Enlarged Virchow-Robin Spaces Research Articles

Variations and natural history of primary intraparenchymal lesions associated with neurofibromatosis type 2.

The study aimed to investigate the clinical implications and natural history of primary intraparenchymal lesions in patients with neurofibromatosis type 2. Radiological findings of 15 neurofibromatosis type 2 cases were retrospectively collected. Twenty-seven primary intraparenchymal lesions were observed in 7 out of 15 patients (47%). Cortical/subcortical T2 hyperintense lesions and enlarged Virchow-Robin spaces were the most common findings in five and four patients, respectively. During the follow-up period (median 84months), one new primary intraparenchymal lesion was identified and increased lesions were observed in two cases on contrast-enhanced MRI. Surgical resection was performed in one case pathologically diagnosed with atypical meningioma. Twenty-five other lesions without contrast enhancement presented no apparent growth during follow-up. Although most primary intraparenchymal lesions are benign, a subset of cases would present newly developed or increased lesions on contrast-enhanced MRI. Careful monitoring is necessary for such cases, and pathological confirmation should be considered.

Chronic kidney disease correlates with MRI findings of cerebral small vessel disease

Objective: Cerebral small vessel disease (CSVD) and chronic kidney disease (CKD) may be part of a multisystem small-vessel disorder. Since the kidney and brain share unique susceptibilities to vascular injury, kidney impairment may be predictive of the presence and severity of CSVD. This study explored the relationship between CSVD and CKD. Methods: Between December 2015 and December 2017 (follow-up 10–20 months) 52 patients with chronic nephritis and CKD were classified into a progressive group (n = 17) and stable group (n = 35). Age, gender, hypertension, diabetes and smoking were matched between groups. CSVD features of both groups, including enlarged Virchow-Robin spaces (VRS), white matter lesions (WML), lacunar infarcts (LI), and cerebral microbleeds (CMB) were evaluated by magnetic resonance (MR) imaging. Results: WML and CMB in the progressive group were exacerbated at follow-up compared to initial exam (p = 0.004 and 0.041, respectively). There was no significant change in VRS, WML, LI, or CMB in the stable group at follow-up compared to initial exam. CMB were significantly different between the progressive group and stable group at follow-up.etimtaed Glomerular filtration rate (eGFR) was significantly correlated with VRS, WML, and CMB at follow-up (p = 0.037, 0.041, and 0.009, respectively). Conclusions: Patients with progressive CKD have a higher prevalence and severity of CSVD, which correlates with deterioration of renal function as assessed by decreased eGFR. Thus EGFR may also be of value in the prediction of cerebral small vessel disease.

Home-measured orthostatic hypotension associated with cerebral small vessel disease in a community-based older population.

This study aimed to investigate the relationship between cerebral small vessel disease (CSVD) and orthostatic hypotension (OH) using self-measured blood pressure at home in community-dwelling older subjects. Between May 2016 and October 2018, 663 community-dwelling adults aged ≥60 years were enrolled in Shandong, China. CSVD, including white matter hyperintensities (WMHs), lacunes, enlarged Virchow-Robin spaces (EVRS) and microbleeds, was assessed using brain magnetic resonance imaging. After receiving appropriate training, the subjects participated in "home-measured (H)OH" by themselves for three consecutive days. Participants were classified into no-HOH, 1 HOH, and ≥2 HOH episode groups according to the presence of HOH episodes. The WMH volume, WMH-to-total intracranial volume (TIV) ratio, total numbers of lacunes and EVRS, and prevalence of Fazekas scale score ≥2, lacunes, and EVRS were elevated in the 1 and ≥2 HOH episode groups compared with the no-HOH episode group (P < 0.05). The prevalence and total number of microbleeds were significantly higher in the ≥2 HOH episodes group than in the no-HOH and 1 HOH episode groups (P < 0.05). HOH episodes were significantly associated with WMH volume, WMH-to-TIV ratio, and the total numbers of lacunes, EVRS, and microbleeds after adjustment for confounders (P < 0.05). The risks of Fazekas scale score ≥2, lacunes, EVRS, and microbleeds were 2.123-, 1.893-, 2.162-, and 1.656-fold higher in the 1 HOH episode group and 4.910-, 5.359-, 3.048-, and 2.418-fold higher in the ≥2 HOH episodes group, respectively, than those in the no-HOH group. The presence of HOH episodes was an independent risk factor for CSVD in the community-based older population.

