Abnormal Accumulation Of Cerebrospinal Fluid Research Articles

Falciform technique in laparoscopic revision of distal catheter obstruction: an evaluation

Background: Ventriculoperitoneal (VP) shunting is a cornerstone treatment for hydrocephalus, a condition characterized by the abnormal accumulation of cerebrospinal fluid in the ventricles of the brain. Despite its efficacy, this procedure is associated with various complications, among which distal catheter obstruction poses significant challenges. This study aimed to evaluate the effectiveness of the ‘falciform technique’ in laparoscopic revision of distal catheter obstructions, offering a novel approach to mitigate this prevalent issue. Materials and methods: This study retrospectively analyzed 28 patients with VP shunt distal catheter obstructions who underwent laparoscopy-assisted shunt revision between January 2016 and June 2022. All of these were done using the ‘falciform technique’ with the fixation of the distal catheter to the falciform ligament in supra-hepatic space. Results: The most common etiology of primary shunt surgery was hydrocephalus, followed by intracranial hemorrhage (42.9%) and traumatic brain injury (32.1%). Normal pressure hydrocephalus occurs in 14.3% of cases. Fifteen patients (53.6%) required revision surgery within 1 year of index surgery. Thirteen patients (46.4%) underwent revision surgery more than 1 year after the index surgery, either as a first revision or subsequent revision. The average surgery time was 32.1±14.7 min and hospital stay was 4.2±1.8 days. After a mean follow-up period of at 20.3±8.7 months, except for three patients who died from other causes (two patients due to pneumonia and one due to exhaustion), there were no shunt-related complications in the remaining 25 patients. Conclusions: Laparoscopy with the application of ‘falciform technique’ is a safe and highly effective method in distal catheter obstruction revision following VP shunt.

Piezo1 regulates meningeal lymphatic vessel drainage and alleviates excessive CSF accumulation

Piezo1 regulates multiple aspects of the vascular system by converting mechanical signals generated by fluid flow into biological processes. Here, we find that Piezo1 is necessary for the proper development and function of meningeal lymphatic vessels and that activating Piezo1 through transgenic overexpression or treatment with the chemical agonist Yoda1 is sufficient to increase cerebrospinal fluid (CSF) outflow by improving lymphatic absorption and transport. The abnormal accumulation of CSF, which often leads to hydrocephalus and ventriculomegaly, currently lacks effective treatments. We discovered that meningeal lymphatics in mouse models of Down syndrome were incompletely developed and abnormally formed. Selective overexpression of Piezo1 in lymphatics or systemic administration of Yoda1 in mice with hydrocephalus or Down syndrome resulted in a notable decrease in pathological CSF accumulation, ventricular enlargement and other associated disease symptoms. Together, our study highlights the importance of Piezo1-mediated lymphatic mechanotransduction in maintaining brain fluid drainage and identifies Piezo1 as a promising therapeutic target for treating excessive CSF accumulation and ventricular enlargement.

Cerebral microvascular markers of intracranial pressure in acute and subacute porcine hydrocephalus model

Hydrocephalus affects 1.1% of infants, resulting in abnormal accumulation of cerebrospinal fluid and elevated intracranial pressure (ICP). Elevated ICP can occur prior to detectable morphological changes using computed tomography, resulting in delayed diagnosis and treatment. To improve early diagnosis, we propose vascular imaging using ultrasound localization microscopy (ULM) and ultrafast Doppler to measure cerebral blood flow changes due to acute to subacute elevations in ICP. We performed a pre-clinical study on a neonatal porcine model with 30 min, 8 h, 12 h, and 24 h ICP elevations while performing brain ultrasound measurements in hourly intervals. Ultrafast plane-wave ultrasound imaging was implemented using the Verasonics Vantage-256 and GE IC59D curved array probe at 400Hz frame rate. Lumason contrast agent was infused into each animal, providing tracers for ULM and enhancing the ultrafast Doppler imaging. Based on preliminary analysis, ultrafast Doppler showed reduced total cerebral blood volume after acute ICP elevation. ULM showed highly correlated parabolic reductions in cortical microcirculation with increasing ICP. Drastic reductions in cerebral microcirculation but less so in microcirculation accompanied microdialysis evidence of brain ischemia. Additionally, there were spatiotemporal changes in cerebral microvascular flow with varying ICP durations that warrant additional investigations using ultrafast ultrasound imaging.

