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Hydrocephalus is the most common neurosurgical disorder worldwide and is characterized by enlargement of the cerebrospinal fluid (CSF)-filled brain ventricles resulting from failed CSF homeostasis. Since the 1840s, physicians have observed inflammation in the brain and the CSF spaces in both posthaemorrhagic hydrocephalus (PHH) and postinfectious hydrocephalus (PIH). Reparative inflammation is an important protective response that eliminates foreign organisms, damaged cells and physical irritants; however, inappropriately triggered or sustained inflammation can respectively initiate or propagate disease. Recent data have begun to uncover the molecular mechanisms by which inflammation - driven by Toll-like receptor 4-regulated cytokines, immune cells and signalling pathways - contributes to the pathogenesis of hydrocephalus. We propose that therapeutic approaches that target inflammatory mediators in both PHH and PIH could address the multiple drivers of disease, including choroid plexus CSF hypersecretion, ependymal denudation, and damage and scarring of intraventricular and parenchymal (glia-lymphatic) CSF pathways. Here, we review the evidence for a prominent role of inflammation in the pathogenic mechanism of PHH and PIH and highlight promising targets for therapeutic intervention. Focusing research efforts on inflammation could shift our view of hydrocephalus from that of a lifelong neurosurgical disorder to that of a preventable neuroinflammatory condition.

Background Many animal models have been used to study the pathophysiology of hydrocephalus; most of these have been rodent models whose lissencephalic cerebral cortex may not respond to ventriculomegaly in the same way as gyrencephalic species and whose size is not amenable to evaluation of clinically relevant neurosurgical treatments. Fewer models of hydrocephalus in gyrencephalic species have been used; thus, we have expanded upon a porcine model of hydrocephalus in juvenile pigs and used it to explore surgical treatment methods. Methods Acquired hydrocephalus was induced in 33–41-day old pigs by percutaneous intracisternal injections of kaolin (n = 17). Controls consisted of sham saline-injected (n = 6) and intact (n = 4) animals. Magnetic resonance imaging (MRI) was employed to evaluate ventriculomegaly at 11–42 days post-kaolin and to plan the surgical implantation of ventriculoperitoneal shunts at 14–38-days post-kaolin. Behavioral and neurological status were assessed. Results Bilateral ventriculomegaly occurred post-induction in all regions of the cerebral ventricles, with prominent CSF flow voids in the third ventricle, foramina of Monro, and cerebral aqueduct. Kaolin deposits formed a solid cast in the basal cisterns but the cisterna magna was patent. In 17 untreated hydrocephalic animals. Mean total ventricular volume was 8898 ± 5917 SD mm3 at 11–43 days of age, which was significantly larger than the baseline values of 2251 ± 194 SD mm3 for 6 sham controls aged 45–55 days, (p Conclusions Kaolin induction of acquired hydrocephalus in juvenile pigs produced an in vivo model that is highly translational, allowing systematic studies of the pathophysiology and clinical treatment of hydrocephalus.

Background: Acquired hydrocephalus (AH) is a common complication in patients with severe brain injury. Brain tissue injury has been proposed to induce a neuroinflammatory reaction reflected by cytokines release, particularly interleukin-6 (IL-6), which associates with early brain damage. The present study measured IL-6 in the cerebrospinal fluid (CSF) of AH patients and determined its relationship to functional outcome following shunt operation.Methods: The study included a total of 32 patients with a shunt operation due to hydrocephalus. CSF samples from 26 AH subjects and 6 iNPH patients were collected via lumbar puncture before surgery. IL-6 level was measured using the micro ELISA immunoassay method. AH subjects were dichotomized into good versus poor outcomes based on modified Rankin Scale (mRS) at 3 months after shunting.Results: CSF analysis demonstrated that IL-6 was significantly elevated in the CSF of the AH group compared to controls (p = 0.023). Within the AH group, eighteen (69.2%) had a good outcome while eight (30.8%) patients had a poor outcome. Mean IL-6 level in the good outcome group was approximately four-times higher than the poor outcome group (p = 0.004). Glasgow Coma Scale (GCS) on admission was significantly different between the two groups (p = 0.014). IL-6 level and admission GCS were significantly correlated with improvement of mRS score (r = 0.473, p = 0.015 and r = 0.691, p＜0.0001, respectively). Receiver operating characteristic curve analysis showed that both factors can accurately differentiate between patients with good versus poor functional outcome (AUC = 0.861, p = 0.0039 and AUC = 0.823, p = 0.0098, respectively). Conclusions: The CSF level of IL-6 is elevated in AH patients and higher levels correlate with improvement of post-shunt functional outcome. Therefore, IL-6 CSF level might serve as a complementary surrogate parameter for operative indication. A possible IL-6 threshold in clinical routine might be a 6.98-pg/ml cutoff value to rule out unresponsive and poor outcome AH patients that are under the 6.98-pg/ml threshold.

