Fourth Ventricle Research Articles

Endoscopic-Assisted Trans-Lateral Ventricular Transchoroidal Fissure Trans-Aqueductal Approach for Evacuation of Severe Intraventricular Hemorrhage

Intraventricular hemorrhage (IVH) is one of the most fatal types of intracerebral hemorrhage (ICH), especially when the third and the fourth ventricles are involved. The use of external ventricular drainage is limited for evacuation of hemorrhage in the lateral ventricles. Endoscopic surgery can provide visualized evacuation of the hemorrhage in the lateral and third ventricles. However, it is usually challenging to access the fourth ventricle using a routine endoscopic approach. We have reported 3 cases of severe IVH with cast fourth ventricles treated using an endoscopic-assisted trans-lateral ventricular transchoroidal fissure trans-aqueductal approach. The average preoperative Graeb score was 11, and the average IVH volume was 75.12 mL. The IVH evacuation rate was 97.5%-100%. The average Glasgow coma scale score had increased to 12 at discharge from 6.6 at admission. At 3 months after surgery, the average modified Rankin scale score was 3. No cerebrospinal fluid shunt had been required and no surgery-related complication had occurred in any patient. Our results have shown that the endoscopic-assisted trans-lateral ventricular transchoroidal fissure trans-aqueductal approach is a feasible and safe endoscopic option that can achieve one-off complete removal of clots in all 4 ventricles in patients with severe IVH.

Genetics of Hydrocephalus (HC)

Genetic hydrocephalus is a neurological condition in which the cerebrospinal fluid (CSF) flows with subsequent and it results in enlargement of the cerebral ventricular cavities. The common cause of the congenital hydrocephalus is the variation in the L1CAM gene, and there is the narrow passage between the third and fourth ventricles. It is suggesting that hydrocephalus is more complicated than the simple CSF , and are many factors which are associated with the genetics of hydrocephalus, the major two factors are the i) telomeres proximity ii) and the more content of Adenine and Thymine [A, T] in the human CH as compared to the other nervous disorders. It is also suggesting that genetics of hydrocephalus is a crucial birth defect, and its genetics is still not completely understood, so it is the most important clinical feature. There are about 43 mutant loci associated with the animals and human hydrocephalus. Among them 9 are associated with animal models and 1 with the human. The most important hydrocephalus gene products are the growth factors, cytokines and many cellular signal pathways in the starting stage of brain development. In this study we will aim to understand the abnormalities in brain cause by the abnormal cellular functioning, and all these cellular events results congenital hydrocephalus. All these studies show that it is the mandolin type of disease with reference to the cellular, molecular genetics, physiological and pathological studies.

Morphometric assessment of the external anatomy of fourth ventricle & dorsal brainstem in South Indian population

Background: Morphometric analysis of ventricular system is helpful in the diagnosis and classification of hydrocephalus and in the assessment and follow-up of enlargement of ventricular system during therapy (ventricular shunts). Present study was aimed to study morphometric assessment of the external anatomy of fourth ventricle & dorsal brainstem. Material and Methods: Present study was descriptive, observational study, conducted in apparently normal brain specimens from human cadavers with known age and sex. The brainstem was separated and the measurements of fourth ventricle & cadaver brainstem were made using millimeter rulers. Results: In present study among 60 fresh adult human cadavers, height & width of fourth ventricle was comparable among male & females, difference was not significant statistically. Various brainstem measurements were comparable among male & females, difference was not significant statistically. Conclusion: Baseline anatomical baseline data helps in interpreting pathological changes, planning surgery, and determining presence and progress of some neurological diseases.

