Characteristics of children with infantile hydrocephalus

Characteristics of children with infantile hydrocephalus

Source publication
Article
Full-text available
Purpose Little is known about the prognosis for school performance among children with all-cause infantile hydrocephalus (IHC). Using detailed educational data, we investigated the school performance for IHC patients compared to other children in Denmark. Patients and methods We conducted a population-based cohort study of all live-born children i...

Contexts in source publication

Context 1
... is recorded by ICD (International Classification of Diseases) codes (ICD 8th edi- tion until 1995 and ICD 10th edition hereafter). ICD codes used in this study are listed in Table S1. ...
Context 2
... total of 61.8% of the IHC patients were males and 38.2% females, compared to 51.3% males and 48.7% females among other children. Characteristics for the IHC children are presented in Table 1. The cumulative mortality at 4 years of age was 20.0% for the IHC children as compared to 0.7% among other children, and increased to 24.9% for IHC children and 1.0% for other children at 20 years of age. ...

Citations

... Hydrocephalus is a complex cerebrospinal fluid (CSF) disorder, affecting both children and adults. In Denmark, the prevalence of infantile hydrocephalus is reported to be 1.08 in 1000 live-born children (from 1977 to 2015) (Schmidt et al., 2018). It is a life-long chronic condition, with a risk of both structural brain damage and repetitive hospital admissions and surgeries due to treatment failure. ...
Article
Full-text available
Introduction Telemetric monitoring of intracranial pressure (ICP) in children with a complex cerebrospinal disorder might help parents distinguish acute and potential life-threatening symptoms of hydrocephalus from other illnesses. Research question What is patient and parent perceptions of system utility of telemetric ICP monitoring, and how does a long-term telemetric implant influence daily life of both patients and their families? Material and methods A qualitative case study design with a focus group interview including parents of children with a complex cerebrospinal fluid disorder and an implanted telemetric ICP sensor. Results Three parents participated. Based on thematic analysis, three themes were created: ‘Daily living with telemetric ICP monitoring’, ‘Parenting a child with a CSF disorder’, and ‘The healthy sibling’. The ICP sensor provided the parents with security and made them trust their intuition, while the possibility of home monitoring ensured stability for the entire family and had a calming effect on healthy siblings. Home monitoring was seen as the system's greatest advantages, whereas size, weight, and functionality of the external monitoring equipment were highlighted as disadvantages. Discussion and conclusion All parents supported the telemetric ICP sensor as a valued tool in treatment guidance of their child and stated that advantages exceeded disadvantages. It was stated that the possibility of conducting ICP measurements at home reduced the need for acute hospital admissions, which consequently led to a more stable daily life for the entire family. Suggestions regarding technical improvements with focus on more compatible external monitoring equipment were raised by all parents included.
... [14,15] Other studies were indicated that factors that influence the quality of life of pediatric patients after neurosurgery include the perceptions of parents and patients, the impact of the illness, and the presence of risk factors where parents often have more negative assessments of their children's health-related quality of life (HRQoL) compared to their children themselves [16], as well as brain tumor, a common condition in pediatric neurosurgery, has been found to have a major impact on children's quality of life, with risk factors including tumor location, radiation, hydrocephalus, lower IQ, and behavioral problems. [17] French study was found that factors that contribute to a good quality of life for pediatric patients after neurosurgery include understanding parents' perceptions of their children's health-related quality of life (HRQoL) [18] as well as the use of validated HRQOL instruments is important in assessing outcomes and monitoring progress and Intraoperative neurophysiological monitoring and maximal tumor removal with minimal neurological morbidity are crucial in minimizing postoperative complications and preserving quality of life. [19,20] ...
