Case Report
Giant Mesencephalic Dilated Virchow Robin Spaces Causing Obstructive Hydrocephalus Treated by Endoscopic Third Ventriculostomy Amandeep Kumar1, Rishab Gupta2, Ajay Garg3, Bhawani S. Sharma1
Key words Dilated - Giant - Mesencephalic - Virchow Robin spaces -
Abbreviations and Acronyms CSF: Cerebrospinal fluid dVRS: Dilatation of VRS MRI: Magnetic resonance imaging VRS: Virchow Robin space
- BACKGROUND:
Virchow Robin spaces (VRSs) are perivascular spaces that act as drainage pathways for interstitial fluid. Dilatation of VRSs is visible on magnetic resonance images in asymptomatic individuals. However, giant dilatation of VRSs (dVRSs) is very rare. Such giant dVRSs may produce a pressure effect on surrounding structures and can be confused with more sinister conditions such as cystic neoplasm.
- CASE
To whom correspondence should be addressed: Amandeep Kumar, M.Ch. [E-mail: [email protected]]
DESCRIPTION: We describe a 30-year-old man who presented with complaints of headache, poor attention and concentration, forgetfulness, polyuria, urinary incontinence for past 3 years and hypersomnia for 3 months. He was found to have a multicystic lesion in the midbrain with extension into the third ventricle, causing hydrocephalus due to aqueductal obstruction. Although at first look, the radiologic features suggested a cystic neoplasm, a careful magnetic resonance imaging evaluation helped to establish the correct diagnosis of dVRS. Endoscopic third ventriculostomy resolved his symptoms.
Citation: World Neurosurg. (2015) 84, 6:2074.e11-2074.e14. http://dx.doi.org/10.1016/j.wneu.2015.07.010
- CONCLUSIONS:
From the Departments of 1Neurosurgery, 2Psychiatry, and 3 Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2015 Elsevier Inc. All rights reserved.
Dilatation of VRS may produce multicystic giant lesions that can easily be confused with other pathologic condition that have a completely different prognosis and management. Knowledge about the existence of such an entity and their radiologic features is important to prevent such misdiagnosis and mismanagement in the form of biopsy/excision, which can have devastating consequences.
INTRODUCTION Virchow Robin spaces (VRSs) are perivascular spaces that course along the cerebral vessels as they traverse from subarachnoid space to the brain parenchyma. They act as a pathway for egress of interstitial fluid. VRSs frequently become dilated and can be seen on magnetic resonance images of patients of all ages (12). They are usually bilateral, smaller than 5 mm, and asymptomatic (12). However, on rare occasions, extreme dilatation may lead to giant VRSs. Such giant tumefactive dilated VRSs (dVRSs) can produce pressure symptoms and can easily be confused with other more sinister cystic intracranial conditions (4, 6, 12, 13, 18, 25, 27). We present a case of giant tumefactive midbrain dVRSs causing obstructive hydrocephalus. The patient’s symptoms were relieved after he underwent endoscopic third ventriculostomy (ETV) after a failed attempt at ventriculoperitoneal shunt placement. The case is being presented to highlight that, radiologically,
2074.E11
www.SCIENCEDIRECT.com
dVRS can be confused with and misinterpreted as other intracranial cystic conditions. Awareness of these innocuous lesions is important to prevent misdiagnosis and consequent mismanagement because dVRSs are benign lesions and do not require any surgical intervention per se. CASE REPORT A 30-year-old man presented to the psychiatric outpatient department with complaints of headache, poor attention and concentration, forgetfulness, polyuria, urinary incontinence for the past 3 years, and hypersomnia for 3 months. On psychiatric evaluation, the patient denied any low mood, anhedonia, or any pessimistic ideas. There was no history of excessive food intake, change in libido, or snoring, and no past history of any psychiatric illness or any chronic medical illness. Considering the nature of the
symptoms, routine blood work was advised along with assessment of serum thyroid-stimulating hormone level and magnetic resonance imaging (MRI) of the brain. No abnormality was detected on the blood tests. MRI revealed a multicystic lesion in the midbrain with extension into the third ventricle causing aqueductal obstruction with consequent hydrocephalus (Figure 1AeD). With a presumptive diagnosis of a cystic neoplasm, the patient was referred to the neurosurgery outpatient department. However, a careful review of patient’s MRI revealed certain features that were contrary to the diagnosis of cystic neoplasm. The cysts were thin walled and the cyst contents had intensities similar to cerebrospinal fluid (CSF) (Figure 1). The cyst walls showed no enhancement. There were no signal alterations in the surrounding normal brain parenchyma. A nonenhancing, thin-walled CSF-filled multicystic lesion in the midbrain
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.07.010
CASE REPORT AMANDEEP KUMAR ET AL.