Spatiotemporal characterization of cellular tau pathology in the human locus coeruleus–pericoerulear complex by three-dimensional imaging

Tau pathology of the noradrenergic locus coeruleus (LC) is a hallmark of several age-related neurodegenerative disorders, including Alzheimer’s disease. However, a comprehensive neuropathological examination of the LC is difficult due to its small size and rod-like shape. To investigate the LC cytoarchitecture and tau cytoskeletal pathology in relation to possible propagation patterns of disease-associated tau in an unprecedented large-scale three-dimensional view, we utilized volume immunostaining and optical clearing technology combined with light sheet fluorescence microscopy. We examined AT8+ pathological tau in the LC/pericoerulear region of 20 brains from Braak neurofibrillary tangle (NFT) stage 0–6. We demonstrate an intriguing morphological complexity and heterogeneity of AT8+ cellular structures in the LC, representing various intracellular stages of NFT maturation and their diverse transition forms. We describe novel morphologies of neuronal tau pathology such as AT8+ cells with fine filamentous somatic protrusions or with disintegrating soma. We show that gradual dendritic atrophy is the first morphological sign of the degeneration of tangle-bearing neurons, even preceding axonal lesions. Interestingly, irrespective of the Braak NFT stage, tau pathology is more advanced in the dorsal LC that preferentially projects to vulnerable forebrain regions in Alzheimer’s disease, like the hippocampus or neocortical areas, compared to the ventral LC projecting to the cerebellum and medulla. Moreover, already in the precortical Braak 0 stage, 3D analysis reveals clustering tendency and dendro-dendritic close appositions of AT8+ LC neurons, AT8+ long axons of NFT-bearing cells that join the ascending dorsal noradrenergic bundle after leaving the LC, as well as AT8+ processes of NFT-bearing LC neurons that target the 4th ventricle wall. Our study suggests that the unique cytoarchitecture, comprised of a densely packed and dendritically extensively interconnected neuronal network with long projections, makes the human LC to be an ideal anatomical template for early accumulation and trans-neuronal spreading of hyperphosphorylated tau.

Subarachnoid Haemorrhage: A Systematic Review

Subarachnoid haemorrhage arises from the accumulation of blood between the arachnoid and pia mater resulting from an aneurysmal rupture or traumatic head injury. Subarachnoid haemorrhage is a life-threatening emergency that requires prompt treatment. The characteristic presenting symptom is the thunderclap headache, which patients may describe as the “worst headache of life” and may prompt further imaging. Associated symptoms include nausea, vomiting, and diplopia. Often signs of meningismus are present due to the presence of blood in the fourth ventricle and further down the spinal cord, causing neck and back pain. Findings of focal deficit increase the grade of subarachnoid haemorrhage and patients with a high-grade subarachnoid haemorrhage report to the Emergency room in a state of coma requiring evaluation and urgent treatment, as the coma can be reversible. Sometimes concurrent intraocular haemorrhage, known as Terson syndrome may be present warranting an ophthalmology service. Eighty per cent of patients that develop Terson syndrome require regular follow-up visits but may not require further intervention. This activity reviews the evaluation and management of subarachnoid haemorrhage (SAH).

Craniopharyngiomas Invading the Ventricular System: A Systematic Review.

Craniopharyngiomas involving the ventricular system are rare but pose significant surgical challenges. We systematically reviewed the literature on craniopharyngiomas invading the ventricles (CP-V). PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched to include studies reporting clinical data of patients with CP-Vs. Clinico-radiological features, management, and treatment outcomes were analyzed. We included 73 studies encompassing 407 patients. Patients were mostly male (61.5%), presenting with headache (57.9%) and/or endocrine disorders (52.1%). CP-Vs mostly involved the third ventricle (96.3%), followed by the lateral ventricles (2.9%), and the fourth ventricle (1%). Tumors had cystic components in 59% of cases and were mostly adamantinomatous (70.8%). Open resection was performed in 232 cases (57%), mostly with trans-lamina terminalis (36.6%) and trans-callosal (31.9%) approaches. Endoscopic resection was performed in 169 cases (41.5%), mostly with trans-sphenoidal (74.6%) and transventricular (24.9%) approaches. Gross-total tumor resection was obtained in most cases (62.9%). Adjuvant radiotherapy was delivered in 22.8% cases. A total of 178 patients experienced persistent complications, mostly including diabetes insipidus (47.1%) and panhypopituitarism (12.7%), not significantly different after open versus endoscopic resection (p=0.117). Symptom improvement was obtained in 88% of cases. CP-Vs recurrences were reported in 94 patients (23.1%), with median progression-free survival of 13.5 months (range=0.5-252.0 months). Fifty-nine patients died (14.5%), with median overall survival of 32.0 months (range=0.5-252.0 months), significantly longer after endoscopic resection than open resection (p=0.019). CP-Vs are uncommon and challenging entities. Surgical resection is feasible, but patient-tailored selection of open/endoscopic approaches is necessary to achieve optimal outcomes and minimize complication risks.