Article
Full-text available
Background: Neurological disorders constitute approximately 30% of all diseases globally, with a significant majority prevalent in developing nations. Aim: This study focuses on assessing the health-related quality of life (HRQoL) in pediatric patients subjected to neurosurgery. Methods: Clinical data were procured from different hospitals in Iraq during the period between July 14th, 2022, and August 18th, 2023, involving both pediatric and adult patients undergoing neurosurgery. The study encompassed 67 patients with an age range of 2 to 13 years. The data included test results and identified etiology in children. Additionally, it accounted for anesthetic outcomes for additives such as remifentanil, sevoflurane, and propofol. The HRQoL scale was employed to evaluate the health-related quality of life in pediatric patients post-surgery, focusing on physical, mental, emotional, and social functions. Results: Demographic data indicated a higher male population (41 patients) compared to females (26 patients). The clinical data recorded included the duration of surgery, anesthesia, estimated blood loss during surgery, and postoperative complications. The surgery lasted 342.57 ± 12.1 minutes, the anesthesia duration was 318.41 ± 100.50 minutes, and the estimated blood loss was 110.5 ± 71.2 ml. Postoperative complications were recorded in 21 cases, predominantly involving infection and bleeding. The average hospital stay was 9.1 ± 3.5 days, with a follow-up period of 10 months. The recovery period spanned between 6 to 10 weeks, and there were five recorded fatalities. The HRQoL assessment revealed physical functions at 76.8, mental functions at 60, emotional functions at 71.33, and social functions at 57.35. Conclusion: This study validates the effectiveness of meticulous surgical procedure management in preserving the quality of life for pediatric patients across diverse age groups. It also highlights the postoperative 125 A journal of the AMERICAN Journal of Pediatric Medicine and Health Sciences www. grnjournal.us complication rate, with infection and bleeding being the most prevalent. The mortality rate was less than 6%, reflecting the overall patient outcomes.
... 3 At the same time, it is one of the most common congenital disorders of the central nervous system affecting 1.08 in 1,000 live-born children, when all subtypes are included. 4 At the time of writing, 100+ candidate genes for hydrocephalus have been described in referred patients and animal studies, [5][6][7][8] including eight genes known to exhibit a Mendelian pattern of inheritance, namely two X-linked genes; L1CAM and AP1S2, and six autosomal genes; CCDC88C, MPDZ, TRIM71, SMARCC1, PTCH1, and SHH. [5][6][7][8][9] There are no findings from genome-wide association studies, which is probably due to difficulties to identify sufficient case numbers to identify moderate or small effect sizes, and the situation is further complicated by the heterogenous presentations of hydrocephalus. ...
... In the Danish population, children with infantile congenital/hydrocephalus have a cumulative mortality of 20 % at 4 years of age, compared to a mortality of 0.7 % among children from the background population. 4 Furthermore, this study did not include elective termination of pregnancies due to prenatal diagnosis of hydrocephalus, as abortions are not registered by diagnosis in Denmark. This differential mortality may have led to exclusion of hydrocephalus subtypes with early and severe presentations. ...
Article
Full-text available
Hydrocephalus is one of the most common congenital disorders of the central nervous system and often displays psychiatric co-morbidities, in particular autism spectrum disorder. The disease mechanisms behind hydrocephalus are complex and not well understood, but some association with dysfunctional cilia in the brain ventricles and subarachnoid space has been indicated. A better understanding of the genetic aetiology of hydrocephalus, including the role of ciliopathies, may bring insights into a potentially shared genetic aetiology. In this population-based case-cohort study we, for the first time, investigated variants of postulated hydrocephalus candidate genes. Using this data, we aimed to investigate potential involvement of the ciliome in hydrocephalus and describe genotype-phenotype associations with autism spectrum disorder. One-hundred and twenty-one hydrocephalus candidate genes were screened in a whole-exome sequenced sub cohort of the Lundbeck Foundation Initiative for Integrative Psychiatric Research study, comprising 72 hydrocephalus patients and 4,181 background population controls. Candidate genes containing high-impact variants of interest were systematically evaluated for their involvement in ciliary function and autism spectrum disorder. The median age at diagnosis for the hydrocephalus patients was 0 years of age (range 0-27 years), the median age at analysis was 22 years (11-35 years), and 70.5 % were males. Median age for controls was 18 years (range 11-26 years) and 53.3 % were males. Fifty-two putative hydrocephalus-associated variants in 34 genes, were identified in 42 patients (58.3 %). In hydrocephalus cases, we found increased, but not significant, enrichment of high-impact protein altering variants (odds ratio 1.51, 95% CI 0.92-2.51, P = 0.096), which was driven by a significant enrichment of rare protein truncating variants (odds ratio 2.71, 95% CI 1.17-5.58, P = 0.011). Fourteen of the genes with high-impact variants are part of the ciliome, whereas another six genes affect cilia-dependent processes during neurogenesis. Furthermore, 15 of the 34 genes with high-impact variants and three of eight genes with protein truncating variants were associated with autism spectrum disorder. Because symptoms of other diseases may be neglected or masked by the hydrocephalus-associated symptoms, we suggest that patients with congenital hydrocephalus undergo clinical genetic assessment with respect to ciliopathies and autism spectrum disorder. Our results point to the significance of hydrocephalus as a ciliary disease in some cases. Future studies in brain ciliopathies may not only reveal new insights into hydrocephalus, but also, brain disease in the broadest sense, given the essential role of cilia for neurodevelopment.