GIANT MESENCEPHALIC DILATED VIRCHOW ROBIN SPACES
Figure 1. (A) Axial T2-weighted image shows a multicystic lesion in the midbrain with hydrocephalus. Axial (B), sagittal (C), and coronal (D) reformatted CISS (constructive interference in steady state) images show multiple cysts within the midbrain causing obstruction of cerebrospinal fluid flow at the level of the aqueduct with dilated pineal recess (* in C) and dilated optic recess of the third ventricle (black arrow in C) suggesting
suggests a diagnosis of dilated VRSs rather than a cystic neoplasm. The patient underwent placement of a ventriculoperitoneal shunt after which he had temporary resolution of his symptoms; the symptoms recurred after 1 month because of malfunction of the shunt. He then underwent ETV after which his symptoms completely resolved and he is doing well at 4 months follow-up. An analysis of CSF revealed normal findings. On postoperative MRI at 3 months, ventricle size had decreased compared with the preoperative MRI, however, the cysts showed no significant change in size (Figure 1EeH). DISCUSSION VRSs, named after Rudolf Virchow and Charles Phillippe Robin (23, 30), can be seen on MRI scans of normal people (11). The VRSs are termed dilated based on their size (>1 mm) (3) or based on their shape (irregular focal expansion or ectasia) (11). Although dilatation of VRSs was first described by Durant-Fardel in
hydrocephalus. After third ventriculostomy, follow-up MRI shows no change in cyst morphology on axial T2-weighted images (E). Axial (F), sagittal (G), and coronal (H) reformatted CISS images show reduction in the size of the pineal recess (* in G) and the optic recess of third ventricle (black arrow in G).
1843 (8), the mechanism of dilatation is still unknown. Different hypotheses have been proposed to explain dilatation of VRSs including impaired drainage of interstitial fluid into the subarachnoid space (13, 17), segmental necrotizing angiitis of cerebral vessels or increased permeability of vessels (14), fibrosis and obstruction of VRSs leading to impaired drainage of fluid (22), and leakage of interstitial fluid from the intracellular compartment into the VRSs (1). dVRSs are characteristically seen in 3 locations and have been classified based on their location into 3 types. Type I dVRSs occur in basal ganglia along the lenticulostriate arteries entering through anterior perforated substance (12). Type II dVRSs are found in the centrum semiovale along the perforating medullary arteries as they enter the cortex over the cerebral convexity and reach white matter (12). Type III or mesencephalic dVRSs are found in the midbrain, where they occur along the perforating branches of the collicular and accessory collicular arteries at the
WORLD NEUROSURGERY 84 [6]: 2074.e11-2074.e14, DECEMBER 2015
pontomesencephalic junction and along the posterior thalamoperforating arteries, paramedic mesencephalothalamic artery, and short and long circumferential arteries at the mesencephalothalamic junction (26). Giant dVRSs are significantly enlarged VRSs and most commonly involve the mesencephalothalamic region (supplied by the paramedial mesencephalothalamic artery) and cerebral white matter (12, 13, 21, 27). All cases of giant dVRS produce a mass effect of varying degrees, and may occasionally produce hydrocephalus (25, 27). Although the adjacent parenchyma usually does not show any alteration in signal intensity, cases with foci of hyperintensity on T2-weighted and fluid attenuation inversion recovery images have been reported (15, 27). As the giant dVRSs can assume bizarre configurations and can produce a mass effect, misinterpretation as other cystic lesions is quite possible (4, 6, 12, 13, 18, 27). They can easily be misdiagnosed as cystic neoplasms, parasitic cysts, non-neoplastic neuroepithelial cysts,
www.WORLDNEUROSURGERY.org
2074.E12
CASE REPORT AMANDEEP KUMAR ET AL.