Human CSF movement influenced by vascular low frequency oscillations and respiration.

Cerebrospinal fluid (CSF) movement through the pathways within the central nervous system is of high significance for maintaining normal brain health and function. Low frequency hemodynamics and respiration have been shown to drive CSF in humans independently. Here, we hypothesize that CSF movement may be driven simultaneously (and in synchrony) by both mechanisms and study their independent and coupled effects on CSF movement using novel neck fMRI scans. Caudad CSF movement at the fourth ventricle and hemodynamics of the major neck blood vessels (internal carotid arteries and internal jugular veins) was measured from 11 young, healthy volunteers using novel neck fMRI scans with simultaneous measurement of respiration. Two distinct models of CSF movement (1. Low-frequency hemodynamics and 2. Respiration) and possible coupling between them were investigated. We show that the dynamics of brain fluids can be assessed from the neck by studying the interrelationships between major neck blood vessels and the CSF movement in the fourth ventricle. We also demonstrate that there exists a cross-frequency coupling between these two separable mechanisms. The human CSF system can respond to multiple coupled physiological forces at the same time. This information may help inform the pathological mechanisms behind CSF movement-related disorders.

Effects of stereotactic aspiration on brainstem hemorrhage in a case series.

PurposeBrainstem hemorrhage is usually treated conservatively with medication and has high mortality and morbidity rates. Stereotactic aspiration can directly and microinvasively draw out the hemorrhage within a narrow space in the brainstem, thus promoting quick recovery and potentially saving the life of the patient. This study was conducted to investigate the effect of stereotactic aspiration on patients with brainstem hemorrhage in a case series.Materials and methodsA total of 42 patients with brainstem hemorrhage were enrolled for stereotactic aspiration of the brain hemorrhage, and another 30 patients with brainstem hemorrhage were enrolled for conservative treatment. The clinical and imaging data were analyzed and compared.ResultsStereotactic aspiration was successful in all patients (100%), with immediate elimination of hematoma in the brainstem. In five patients with the hemorrhage extending to the fourth ventricle (n = 1) and basal ganglia (n = 4), the hemorrhage was eliminated, resulting in good outcomes. However, four patients died of multiple organ failure after aspiration, resulting in a mortality rate of 9.5%. One week after surgery, the Glasgow Coma Scale (GCS) score ranged from 3 to 11 (mean 5.9 ± 2.3). At 1-month follow-up, 4 patients died, and 36 patients survived, with the GCS score ranging between 3 and 15 (mean 8.6 ± 2.1), which was significantly (P < 0.01) higher than that before surgery. The Modified Rankin Scale (mRS) score was 5 before treatment, 5 (4.4, 6) at 1 week after surgery, and 5 (4, 6) at 1 month. In the conservative group, 16 (53.3%) patients died during hospitalization. The GCS score was 0–6 (mean 2.3 ± 1.1), which was significantly (P < 0.05) worse than at admission or of that in the aspiration group at 1 month. The mRS score at 1 month was 6 (5, 6), which was significantly (P < 0.05) worse than that in the aspiration group.ConclusionStereotactic aspiration for brainstem hemorrhage as an approach of microinvasiveness may be effective in evacuating brainstem hemorrhage and may promote quick recovery of the patient, resulting in better clinical outcomes.