The clinical and neurological features of prematurely born children with perinatal hypoxic-ischemic lesions of the central nervous system were studied. A comprehensive examination of 79 preterm infants of both sexes who underwent perinatal hypoxic-ischemic lesions of the central nervous system was performed. 37.9 % of the surveyed were diagnosed with spastic cerebral palsy, 20.3 % were diagnosed with spastic diplegia, 27.8 % were diagnosed with childhood hemiplegia, hydrocephalus was acquired in 13.9 % as a result of non-traumatic intraventricular hemorrhage. In 10.3 % of children, severe movement disorders (patients with spastic cerebral palsy and acquired hydrocephalus without surgery) were noted, 37.7 % of the examined did not master walking (patients with spastic diplegia, spastic cerebral palsy and acquired hydrocephalus operated on after 6 months of life), 35.5 % of children have mastered walking with assistive devices (patients with spastic diplegia and acquired hydrocephalus operated on up to 6 months of age), 16.5 % of children have mastered walking without restrictions (children with childhood hemiplegia and acquired hydrocephalus, operated on up to 6 months of age). Characteristic structural changes in perinatal hypoxic-ischemic lesions of the central nervous system are periventricular leukomalacia of the II–III degree and intraventricular hemorrhage of the I–III degree. The study of somatosensory evoked potentials conducted in the course of the work showed that at the 3rd, 4th levels of disorders of motor functions by GMFCP, there is a significant decrease in the function of conducting along the somatosensory pathway, depression of the amplitude of the peaks. It has been established, that in the structure of severe motor disorders in children with perinatal hypoxic-ischemic lesions of the central nervous system, violations of arbitrary regulation of movements and postural control are significant. A clear manifestation of the close correlation between motor function impairment, cognitive activity and anxiety level in the examined children has been established, which testifies to the negative impact of a negative emotional state on the development of a child with severe motor disorders.

Background and Objective: Ventricle volume is closely related to hydrocephalus, brain atrophy, Alzheimer's, Parkinson's syndrome, and other diseases. To accurately measure the volume of the ventricles for elderly patients, we use deep learning to establish a systematic and comprehensive automated ventricle segmentation framework.Methods: The study participation included 20 normal elderly people, 20 patients with cerebral atrophy, 64 patients with normal pressure hydrocephalus, and 51 patients with acquired hydrocephalus. Second, get their imaging data through the picture archiving and communication systems (PACS) system. Then use ITK software to manually label participants' ventricular structures. Finally, extract imaging features through machine learning.Results: This automated ventricle segmentation method can be applied not only to CT and MRI images but also to images with different scan slice thicknesses. More importantly, it produces excellent segmentation results (Dice > 0.9).Conclusion: This automated ventricle segmentation method has wide applicability and clinical practicability. It can help clinicians find early disease, diagnose disease, understand the patient's disease progression, and evaluate the patient's treatment effect.

Purpose: To investigate differences in cerebrospinal fluid (CSF) flow through the aqueduct and to determine whether there is a relationship between CSF flow and ventricular volume parameters in idiopathic normal pressure hydrocephalus (iNPH) patients, elderly acquired hydrocephalus patients and age-matched healthy volunteers by phase-contrast MR (PC-MR). Methods: A total of 40 iNPH patients and 41 elderly acquired hydrocephalus patients and 26 age-matched healthy volunteers in the normal control (NC) group were included between November 2017 and October 2019 in this retrospective study. The following CSF flow parameters were measured with PC-MR: peak velocity, average velocity (AV), aqueductal stroke volume (ASV), net ASV, and net flow. The following ventricular volume parameters were measured: ventricular volume (VV), brain volume, total intracranial volume, and relative VV. Differences between the iNPH and acquired hydrocephalus groups were compared Mann–Whitney U test and correlations between CSF flow and ventricular volume parameters were assessed using the Spearman correlation coefficient. Results: Aqueductal stroke volume was significantly higher in the iNPH and acquired hydrocephalus groups than in the NC group, but did not differ significantly between the iNPH group and acquired hydrocephalus group. The AV, net ASV, and net flow in the iNPH and acquired hydrocephalus groups were significantly higher than those in the NC group (P < 0.0001), and those in the acquired hydrocephalus group were significantly higher than those in the iNPH group (P = 0.01, P = 0.007, P = 0.002, respectively). The direction of the AV and net ASV significantly differed among the three groups. There were no associations between the volume parameters and CSF flow according to PC-MR among the three groups. Conclusion: Compared with iNPH, elderly acquired hydrocephalus demonstrated higher CSF hyperdynamic flow. Although increased CSF flow may contribute to further changes in ventricular morphology, there is no linear relationship between them. These findings might help increase our understanding of flow dynamics in iNPH and elderly acquired hydrocephalus.

Chiari malformations are structural defects in the posterior fossa where the cerebellum displaces caudally into the foramen magnum and upper spinal canal. These malformations are classified by severity as Types 1-4, each presenting with different associated and/or concurrent conditions and anomalies. The aim of this study was to utilize a nationwide database to study patients with Chiari malformations including their concurrent diagnoses and associated anomalies. Using a retrospective review of the Nationwide Inpatient Sample (NIS) database from 2003 to 2012, Chiari malformations were assessed by Chiari type and rates of concurrence for various additional anomalies were evaluated using cross-tabulations. There were 305,726 national cases of Chiari Type 1, 119,632 cases of Chiari Type 2, 15,540 cases of Type 3, and 79,663 cases of Type 4. Overall 44.3% of Chiari patients have at least one concurrent anomaly. Stratified by Chiari Type, 7.1% of Type 1 patients, 12.3% of Type 2, and 100% of Type 3 and 4 have at least one concurrent anomaly. The most common isolated neurologic associations were tethered cord, syringomyelia, and hydrocephalus, while the most common anomaly clusters were syringomyelia and scoliosis in Type 1 (0.63), tethered cord syndrome and scoliosis (0.72%) in Type 2, encephalocele and acquired hydrocephalus (11.45%) in Type 3, and reduction deformity of the brain with acquired hydrocephalus (15.95%) in Type 4. Chiari malformations have strong associations with other abnormalities outside of known relationships in the current classification. While neurologic abnormalities are most common, additional body systems are frequently involved especially with worsening hindbrain defects.

