- Department of Neurosurgery, Faculty of Life Sciences, Kumamoto University School of Medicine, 1-1-1, Honjo, Kumamoto, Kumamoto, Japan
Department of Neurosurgery, Faculty of Life Sciences, Kumamoto University School of Medicine, 1-1-1, Honjo, Kumamoto, Kumamoto, Japan
DOI:10.4103/2152-7806.102942Copyright: © 2012 Fujimoto K. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Fujimoto K, Kuroda J, Hide T, Hasegawa Y, Yano S, Kuratsu J. Giant tumefactive perivascular spaces that expanded and became symptomatic 14 years after initial surgery. Surg Neurol Int 27-Oct-2012;3:127
How to cite this URL: Fujimoto K, Kuroda J, Hide T, Hasegawa Y, Yano S, Kuratsu J. Giant tumefactive perivascular spaces that expanded and became symptomatic 14 years after initial surgery. Surg Neurol Int 27-Oct-2012;3:127. Available from: http://sni.wpengine.com/surgicalint_articles/giant-tumefactive-perivascular-spaces-that-expanded-and-became-symptomatic-14-years-after-initial-surgery/
Background:Perivascular spaces (PVSs) or Virchow–Robin spaces in the brain are pial-lined interstitial fluid (ISF)-filled structures surrounding the penetrating arteries and arterioles. These spaces appear as 1- to 2-mm in diameter, round, oval, or curvilinear smooth-walled structures on magnetic resonance imaging (MRI). Typical PVSs are asymptomatic. Occasionally, they become enlarged and cause specific clinical manifestations that depend on location and the degree of tissue compression. In this case, they are referred to as giant tumefactive PVSs. To our knowledge, there have been no reported cases in which giant PVSs increased remarkably in number and size during both the natural course and postoperative course. We describe a rare progression of giant tumefactive PVSs 14 years after initial surgery.
Case Description:On first admission at age 17, endoscopic ventriculocystostomy and third ventriculostomy were performed to relieve hydrocephalus caused by cysts compressing the cerebral aqueduct. Fourteen years later, the multicystic lesion reappeared with an increase in both cyst number and size. The patient showed no hydrocephalus but presented with oculomotor and trochlear nerve palsies, which were caused by a mass effect on the midbrain. Endoscopic ventriculocystostomy was performed and symptoms improved.
Conclusion:This is the first case report in which giant PVSs increased significantly in number and size.
Keywords: Hydrocephalus, neuroendoscopic surgery, Virchow–Robin space
Perivascular spaces (PVSs) or Virchow–Robin spaces in the brain are pial-lined interstitial fluid (ISF)-filled structures surrounding the penetrating arteries and arterioles.[
A 17-year-old male was referred to our hospital with progressive headache and nausea. Neurological examination showed papilledema, and MRI [Figure
Initial MRI findings in a 17-year-old patient who presented with progressive headache and nausea. Axial T1-weighted (a) T2-weighted (b) contrast-enhanced T1-weighted (c) and sagittal T2-weighted (d) MR images revealed a multicystic lesion in the midbrain and thalamus causing aqueduct stenosis. A T2-weighted image (e) obtained after endoscopic ventriculocystostomy and third ventriculostomy showed a reduction in ventricular size, with no change in the multicystic lesion
Fourteen years later, at age 31, the patient was readmitted to our hospital with progressive double vision and nausea. Neurological examination revealed anisocoria (right pupil: 3 mm, left pupil: 5 mm), and the light reflex was dull in both pupils. Left eye exotropia in the primary position was present. When the patient tilted his head to the right, double vision worsened, while titling to the left resolved the double vision (Bielshowsky sign positive). Eye movement during tracking of moving objects was normal. These findings indicated left oculomotor and right trochlear nerve palsies. MRI [Figure
Increase in cyst number and size 14 years after initial surgery. T2-weighted (a) and sagittal T2-weighted (b) MR images revealed a multicystic lesion in the midbrain and thalamus that had increased in number and size, but no hydrocephalus was observed. Axial T2-weighted (c) and sagittal T2-weighted (d) MR images obtained after the second surgery showed a slight reduction in the size of the multicystic lesion
We speculated that compression of the midbrain was the most probable cause of symptoms and performed neuroendoscopic surgery. Two frontal precoronal burr holes were drilled. Through the left burr hole, we inserted the rigid endoscope with an operating sheath (Karl Storz Inc., Germany) and a neuronavigational guidance probe (Medtronic Inc., USA) into the third ventricle. Through the right burr hole, we inserted the flexible endoscope VEF-IV (Olympus, INC. Japan) with EMF System Pal-1 (Japan Medical Dynamic Marketing Inc., Japan) into the third ventricle. Thin-walled cysts were observed in the third ventricle [
After surgery, the symptoms and neurological disorder improved. Postsurgical MRI [Figure
Histological section of a biopsied cyst wall strained with hematoxylin and eosin (a) and immunostained for the astrocytic marker glial fibrillary acidic protein (GFAP) (b) or for the neuronal marker microtubule-associated protein (MAP2) (c) GFAP immunostaining revealed extensive gliosis in the cyst wall. Epithelial cells on the outer aspects appeared to be ependymal cells compressed by the multicystic lesion. No neurons were identified by MAP2 staining. Original magnification: ×400
Virchow–Robin spaces in the brain are normal anatomical structures, but they are often too small for MRI detection.[
The precise etiology of the dilation of Virchow–Robin spaces remains obscure. Several hypotheses have been suggested as follows:[
Giant tumefactive PVSs have typical MR imaging features. These are round to oval in shape with a smooth margin along the path of penetrating arteries, are isointense relative to CSF, and show no enhancement with contrast media.[
The most common location of giant tumefactive PVSs is the mesencephalothalamic region in the territory of the paramedian mesencephalothalamic artery.[
According to Salzman et al.,[
Asymptomatic cases do not necessarily require any treatment. Longitudinal studies of patients with dilation of PVSs have not been conducted, but follow-up imaging studies in cases published to date have yet to document an increase in PVS size during the natural course.[
To the best of our knowledge, there are no published reports documenting giant PVSs that increased in number and size during both the natural course and postoperative course with emergence of new symptoms. In our case, giant PVSs were sufficiently large to produce a symptomatic mass effect (left oculomotor and right trochlear nerve palsies) 14 years after third ventriculostomy and fenestration of the cysts to relieve obstructive hydrocephalus. As observed in many previous cases, the etiology of giant PVSs progression after initial surgery is obscure. This progression may be affected by initial surgery or may be part of the natural course.
Our case demonstrated that giant PVSs can increase in number and size in the intervening years following surgery.
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