ventricular diverticula, cystic infarction, and mucopolysaccharidosis, especially because dVRSs are usually periventricular in location (4, 6, 12, 13, 18, 27). A careful analysis of MRI, however, can help in differentiation. The presence of a solid component, contrast enhancement, perilesional edema, intensity of cyst contents different from intensity of CSF, and low signal intensity on diffusion-weighted images with high apparent diffusion values point toward the lesion being a cystic neoplasm (28). Giant dVRSs on the other hand are sharply demarcated, nonenhancing, purely cystic lesions in a characteristic location of perforating vessels with cyst contents displaying signal intensity similar to CSF in all MRI sequences. The surrounding brain parenchyma appears normal (3, 27). Although these radiologic features are almost pathognomonic of giant dVRSs, differentiation from other intracranial cystic lesions may be difficult in certain cases and in such a situation, clinical and radiologic follow-up may be helpful. The symptoms due to a large dVRS can be either due to midbrain compression (Benedikt syndrome or Parkinson-like symptoms) or due to obstructive hydrocephalus. Such patients can present with headache, dizziness, decreased concentration, impaired memory, impaired balance, dementia, and parkinsonism (9, 31); however, a direct correlation between clinical manifestations and radiologic findings is lacking (18, 29), except when the dVRS obstructs CSF pathways leading to hydrocephalus (27). dVRS may also be associated with certain metabolic and genetic disorders including mucopolysaccharidosis (16), Lowe syndrome (2), Coffin-Lowry syndrome (20), and myotonic dystrophy (7). The management of mesencephalic dVRSs should be aimed at addressing the symptoms due to hydrocephalus and those due to midbrain compression. Hydrocephalus due to dVRSs can be managed either with placement of a ventriculoperitoneal shunt (17) or with ETV (5, 10, 19, 24). However, symptoms due to midbrain compression cannot be relieved by CSF diversion alone (5, 10, 24) and in such cases, cyst fenestration and endoscopic cystoventriculostomy or cystocisternostomy is needed to relieve the midbrain compression (5, 10, 24). However,
2074.E13
www.SCIENCEDIRECT.com
GIANT MESENCEPHALIC DILATED VIRCHOW ROBIN SPACES
intraoperative bleeding in cysts has been reported in patients undergoing cyst fenestration (10, 24). Fiorendi et al. (10) have cautioned against internal exploration of cysts and have advised against taking a biopsy of the cyst wall as even slight traction can lead to rupture of intracystic arteries leading to hemorrhage and consequent neurologic deficits. Endoscopy offers the advantage of performing both CSF diversion by doing ETV and cyst decompression by doing cystoventriculostomy/cystocisternostomy. In the present case, the patient had symptoms caused by hydrocephalus only and not due to midbrain compression. Thus, we only performed ETV; cyst fenestration was not attempted. CONCLUSIONS dVRSs are commonly seen on MRI scans done for various reasons and are asymptomatic. However, giant dVRSs are a rare entity and are uncommonly encountered in clinical practice. Radiologically, dVRSs can closely mimic other intracranial cystic lesions. Knowledge about the existence of such lesions, their characteristic location, and careful analysis of MRI can help in making a correct diagnosis. Endoscopy can be used to perform third ventriculostomy as well as cyst fenestration in patients with symptomatic midbrain compression. REFERENCES 1. Adachi M, Hosoya T, Haku T, Yamaguchi K: Dilated Virchow-Robin spaces: MRI pathological study. Neuroradiology 40:27-31, 1998. 2. Barkovich AJ: Pediatric neuroimaging. Philadelphia: Lippincott Williams & Wilkins; 1999. 3. Bokura H, Kobayashi S, Yamaguchi S: Distinguishing silent lacunar infarction enlarged Virchow-Robin spaces: a magnetic resonance imaging and pathological study. J Neurol 245: 116-122, 1998. 4. Cakirer S: MR imaging findings in tumefactive perivascular spaces. Acta Radiol 44:673-674, 2003. 5. Conrad J, Welschehold S, Charalampaki P, van Lindert E, Grunert P, Perneczky A: Mesencephalic ependymal cysts: treatment under pure endoscopic or endoscope-assisted keyhole conditions. J Neurosurg 109:723-728, 2008. 6. Davis G, Fitt GJ, Kalnins RM, Mitchell LA: Increased perivascular spaces mimicking frontal lobe tumor. J Neurosurg 97:723, 2002.