Intraventricular hemorrhage in the setting of prematurity remains the most common cause of acquired hydrocephalus. Neonates with progressive post-hemorrhagic hydrocephalus are at risk for adverse neurodevelopmental outcomes. The goal of this review is to describe the distinct and often overlapping types of brain injury in the preterm neonate, with a focus on neonatal hydrocephalus, and to connect injury on imaging to neurodevelopmental outcome risk. Head ultrasound and magnetic resonance imaging findings are described separately. The current state of the literature is imprecise and we end the review with recommendations for future radiologic and neurodevelopmental research.

A 76-year-old man presented with progressive dementia, gait disturbance, and urinary incontinence for 1 year. Computed tomography scan revealed nonobstructive hydrocephalus, but abnormal papillary structures at the ventricular wall were noted. Before cerebrospinal fluid (CSF) diversion surgery for hydrocephalus, we performed magnetic resonance angiography and magnetic resonance venography, which revealed multiple engorged vessels over the ventricular wall and bilateral hemispheres. Digital subtraction angiography revealed 2 dural arteriovenous fistulas (DAVFs) at the left transverse-sigmoid sinus and superior sagittal sinus. Signs of angioarchitecture characteristic of cerebral venous hypertension (CVH) were noted, including cortical vein regurgitation and severe pseudophlebitic pattern. DAVFs with CVH might be a factor contributing to acquired hydrocephalus. DAVFs should be considered when patients with hydrocephalus exhibit abnormal papillary structures at the ventricular wall. Performing CSF diversion surgery for hydrocephalus before downgrading or curing such aggressive DAVFs may lead to major complications.

The authors report on the clinical course of two infants with severe hemophilia A (HA) and concomitant progressive hydrocephalus that required management with a ventriculoperitoneal shunt. The first child, with known HA, presented with a spontaneous intracranial hemorrhage and acquired hydrocephalus. He underwent cerebrospinal fluid diversion with a temporary external ventricular drain, followed by placement of a ventriculoperitoneal shunt. The second child had hydrocephalus secondary to a Dandy-Walker malformation and was diagnosed with severe HA during preoperative evaluation. He underwent placement of a ventriculoperitoneal shunt after progression of the hydrocephalus. The authors also review the treatment of hydrocephalus in patients with HA and describe the perioperative protocols used in their two cases. Treatment of hydrocephalus in infants with HA requires unique perioperative management to avoid complications.

Introduction: A multitude of underly- ing reasons result in hydrocephalus (HC), and its clas- sification remains controversial. The current study lo- oks at patients with the Syndrome of Hydrocephalus in Young and Middle-Aged adults (SHYMA) through a case series. Patients and methods: We report 35 patients with HC referred to St. Anna Multiprofile Hospital during the period 2008-2012. Inclusion criteria were decom- pensated congenital hydrocephalus, (DCH), acquired hydrocephalus (AHC), or idiopathic hydrocephalus (IHC) in the age range of 16-55 years, treated with a ventriculo-peritoneal shunt (VPS) — 17 patients were treated with Strata Adjustable Delta Valve ("Strata" group) and 18 patients had Medtronic Orbis Sigma val- ves inserted ("Orbis Sigma" group). Results: Eight patients (22.86%) had DCH, 14 (40%) had AHC, and 13 (37.14%) had IHC. Regardless the underlying cause for HC, all the pati- ents had similar symptoms, mainly related to gait in 26 (74.3%), cognition in 30 (85.7%), bladder control in 20 (57.14%) and chronic headaches in 24 patients (68.57%). Symptomatic improvement was achieved in 34 of the shunted 35 patients (97.14%), but the postoperative complications rate was found to be significantly lower in the "Strata" group. Conclusion: The clinical presentation of hydro- cephalus in the age 16-55 years has common features presenting with syndrome of hydrocephalus in young and middle-aged adults as separate clinical entity. VPS is a feasible treatment option in SHYMA. Due to the excessive, long standing ventriculomegaly, thus sensitive compliance of brain parenchyma and high tendency to develop subdural hematomas, adjust- able VPS are advisable option.

OBJECTIVEHydrocephalus is one of the most common brain disorders, yet a reliable assessment of the global burden of disease is lacking. The authors sought a reliable estimate of the prevalence and annual incidence of hydrocephalus worldwide.METHODSThe authors performed a systematic literature review and meta-analysis to estimate the incidence of congenital hydrocephalus by WHO region and World Bank income level using the MEDLINE/PubMed and Cochrane Database of Systematic Reviews databases. A global estimate of pediatric hydrocephalus was obtained by adding acquired forms of childhood hydrocephalus to the baseline congenital figures using neural tube defect (NTD) registry data and known proportions of posthemorrhagic and postinfectious cases. Adult forms of hydrocephalus were also examined qualitatively.RESULTSSeventy-eight articles were included from the systematic review, representative of all WHO regions and each income level. The pooled incidence of congenital hydrocephalus was highest in Africa and Latin America (145 and 316 per 100,000 births, respectively) and lowest in the United States/Canada (68 per 100,000 births) (p for interaction < 0.1). The incidence was higher in low- and middle-income countries (123 per 100,000 births; 95% CI 98–152 births) than in high-income countries (79 per 100,000 births; 95% CI 68–90 births) (p for interaction < 0.01). While likely representing an underestimate, this model predicts that each year, nearly 400,000 new cases of pediatric hydrocephalus will develop worldwide. The greatest burden of disease falls on the African, Latin American, and Southeast Asian regions, accounting for three-quarters of the total volume of new cases. The high crude birth rate, greater proportion of patients with postinfectious etiology, and higher incidence of NTDs all contribute to a case volume in low- and middle-income countries that outweighs that in high-income countries by more than 20-fold. Global estimates of adult and other forms of acquired hydrocephalus are lacking.CONCLUSIONSFor the first time in a global model, the annual incidence of pediatric hydrocephalus is estimated. Low- and middle-income countries incur the greatest burden of disease, particularly those within the African and Latin American regions. Reliable incidence and burden figures for adult forms of hydrocephalus are absent in the literature and warrant specific investigation. A global effort to address hydrocephalus in regions with the greatest demand is imperative to reduce disease incidence, morbidity, mortality, and disparities of access to treatment.