Neuroimaging consequences of cerebral small vessel disease in patients with obstructive sleep apnea–hypopnea syndrome

ObjectiveThis study aimed to investigate the association between severity of obstructive sleep apnea–hypopnea syndrome (OSAHS) and the neuroimaging consequences of cerebral small vessel disease (SVD).MethodsPatients with OSAHS and age‐ and gender‐matched healthy control subjects completed the mini‐mental state examination and underwent an evoked‐related potential study and overnight polysomnographic monitoring. Magnetic resonance imaging (MRI) was performed to detect markers of silent cerebral SVD, including Virchow–Robin spaces (VRS) rated on a five‐point scale, white matter lesions, lacunar infarcts, and deep microbleeds. Multinomial logistic regression models were used to examine the associations of the apnea–hypopnea index (AHI) and arousal index (AI) values, mean oxyhemoglobin saturation, the duration of snoring history, and MRI markers of small vessel disease with the incidence of enlarged VRS.ResultsThe study included 72 patients with severe OSAHS and 53 volunteers without OSAHS. The duration of snoring history ranged from 5 to 22 years in the OSAHS group. Smaller P3 amplitudes at Cz were found in OSAHS patients than control subjects (p < .05), which is associated with neurocognitive impairment. Enlarged VRS were more prevalent in the basal ganglia and centrum semiovale of patients with OSAHS than in the control group. No significant between‐group differences were observed in the number of white matter lesions, lacunar infarcts, and deep microbleeds. Enlarged VRS were positively correlated with AHI and AI values in the OSAHS group (r = .63, p < .001; r = .55, p < .001, respectively).ConclusionsSilent cerebral SVD was more prevalent in patients with OSAHS than in the controls. Enlarged VRS observed in the basal ganglia and centrum semiovale were positively correlated with severity of OSAHS, which may contribute to cognitive impairment.

Enlarged Virchow Robin spaces associate with cognitive decline in multiple sclerosis.

The clinical significance of Virchow Robin spaces (VRS) in inflammatory brain disorders, especially in multiple sclerosis (MS), is still undefined. We analysed enlarged VRS (eVRS) by means of phase sensitive inversion recovery (PSIR) MRI sequence and investigated their association with inflammation or brain atrophy, and to clinical or physical disability. Forty-three MS patients (21 clinically isolated syndrome suggestive of MS [CIS], 15 RRMS, 7 progressive [PMS]) and 10 healthy controls (HC) were studied. 3DT1, 3DFLAIR and 2DPSIR images were obtained with a 3T MRI scanner. eVRS number and volume were calculated by manual segmentation (ITK-SNAP). Freesurfer was used to assess brain parenchymal fraction (BPF). All patients underwent clinical (EDSS) and cognitive (Rao’s BRB and DKEFS) evaluation.eVRS number and volume resulted significantly higher on 2D-PSIR compared to both 3D-T1 (p<0.001) and 3D-FLAIR (p<0.001) and were significantly increased in CIS compared to HC (p<0.05), in PMS and RRMS compared to CIS (p<0.001) and in male versus female patients (p<0.05). eVRS volume increased significantly with disease duration (r = 0.6) but did not correlate with EDSS. eVRS significantly correlated with SPARTd (r = -0.47) and DKEFSfs (r = -0.46), especially when RRMS and PMS were merged in a single group (r = 0.89, p = 0.002 and r = 0.66, p = 0.009 respectively), while no correlation was found with BPF (r = 0.3), gadolinium-enhancing lesions (r = 0.2) and WMT2 lesion volume (r = 0.2). 2DPSIR allowed the detection of an impressive higher number of eVRS compared to 3DT1 and 3DFLAIR. eVRS associate with SPARTd and DKEFSfs failure in relapse-onset MS, suggesting they may contribute to cognitive decline in MS.