An Overview of Hydrocephalus and Shunts Used in The Clinical Management of Hydrocephalus

Hydrocephalus is a complex neurological condition characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain, leading to increased intracranial pressure. The clinical management of hydrocephalus often involves the surgical placement of a shunt system to divert CSF and restore normal fluid dynamics. This article provides a comprehensive overview of hydrocephalus, its etiology, clinical presentation, and various types of shunts used in its management. The outline of this report encompasses the principles of shunt surgery, indications for shunt placement and primary types of shunts used in the clinical management of hydrocephalus, including ventriculoperitoneal (VP), ventriculoatrial (VA), ventriculopleural (VPL), lumboperitoneal (LP) and ventriculo-subgaleal (VS) shunts. Additionally, some non-conventional shunts such as ventriculo-osseous, ventriculo renal, ventriculo gall bladder, intraventricular (Aqueductal stents) and long-tunnelled external ventricular drains are also discussed. The distinct features of each shunt type, its associated advantages, limitations and complications are also outlined together with a comparison between pressure vs flow regulated valves. An understanding of the different shunt types and their specific characteristics is essential for clinicians to make informed decisions in tailoring treatment to individual patients. This review offers a comprehensive understanding of shunt characteristics which we believe will empower clinicians to make informed decisions tailored to individual patients, ensuring effective CSF diversion and long-term management of hydrocephalus.

Evaluation of Overshunting between Low and Medium Pressure Ventriculoperitoneal Shunts in Dogs with Severe Hydrocephalus Using Frameless Stereotactic Ventricular Shunt Placement

Hydrocephalus is a neurological disorder characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricular system of the brain, leading to cerebral ventricular dilation, brain parenchyma compression, and neuronal cell loss. Surgery is an effective method of draining excessive amounts of CSF. Ventriculoperitoneal shunt (VPS) allows excess CSF to divert into the abdomen; this device is the most commonly used in the treatment of hydrocephalus both in veterinary and human patients. This study aims to describe the application of two types of VPS, low-pressure valve and medium-pressure valve, using a frameless stereotactic neuronavigational system in eight severe hydrocephalus in dogs and, in particular, analyze the prevalence of postoperative overshunting. Non-communicating hydrocephalus was found in seven dogs, whereas the rest of them had communicating hydrocephalus caused by traumatic brain injury with a skull fracture. The criteria for pressure valve selection depended on the intraoperative intraventricular pressure (IVP) that was determined by the adaptive manometer, according to the human protocol. Low-pressure valve placement was performed in five dogs, and the others received medium-pressure valve placement. The follow-up period was 2 weeks, 4–12 weeks, and 12 weeks to 12 months. Pre- and postoperative information including neurological signs, CT-Scan or MRI, medical treatment, complications, and ventricular volume were compared in all dogs. Seven dogs showed neurological improvement within 2 weeks after surgery. Overshunting was seen in four dogs who received low-pressure valve placement. Three of them had shunt infections within 4 to 6 weeks after surgery. One dog underwent shunt revision from a low-pressure valve to a medium-pressure valve caused by severe overshunting and progressive neurological signs. In addition, cognitive and learning improvements were evaluated based on the owners’ feedback, and neurological signs were examined during the follow-up period in two dogs that received low-pressure valve placement. We conclude that a medium-pressure valve is recommended for overshunting prevention. However, low-pressure valve placement seems to improve cognitive function and learning ability, which is related to an increase in the brain parenchyma observed during long-term monitoring. Moreover, we also report our experience and surgical procedure for frameless stereotactic ventricular shunt placement (FSVSP) in VPS surgery in dogs affected by hydrocephalus.