Hydrocephalus refers to a pathological condition that occurs when there is an excessive accumulation of cerebrospinal fluid (CSF) in the head. Hydrocephalus can either be congenital, resulting from disturbance in the development of the CSF pathways or alternatively, it is acquired due to secondary events resulting in blockage to circulation channels. Hydrocephalus can be further categorised into obstructive and non-obstructive subcategories. It is known that hydrocephalus occurs as a result of either an obstruction to the flow of CSF and/or the body's inability to absorb it. This ultimately increases the pressures within the brain due to the swelling caused by the build-up of CSF within the ventricles and subarachnoid spaces. In Australia, one in a thousand children are born with hydrocephalus per year. This figure is excluding acquired hydrocephalus in older children and adults. In infants, hydrocephalus is often associated with an increase in head circumference. They may also present with lethargy, vomiting, poor feeding, seizures, nystagmus and low muscle tone. Without proper diagnosis for early intervention and management, hydrocephalus may result in compromised mental functioning, visual disturbances, walking difficulty, incontinence and reduced conscious state. Cranial ultrasound has emerged as an effective imaging modality in diagnosing progressive ventricle dilation in the infant population and may be used for continuous follow-up. This talk will cover the role of ultrasound and its major benefits in the diagnosis and detection of hydrocephalus for the paediatric population.

Objective: To determine the rate of conversion to ventriculoperitoneal (VP) shunt in children treated with ventriculosubgaleal (VSG) shunt for hydrocephalus of infective and noninfective etiology. Materials and Methods: A prospective, observational study was conducted at the department of neurosurgery in a tertiary care medical institute in children who underwent VSG shunt for congenital and acquired hydrocephalus of infective and noninfective etiology. Primary outcome measure was rate of conversion to VP shunt. Results: In total 58 children, 65.5% were aged 5 days to 12 months and 67.2% were male. Meningitis (70.7%) was the most common cause of hydrocephalus, followed by hemorrhage (20.7%) and congenital causes (8.6%). The rate of conversion to VP shunt was 58.6%. Moreover, 25.9% of the children had spontaneous resolution of hydrocephalus with no further requirement of shunt, whereas 15.5% required endoscopic third ventriculostomy. Shunt revision was required in 6.9% of the children and shunt malfunction rate was 5.2%. Other complications such as cerebrospinal fluid (CSF) leak (3.4%), wound infection (1.7%), and shunt tip migration (1.7%) were uncommon. Conclusion: VSG shunt is a simple and effective treatment for temporary diversion of CSF for infective and noninfective hydrocephalus in children who are not ideal for a permanent method of CSF diversion even in the presence of active meningitis and shunt infection. In experienced hands and in selected cases, VSG shunt alone can help resolve the hydrocephalus.

Objective: Although endoscopic third ventriculostomy (ETV) is a well established procedure for the treatment of congenital and acquired hydrocephalus, there is still a controversy with regard to the higher risk of failure in younger infants compared to older children after such a procedure. The aim of this article is to define the effectiveness of third ventriculostomy for hydrocephalus in infants less than 6 months of age better and to determine possible positive predictive factors. Materials and Methods: In a series of 126 consecutive patients who underwent ETV between January 2000 and December 2002, 21 procedures were performed on infants ranging in age from 23 to 180 days, and without a previous history of shunting. The follow-up period was 49 to 82 months. Results: The overall success rate of ETV was 67% (14/21). All failures occurred in the early postoperative period (average 38 days, range 25-88 days). The success rate varied with the etiology of the patient's hydrocephalus. The best results were obtained in patients with acqueductal stenosis, Dandy-Walker malformation and myelomeningocele. The least favorable results (50% failure rate) occurred in infants treated for post-hemorrhagic and post-meningitic hydrocephalus. Conclusion: This study does not demonstrate a correlation between the age of the infants and the ETV success rate. The specific etiology of hydrocephalus is the most relevant prognostic factor in infants under six months of age.

Hydrocephalus is a progressive brain disorder characterized by abnormalities in the flow of cerebrospinal fluid (CSF) and ventricular dilatation that leads to cerebral atrophy, and if left untreated, can be fatal. Genetic mutations, congenital malformations, infectious diseases, intracerebral hemorrhages and tumors are common conditions resulting in hydrocephalus. Although the causes of obstructive hydrocephalus are better understood, the mechanisms resulting in chronic, progressive communicating congenital and acquired hydrocephalus are less well understood. In this regard, recent studies in transgenic (tg) mice suggest that increased expression of cytokines such as TGF-beta1 might play an important role by disrupting the vascular extracellular matrix (ECM) remodeling, promoting hemorrhages, and altering the reabsorption of CSF. In this context, the main objective of this manuscript is to provide an overview on the cellular and molecular mechanisms of hydrocephalus based on studies derived from tg and experimental animal models.