Enlarged Virchow-Robin spaces in a young man: a constrained spherical deconvolution tractography study

Background and aim:Virchow-Robin spaces are mainly located along the path of the lenticulostriate arteries in the basal ganglia through the anterior perforate substance, and can be found both in normal subjects, as a rare phenomenon, and in patients with different diseases. We report a case of a healthy young man with unilateral enlarged Virchow-Robin spaces in the left capsule-lenticulostriate area. Aim of this case report is to show the potential of probabilistic Constrained Spherical Deconvolution (CSD) tractography in showing abnormal diffusion tensor imaging and tractography of the anterior thalamic tracts caused by mass effect from adjacent enlarged Virchow-Robin spaces.Methods:The study was performed with a 3T magnetic resonance imaging (MRI) scanner (Achieva, Philips Healthcare, Best, Netherlands); equipped with a 32-channel SENSE head coil. Diffusion Weighted Images were analyzed by using CSD, a fast computation method that overcomes major limitations of Diffusion Tensor Imaging allowing reliable estimation of one or more fiber orientations in the presence of intravoxel orientational heterogeneity.Results:Tractography showed increased Fractional Anisotropy and reduced Apparent Diffusion Coefficient values, a displacement and compression of the anterior thalamic projections by part of the enlarged VRS, and a decrease of white matter fibers in the left side in comparison to the right one.Conclusions:We report on a case of a healthy individual with unilateral dilated VRS in the capsulo-lenticulostriatal area, proving the utility of diffusion MRI and tractography in understanding the abnormal neuroanatomy of this particular condition. (www.actabiomedica.it)

Imaging in Myotonic Dystrophy Type 1 – Case Reports

Myotonic dystrophy type 1 (DM1) is the most common of the muscular dystrophies. It is an autosomal dominant neuromuscular disorder with multisystem involvement, including the central nervous system. Two DNA-proven cases are presented. Patients reported are siblings showing features of DM1 on magnetic resonance imaging (MRI). These features include T2 and FLAIR hyperintensities in the periventricular, deep, and subcortical white matter, with frequent involvement of the anterior temporal lobe. Other features include general brain atrophy and enlarged Virchow-Robin spaces. Subcortical white matter lesions anterior in the temporal lobe are the most specific imaging finding, and a short differential diagnosis is discussed.

Prefrontal white matter pathology in air pollution exposed Mexico City young urbanites and their potential impact on neurovascular unit dysfunction and the development of Alzheimer's disease

Millions of urban children are chronically exposed to high concentrations of air pollutants, i.e., fine particulate matter (PM2.5) and ozone, associated with increased risk for Alzheimer's disease. Compared with children living with clear air those in Mexico City (MC) exhibit systemic, brain and intrathecal inflammation, low CSF Aβ42, breakdown of the BBB, attention and short-term memory deficits, prefrontal white matter hyperintensities, damage to epithelial and endothelial barriers, tight junction and neural autoantibodies, and Alzheimer and Parkinson's hallmarks. The prefrontal white matter is a target of air pollution. We examined by light and electron microscopy the prefrontal white matter of MC dogs (n: 15, age 3.17±0.74 years), children and teens (n: 34, age: 12.64±4.2 years) versus controls. Major findings in MC residents included leaking capillaries and small arterioles with extravascular lipids and erythrocytes, lipofuscin in pericytes, smooth muscle and endothelial cells (EC), thickening of cerebrovascular basement membranes with small deposits of amyloid, patchy absence of the perivascular glial sheet, enlarged Virchow–Robin spaces and nanosize particles (20–48nm) in EC, basement membranes, axons and dendrites. Tight junctions, a key component of the neurovascular unit (NVU) were abnormal in MC versus control dogs (χ2<0.0001), and white matter perivascular damage was significantly worse in MC dogs (p=0.002). The integrity of the NVU, an interactive network of vascular, glial and neuronal cells is compromised in MC young residents. Characterizing the early NVU damage and identifying biomarkers of neurovascular dysfunction may provide a fresh insight into Alzheimer pathogenesis and open opportunities for pediatric neuroprotection.

Cerebrospinal fluid diversion in patients with enlarged Virchow-Robin spaces without ventriculomegaly.

Enlarged Virchow-Robin spaces (eVRS) are an MRI biomarker in several neurological diseases of inflammatory, neurodegenerative, vascular, metabolic, or genetic origin. We report on a further condition in which eVRS were observed in patients with an ongoing chronic hydrocephalus-like clinical picture without ventriculomegaly who improved after CSF diversion, and we discuss the possible mechanisms underlying this finding. A retrospective study of seven patients presenting progressive gait, cognitive, and urinary disturbances in association with eVRS was undertaken. All patients presented an Evans ratio <0.30 and >20 eVRS at the level of basal ganglia and periventricular parenchyma as assessed by T2-weighted MRI. All patients underwent prolonged external lumbar drainage (PELD) with good response. Six patients received ventriculoperitoneal shunt with improvement of their clinical status compared to that before PELD (follow-up: 8-58 months, mean 24.6). The seventh patient did not undergo ventriculoperitoneal shunt and received a second PELD with persistent improvement (follow-up: 14 months). Our results indicate that a mechanism involving CSF accumulation and stasis in the subarachnoid space was at least a concurrent factor of this clinical picture. This study should stimulate new perspectives on the role of CSF disturbances in the pathogenesis of diseases associated with VRS enlargement.