Biomechanical effects of hyper-dynamic cerebrospinal fluid flow through the cerebral aqueduct in idiopathic normal pressure hydrocephalus patients

Normal pressure hydrocephalus (NPH) is an intracranial disease characterized by an abnormal accumulation of cerebrospinal fluid (CSF) in brain ventricles within the normal range of intracranial pressure. Most NPH in aged patients is idiopathic (iNPH) and without any prior history of intracranial diseases. Although an abnormal increase of CSF stroke volume (hyper-dynamic CSF flow) in the aqueduct between the third and fourth ventricles has received much attention as a clinical evaluation index in iNPH patients, the biomechanical effects of this flow on iNPH pathophysiology are poorly understood. This study aimed to clarify the potential biomechanical effects of hyper-dynamic CSF flow through the aqueduct of iNPH patients using magnetic resonance imaging-based computational simulations. Ventricular geometries and CSF flow rates through aqueducts of 10 iNPH patients and 10 healthy control subjects were obtained from multimodal magnetic resonance images, and these CSF flow fields were simulated using computational fluid dynamics. As biomechanical factors, we evaluated wall shear stress on the ventricular wall and the extent of flow mixing, which potentially disturbs the CSF composition in each ventricle. The results showed that the relatively high CSF flow rate and large and irregular shapes of the aqueduct in iNPH resulted in large wall shear stresses localized in relatively narrow regions. Furthermore, the resulting CSF flow showed a stable cyclic motion in control subjects, whereas strong mixing during transport through the aqueduct was found in patients with iNPH. These findings provide further insights into the clinical and biomechanical correlates of NPH pathophysiology.

Normal Pressure Hydrocephalus: Clinical Approach and Practical Considerations

Normal pressure hydrocephalus is defined as an abnormal cerebrospinal fluid accumulation in the ventricles. The condition is more commonly seen in the elderly population. It is present in more than 700,000 Americans between the ages of 60 - 70, but less than 20% receive an accurate diagnosis. It may be idiopathic without a known cause or the result of any circumstance that interferes with the normal cerebral spinal fluid flow (i.e., subarachnoid hemorrhage, head trauma, infection, tumor, or complications from surgery). Clinical manifestations include the presence of cognitive impairment, gait disturbance, and urinary incontinence. The diagnosis of normal pressure hydrocephalus is complex since it may present similar symptoms to those associated with other neurological conditions, particularly dementia syndromes. Therefore, a combination of classic clinical presentation, brain imaging findings, and dynamic evaluation of cerebrospinal fluid is necessary to identify this disorder. Depending on the patient’s characteristics, medical/conservative treatment or ventriculoperitoneal surgical shunting can be effective management methods. On an individual basis, the prognosis of the disease and the outcome of treatment may vary.

Modulation of striatal glutamatergic, dopaminergic and cholinergic neurotransmission pathways concomitant with motor disturbance in rats with kaolin-induced hydrocephalus

BackgroundHydrocephalus is characterized by abnormal accumulation of cerebrospinal fluid in the cerebral ventricles and causes motor impairments. The mechanisms underlying the motor changes remain elusive. Enlargement of ventricles compresses the striatum of the basal ganglia, a group of nuclei involved in the subcortical motor circuit. Here, we used a kaolin-injection juvenile rat model to explore the effects of acute and chronic hydrocephalus, 1 and 5 weeks post-treatment, respectively on the three major neurotransmission pathways (glutamatergic, dopaminergic and cholinergic) in the striatum.MethodsRats were evaluated for motor impairments. Expressions of presynaptic and postsynaptic protein markers related to the glutamatergic, dopaminergic, and cholinergic connections in the striatum were evaluated. Combined intracellular dye injection and substance P immunohistochemistry were used to distinguish between direct and indirect pathway striatal medium spiny neurons (d and i-MSNs) for the analysis of their dendritic spine density changes.ResultsHydrocephalic rats showed compromised open-field gait behavior. However, male but not female rats displayed stereotypic movements and compromised rotarod performance. Morphologically, the increase in lateral ventricle sizes was greater in the chronic than acute hydrocephalus conditions. Biochemically, hydrocephalic rats had significantly decreased striatal levels of synaptophysin, vesicular glutamate transporter 1, and glutamatergic postsynaptic density protein 95, suggesting a reduction of corticostriatal excitation. The expression of GluR2/3 was also reduced suggesting glutamate receptor compositional changes. The densities of dendritic spines, morphological correlates of excitatory synaptic foci, on both d and i-MSNs were also reduced. Hydrocephalus altered type 1 (DR1) and 2 (DR2) dopamine receptor expressions without affecting tyrosine hydroxylase level. DR1 was decreased in acute and chronic hydrocephalus, while DR2 only started to decrease later during chronic hydrocephalus. Since dopamine excites d-MSNs through DR1 and inhibits i-MSNs via DR2, our findings suggest that hydrocephalus downregulated the direct basal ganglia neural pathway persistently and disinhibited the indirect pathway late during chronic hydrocephalus. Hydrocephalus also persistently reduced the striatal choline acetyltransferase level, suggesting a reduction of cholinergic modulation.ConclusionsHydrocephalus altered striatal glutamatergic, dopaminergic, and cholinergic neurotransmission pathways and tipped the balance between the direct and indirect basal ganglia circuits, which could have contributed to the motor impairments in hydrocephalus.