... At the same time, it is one of the most common congenital disorders of the central nervous system affecting 1.08 in 1,000 live-born children, when all subtypes are included. 1 At the time of writing, 100+ candidate genes for hydrocephalus have been described in referred patients and animal studies, [2][3][4][5] including eight genes known to exhibit a Mendelian pattern of inheritance, namely two X-linked genes; L1CAM and absorption. 14,15 Motile ciliopathies can lead to hydrocephalus due to impaired circulation and subsequent accumulation of CSF. ...
Preprint
Background Hydrocephalus is one of the most common congenital disorders of the central nervous system and often displays psychiatric co-morbidities, in particular autism spectrum disorder. The disease mechanisms behind hydrocephalus are complex and not well understood, but some association with dysfunctional cilia in the brain ventricles and subarachnoid space has been indicated. A better understanding of the genetic aetiology of hydrocephalus, including the role of ciliopathies, may bring insights into a potentially shared genetic aetiology. In this population-based case-cohort study we, for the first time, investigated variants of postulated hydrocephalus candidate genes. Using this data, we aimed to investigate potential involvement of the ciliome in hydrocephalus and describe genotype-phenotype associations with autism spectrum disorder, identified in the iPSYCH cohort. Methods One-hundred and twenty-one hydrocephalus candidate genes were screened in a whole-exome sequenced sub cohort of the iPSYCH study, comprising 72 hydrocephalus patients and 4,181 background population controls. Candidate genes containing high-impact variants of interest were systematically evaluated for their involvement in ciliary function and autism spectrum disorder. Results The median age at diagnosis for the hydrocephalus patients was 0 years of age (range 0-27 years), the median age at analysis was 22 years (11-35 years), and 70.5 % were males. Median age for controls was 18 years (range 11-26 years) and 53.3 % were males. Fifty-two putative hydrocephalus-associated variants in 34 genes, were identified in 42 patients (58.3 %). In hydrocephalus cases, we found increased, but not significant, enrichment of high-impact protein altering variants (OR 1.51, 95% CI 0.92-2.51, p = 0.096), which was driven by a significant enrichment of rare protein truncating variants (OR 2.71, 95% CI 1.17-5.58, p = 0.011). Fourteen of the genes with high-impact variants are part of the ciliome, whereas another six genes affect cilia-dependent processes during neurogenesis. Furthermore, 15 of the 34 genes with high-impact variants and three of eight genes with protein truncating variants were associated with autism spectrum disorder. Conclusions Because symptoms of other diseases may be neglected or masked by the hydrocephalus-associated symptoms and identification of co-morbidities may be of clinical significance, we suggest that patients with congenital hydrocephalus undergo clinical genetic assessment with respect to ciliopathies and autism spectrum disorder. Our results point to the significance of hydrocephalus as a ciliary disease in some cases. Future studies in brain ciliopathies may not only reveal new insights into hydrocephalus, but also, brain disease in the broadest sense, given the essential role of cilia for neurodevelopment.