7. Di Costanzo A, Di Salle F, Santoro L, Bonavita V, Tedeschi G: Dilated Virchow-Robin spaces in myotonic dystrophy: frequency, extent and significance. Eur Neurol 46:131-139, 2001. 8. Durant-Fardel M: Traite du ramollissement du cerveau. Paris, France: Balliere; 1843. 9. Fénelon G, Gray F, Wallace C, Poirier J, Guillard A: Parkinsonism and dilatation of the perivascular spaces (état criblé) of the striatum: a clinical magnetic resonance imaging and pathological study. Mov Disord 10:754-760, 1995. 10. Fiorindi A, Delitala A, Francaviglia N, Longatti P: Neuroendoscopic options in the treatment of mesencephalic expanding cysts: report of four cases and review of the literature. Clin Neurol Neurosurg 115:2370-2376, 2013. 11. Groeschel S, Chong WK, Surtees R, Hanefeld F: Virchow-Robin spaces on magnetic resonance images: normative data, their dilatation, and a review of the literature. Neuroradiology 48: 745-754, 2006. 12. Heier LA, Bauer CJ, Schwartz L, Zimmerman RD, Morgello S, Deck MD: Large Virchow-Robin spaces: MR-clinical correlation. AJNR Am J Neuroradiol 10:929-936, 1989. 13. Homeyer P, Cornu P, Lacomblez L, Chiras J, Derouesne C: A special form of cerebral lacunae: expanding lacunae. J Neurol Neurosurg Psychiatry 61:200-202, 1996. 14. Hughes W: Origin of lacunes. Lancet 2:19-21, 1965. 15. Komiyama M, Yasui T, Izumi T: Magnet resonance imaging features of unusually dilated VirchowRobin spaces—two case reports. Neurol Med Chir 38:161-164, 1998. 16. Lee C, Dineen TE, Brack M, Kirsch JE, Runge VM: The mucopolysaccharidoses: characterization by cranial MR imaging. AJNR Am J Neuroradiol 14: 1285-1292, 1993. 17. Mascalchi M, Salvi F, Godano U, Nistri M, Taluti R, Tosetti M, Villari N, Calbucci F: Expanding lacunae causing triventricular hydrocephalus: report of two cases. J Neurosurg 91: 669-674, 1999. 18. Ogawa R, Okudera T, Fukasawa H, Hashimoto M, Inugami A, Fujita H, Hatajawa J, Shimosegawa E, Noguchi K, Uemura K: Unusual widening of Virchow-Robin spaces: MR appearance. AJNR Am J Neuroradiol 16:1238-1242, 1995. 19. Ottenhausen M, Meier U, Tittel A, Lemcke J: Acute decompensation of noncommunicating hydrocephalus caused by dilated Virchow-Robin spaces type III in a woman treated by endoscopic third ventriculostomy: a case report and review of the literature. J Neurol Surg A Cent Eur Neurosurg 74 (Suppl 1):e242-247, 2013. 20. Patlas M, Joseph A, Cohen JE, Gomori JM: MRI and MRS of Coffin-Lowry syndrome: a case report. Neurol Res 25:285-286, 2003. 21. Poirier J, Barbizet J, Gaston A, Meyrignac C: Thalamic dementia. Expansive lacunae of the thalamo-paramedian mesencephalic area.
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.07.010
CASE REPORT AMANDEEP KUMAR ET AL.
Hydrocephalus caused by stenosis of the aqueduct of Sylvius. Rev Neurol (Paris) 139:349-358, 1983. 22. Pollock H, Hutchings M, Weller RO, Zhang ET: Perivascular spaces in the basal ganglia of the human brain: their relationship to lacunes. J Anat 191:337-346, 1997. 23. Robin C: Recherches sur quelques particularités de la structure des capillaires de l’encephale. J Physiol Homme Anim 2:537-548, 1859. 24. Rohlfs J, Riegel T, Khalil M, Iwinska-Zelder J, Mennel H-D, Bertalanffy H, Hellwig D: Enlarged perivascular spaces mimicking multicystic brain tumors. Report of two cases and review of the literature. J Neurosurg 102:1142-1146, 2005. 25. Romi F, Tysnes OB, Krakenes J, Savoiardo M, Aarli JA, Bindoff L: Cystic dilation of Virchow Robin spaces in the midbrain. Eur Neurol 47: 186-188, 2002. 26. Saeki N, Sato M, Kubota M, Uchino Y, Murai H, Nagai Y, Ishikura H, Nomura S, Matsuura I,
GIANT MESENCEPHALIC DILATED VIRCHOW ROBIN SPACES
Yamaura A: MR imaging of normal perivascular space expansion at midbrain. AJNR Am J Neuroradiol 26:566-571, 2005. 27. Salzman KL, Osborn AG, House P, Jinkins JR, Ditchfield A, Cooper JA, Weller RO: Giant tumefactive perivascular spaces. AJNR Am J Neuroradiol 26:298-305, 2005. 28. Tien RD, Felsberg GJ, Friedman H, Brown M, MacFall J: MR imaging of high-grade cerebral gliomas: value of diffusion-weighted echoplanar pulse sequences. AJR Am J Roentgenol 162: 671-677, 1994. 29. Ugawa Y, Shirouzu I, Terao Y, Hanajima R, Machii K, Mochijuki H, Furubyashi T, Kanazawa I: Physiological analyses of a patient with extreme widening of Virchow-Robin spaces. J Neurol Sci 159:25-27, 1998.
31. Vital C, Julian J: Widespread dilatation of perivascular spaces: A leukoencephalopathy causing dementia. Neuroradiology 48:1310-1313, 1997.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Patient consent: The patient /next of kin/guardian has consented to the submission of the case report for submission to the journal. Received 25 March 2015; accepted 1 July 2015 Citation: World Neurosurg. (2015) 84, 6:2074.e11-2074.e14. http://dx.doi.org/10.1016/j.wneu.2015.07.010 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com
30. Virchow R: Ueber die Erweiterung kleinerer Gefaesse. Arch Pathol Anat Physiol Klin Med 3: 427-462, 1851.
WORLD NEUROSURGERY 84 [6]: 2074.e11-2074.e14, DECEMBER 2015
1878-8750/$ - see front matter ª 2015 Elsevier Inc. All rights reserved.