Intraventricular hemorrhage is the most common cause of infantile acquired hydrocephalus. Our objective is to determine if the implantation of ventriculoperitoneal shunt in posthemorrhagic hydrocephalus as a primary and definitive neurosurgical treatment, with no previous temporary procedures, would decrease complication rates with good functional outcomes. Two hundred seventy-one patients with germinal matrix hemorrhage were diagnosed at the Carlos Haya Hospital between 2003 and 2010. Forty-seven patients underwent ventriculoperitoneal shunt after developing symptomatic hydrocephalus. The minimum weight required for shunt implantation was 1,500 g. We recorded complications related to the surgical procedure and analyzed functional state with a self-developed four-grade scale. One hundred thirty-nine (51.3 %) patients with intraventricular hemorrhage developed ventricular dilatation, but only 47 patients (17.34 %) needed shunting. In seven cases, temporary neurosurgical procedures were performed, but in all of them, this was followed by ventriculoperitoneal shunt implantation. The infection rate was 4.25 %, and shunt obstruction rate was 4.25 %. More than 80 % of patients were classified as good or excellent functional state. Mean follow-up period was 38.75 months (SD, 27.09; range, 1–102 months). Ventriculoperitoneal shunting as a primary neurosurgical treatment in posthemorrhagic hydrocephalus would decrease surgical morbidity with good functional outcome.

The quaking viable ( qk v ) mice harbor an autosomal recessive mutation that deletes the parkin co-regulated gene ( pacrg ) and parkin ( park2 ) genes, and alters the expression of the quaking ( qkI ) gene. qk v mice have been well-studied for their dysmyelination phenotype caused by the altered expression of the qkI gene. The qk v mice exhibit sterility in males and develop acquired mild hydrocephalus due to the lack of PACRG expression. To identify genetic interactors of the pacrg–parkin–qkI locus, we crossbred the qk v mice with various mouse strains including the patched1 ( ptch1 )-deficient mice. The ptch1 heterozygous mice exhibit increased Sonic Hedgehog (Shh) signaling and are prone to several malignancies including tumorigenesis. In the present study, we show that the qk v/v ; ptch1 +/− mice are distinguished by a dome-shaped skull at 4 to 6 weeks of age and exhibit dilation of the lateral and third ventricles leading to fatal acquired hydrocephalus by ~ 5 months of age, unlike their littermate controls that did not develop the condition. The qk v/v ; ptch1 +/− mice contained normal ciliated ependymal cells lining the ventricles of the brain, but these cells were functionally compromised with a severe cilial mediated flow defect. Our findings suggest that the ptch1 and the pacrg–parkin–qkI loci genetically interact to regulate cilia function of the ependymal cells.

Latex allergy is a potentially life-threatening intraoperative occurrence which is relatively rare in our environment. We report the case of a 3 month old male infant with acquired hydrocephalus undergoing ventriculo-peritoneal shunt insertion who developed wheals and suffered a respiratory arrest following contact with latex gloves. The need for anaesthetists to effectively diagnose and properly manage this rare clinical entity is emphasized. Keywords: latex allergy; perioperative

The following patients with multiple complex diagnoses define the adult hydrocephalus population: (1) transition patients (previously treated for hydrocephalus as children), (2) adults with previously untreated congenital hydrocephalus, (3) adults with acquired hydrocephalus with an identifiable etiology, and (4) patients with suspected or proven idiopathic normal pressure hydrocephalus. Restricting the outpatient care or hydrocephalus clinic definition to a single hydrocephalus patient subpopulation limits our understanding of these patients and effectively abandons the remaining adult patients with hydrocephalus to a less focused and potentially less effective healthcare arrangement. A comprehensive adult hydrocephalus clinic model is described and recommended to advance our understanding of this diverse patient population, which will ultimately lead to the development and provision of a better standard of patient care.

Nonketotic hyperglycinemia is an autosomal recessive disorder of glycine metabolism. Patients generally present in the neonatal period with lethargy, feeding difficulty, hypotonia, apnea, poorly controlled convulsions, and coma. Myoclonic seizures and burst suppression pattern on electroencephalography are major findings of disease, but development of hydrocephalus is not an expected finding. The present case is that of an infant with acquired hydrocephalus, psychomotor retardation, and myoclonic seizures in whom the final diagnosis was nonketotic hyperglycinemia. (C) 2009 by Elsevier Inc. All rights reserved.

The inflammatory process of meningitis may obstruct the circulation of cerebrospinal fluid culminating into increased intracranial tension and in children before closure of their frontanel may lead to hydrocephalus. This study was constructed to find out the impact of the out break of meningococcal meningitis into the frequency of hydrocephalus in young children. We found that the peak of the incidence of acquired hydrocephalus in young children follows that of the seasonal cerebro-spinal meningitis (CSM) by few weeks. The age group affected by post-meningetic hydrocephalus was that below two years. Children of this age group were not included in the national vaccination program against cerebro-spinal meningitis at the time of the study, which may be the cause of their high susceptibility. Keywords : hydrocephalus, meningococcal meningitis Sudan Journal of Medical Sciences Vol. 1(1) 2006: 41-42

