 ± 4.7 years, range 1-16 years]) presented with hemangioblastomas. Tumors were detected in the cerebellum, brainstem, and spinal cord in 40, 28, and 32% of patients, respectively. Sixteen children (64%) had VHL syndrome. The most frequent symptoms were those related to increased intracranial pressure. The mean duration of symptoms was 1.5 ± 2.1 months. Preoperative hydrocephalus was noted in 11 children (44%). Gross total resection was achieved in all children. Clinical symptoms improved in 19 children (76%), unchanged in four children (16%), and aggravated in two children (8%), respectively. The mean follow-up was 44.5 ± 32.3 months. Five patients (20%) experienced disease progression. Using univariate analysis, both tumor-associated cysts ( P = 0.027) and VHL disease ( P = 0.032) were significantly related to postoperative outcomes. Conclusions: Pediatric hemangioblastomas have many different clinical features compared with adult cases. A high degree of suspicion for VHL disease should be raised in pediatric hemangioblastomas. Despite many challenges involved, surgical outcomes for pediatric hemangioblastomas are favorable. Lifelong follow-up is mandatory to detect the disease progression. [ABSTRACT FROM AUTHOR]

Purpose: Presence of metastases in newly diagnosed pediatric posterior fossa tumors (PFT) is not a rare situation, but optimal treatment of associated hydrocephalus in these children has remained undetermined. Methods: Twenty-nine children treated between January 2005 and December 2015 for a metastatic PFT associated with hydrocephalus constituted the study cohort. Patients were divided into three groups: ventriculoperitoneal shunt (VPS), endoscopic third ventriculostomy (ETV), and temporary ventricular drainage before or during tumor resection (PVD). Results: There were 4 VPS, 18 ETV, and 7 PVD. The global incidence of CSF diversion failure was 52%. No case of dysfunction or dissemination of metastatic cells occurred in the VPS group. Recurrence of hydrocephalus occurred in 55% of the ETV group. Presence of multiple macroscopic metastases and CSF metastatic cells after tumor surgery was associated with ETV failure. Fifty-seven percent of the children in the PVD group were reoperated after an average time of 53 days. Specific oncologic treatment was initiated earlier in the VPS group (11 days) compared to ETV (27 days) and PVD (23 days) groups. Conclusions: ETV should be avoided in cases of multiple macroscopic metastases, and children who underwent ETV must be followed carefully when metastatic cells are present in CSF after tumor surgery. External ventricular drainage before or during surgical removal should not be considered as a final option to treat hydrocephalus. VPS remains a safe alternative in this situation and allows an early specific oncologic treatment. [ABSTRACT FROM AUTHOR]

Introduction: The standard treatment for hydranencephaly and maximal hydrocephalus consists of inserting shunts, although complications frequently occur. Choroid plexus cauterization (CPC) is an alternative, but its long-term efficacy and the factors associated with the success and failure of controlling head circumference (HC) are not well defined. Objective: This study aims to evaluate the long-term efficacy and factors related to the success rate of CPC in the treatment of hydranencephaly and maximal hydrocephalus. Method: Forty-two children with maximal hydrocephalus and hydranencephaly underwent CPC from 2006 to 2014 and were retrospectively evaluated. Children with less than 3 months of follow-up were excluded. The long-term efficacy and success rate of possible variables (i.e., sex, type of malformation, type of surgery performed, treatment hospital, age, and HC at the time of surgery and birth) were evaluated. Results: Thirty-four children were considered for the effectiveness analysis. Treatment was successful in 24 children (70.6%), and failure occurred in 10 children (29.4%). Failure was detected soon after the endoscopic procedure (average 116 days). There was no difference in effectiveness when comparing the age at the moment of surgery ( p = 0.473), type of malformation ( p = 1), HC at birth (0.699), and HC at the time of surgery ( p = 0.648). The surgical death rate was 7.14%. Conclusion: Endoscopic CPC was a valid procedure used to treat hydranencephaly and maximal hydrocephaly, and it was effective in 70.6% of cases, with an average follow-up period of 32 months. When failures occurred, they occurred early. None of the analyzed variables interfered with the success of the treatment. [ABSTRACT FROM AUTHOR]