www.WORLDNEUROSURGERY.org
2074.E14
Giant Mesencephalic Dilated Virchow Robin Spaces Causing Obstructive Hydrocephalus Treated by Endoscopic Third Ventriculostomy Amandeep Kumar1, Rishab Gupta2, Ajay Garg3, Bhawani S. Sharma1
Key words Dilated - Giant - Mesencephalic - Virchow Robin spaces -
Abbreviations and Acronyms CSF: Cerebrospinal fluid dVRS: Dilatation of VRS MRI: Magnetic resonance imaging VRS: Virchow Robin space
- BACKGROUND:
Virchow Robin spaces (VRSs) are perivascular spaces that act as drainage pathways for interstitial fluid. Dilatation of VRSs is visible on magnetic resonance images in asymptomatic individuals. However, giant dilatation of VRSs (dVRSs) is very rare. Such giant dVRSs may produce a pressure effect on surrounding structures and can be confused with more sinister conditions such as cystic neoplasm.
- CASE
To whom correspondence should be addressed: Amandeep Kumar, M.Ch. [E-mail: [email protected]]
DESCRIPTION: We describe a 30-year-old man who presented with complaints of headache, poor attention and concentration, forgetfulness, polyuria, urinary incontinence for past 3 years and hypersomnia for 3 months. He was found to have a multicystic lesion in the midbrain with extension into the third ventricle, causing hydrocephalus due to aqueductal obstruction. Although at first look, the radiologic features suggested a cystic neoplasm, a careful magnetic resonance imaging evaluation helped to establish the correct diagnosis of dVRS. Endoscopic third ventriculostomy resolved his symptoms.
Citation: World Neurosurg. (2015) 84, 6:2074.e11-2074.e14. http://dx.doi.org/10.1016/j.wneu.2015.07.010
- CONCLUSIONS:
From the Departments of 1Neurosurgery, 2Psychiatry, and 3 Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2015 Elsevier Inc. All rights reserved.
Dilatation of VRS may produce multicystic giant lesions that can easily be confused with other pathologic condition that have a completely different prognosis and management. Knowledge about the existence of such an entity and their radiologic features is important to prevent such misdiagnosis and mismanagement in the form of biopsy/excision, which can have devastating consequences.
INTRODUCTION Virchow Robin spaces (VRSs) are perivascular spaces that course along the cerebral vessels as they traverse from subarachnoid space to the brain parenchyma. They act as a pathway for egress of interstitial fluid. VRSs frequently become dilated and can be seen on magnetic resonance images of patients of all ages (12). They are usually bilateral, smaller than 5 mm, and asymptomatic (12). However, on rare occasions, extreme dilatation may lead to giant VRSs. Such giant tumefactive dilated VRSs (dVRSs) can produce pressure symptoms and can easily be confused with other more sinister cystic intracranial conditions (4, 6, 12, 13, 18, 25, 27). We present a case of giant tumefactive midbrain dVRSs causing obstructive hydrocephalus. The patient’s symptoms were relieved after he underwent endoscopic third ventriculostomy (ETV) after a failed attempt at ventriculoperitoneal shunt placement. The case is being presented to highlight that, radiologically,
2074.E11
www.SCIENCEDIRECT.com
dVRS can be confused with and misinterpreted as other intracranial cystic conditions. Awareness of these innocuous lesions is important to prevent misdiagnosis and consequent mismanagement because dVRSs are benign lesions and do not require any surgical intervention per se. CASE REPORT A 30-year-old man presented to the psychiatric outpatient department with complaints of headache, poor attention and concentration, forgetfulness, polyuria, urinary incontinence for the past 3 years, and hypersomnia for 3 months. On psychiatric evaluation, the patient denied any low mood, anhedonia, or any pessimistic ideas. There was no history of excessive food intake, change in libido, or snoring, and no past history of any psychiatric illness or any chronic medical illness. Considering the nature of the
symptoms, routine blood work was advised along with assessment of serum thyroid-stimulating hormone level and magnetic resonance imaging (MRI) of the brain. No abnormality was detected on the blood tests. MRI revealed a multicystic lesion in the midbrain with extension into the third ventricle causing aqueductal obstruction with consequent hydrocephalus (Figure 1AeD). With a presumptive diagnosis of a cystic neoplasm, the patient was referred to the neurosurgery outpatient department. However, a careful review of patient’s MRI revealed certain features that were contrary to the diagnosis of cystic neoplasm. The cysts were thin walled and the cyst contents had intensities similar to cerebrospinal fluid (CSF) (Figure 1). The cyst walls showed no enhancement. There were no signal alterations in the surrounding normal brain parenchyma. A nonenhancing, thin-walled CSF-filled multicystic lesion in the midbrain
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.07.010
CASE REPORT AMANDEEP KUMAR ET AL.