Congenital hydrocephalus has an estimated population incidence of 0.2 to 0.8/1000 live births. With improvements in techniques for cerebrospinal fluid shunting, treatment of hydrocephalus has become safe and routine, yet data describing mortality from congenital hydrocephalus or demonstrating improvements in mortality with the advent of modern treatment are scarce. The authors' analysis sought to rectify this situation.The authors performed an electronic search of National Center for Health Statistics death certificate databases to identify deaths from 1979 to 1998 attributed to congenital hydrocephalus, spina bifida with hydrocephalus, and acquired hydrocephalus (both obstructive and communicating) in all children in the US aged 1 day to 20 years. Mortality rates were defined as deaths per 100,000 person-years and were analyzed for differences on the basis of age, race, sex, and year. The authors identified 10,406 deaths attributed to childhood hydrocephalus within the 20-year study period. The overall mortality rate was 0.71 per 100,00 person-years. Mortality rates were highest in infants, with 3979 deaths; they were similar between girls and boys. Compared with white infants, black infants had higher relative risk (RR) for death caused by congenital hydrocephalus (RR 1.46, p value0.0001) and acquired hydrocephalus (RR 2.58, p value0.0001) but not for that caused by hydrocephalus with spina bifida (RR 0.65, p value0.0001). From 1979 to 1998, the mortality rate due to congenital hydrocephalus declined 66.3%, from spina bifida with hydrocephalus it declined by 30.4%, and from acquired hydrocephalus it declined by 67.5%.Mortality rates from childhood hydrocephalus have declined in US children over the previous 20 years. Black race is associated with higher mortality rates in infants for both congenital and acquired hydrocephalus, whereas sex has no effect.

Cysts of the third ventricle are rare congenital suprasellar malformations of arachnoidal, endodermal, or neuroepithelial origin. Depending on their size and location, they can cause space-occupying intracranial lesions and hydrocephalus occlusus by obstruction of the aqueduct or foramen of Monro. They can be missed on routine computer tomography and magnetic resonance imaging. A 2-year-old boy presented signs of intracranial pressure. Initial magnetic resonance imaging revealed a triventricular internal hydrocephalus with no visible etiologic lesion. An extended investigation with the constructive interference in steady state-technique showed a mobile cystic cerebrospinal fluid-intense lesion within the third ventricle, causing transient occlusion of the foramen of Monro. A suprasellar cyst of the third ventricle is an important differential diagnosis in apparently "idiopathic" internal hydrocephalus. In such cases, magnetic resonance imaging using the constructive interference in steady state-technique with a slice thickness of 1 mm is the method of choice for detecting intraventricular cysts. Neurosurgical fenestration and/or resection of the cyst by neuroendoscopy can resolve cerebrospinal fluid circulation disturbances and seems to be superior to a shunt.

Objective Infantile posthemorrhagic hydrocephalus (IPHH) is the most common cause of infantile acquired hydrocephalus. We present and discuss our experience of treatment of six IPHH patients treated by a ventriculo-peritoneal (VP) shunt. Methods Six preterm infants treated by a VP shunt due to germinal matrix hemorrhage and hydrocephalus were included in our study. External ventricular drainage (EVD) was performed in patients with symptomatic ventricular dilatation, and a VP shunt was placed in the case of no improvement of the ventricular index despite several rounds of EVD. Radiographic findings and surgical outcomes were analyzed retrospectively. Results Four patients were male and two were female. Mean gestational age was 25 weeks and mean weight at birth was 868.3 g. One patient had a Papile grade II (16.7%) hemorrhage, three had a grade III (50%) hemorrhage, and two had a grade IV (33.3%) hemorrhage. EVD complications (one case of ventriculitis and one case of a ventricular abscess) occurred in two patients. VP shunt complications occurred in two patients (33.3%). Three cases had an isolated 4th ventricle; two of these cases had a VP shunt placed whereas the other case had a VP shunt placed in addition to aqueductoplasty using a neuroendoscope. At the last follow-up, three of the six patients had severe neurodevelopmental delay, two had mild neurodevelopmental delay, and one had normal development status. Conclusion In our study, although it is difficult to present the significant result for management of IPHH, we think that varied efforts are required to treat IPHH patients.

Lymphocytic choriomeningitis virus (LCMV), a human zoonosis caused by a rodent-borne arenavirus, has been associated with both postnatal and intrauterine human disease. Infection in man is acquired after inhalation, ingestion, or direct contact with virus found in the urine, feces, and saliva of infected mice, hamsters, and guinea pigs. Congenital LCMV infection is a significant, often unrecognized cause of chorioretinitis, hydrocephalus, microcephaly or macrocephaly, and mental retardation. Acquired LCMV infection, asymptomatic in approximately one third of individuals, is productive of central nervous system manifestations in one half of the remaining cases. Aseptic meningitis or meningoencephalitis are the predominant syndromes, although transverse myelitis, a Guillain-Barré-type syndrome, as well as transient and permanent acquired hydrocephalus have also been reported. Fatalities are rare. We report a patient with meningoencephalitis attributable to LCMV and discuss the spectrum of central nervous system disease, newer diagnostic modalities, and preventive strategies. lymphocytic choriomeningitis virus, aseptic meningitis, meningoencephalitis, zoonosis, hydrocephalus, arenavirus.

Cerebral aqueductal stenosis is one of the most common causes of congenital and acquired hydrocephalus, but the etiology, pathophysiology and cerebrospinal fluid (CSF) dynamics of aqueductal stenosis have yet to be clarified. Utilizing cardiac gated cine magnetic resonance (MR) imaging, we evaluated aqueductal configuration and pulsatile motion of brain and CSF flow stimulated by cardiac pulsation in five patients with non-tumoral aqueductal stenosis. Cine MR of four cases revealed obliteration of the aqueduct by thickening mesencephalic tectum, turbulent CSF flow in the III ventricle, and absence of flow-related signal void, which in all normal cases indicates CSF movement within the aqueduct. In the remaining fifth case, with proximal dilation of the aqueduct resulting from thinning of the tectum, distortion of caudal (distal) tectum related to pulsatile motion of the brain caused funnel-like narrowing of the aqueduct, leading to incomplete obstruction and the absence of upward CSF flow during diastole.