... Hydrocephalus is an aetiologically heterogeneous neurological condition with the broad definition excessive amount of intracranial CSF relative to the brain volume, which occurs in 1.08/1,000 children up to 2 years of age in Denmark [10]. A relatively common presentation is the Benign Enlargement of the Subarachnoid Space (BESS) or benign external hydrocephalus, that is asymptomatic and usually resolves spontaneously before the child reaches the age of 2 years [11,12]. ...
... However, one could argue that patients with autism spectrum disorder are more likely to undergo brain imaging studies than patients with the other psychiatric disorders investigated in this study. But the very strong association observed in this study (OR 3.77, 95% CI 2.48-5.78) is unlikely to be explained by incidental findings of hydrocephalus only, which generally is a rare disease seen in 1.08/1000 in children up to 2 years of age in Denmark [10]. Thus, we believe the effect of surveillance bias has limited impact on the results. ...
Article
Full-text available
Background The association between autism spectrum disorder and hydrocephalus is not well understood, despite demonstrated links between autism spectrum disorder and cerebrospinal fluid abnormalities. Based on the hypothesis that autism spectrum disorder and hydrocephalus may, at least in some cases, be two manifestations of a shared congenital brain pathology, we investigated the potential association between autism spectrum disorder and hydrocephalus in a large Danish population-based cohort. Methods Patients and controls were obtained from the Lundbeck Foundation Initiative for Integrative Psychiatric Research iPSYCH2012 case-cohort, which includes all patients with selected psychiatric disorders born in Denmark 1981–2005 along with randomly selected population controls (end of follow-up, December 31, 2016). The associations between individual psychiatric disorders and hydrocephalus were estimated using binary logistic regression with adjustment for age and sex. Results The cohort consisted of 86,571 individuals, of which 14,654 were diagnosed with autism spectrum disorder, 28,606 were population controls, and the remaining were diagnosed with other psychiatric disorders. We identified 201 hydrocephalus cases; 68 among autism spectrum disorder patients and 40 among controls (OR 3.77, 95% CI 2.48–5.78), which corresponds to an absolute risk of 0.46 % (i.e. approximately one in 217 children with autism spectrum disorder had co-occurring hydrocephalus). The autism spectrum disorder-hydrocephalus association was significant over the entire subgroup spectrum of autism spectrum disorder. Conclusions Given the considerable risk of hydrocephalus among patients with autism spectrum disorder, we suggest that patients with autism spectrum disorder should be evaluated for co-occurring hydrocephalus on a routine basis as timely neurosurgical intervention is important. Likewise, attention must be paid to traits of autism spectrum disorder in children with hydrocephalus. The results of this study call for future investigations on a potential shared aetiology between hydrocephalus and autism spectrum disorder, including the role abnormal CSF dynamics in the pathogenesis of autism spectrum disorder.
... At present, treatment is limited to surgical diversion (shunt treatment) of cerebrospinal fluid (CSF) from the cerebral ventricles to alternative absorption sites or to endoscopic third ventriculostomy (ETV) with or without choroid plexus cauterization (CPC). Functional outcomes are problematic, with residual neurological and cognitive deficits prevalent in 25-80% of patients [1,[8][9][10][11][12][13]. ...
... Post-shunt periods less than 28 days were characterized by relatively acute onset of neurological symptoms (n = 4/8, cases [11][12][13][14]; often demise began 6-24 h before euthanasia was required, and in two cases animals that appeared normal developed profound seizures just 8-10 h later. Shunt patency studies were not performed in vivo, so it is not possible to know unequivocally if these cases represented shunt malfunction. ...
Article
Full-text available
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 mm ³ at 11–43 days of age, which was significantly larger than the baseline values of 2251 ± 194 SD mm ³ for 6 sham controls aged 45–55 days, (p < 0.001). Past the post-induction recovery period, untreated pigs were asymptomatic despite exhibiting mild-moderate ventriculomegaly. Three out of 4 shunted animals showed a reduction in ventricular volume after 20–30 days of treatment, however some developed ataxia and lethargy, from putative shunt malfunction. 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.