Delayed cerebrospinal fluid ascites following ventriculoperitoneal shunt

Background: Cerebrospinal fluid (CSF) ascites is an abnormal accumulation of CSF within the peritoneal cavity caused by the peritoneum's inability to absorb the CSF, following a ventriculoperitoneal (VP) shunt surgery. Excessive CSF production (e.g, choroid plexus papilloma and choroid plexus villous hypertrophy), high CSF protein secondary to chronic infection (e.g. tuberculosis), and brain tumours (e.g, optic gliomas and craniopharyngiomas) have all been suggested as contributing factors to the formation of CSF ascites. Peritoneal inflammation as a result of several shunt revisions or some non-specific inflammatory reaction to shunt material has also been explored.
 Case Presentation: A 3-year-old girl with lumbar myelomeningocele and delayed CSF ascites following VP shunt is reported. Therapeutic paracentesis was employed to relieve abdominal distension, although recurring accumulation was common. The VP shunt was removed and instead of a Ventriculo-atrial shunt, she underwent Endoscopic Third Ventriculostomy (ETV). CSF ascites gradually disappeared after ETV over a two-week period.
 Conclusions: Abdominal paracentesis to relieve ascites and conversion of a Ventriculoperitoneal shunt to a Ventriculo-atrial shunt are commonly used to treat CSF ascites, however Endoscopic Third Ventriculostomy, where feasible, is another alternative treatment that can be performed to treat this condition.

Reactive microglia and mitochondrial unfolded protein response following ventriculomegaly and behavior defects in kaolin-induced hydrocephalus

Ventriculomegaly induced by the abnormal accumulation of cerebrospinal fluid (CSF) leads to hydrocephalus, which is accompanied by neuroinflammation and mitochondrial oxidative stress. The mitochondrial stress activates mitochondrial unfolded protein response (UPRmt), which is essential for mitochondrial protein homeostasis. However, the association of inflammatory response and UPRmt in the pathogenesis of hydrocephalus is still unclear. To assess their relevance in the pathogenesis of hydrocephalus, we established a kaolin-induced hydrocephalus model in 8-week-old male C57BL/6J mice and evaluated it over time. We found that kaolin-injected mice showed prominent ventricular dilation, motor behavior defects at the 3-day, followed by the activation of microglia and UPRmt in the motor cortex at the 5-day. In addition, PARP-1/NF-κB signaling and apoptotic cell death appeared at the 5-day. Taken together, our findings demonstrate that activation of microglia and UPRmt occurs after hydrocephalic ventricular expansion and behavioral abnormal-ities which could be lead to apoptotic neuronal cell death, providing a new perspective on the pathogenic mechanism of hydrocephalus.

Cerebral microcirculation mapped by echo particle tracking velocimetry quantifies the intracranial pressure and detects ischemia

Affecting 1.1‰ of infants, hydrocephalus involves abnormal accumulation of cerebrospinal fluid, resulting in elevated intracranial pressure (ICP). It is the leading cause for brain surgery in newborns, often causing long-term neurologic disabilities or even death. Since conventional invasive ICP monitoring is risky, early neurosurgical interventions could benefit from noninvasive techniques. Here we use clinical contrast-enhanced ultrasound (CEUS) imaging and intravascular microbubble tracking algorithms to map the cerebral blood flow in hydrocephalic pediatric porcine models. Regional microvascular perfusions are quantified by the cerebral microcirculation (CMC) parameter, which accounts for the concentration of micro-vessels and flow velocity in them. Combining CMC with hemodynamic parameters yields functional relationships between cortical micro-perfusion and ICP, with correlation coefficients exceeding 0.85. For cerebral ischemia cases, the nondimensionalized cortical micro-perfusion decreases by an order of magnitude when ICP exceeds 50% of the MAP. These findings suggest that CEUS-based CMC measurement is a plausible noninvasive method for assessing the ICP and detecting ischemia.