Thirty-nine patients aged 21-82 years (mean 62 years) under investigation for suspected acquired hydrocephalus were investigated by dynamic posturogrdphy before and 6-8 h after a cerebrospinal fluid tap of 20-40 ml. Dynamic posturography comprises a sensory organization (SO) part in which the platform and visual surround are either stable or referenced to the patient's sway. The eyes are closed or open. In a movement coordination (MC) part the platform makes active movements, thus introducing external disturbances in the procedure. The patients improved their balancing ability in all tests on the sway referenced platform in SO, while MC remained unaffected. It is concluded that dynamic posturography is valuable for dysequilibrium assessment in clinical evaluation of suitable candidates for ventricular shunts in acquired hydrocephalus.

Hydranencephaly is a rare devastating malformation where greater part of the cerebral hemisphere is replaced by large membranous sac filled with cerebrospinal fluid (CSF) [1, 2]. Similar condition can be produced secondary to a congenital / perinatally acquired hydrocephalus. Both these conditions mimic each other on CT and are trans-illumination positive. Literature search does not give any definite clinical criteria to distinguish these two. We therefore present a similar case, which posed a diagnostic problem.

On the basis of the laws of Pascal and Laplace, it is shown that the ventricular dilatation in acquired hydrocephalus is due to a primary increase in the intraventricular pressure (IVP), and that a new steady state can be reached, whether the IVP is increased or normal. The pressure increase is due to a disproportion between the production and reabsorption of cerebrospinal fluid (CSF). As water and salts pass freely across the ependyma and the choroid plexus in hydrocephalus, the pressure increase is caused by an increased protein concentration in the ventricular CSF, leading to increased fluid contents according to the Gibbs-Donnan equilibrium. During the ventricular dilatation, the ependyma is destroyed, and the protein molecules penetrate into the subependymal part of the white matter. This results in a reduction in the colloid osmotic pressure of the ventricular CSF, and a new steady state can be reached, with a normal protein concentration in an increased volume. The attendant microscopic changes in the ventricular wall were demonstrated in a patient with acquired hydrocephalus, and the observations made were in conformity with the results of a number of animal experiments. The symptomatology of acquired hydrocephalus is in agreement with a primary affection of the axons running in the juxtaventricular part of the white matter.

W E HAVE found tha t the use of the Pudenz -Heye r valve with a ventr iculo-peri toneal shunt provides the mos t favorable me thod of t r e a t m e n t for congenital and acquired hydrocephalus. The simple ventr iculo-peri toneal shunt was prone to early malfunct ion because of the insinuation of omen tum into the distal end of the tubing. For this reason, ventr iculo-caval and ventriculo-atrial shunts have been generally adopted. The Pudenz -Heye r shunt ing device has a leaf valve a t the distal end of the tubular system. This va lve remains closed a t all t imes except when fluid is passing outward, thus prevent ing o m e n t u m f rom gaining access to the tubula r system. Consequently, we chose to per form ventriculo-peri toneal shunts when confronted with the problem of a child who a l ready had one internal and two external jugular veins thrombosed from previous ventr iculo-venous shunts. Our resuits in these cases p roved so favorable tha t we ul t imately made it our practice to use this method as a m a t t e r of routine. Although the technique of establishing a ventI iculo-peri toneal shunt is relatively simple in an adult , the appl ica t ion to a newborn or premature child is more complicated. The problems of general anesthesia in the newborn add to the difficulties. We are report ing our simplified technique for performing the ventriculo-peri toneal shunt under local anesthesia. Our experience includes 67 procedures performed on 6~ newborn children and 5 adults , for either congenital or acquired hydrocephalus of either the communica t ing or the obstruct ive varieties.

Intracranial pressure (ICP) monitoring is a critical measure for avoiding severe brain dysfunction or brain death by directing supportive therapy so as to prevent ICP increase severe enough to reduce cerebral blood perfusion. Such situations occur with brain swelling, increased cerebral vascular volume, and increase in cerebrospinal fluid (CSF) volume. Causes include ischemic stroke, subarachnoid bleeding, brain contusion, encephalitis (as in Reye's syndrome), and hydrocephalus from meningitis or neoplasm. When several days of ICP monitoring can direct resolution of the pressure crisis, the invasive direct connection of an intracranial sensor with external recording device carries only minimal infection risk. Prolonged ICP monitoring for weeks or months demands telemetry and becomes desirable in a number of chronic disease problems including both congenital and acquired hydrocephalus where enlarged and pressurized cerebral ventricles develop with reduced absorption of continuously secreted CSF. Although the primary disturbance in CSF circulation can remain incurable, its palliation by valve-regulated CSF diversions or shunting can restore normal brain function and in infants permit normal brain development. Missing this goal can result from failure to maintain a sufficiently normal pattern of CSF dynamics and ICP. Monitoring of the CSF pressure fluctuations transmitted through an intraventricular catheter provides the most accurate record of ICP pulsations. Therefore, a pressure sensing module can be “T'd” into an existing shunt system in continuity with the already placed ventricular tube. The capacity to monitor ICP accurately by telemetry was first established in dogs made hydrocephalic to assure free CSF pulse through a ventricular catheter (1,2,3, 4,5). The subsequent use of ICP monitoring by telemetry in three patients will be described.