Chiari malformation type I and syringomyelia as keys to understanding CSF circulation (literature review)

Pathophysiology of syringomyelia has not been understood completely. The basic «piston» theory is that the downward movement of the cerebellar tonsils that occurs during the cardiac cycle acts like a «piston» on the partially closed spinal subarachnoid space and results in the formation of a syringomyelitic cavity. However, this still needs to be confirmed by additional experimental data. The main pathogenetic question is how the cerebrospinal fluid enters and remains in the syringomyelic cavity, where the pressure is higher than in the subarachnoid space. Based on the literature data on the pathogenesis of Chiari I malformation with syringomyelia, we modeled and presented our vision of the pathogenesis. Our assumption about the formation of syringomyelia in Chiari I malformation is that there may be a CSF channel between the fourth ventricle and the syrinx. This channel takes on the function of a one-way valve when slightly squeezed and with cyclic movements of the cerebellar tonsils. Decompression of the tonsils turns off the one-way valve, collapsing the syrinx. Perhaps this assumption will serve as a working hypothesis for further study of the pathophysiology of Chiari malformation type I and syringomyelia.

Microsurgical Management of Fourth Ventricle Lesions Via the Median Suboccipital Keyhole Telovelar Approach.

In this 2-year retrospective analysis, 13 patients with fourth ventricle lesions who underwent microsurgical resection via the midline suboccipital keyhole telovelar approach were analyzed. This is the first study to investigate the surgical outcome and complications of using this approach to resect various types of lesions in the fourth ventricle. We aimed to clarify whether this approach has met its promise of lesion dissection. Three patients (23.1%) had intraoperative extraventricular drains. There were no immediate postoperative deaths. Gross total resection was achieved in 84.6% of the cases. The Fisher exact test showed there was no statistically significant correlation between lesion location, lesion size, brainstem invasion, and extent of resection. About two third (69.2%) of the cases were free of complications. New or worsening gait/focal motor disturbance (15.4%) was the most common neurological deficit in the immediate postoperative period. One patient (7.7%) had worse gait disturbance/motor deficit following surgical intervention. Two patients (15.4%) developed meningitis. Two patients (15.4%) required postoperative cerebrospinal fluid diversion after tumor resection, of these 2 patients, 1 (7.7%) eventually needed a permanent shunt. There were no cases of cerebellar mutism and bulbar paralysis. The median suboccipital keyhole telovelar approach provides relative wide access to resect most fourth ventricle tumors completely and with satisfactory results. In contrast, this requires the appropriate patient selection and skilled surgeons.

Severe cerebellar malformations in mutant mice demonstrate a role for PDGF-C/PDGFRα signalling in cerebellar development.

ABSTRACTFormation of the mouse cerebellum is initiated in the embryo and continues for a few weeks after birth. Double-mutant mice lacking platelet-derived growth factor C (PDGF-C) and that are heterozygous for platelet-derived growth factor receptor alpha (Pdgfc-/-; PdgfraGFP/+) develop cerebellar hypoplasia and malformation with loss of cerebellar lobes in the posterior vermis. This phenotype is similar to those observed in Foxc1 mutant mice and in a human neuroimaging pattern called Dandy Walker malformation. Pdgfc-Pdgfra mutant mice also display ependymal denudation in the fourth ventricle and gene expression changes in cerebellar meninges, which coincide with the first visible signs of cerebellar malformation. Here, we show that PDGF-C/PDGFRα signalling is a critical component in the network of molecular and cellular interactions that take place between the developing meninges and neural tissues, and which are required to build a fully functioning cerebellum.