Risk Factors, Consequences and Rating Methodology: Enlarged Virchow Robin Spaces in Brain MRIs in the Framingham Study (P6.174)

Risk Factors, Consequences and Rating Methodology: Enlarged Virchow Robin Spaces in Brain MRIs in the Framingham Study (P6.174)

PO-0806 Multi-cystic White Matter Enlarged Virchow Robin Spaces In A 5-year-old Boy

Virchow–Robin spaces (VRS) are perivascular, pial-lined areas in the brain that surrounds small arteries and can be seen on brain magnetic resonance imaging. VRS are typically less than 5 mm in diameter and accepted as normal anatomical structures. VRS are usually asymptomatic, but if there is an expansion to the brain parenchyma, various clinical symptoms can be seen associated with the mass effect. A 5-year-old boy presented with a complaint of developmental delay. He had relative macrocephaly, hair with a double crown, and clinodactyly of the middle phalanx of both the fifth fingers. He had dysmorphic facial features, including triangular face, frontal bossing, and low-set protruding ears. His neurologic examination revealed normal muscular tonus and normal deep tendon reflexes with clumsy gait. Metabolic screening tests, including tandem mass, urine organic acids, plasma, and urine amino acid profiles were also normal. The patient underwent MRI of the brain as a firstline investigation, and T2 images demonstrated an extensive involvement of the hemispheric, subcortical white matter with a cerebrospinal fluid intensity signal change suggestive of multilocular giant VRS. MR spectroscopy showed no significant signal peak. The karyotype analysis of the patient was 46 XY, and FISH for Angelman syndrome also revealed a normal result. In the 2-year follow-up, the patient showed no neuromotor deterioration and radiological progression. In conclusion, VRS must be differentiated from other cystic lesions of the brain. Phenotypic characteristics of our patient were not compatible with the previously defined syndromes.

Eigenspace template matching for detection of lacunar infarcts on MR images.

Detection of lacunar infarcts is important because their presence indicates an increased risk of severe cerebral infarction. However, accurate identification is often hindered by the difficulty in distinguishing between lacunar infarcts and enlarged Virchow-Robin spaces. Therefore, we developed a computer-aided detection (CAD) scheme for the detection of lacunar infarcts. Although our previous CAD method indicated a sensitivity of 96.8% with 0.71 false positives (FPs) per slice, further reduction of FPs remained an issue for the clinical application. Thus, the purpose of this study is to improve our CAD scheme by using template matching in the eigenspace. Conventional template matching is useful for the reduction of FPs, but it has the following two pitfalls: (1) It needs to maintain a large number of templates to improve the detection performance, and (2) calculation of the cross-correlation coefficient with these templates is time consuming. To solve these problems, we used template matching in the lower dimension space made by a principal component analysis. Our database comprised 1,143 T1- and T2-weighted images obtained from 132 patients. The proposed method was evaluated by using twofold cross-validation. By using this method, 34.1% of FPs was eliminated compared with our previous method. The final performance indicated that the sensitivity of the detection of lacunar infarcts was 96.8% with 0.47 FPs per slice. Therefore, the modified CAD scheme could improve FP rate without a significant reduction in the true positive rate.