Role of anaesthesiologist in the management of an infant with hydrocephalus for shunt surgery

Hydrocephalus is a disorder of abnormal accumulation of cerebrospinal fluid (CSF) in the ventricular system. Accumulation of CSF occurs due to an imbalance between CSF production and absorption. In the new-born and infants, hydrocephalus almost entirely presents as an enlarged head resulting from the separation of cranial bone plates, so any new-born or infant with an enlarged head should undergo evaluation. We report the case of a gross hydrocephalus in a 9-month-old infant (head circumference 55 cm) posted for ventriculoperitoneal shunt surgery with anaesthetic consideration of difficult intubation because of such a large head size, positioning, age-related pathophysiology and temperature regulation. The case was managed successfully with an uneventful hospital stay, and a significant decrease in head size was noted.

Full Mouth Rehabilitation of Hydrocephalus Patient ? A Case Report

Dentist’s frequently encounter patients who are medically, physically or mentally compromised. One such medical condition is ‘hydrocephalus’. Hydrocephalus is basically a neurological disorder caused by excessive intracranial pressure due to abnormal accumulation of cerebrospinal fluid within the ventricles or subarachnoid space of brain and raised intracranial pressure. Ventriculo-Peritoneal Shunt (VPS) is the most commonly used treatment though ventriculo-atrial shunt is also another treatment option. To maintain the intracranial pressure within normal range, the patients are kept on medications which make them more prone for dental malformations and caries. Because of the poor oral hygiene, these patients require a regular dental care. This report discusses the dental management process employed for a five years old patient having hydrocephalus.

A Multimodal Treatment Approach on Rare Case of Meningomyelocele

Meningomyelocele, one of the neural tube defect (NTD) variants, is a condition in which meninges and neural tissue protrudes through a defect in vertebral arch and forms a cyst-like sac with an incidence of 0.5–11/1000 live births. Hydrocephalus is a major problem for majority of patients with meningomyelocele due to the abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain. Meticulous preoperative evaluation with planning of the anesthetic technique, especially in regard to managing the airway, plays a crucial role in successful surgical management of these cases. Here, we report a case of lumbosacral meningomyelocele with hydrocephalus underwent a multimodal treatment approach for providing a comprehensive plan of care.

Multimodal Sensing Capabilities for the Detection of Shunt Failure.

Hydrocephalus is a medical condition characterized by the abnormal accumulation of cerebrospinal fluid (CSF) within the cavities of the brain called ventricles. It frequently follows pediatric and adult congenital malformations, stroke, meningitis, aneurysmal rupture, brain tumors, and traumatic brain injury. CSF diversion devices, or shunts, have become the primary therapy for hydrocephalus treatment for nearly 60 years. However, routine treatment complications associated with a shunt device are infection, obstruction, and over drainage. Although some (regrettably, the minority) patients with shunts can go for years without complications, even those lucky few may potentially experience one shunt malfunction; a shunt complication can require emergency intervention. Here, we present a soft, wireless device that monitors distal terminal fluid flow and transmits measurements to a smartphone via a low-power Bluetooth communication when requested. The proposed multimodal sensing device enabled by flow sensors, for measurements of flow rate and electrodes for measurements of resistance in a fluidic chamber, allows precision measurement of CSF flow rate over a long time and under any circumstances caused by unexpected or abnormal events. A universal design compatible with any modern commercial spinal fluid shunt system would enable the widespread use of this technology.

Choroid plexus NKCC1 mediates cerebrospinal fluid clearance during mouse early postnatal development

Cerebrospinal fluid (CSF) provides vital support for the brain. Abnormal CSF accumulation, such as hydrocephalus, can negatively affect perinatal neurodevelopment. The mechanisms regulating CSF clearance during the postnatal critical period are unclear. Here, we show that CSF K+, accompanied by water, is cleared through the choroid plexus (ChP) during mouse early postnatal development. We report that, at this developmental stage, the ChP showed increased ATP production and increased expression of ATP-dependent K+ transporters, particularly the Na+, K+, Cl−, and water cotransporter NKCC1. Overexpression of NKCC1 in the ChP resulted in increased CSF K+ clearance, increased cerebral compliance, and reduced circulating CSF in the brain without changes in intracranial pressure in mice. Moreover, ChP-specific NKCC1 overexpression in an obstructive hydrocephalus mouse model resulted in reduced ventriculomegaly. Collectively, our results implicate NKCC1 in regulating CSF K+ clearance through the ChP in the critical period during postnatal neurodevelopment in mice.