GIANT MESENCEPHALIC DILATED VIRCHOW ROBIN SPACES
Figure 1. (A) Axial T2-weighted image shows a multicystic lesion in the midbrain with hydrocephalus. Axial (B), sagittal (C), and coronal (D) reformatted CISS (constructive interference in steady state) images show multiple cysts within the midbrain causing obstruction of cerebrospinal fluid flow at the level of the aqueduct with dilated pineal recess (* in C) and dilated optic recess of the third ventricle (black arrow in C) suggesting
suggests a diagnosis of dilated VRSs rather than a cystic neoplasm. The patient underwent placement of a ventriculoperitoneal shunt after which he had temporary resolution of his symptoms; the symptoms recurred after 1 month because of malfunction of the shunt. He then underwent ETV after which his symptoms completely resolved and he is doing well at 4 months follow-up. An analysis of CSF revealed normal findings. On postoperative MRI at 3 months, ventricle size had decreased compared with the preoperative MRI, however, the cysts showed no significant change in size (Figure 1EeH). DISCUSSION VRSs, named after Rudolf Virchow and Charles Phillippe Robin (23, 30), can be seen on MRI scans of normal people (11). The VRSs are termed dilated based on their size (>1 mm) (3) or based on their shape (irregular focal expansion or ectasia) (11). Although dilatation of VRSs was first described by Durant-Fardel in
hydrocephalus. After third ventriculostomy, follow-up MRI shows no change in cyst morphology on axial T2-weighted images (E). Axial (F), sagittal (G), and coronal (H) reformatted CISS images show reduction in the size of the pineal recess (* in G) and the optic recess of third ventricle (black arrow in G).
1843 (8), the mechanism of dilatation is still unknown. Different hypotheses have been proposed to explain dilatation of VRSs including impaired drainage of interstitial fluid into the subarachnoid space (13, 17), segmental necrotizing angiitis of cerebral vessels or increased permeability of vessels (14), fibrosis and obstruction of VRSs leading to impaired drainage of fluid (22), and leakage of interstitial fluid from the intracellular compartment into the VRSs (1). dVRSs are characteristically seen in 3 locations and have been classified based on their location into 3 types. Type I dVRSs occur in basal ganglia along the lenticulostriate arteries entering through anterior perforated substance (12). Type II dVRSs are found in the centrum semiovale along the perforating medullary arteries as they enter the cortex over the cerebral convexity and reach white matter (12). Type III or mesencephalic dVRSs are found in the midbrain, where they occur along the perforating branches of the collicular and accessory collicular arteries at the
WORLD NEUROSURGERY 84 [6]: 2074.e11-2074.e14, DECEMBER 2015
pontomesencephalic junction and along the posterior thalamoperforating arteries, paramedic mesencephalothalamic artery, and short and long circumferential arteries at the mesencephalothalamic junction (26). Giant dVRSs are significantly enlarged VRSs and most commonly involve the mesencephalothalamic region (supplied by the paramedial mesencephalothalamic artery) and cerebral white matter (12, 13, 21, 27). All cases of giant dVRS produce a mass effect of varying degrees, and may occasionally produce hydrocephalus (25, 27). Although the adjacent parenchyma usually does not show any alteration in signal intensity, cases with foci of hyperintensity on T2-weighted and fluid attenuation inversion recovery images have been reported (15, 27). As the giant dVRSs can assume bizarre configurations and can produce a mass effect, misinterpretation as other cystic lesions is quite possible (4, 6, 12, 13, 18, 27). They can easily be misdiagnosed as cystic neoplasms, parasitic cysts, non-neoplastic neuroepithelial cysts,
www.WORLDNEUROSURGERY.org
2074.E12
CASE REPORT AMANDEEP KUMAR ET AL.