Swiss Cheese Striatum

Markedly enlarged Virchow-Robin spaces throughout the striatum appear occasionally on magnetic resonance imaging (MRI) scans of the elderly, and this type of striatum is known as the Swiss cheese striatum (SCS); however, its clinical impact is unknown. To determine the clinical features associated with SCS detected on MRI scans. A blinded, retrospective case-control study using medical records from 2000 to 2007 obtained from an MRI database at the Mayo Clinic in Rochester, Minnesota, of residents 40 years of age or older of Olmsted County, Minnesota, who had extensive Mayo Clinic medical records and MRI reports suggestive of SCS. Cases with a severe form of SCS (n = 27) were randomly selected for comparison with age-, sex-, and examination year-matched controls (n = 52) with a minimal form of SCS or no SCS. Magnetic resonance imaging. Associations of clinical and imaging features with the presence of a severe form of SCS. Medical records were reviewed for clinical features such as parkinsonism, dementia, and vascular risk factors. The MRI scans were visually scored for degree of leukoaraiosis, central atrophy, and cortical atrophy. No significant differences were found between those with a severe form of SCS and controls in rates of parkinsonism (19% vs 17%; odds ratio, 1.09 [95% CI, 0.28-4.16]) or dementia of any type (30% vs 21%; odds ratio, 1.57 [95% CI, 0.48-5.13]). Vascular risk factors were not significantly different between groups. Swiss cheese striatum correlated with degree of leukoaraiosis (P < .001). Potential associations with visualized cortical atrophy (P = .01), nonobstructive urinary incontinence (18.5% vs 3.9%; P = .04), and syncope (37% vs 9.6%; P = .01) did not hold up after correction for the false discovery rate. Our study suggests that marked cribriform change in the striatum was not associated with the development of extrapyramidal clinical disorders, including parkinsonism. The association of SCS with leukoaraiosis suggests that it is part of a more generalized cerebrovascular process. Skepticism is called for when attributing clinical symptoms to this MRI finding.

Unusual Imaging Findings in Brain and Spinal Cord in Two Siblings With Maple Syrup Urine Disease

Maple syrup urine disease (MSUD) is a rare metabolic disease affecting the neural tissue. While the brain abnormalities present on MRI are well known, spinal imaging features have not been studied. We herewith report an unusual finding of enlarged Virchow Robin spaces in brain and novel spinal cord changes in two biochemically diagnosed cases of MSUD. To the best of our knowledge, spinal MRI findings in cases of MSUD have not been previously reported. Knowledge of spinal MRI findings may be useful in diagnosis of this rare disorder.

Higher ambulatory blood pressure relates to enlarged Virchow-Robin spaces in first-ever lacunar stroke patients

Enlarged Virchow-Robin spaces (EVRS) are considered to be a sign of cerebral small vessel disease. Hypertension is an important risk factor for cerebral small vessel disease, whereas ambulatory blood pressure (BP) is the strongest predictor of hypertension-related brain damage. However, the association between ambulatory BP levels and EVRS has never been investigated. The aim of this study was to determine the association between ambulatory BP levels and EVRS. In 143 first-ever lacunar stroke patients, we performed 24-h ambulatory BP monitoring after the acute stroke phase. On brain MRI we counted EVRS in the basal ganglia and the centrum semiovale. We graded the number of EVRS at each level into a three-category severity scale. We assessed the association between BP levels and EVRS by ordinal regression analysis. After adjusting for age, sex, extensive white matter lesions, and asymptomatic lacunar infarcts, higher day systolic (OR 1.21; 95 % CI 1.00–1.46 per 10 mmHg), day diastolic (1.18; 95 % CI 1.02–1.37 per 5 mmHg) and 24-h diastolic (OR 1.18; 95 % CI 1.01–1.37 per 5 mmHg) ambulatory BP levels were associated with EVRS at the basal ganglia level. No relation was found between ambulatory BP levels and EVRS in the centrum semiovale. Higher day ambulatory BP levels are associated with EVRS in the basal ganglia. This association was independent of the presence of extensive white matter lesions and asymptomatic lacunar infarcts. Our results imply that basal ganglia EVRS should be regarded as a separate manifestation of BP-related brain damage.

Multi-cystic white matter enlarged Virchow Robin spaces in a 5-year-old boy

Multi-cystic white matter enlarged Virchow Robin spaces in a 5-year-old boy

Diffusion Tensor Imaging and Tractography of the Corticospinal Tract in the Presence of Enlarged Virchow–Robin Spaces

We report a patient with abnormal diffusion tensor imaging (DTI) and tractography of the corticospinal tract caused by mass effect from adjacent enlarged Virchow-Robin spaces. DTI was performed using 25 noncollinear directions. Fractional anisotropy (FA) and mean diffusivity (MD) maps were generated. Region-of-interest measurements of the corticospinal tracts were organized in histograms, and comparisons were made between sides. Statistical analysis consisted of a Wilcoxon rank-sum nonparametric test and a two-sample test of proportions to compare the relative percentage of voxels >.8. The patient had no signs or symptoms of motor weakness. The corticospinal tract adjacent to the enlarged Virchow-Robin spaces showed significant changes in the proportion of FA > .8, distribution of FA and distribution of MD (P < .001). Diffusion tensor changes may be caused by enlarged Virchow-Robin spaces in the absence of clinical signs or symptoms. We hypothesize that the DTI changes are due to alterations in the extravascular extracellular space. Tensor changes should be interpreted with caution in patients with space occupying mass lesions such as brain tumors.