Managing Chronic Health Needs in Child Care and Schools—Hydrocephalus and Shunts

Patient Education| 2021 Managing Chronic Health Needs in Child Care and Schools—Hydrocephalus and Shunts Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Share Icon Share Facebook Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Managing Chronic Health Needs in Child Care and Schools—Hydrocephalus and Shunts. Pediatric Patient Education 2021; 10.1542/ppe_document025 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All AAP SitesAll PublicationsPediatric Patient Education Search Advanced Search Subjects: Managing Chronic Health Needs in Child Care Hydrocephalus is the abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain. Hydrocephalus can be caused by a blockage of CSF flow (obstructive hydrocephalus) or failure to absorb CSF (communicating hydrocephalus). Hydrocephalus can be present at birth (congenital hydrocephalus) or develop later in life (acquired hydrocephalus). There are numerous causes of hydrocephalus, the most common being hydrocephalus associated with myelomeningocele and post- hemorrhagic hydrocephalus as a complication of preterm birth. One treatment of hydrocephalus is the placement of a shunt, which is a flexible tube, to carry the extra CSF from the ventricle of the brain to another area of the body. The most common distal site for a shunt is the peritoneal cavity in the abdomen. These shunts are ventriculoperitoneal shunts. A successful shunt relieves pressure on the brain, controlling the rate of head growth and maximizing... © 2019 American Academy of Pediatrics. All rights reserved. You do not currently have access to this content.

Full-mouth rehabilitation of a hydrocephalus patient

Dentists frequently encounter patients who are either medically, physically, or mentally compromised. One such medically compromised condition is “hydrocephalus.” Hydrocephalus is basically a neurological disorder caused by excessive intracranial pressure due to abnormal accumulation of cerebrospinal fluid within the ventricles or subarachnoid space of the brain and raised intracranial pressure. Ventriculoperitoneal shunt (VPS) is the most commonly used treatment, but ventriculoatrial shunt is another treatment option. To keep the intracranial pressure within normal range, the patients are kept on medications which make them more prone to dental caries. Because of poor oral hygiene, these patients require a regular dental care. This report discusses the dental management process employed for a 5-year-old patient with VPS-treated hydrocephalus.

An Abnormal Accumulation of Cerebrospinal Fluid in the Cavities Deep Within the Brain

An Abnormal Accumulation of Cerebrospinal Fluid in the Cavities Deep Within the Brain

Fetal brain damage in congenital hydrocephalus.

Congenital hydrocephalus (HCP) is a developmental brain disorder characterized by the abnormal accumulation of cerebrospinal fluid within the ventricles. It is caused by genetic and acquired factors that start during early embryogenesis with disruption of the neurogerminal areas. As might be expected, early-onset hydrocephalus alters the process of brain development leading to irreparable neurological deficit. A primary alteration of the ependyma/neural stem cells (affecting vesicle trafficking and abnormal cell junctions) leads to its loss or denudation and translocation of neural progenitor cells (NPCs) and neural stem cells (NSCs) into the cerebrospinal fluid (CSF). Under these abnormal conditions, morphological and functional processes, underlying the concept of astroglial reaction, are initiated in an attempt to recover homeostasis in the periventricular zone. This astroglial reaction includes astrocyte hypertrophy, hyperplasia, and development of a new layer with reorganized functional features that resemble the ependyma. Despite decades of research, there is a lack of information concerning the biological basis of the brain abnormalities that are associated with HCP. The present review of current literature discusses the neuropathological changes during gestation following the onset of congenital hydrocephalus and the unanswered questions into the pathophysiology of the disease. A better understanding of those missing points might help create novel therapeutic strategies that can reverse or even prevent the ultimate neurological impairment that affects this population and improve long-term clinical outcome.