ventricular diverticula, cystic infarction, and mucopolysaccharidosis, especially because dVRSs are usually periventricular in location (4, 6, 12, 13, 18, 27). A careful analysis of MRI, however, can help in differentiation. The presence of a solid component, contrast enhancement, perilesional edema, intensity of cyst contents different from intensity of CSF, and low signal intensity on diffusion-weighted images with high apparent diffusion values point toward the lesion being a cystic neoplasm (28). Giant dVRSs on the other hand are sharply demarcated, nonenhancing, purely cystic lesions in a characteristic location of perforating vessels with cyst contents displaying signal intensity similar to CSF in all MRI sequences. The surrounding brain parenchyma appears normal (3, 27). Although these radiologic features are almost pathognomonic of giant dVRSs, differentiation from other intracranial cystic lesions may be difficult in certain cases and in such a situation, clinical and radiologic follow-up may be helpful. The symptoms due to a large dVRS can be either due to midbrain compression (Benedikt syndrome or Parkinson-like symptoms) or due to obstructive hydrocephalus. Such patients can present with headache, dizziness, decreased concentration, impaired memory, impaired balance, dementia, and parkinsonism (9, 31); however, a direct correlation between clinical manifestations and radiologic findings is lacking (18, 29), except when the dVRS obstructs CSF pathways leading to hydrocephalus (27). dVRS may also be associated with certain metabolic and genetic disorders including mucopolysaccharidosis (16), Lowe syndrome (2), Coffin-Lowry syndrome (20), and myotonic dystrophy (7). The management of mesencephalic dVRSs should be aimed at addressing the symptoms due to hydrocephalus and those due to midbrain compression. Hydrocephalus due to dVRSs can be managed either with placement of a ventriculoperitoneal shunt (17) or with ETV (5, 10, 19, 24). However, symptoms due to midbrain compression cannot be relieved by CSF diversion alone (5, 10, 24) and in such cases, cyst fenestration and endoscopic cystoventriculostomy or cystocisternostomy is needed to relieve the midbrain compression (5, 10, 24). However,
2074.E13
www.SCIENCEDIRECT.com
GIANT MESENCEPHALIC DILATED VIRCHOW ROBIN SPACES
intraoperative bleeding in cysts has been reported in patients undergoing cyst fenestration (10, 24). Fiorendi et al. (10) have cautioned against internal exploration of cysts and have advised against taking a biopsy of the cyst wall as even slight traction can lead to rupture of intracystic arteries leading to hemorrhage and consequent neurologic deficits. Endoscopy offers the advantage of performing both CSF diversion by doing ETV and cyst decompression by doing cystoventriculostomy/cystocisternostomy. In the present case, the patient had symptoms caused by hydrocephalus only and not due to midbrain compression. Thus, we only performed ETV; cyst fenestration was not attempted. CONCLUSIONS dVRSs are commonly seen on MRI scans done for various reasons and are asymptomatic. However, giant dVRSs are a rare entity and are uncommonly encountered in clinical practice. Radiologically, dVRSs can closely mimic other intracranial cystic lesions. Knowledge about the existence of such lesions, their characteristic location, and careful analysis of MRI can help in making a correct diagnosis. Endoscopy can be used to perform third ventriculostomy as well as cyst fenestration in patients with symptomatic midbrain compression. REFERENCES 1. Adachi M, Hosoya T, Haku T, Yamaguchi K: Dilated Virchow-Robin spaces: MRI pathological study. Neuroradiology 40:27-31, 1998. 2. Barkovich AJ: Pediatric neuroimaging. Philadelphia: Lippincott Williams & Wilkins; 1999. 3. Bokura H, Kobayashi S, Yamaguchi S: Distinguishing silent lacunar infarction enlarged Virchow-Robin spaces: a magnetic resonance imaging and pathological study. J Neurol 245: 116-122, 1998. 4. Cakirer S: MR imaging findings in tumefactive perivascular spaces. Acta Radiol 44:673-674, 2003. 5. Conrad J, Welschehold S, Charalampaki P, van Lindert E, Grunert P, Perneczky A: Mesencephalic ependymal cysts: treatment under pure endoscopic or endoscope-assisted keyhole conditions. J Neurosurg 109:723-728, 2008. 6. Davis G, Fitt GJ, Kalnins RM, Mitchell LA: Increased perivascular spaces mimicking frontal lobe tumor. J Neurosurg 97:723, 2002.