... (6)(7)(8)(9) Hydrocephalus is an aetiologically heterogeneous neurological condition with the broad de nition excessive amount of intracranial CSF relative to the brain volume, which occurs in 1.08/1,000 children up to two years of age in Denmark. (10) A relatively common presentation is the Benign Enlargement of the Subarachnoid Space (BESS) or benign external hydrocephalus, that is asymptomatic and usually resolves spontaneously before the child reaches the age of two years. (11,12) The signs and symptoms which most hydrocephalus patients present with do not necessarily include the well-known acute symptoms of increased intracranial pressure or obvious increased head circumference. ...
Preprint
Full-text available
Background The association between autism spectrum disorder and hydrocephalus is not well understood, despite demonstrated links between autism spectrum disorder and cerebrospinal fluid abnormalities. Based on the hypothesis that autism spectrum disorder and hydrocephalus may, at least in some cases, be two manifestations of a shared congenital brain pathology, we investigated the potential association between autism spectrum disorder and hydrocephalus in a large Danish population-based cohort. Methods Patients and controls were obtained from the Lundbeck Foundation Initiative for Integrative Psychiatric Research iPSYCH2012 case-cohort, which includes all patients with selected psychiatric disorders born in Denmark 1981-2005 along with randomly selected population controls (end of follow-up, December 31, 2016). The associations between individual psychiatric disorders and hydrocephalus were estimated using binary logistic regression with adjustment for age and sex. Results The cohort consisted of 86,571 individuals, of which 14,654 were diagnosed with autism spectrum disorder, 28,606 were population controls, and the remaining were diagnosed with other psychiatric disorders. We identified 201 hydrocephalus cases; 68 among autism spectrum disorder patients and 40 among controls (OR 3.77, 95% CI 2.48-5.78), which corresponds to an absolute risk of 0,46 %. The autism spectrum disorder-hydrocephalus association was significant over the entire subgroup spectrum of autism spectrum disorder. Conclusions Given the considerable risk of hydrocephalus among patients with autism spectrum disorder, we suggest that patients with autism spectrum disorder should be evaluated for co-occurring hydrocephalus on a routine basis as timely neurosurgical intervention is important. Likewise, attention must be paid to traits of autism spectrum disorder in children with hydrocephalus. The results of this study call for future investigations on a potential shared aetiology between hydrocephalus and autism spectrum disorder, including the role abnormal CSF dynamics in the pathogenesis of autism spectrum disorder.
... (6)(7)(8)(9) Hydrocephalus is an aetiologically heterogeneous neurological condition with an excessive amount of intracranial CSF relative to the brain volume, which occurs in 1.08/1,000 children up to two years of age in Denmark. (10) A relatively common presentation is the Benign Enlargement of the Subarachnoid Space (BESS) or benign external hydrocephalus, that is asymptomatic and usually resolves spontaneously before the child reaches the age of two years. (11,12) Most hydrocephalus patients though, present with symptoms, but not necessarily the well-known acute symptoms of increased intracranial pressure or an obviously increased head circumference. ...