Vascular inflammation in cerebral small vessel disease

Cerebral small vessel disease (CSVD) is considered to be caused by an increased permeability of the blood-brain barrier and results in enlargement of Virchow Robin spaces (VRs), white matter lesions, brain microbleeds, and lacunar infarcts. The increased permeability of the blood-brain barrier may relate to endothelial cell activation and activated monocytes/macrophages. Therefore, we hypothesized that plasma markers of endothelial activation (adhesion molecules) and monocyte/macrophage activation (neopterin) relate to CSVD manifestations. In 163 first-ever lacunar stroke patients and 183 essential hypertensive patients, we assessed CSVD manifestations on brain magnetic resonance imaging (MRI) and levels of C-reactive protein (CRP), neopterin, as well as circulating soluble adhesion molecules (sICAM-1, sVCAM-1, sE-selectin, sP-selectin). Neopterin, sICAM-1 and sVCAM-1 levels were higher in patients with extensive CSVD manifestations than in those without (p < 0.01). Neopterin levels independently related to higher numbers of enlarged Virchow Robin spaces (p < 0.001). An inflammatory process with activated monocytes/macrophages may play a role in the increased permeability of the blood brain barrier in patients with CSVD.

Further delineation of the 17p13.3 microdeletion involving YWHAE but distal to PAFAH1B1: Four additional patients

Background The 17p13.3 deletion syndrome (or Miller-Dieker syndrome, MDS, MIM 247200) is characterized by lissencephaly, mental retardation and facial dysmorphism. The phenotype is attributed to haploinsufficiency of two genes present in the minimal critical region of MDS: PAFAH1B1 (formerly referred to as LIS1) and YWHAE. Whereas isolated PAFAH1B1 deletion causes lissencephaly, YWHAE is a candidate for the dysmorphic phenotype associated with MDS. Objective We describe clinical, neuroradiological and molecular data in four patients with a 17p13.3 deletion distal to PAFAH1B1 involving YWHAE. Results All patients presented with mild or moderate developmental delay and pre and/or post-natal growth retardation. Patients A, B and C had neuro-imaging anomalies: leucoencephalopathy with macrocephaly (patients A and C), Chiari type 1 malformation (patient A) and paraventricular cysts (patient C). Patient B had patent ductus arteriosus and pulmonary arterial hypertension. Patient C had unilateral club foot. Patient D had enlarged Virchow Robin spaces, microcornea, and chorioretinal and lens coloboma. Array-CGH revealed de novo terminal 17p13.3 deletions for patient A and B, and showed interstitial 17p13.3 deletions of 1.4 Mb for patient C and of 0.5 Mb for patient D. In all patients, PAFAH1B1 was not deleted. Conclusion Our patients confirm that 17p deletion distal to PAFAH1B1 have a distinctive phenotype : mild mental retardation, moderate to severe growth restriction, white matter abnormalities and developmental defects including Chiari type 1 malformation and coloboma. Our patients contribute to the delineation and clinical characterization of 17p13.3 deletion distal to PAFAH1B1 and highlight the role of the region containing YWHAE in brain and eye development and in somatic growth.

Mesencephalic enlarged Virchow–Robin spaces in a 6-year-old boy: a case-based update

Perivascular spaces or Virchow-Robin spaces are normal anatomical structures within the brain, typically less than 5 mm in diameter. Rarely, they can reach much larger sizes and adopt bizarre single or multi-cystic configurations, as was the case in the patient that we report on. When there is such markedly gross dilatation, the phenomenon is known as Giant or tumefactive perivascular space enlargement. We report a 6-year-old boy with a multi-cystic mesencephalic lesions presenting with obstructive hydrocephalus secondary to obstruction of the aqueduct of Sylvius due to tumefactive dilatation of Virchow-Robin spaces. The patient underwent an endoscopic fenestration and biopsy of the cystic portion abutting into the ventricular system. In this paper, we discuss the phenomenon of perivascular (Virchow-Robin) spaces and their treatment options and review the relevant literature. To our knowledge, this is the first pediatric case of tumefactive dilatation of the Virchow-Robin spaces causing obstructive hydrocephalus that were directly fenestrated using neuroendoscopy.