7. Di Costanzo A, Di Salle F, Santoro L, Bonavita V, Tedeschi G: Dilated Virchow-Robin spaces in myotonic dystrophy: frequency, extent and significance. Eur Neurol 46:131-139, 2001. 8. Durant-Fardel M: Traite du ramollissement du cerveau. Paris, France: Balliere; 1843. 9. Fénelon G, Gray F, Wallace C, Poirier J, Guillard A: Parkinsonism and dilatation of the perivascular spaces (état criblé) of the striatum: a clinical magnetic resonance imaging and pathological study. Mov Disord 10:754-760, 1995. 10. Fiorindi A, Delitala A, Francaviglia N, Longatti P: Neuroendoscopic options in the treatment of mesencephalic expanding cysts: report of four cases and review of the literature. Clin Neurol Neurosurg 115:2370-2376, 2013. 11. Groeschel S, Chong WK, Surtees R, Hanefeld F: Virchow-Robin spaces on magnetic resonance images: normative data, their dilatation, and a review of the literature. Neuroradiology 48: 745-754, 2006. 12. Heier LA, Bauer CJ, Schwartz L, Zimmerman RD, Morgello S, Deck MD: Large Virchow-Robin spaces: MR-clinical correlation. AJNR Am J Neuroradiol 10:929-936, 1989. 13. Homeyer P, Cornu P, Lacomblez L, Chiras J, Derouesne C: A special form of cerebral lacunae: expanding lacunae. J Neurol Neurosurg Psychiatry 61:200-202, 1996. 14. Hughes W: Origin of lacunes. Lancet 2:19-21, 1965. 15. Komiyama M, Yasui T, Izumi T: Magnet resonance imaging features of unusually dilated VirchowRobin spaces—two case reports. Neurol Med Chir 38:161-164, 1998. 16. Lee C, Dineen TE, Brack M, Kirsch JE, Runge VM: The mucopolysaccharidoses: characterization by cranial MR imaging. AJNR Am J Neuroradiol 14: 1285-1292, 1993. 17. Mascalchi M, Salvi F, Godano U, Nistri M, Taluti R, Tosetti M, Villari N, Calbucci F: Expanding lacunae causing triventricular hydrocephalus: report of two cases. J Neurosurg 91: 669-674, 1999. 18. Ogawa R, Okudera T, Fukasawa H, Hashimoto M, Inugami A, Fujita H, Hatajawa J, Shimosegawa E, Noguchi K, Uemura K: Unusual widening of Virchow-Robin spaces: MR appearance. AJNR Am J Neuroradiol 16:1238-1242, 1995. 19. Ottenhausen M, Meier U, Tittel A, Lemcke J: Acute decompensation of noncommunicating hydrocephalus caused by dilated Virchow-Robin spaces type III in a woman treated by endoscopic third ventriculostomy: a case report and review of the literature. J Neurol Surg A Cent Eur Neurosurg 74 (Suppl 1):e242-247, 2013. 20. Patlas M, Joseph A, Cohen JE, Gomori JM: MRI and MRS of Coffin-Lowry syndrome: a case report. Neurol Res 25:285-286, 2003. 21. Poirier J, Barbizet J, Gaston A, Meyrignac C: Thalamic dementia. Expansive lacunae of the thalamo-paramedian mesencephalic area.
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2015.07.010
CASE REPORT AMANDEEP KUMAR ET AL.
Hydrocephalus caused by stenosis of the aqueduct of Sylvius. Rev Neurol (Paris) 139:349-358, 1983. 22. Pollock H, Hutchings M, Weller RO, Zhang ET: Perivascular spaces in the basal ganglia of the human brain: their relationship to lacunes. J Anat 191:337-346, 1997. 23. Robin C: Recherches sur quelques particularités de la structure des capillaires de l’encephale. J Physiol Homme Anim 2:537-548, 1859. 24. Rohlfs J, Riegel T, Khalil M, Iwinska-Zelder J, Mennel H-D, Bertalanffy H, Hellwig D: Enlarged perivascular spaces mimicking multicystic brain tumors. Report of two cases and review of the literature. J Neurosurg 102:1142-1146, 2005. 25. Romi F, Tysnes OB, Krakenes J, Savoiardo M, Aarli JA, Bindoff L: Cystic dilation of Virchow Robin spaces in the midbrain. Eur Neurol 47: 186-188, 2002. 26. Saeki N, Sato M, Kubota M, Uchino Y, Murai H, Nagai Y, Ishikura H, Nomura S, Matsuura I,
GIANT MESENCEPHALIC DILATED VIRCHOW ROBIN SPACES
Yamaura A: MR imaging of normal perivascular space expansion at midbrain. AJNR Am J Neuroradiol 26:566-571, 2005. 27. Salzman KL, Osborn AG, House P, Jinkins JR, Ditchfield A, Cooper JA, Weller RO: Giant tumefactive perivascular spaces. AJNR Am J Neuroradiol 26:298-305, 2005. 28. Tien RD, Felsberg GJ, Friedman H, Brown M, MacFall J: MR imaging of high-grade cerebral gliomas: value of diffusion-weighted echoplanar pulse sequences. AJR Am J Roentgenol 162: 671-677, 1994. 29. Ugawa Y, Shirouzu I, Terao Y, Hanajima R, Machii K, Mochijuki H, Furubyashi T, Kanazawa I: Physiological analyses of a patient with extreme widening of Virchow-Robin spaces. J Neurol Sci 159:25-27, 1998.
31. Vital C, Julian J: Widespread dilatation of perivascular spaces: A leukoencephalopathy causing dementia. Neuroradiology 48:1310-1313, 1997.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Patient consent: The patient /next of kin/guardian has consented to the submission of the case report for submission to the journal. Received 25 March 2015; accepted 1 July 2015 Citation: World Neurosurg. (2015) 84, 6:2074.e11-2074.e14. http://dx.doi.org/10.1016/j.wneu.2015.07.010 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com
30. Virchow R: Ueber die Erweiterung kleinerer Gefaesse. Arch Pathol Anat Physiol Klin Med 3: 427-462, 1851.
WORLD NEUROSURGERY 84 [6]: 2074.e11-2074.e14, DECEMBER 2015
1878-8750/$ - see front matter ª 2015 Elsevier Inc. All rights reserved.
www.WORLDNEUROSURGERY.org
2074.E14