Preprint
Full-text available
Background The association between autism spectrum disorder and hydrocephalus is not well understood, despite demonstrated links between autism spectrum disorder and cerebrospinal fluid abnormalities. Based on the hypothesis that autism spectrum disorder and hydrocephalus may, at least in some cases, be two manifestations of a shared congenital brain pathology, we investigated the potential association between autism spectrum disorder and hydrocephalus in a large Danish population-based cohort, and whether the polygenic risk scores for autism spectrum disorder changed as a function of the presence of hydrocephalus. Methods Patients and controls were obtained from the Lundbeck Foundation Initiative for Integrative Psychiatric Research iPSYCH2012 case-cohort, which includes all patients with selected psychiatric disorders born in Denmark 1981–2005 along with randomly selected population controls (end of follow-up, December 31, 2016). The associations between individual psychiatric disorders and hydrocephalus were estimated using binary logistic regression with adjustment for age and sex. Polygenic risk scores for autism spectrum disorder were used to compare the genetic architecture of autism spectrum disorder as a function of the presence of hydrocephalus. Results The cohort consisted of 86,571 individuals, of which 14,654 were diagnosed with autism spectrum disorder, 28,606 were population controls, and the remaining were diagnosed with other psychiatric disorders. We identified 201 hydrocephalus cases; 68 among autism spectrum disorder patients and 40 among controls (OR 3.77, 95% CI 2.48–5.78). The autism spectrum disorder-hydrocephalus association was significant over the entire subgroup spectrum of autism spectrum disorder. The presence of hydrocephalus did not markedly influence the polygenic risk scores in patients with autism spectrum disorder, which may indicate overlapping genetic architectures or other common aetiology. Conclusions Given the very strong association, we suggest that patients with autism spectrum disorder should be evaluated for co-occurring hydrocephalus on a routine basis as timely neurosurgical intervention is important. Further clarification of the genetic aetiology of both diseases, may help in elucidating shared genetic pathways between autism spectrum disorder and hydrocephalus, and it may elucidate the role of abnormal CSF dynamics in the pathogenesis of autism spectrum disorders.
Article
Full-text available
OBJECTIVE The objective of this study was to assess the relevance of shunted hydrocephalus in regard to participation by young patients in physical education (PE) classes. Students diagnosed with this condition are very often restricted in PE classes owing to the lack of official and well-defined guidelines. However, the medical literature suggests that there is no relationship between the disease and risk of sport-related injuries. In this study, the authors intended to evaluate not only the accuracy of this statement, but also to explore the factors that delay or foreclose return to exercise. METHODS The analysis was conducted on patients aged < 18 years with a diagnosis of shunt-treated hydrocephalus who received follow-up for a minimum of 1 year. Collected medical data were examined for factors limiting participation in PE at school. Indicators of both sport-related injuries and conditions acceptable for return to exercise were gathered during follow-up visits. RESULTS In this study, 72.72% of patients attended sport activities in schools. The group based on return to PE class differed significantly in the occurrence of neurological deficits, as well as presence of comorbidities. In univariate analysis, the authors identified these parameters as risk factors limiting participation in PE. On the contrary, etiology of hydrocephalus, type of shunting device, number of shunt malfunctions, and presence of epilepsy did not significantly influence sport engagement. CONCLUSIONS This study shows that many patients with shunt-treated hydrocephalus can safely participate in PE. Presence of neurological deficits before and after neurosurgical treatment, as well as presence of comorbidities, are factors that negatively impact the possibility of a patient returning to physical activity. Sport-related injuries do occur, but at a low incidence.
Preprint
Full-text available
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 ways similar to 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. Methods. Acquired hydrocephalus was induced in 30-35-day old pigs by percutaneous intracisternal injections of kaolin. Intracisternal and intraventricular injections of autologous blood was attempted in 2 cases to induce post-hemorrhagic hydrocephalus. Magnetic resonance imaging (MRI) was employed to evaluate the progression of ventriculomegaly and plan the surgical implantation of ventriculoperitoneal shunts at approximately 1–4 weeks post-kaolin. Behavioral and neurological status was assessed continuously. Results. Bilateral ventriculomegaly occurred post-induction and was characterized by enlargement of all portions 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 basal cisterns but the cisterna magna was patent. In 14 untreated hydrocephalic animals, mean total ventricular volumes were 6786 ± 4336 SD mm³ at 17–57 days post-kaolin, which was significantly larger than the baseline values of 2251 ± 194 SD mm³ in sham controls. Consistent with human hydrocephalus, intermittent disruption of the ventricular zone of the lateral ventricles was characterized by loss of multiciliated ependymal cells and the appearance of reactive astrocytes. Past the post-induction recovery period, untreated pigs were asymptomatic in spite of exhibiting mild-moderate ventriculomegaly. Shunted animals developed ataxia and lethargy only when obstruction of the ventricular catheter and/or distal valve occurred. Conclusions. Mechanical induction of acquired hydrocephalus produces a reliable in vivo model that is highly translational, allowing systematic studies of the pathophysiology and clinical treatment of hydrocephalus.