A hidden infection: Racemose neurocysticercosis causing hydrocephalus; a case report

Introduction and importanceNeurocysticercosis (NCC) is the most common helminthic central nervous system infection (CNS) in the Western hemisphere and the most common cause of acquired epilepsy worldwide. Due to its relatively prolonged latent period and clinical similarity to other infectious diseases – including bacterial or viral meningitis and other helminthic infections – NCC may be difficult to diagnose, especially for clinicians who rarely encounter it.Case presentationThis case report discusses a patient with obstructive hydrocephalus and eosinophilic meningitis secondary to racemose NCC. The diagnosis process was initially complicated by the patient's history of pork allergy and absence of radiographic evidence of helminthic CNS infection. Further investigation showed a 4th ventricle multi-cystic lesion causing hydrocephalus which prompted a surgical intervention with a ventriculoperitoneal shunt (VPS) in conjunction with anti-helminthic medical treatment. At 1-year follow-up, the patient has reported recurrence of VPS related complications.Clinical discussionLarval cysts typically deposit within the brain parenchyma, making them easily detected on head computed tomography (CT) scans and leading to neurologic sequelae such as epilepsy. In this case, the absence of CT evidence of NCC and the patient's lifelong history of pork allergy slowed the diagnosis process.ConclusionRacemose NCC is a rare subset of the disease in which cyst clusters occupy the extra parenchymal space, thereby changing the symptomatic profile and making the cysts more difficult to visualize in imaging studies. In this case, magnetic resonance imaging (MRI) was the best imaging modality to diagnosis extra parenchymal NCC and guide its surgical management.

Congenital Hydrocephalus with Aqueductal Stenosis: A Rare Condition Case Report

Congenital Hydrocephalus is due to buildup of excess cerebrospinal fluid in the brain at birth. Fluid buildup leads to neurological and psychological damages to the child. Aqueductal stenosis means a narrowing the passageway between the third and fourth ventricles. In this case report, Ventriculoperitoneal shunt is placed to relieve the pressure, but was removed due to Meningitis. As per review of literature, incidence of shunt failure in infants below 2 years is 5-12% and with more than 60% mortality rate.

Organization of the ventricular zone of the cerebellum.

The roof of the fourth ventricle (4V) is located on the ventral part of the cerebellum, a region with abundant vascularization and cell heterogeneity that includes tanycyte-like cells that define a peculiar glial niche known as ventromedial cord. This cord is composed of a group of biciliated cells that run along the midline, contacting the ventricular lumen and the subventricular zone. Although the complex morphology of the glial cells composing the cord resembles to tanycytes, cells which are known for its proliferative capacity, scarce or non-proliferative activity has been evidenced in this area. The subventricular zone of the cerebellum includes astrocytes, oligodendrocytes, and neurons whose function has not been extensively studied. This review describes to some extent the phenotypic, morphological, and functional characteristics of the cells that integrate the roof of the 4V, primarily from rodent brains.

RADIOLOGICAL EVALUATION OF A RARE CASE OF DANDY-WALKER MALFORMATION IN A 25 YEARS OLD MALE

Dandy-Walker Malformation (DWM) is a rare congenital malformation characterized by hypoplasia of the cerebellar vermis and its upward rotation and cystic enlargement of the fourth ventricle. The clinical manifestations include headache, psychomotor retardation, ataxia, urinary incontinence and hydrocephalus. This is a case of 25 years male presented to general medicine opd in our institute with intermittent episodes of nausea, vomiting, seizures, ataxia for last few years which was further evaluated by CT and MRI of brain in Dept of Radiodiagnosis suggesting diagnosis of Dandy- Walker malformation. patient was referred to Dept of Neurology for exact management.