Three premature infants with post-haemorrhagic hydrocephalus were treated by removing large volumes of cerebrospinal fluid with repeated lumbar punctures. After this treatment, a computerised tomographic brain scan showed that ventricle size had decreased, and subsequent head growth was normal in all three patients. The results suggest that acquired hydrocephalus in premature infants may be transient and that ventricular shunts may not be necessary in all cases.

This report presents a comparison of nine models of experimental hydrocephalus from different etiologies and discusses the diverse and common pathological features among them. Using rabbits, dogs, rats, and mice, the following congenital and acquired models of 105 individuals and 43 litters of hydrocephalic animals were developed. These animals were investigated morphologically and RI study was done with Hy-3 mouse. It was concluded that the pathology of hydrocephalus depended on the age of the animals and the method of production rather than the dosis of each insults.

By quantitative isotope ventriculography (QIV), the formation rate of albumin in the ventricular system (FRalb) was determined in 14 hydrocephalic and 14 nonhydrocephalic patients. The FRalb was significantly reduced in the patients with acquired hydrocephalus. By a comparison of the mean formation rate of albumin (MFRalb) and the mean absorption rate of albumin (MARalb), a significant biological difference in the two groups of patients was demonstrated.

Six dogs that had a sudden onset and rapid progression of neurological signs with enlargement of the cranial vault at 6-8 weeks old were believed to have congenital hydrocephalus. There were severe inflammatory and necrotizing lesions in periventricular sites in the brain. A bacterial infection may have been the cause of this process. Recent studies on hydrocephalus in animals, including the dog, have changed the old and rather simplified concepts on cerebrospinal fluid dynamics and contributed greatly to the understanding of the pathogenesis of hydrocephalus (24, 391. Spon- taneously occurring cases of hydrocephalus, however, remain a clinical and patho- logical challenge. It has been stated that hydrocephalus is the most common congenital malforma- tion of the canine nervous system (23). There is a higher incidence in miniature breeds and brachycephalic types (7, 271. This condition is usually detected soon after birth (lo) and is often associated with malformation of the mesencephalic aqueduct (6, 331. There are few pathological studies on spontaneously acquired hydrocephalus in the dog. One study (7) describes hydrocephalus ex vucuo resulting from senile atrophy of the brain, and others examined five hydrocephalic brains but found histological lesions in only one (34, 35). Our report of clinical and histologic studies on a group of young dogs indicates that these dogs had a type of acquired internal hydrocephalus of probable infec- tious cause not previously reported.

Based on the results of quantitative isotope ventriculography (QIV) a group of 25 patients was divided into 17 hydrocephalic and 8 non- or doubtfully hydrocephalic patients. An atrio-ventricular shunt (A-V shunt) was inserted in all 25 patients. Eight of the hydrocephalic patients improved or became well, while nine hydrocephalic patients were unchanged, worse or dead at the time of follow-up. These nine patients suffered either from severe brain damage, chronic alcoholism over several years, arterial hypertension, severe diabetes, or acute meningo-encephalitis caused by a virus infection. None of the eight non- or doubtfully hydrocephalic patients improved after the operation. From this it was concluded that QIV is of considerable diagnostic value in acquired hydrocephalus.

The authors present the results of a prospective study of 20 children with congenital or acquired hydrocephalus of nontumoral etiology and submitted to ventriculo- (or cyst-) peritoneal shunting with valve. The diagnosis was established by B-mode or real-time brain sonography, in association with another neuroradiological procedure (computed tomography, ventriculography with air or Dimer-X, cerebral angiography). Among the proposed measurements (cortical thickness, lateral ventricle height, III ventricle width and ventricular ratio) for pre- and postoperative comparison, the cortical thickness and the lateral ventricle height were the ones that changed significantly when analyzed by sonography. The routine use of brain sonography allowed the visualization of the ventricular catheter position and the diagnosis of complications, such as subdural collection, progressive enlargement of cysts, isolated IV ventricle, etc, even before symptoms arise. The authors conclude that sonography is easily performed, inexpensive and innocuous, and should be used routinely during the follow-up of children with hydrocephalus.

A total of 160 patients suspected of having acquired hydrocephalus were studied either by quantitative isotope ventriculography (QIV) or by lumbar isotope cisternography (LIC). Of these patients, 56 had hydrocephalus. Mental deterioration, gait disturbances, ataxia, spasticity, and incontinence were most frequently present in the hydrocephalic patients, but none of the signs or combinations thereof are pathognomonic of acquired hydrocephalus. These signs are independent of the intracranial pressure (ICP) and the type of hydrocephalus. Surgical shunt procedures were in most cases followed by the disappearance of mental deterioration, gait disturbances, ataxia, and spasticity.

The authors retrospectively reviewed all cases of acquired hydrocephalus admitted to the Institute of Neurological Sciences, Glasgow, Scotland, within a 5-year period and encountered 17 cases of posttraumatic hydrocephalus. These represented 0.7% of 2374 cases of severe head injury. Hydrocephalus became symptomatic within 1 year from the time of injury in 16 of 17 cases. Meningitis, traumatic subarachnoid hemorrhage, posterior fossa mass, supratentorial clot with contralateral ventricular dilatation, and craniotomy contributed to the development of posttraumatic hydrocephalus. After shunting, eight patients (50%) improved markedly and four (25%) slightly.