An indolent presentation of brain abscess due to a slow growing organism

Propioni bacterium propionicum is an anaerobic gram positive bacteria. It’s rarely described as to cause brain abscess. We report the isolation of this organism from a brain abscess and emphasise the problems associated with it’s diagnosis. A 62 year old lady with a history of acromegaly underwent emergency surgery for recurrent pituitary lesion felt to be apoplexy. Four weeks later she presented with headache and confusion. Co morbidities included autoimmune diseases on long term steroids and previous use of immunosuppressants. Clinical examination showed neck stiffness. Brain MRI demonstrated an enhancing lesion in the fourth ventricle causing secondary obstructive hydrocephalus requiring endoscopic third ventriculostomy. Blood tests, CSF analysis and whole body PET scan were unable to differentiate between infection and malignancy. Excision of the lesion was felt very high risk as deep in the tectal area. A sporadic trial of antibiotics, high dose steroids and immunotherapy was tried. Patient developed worsening headache with progression of the lesion. Underwent craniotomy and pus was found which isolated P. propionicum sensitive to penicillin and betalactams. Treated with intravenous meropenem for a year. There was significant reduction in the size of the abscess and hydrocephalus on brain MRI at 8 months post-diagnosis. P. propionicum has been isolated and associated with lesions of the lacrimal glands, lung and abdomen. We could find only two case reports of brain abscess due to P. propionicum. Identification of P.propionicum in the microbiology laboratory can be difficult requiring anaerobic growth conditions and extended cultures. Treatment is prolonged antimicrobial therapy.

Symptomatic care of late-onset Alexander disease presenting with area postrema-like syndrome with prednisolone; a case report

BackgroundAlexander disease (AxD) is classified into AxD type I (infantile) and AxD type II (juvenile and adult form). We aimed to determine the potential genetic cause(s) contributing to the AxD type II manifestations in a 9-year-old male who presented area postrema-like syndrome and his vomiting and weight loss improved after taking prednisolone.Case presentationA normal cognitive 9-year-old boy with persistent nausea, vomiting, and a significant weight loss at the age of 6 years was noticed. He also experienced an episode of status epilepticus with generalized atonic seizures. He showed non-febrile infrequent multifocal motor seizures at the age of 40 days which were treated with phenobarbital. He exhibited normal physical growth and neurologic developmental milestones by the age of six. Occasionally vomiting unrelated to feeding was reported. Upon examination at 9 years, a weak gag reflex, prominent drooling, exaggerated knee-deep tendon reflexes (3+), and nasal tone speech was detected. All gastroenterological, biochemical, and metabolic assessments were normal. Brain magnetic resonance imaging (MRI) revealed bifrontal confluent deep and periventricular white matter signal changes, fine symmetric frontal white matter and bilateral caudate nucleus involvements with garland changes, and a hyperintense tumefactive-like lesion in the brain stem around the floor of the fourth ventricle and area postrema with contrast uptake in post-contrast T1-W images. Latter MRI at the age of 8 years showed enlarged area postrema lesion and bilateral middle cerebellar peduncles and dentate nuclei involvements. Due to clinical and genetic heterogeneities, whole-exome sequencing was performed and the candidate variant was confirmed by Sanger sequencing. A de novo heterozygous mutation, NM_001242376.1:c.262 C > T;R88C in exon 1 of the GFAP (OMIM: 137,780) was verified. Because of persistent vomiting and weight loss of 6.0 kg, prednisolone was prescribed which brought about ceasing vomiting and led to weight gaining of 3.0 kg over the next 3 months after treatment. Occasional attempts to discontinue prednisolone had been resulting in the reappearance of vomiting.ConclusionsThis study broadens the spectrum of symptomatic treatment in leukodystrophies and also shows that R88C mutation may lead to a broad range of phenotypes in AxD type II patients.

Ependymal Cilia: Physiology and Role in Hydrocephalus.

Cerebrospinal fluid (CSF), a colorless liquid that generally circulates from the lateral ventricles to the third and fourth ventricles, provides essential nutrients for brain homeostasis and growth factors during development. As evidenced by an increasing corpus of research, CSF serves a range of important functions. While it is considered that decreased CSF flow is associated to the development of hydrocephalus, it has recently been postulated that motile cilia, which line the apical surfaces of ependymal cells (ECs), play a role in stimulating CSF circulation by cilia beating. Ependymal cilia protrude from ECs, and their synchronous pulsing transports CSF from the lateral ventricle to the third and fourth ventricles, and then to the subarachnoid cavity for absorption. As a result, we postulated that malfunctioning ependymal cilia could disrupt normal CSF flow, raising the risk of hydrocephalus. This review aims to demonstrate the physiological functions of ependymal cilia, as well as how cilia immobility or disorientation causes problems. We also conclude conceivable ways of treatment of hydrocephalus currently for clinical application and provide theoretical support for regimen improvements by investigating the relationship between ependymal cilia and hydrocephalus development.

Curcumin analogue BDDD-721 exhibits more potent anticancer effects than curcumin on medulloblastoma by targeting Shh/Gli1 signaling pathway.

Medulloblastoma (MB) is a malignant tumor in the fourth ventricle of children. The clinical treatment is mainly surgical resection combined with radiotherapy and chemotherapy, but the curative effect is not ideal, and the 3-year survival rate is very low. Previous study confirmed that curcumin attenuated the proliferation of medulloblastoma both in vitro and in vivo. In present study, we found a curcumin analogue named BDDD-721, exhibited more potent anti-tumor activity than curcumin. Compared with curcumin, BDDD-721 more effectively inhibited the proliferation, migration, invasion, and increased apoptosis of medulloblastoma cells. Furthermore, BDDD-721 treatment led to activation of glioma-associated oncogene homolog (Gli), reduced expression of Shh and its downstream target Smo, Gli1 and Ptch1. In addition, SAG (Shh signaling pathway agonist) antagonized the pro-apoptotic effects of BDDD-721 on medulloblastomas as confirmed by CCK8 assays and flow cytometry; while cyclopamine (Shh signaling pathway inhibitor) enhanced its effects on medulloblastomas. In conclusion, these results indicate that curcumin analogue BDDD-721 has more potent anticancer effects than curcumin on medulloblastomas by targeting Shh/Gli1 signaling pathway.

Four-dimensional ultrasound in the diagnosis of intrauterine central nervous system anomalies: four dimensional in CNS congenital anomalies

Background Central nervous system (CNS) anomalies are an important factor of neonatal deaths. Three-dimensional (3D)/4D ultrasound (US) has an advantage over the conventional 2D US as it provides additional multiple options like multiplanar view, tomographic view, and surface rendering view leading to accurate diagnosis of fetal congenital anomalies. Aim The aim of this study was to evaluate the role of the 4D US in the diagnosis of in utero congenital CNS anomalies. Patients and methods The current study was conducted on 30 pregnant women with suspected congenital CNS anomalies by 2D US in Tanta University Hospitals. All the patients were subjected to 2D and 4D US examination in cases with suspected anomalies by 2D US. Results Out of 30 anomalies, two (6.5%) anomalies were lobar holoprosencephaly; six (20%) anomalies were anencephaly, three (10%) anomalies were exencephaly, one (3.5%) anomaly was aqueductal stenosis, three (10%) anomalies were chiari malformation with its two types I and II, and three (10%) anomalies were Dandy–Walker. Three anomalies were hydranencephaly (10%), three (10%) meningocele, one (3.5%) spina bifida, and two cases were obstructive hydrocephalus distal to the 4th ventricle (obstruction of foramen of luschka and magendie) (6.5%). As regards brain anomalies, sensitivity of 4D US=96.4%, specificity of 4D US=100%, positive predictive value=100%, negative predictive value=50.0%, and accuracy=96%. So, 4D US is more superior to 2D US in demonstrating anomalies of the CNS (through multiplanar view and tomographic views), which may affect the postnatal outcome. 4D US offers precise demonstration of normal and abnormal fetal structures through its different modes, so it helps in accurate prenatal diagnosis of CNS anomalies. Conclusion 4D US is considered now an important diagnostic tool in fetal medicine as it provides many advanced diagnostic options such as multiplanar view or tomographic imaging, volume calculation, and surface rendering mode, which aided in early diagnosis of fetal anomalies and improved postnatal outcome.