British Journal of Neurosurgery, February 2015; 29(1): 100–102 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.957160

SHORT REPORT

Giant perivascular spaces causing hemiparesis successfully treated by cystoventriculoperitoneal shunt Roland Roelz1,2, Karl Egger3 & Peter Reinacher1 1Department of Stereotactic and Functional Neurosurgery, University Medical Center Freiburg, Freiburg, Germany, 2Department of Neurosurgery, University Medical Center Freiburg, Freiburg, Germany, and 3Department of Neuroradiology,

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University Medical Center Freiburg, Freiburg, Germany symptoms. Very rarely, massive expansion of these spaces as single or multicystic lesions can cause neurological symptoms and hydrocephalus requiring surgical intervention.

Abstract We report a 55-year-old man affected by a slowly progressive hemiparesis. Imaging revealed giant dilations of brain perivascular spaces, or Virchow–Robin spaces of the thalamomesencephalic region and occlusive hydrocephalus. Stereotactic catheter cystoventriculostomy alone reverted hemiparesis. However, conversion of the catheter into a peritoneal shunt system was necessary to treat hydrocephalus.

Case report A 55-year-old man reported about a mild and very slowly progressing weakness of his left arm and leg, first noticed 5 years before his presentation to our department. He did not seek medical attention until his colleagues noticed limping of his left leg. There was no other significant past medical history, but a medically treated hypertension and obesity (BMI, 31). Neurological examination revealed a grade-4/5 left hemiparesis. Stretch reflexes were slightly increased on the left side with a negative Babinski sign. The patient was able to walk without limitations, but a slight circumduction of the left leg was present. Further neurological and general examination was unremarkable. Notably, there was no cognitive impairment, urinary incontinence or unsteady gait. A cranial magnetic resonance imaging (MRI) (Fig. 1) showed a polycystic space occupying lesion (5 ⫻ 3.5 cm in

Keywords: cyst; hydrocephalus; supratentorial tumour; surgical management

Introduction Small vessels extending into the brain parenchyma are surrounded by perivascular spaces (PVS), or Virchow–Robin spaces, formed by pial extensions filled with interstitial fluid. Dilated PVS (dPVS) are highly prevalent in healthy individuals of all ages and can be detected in most brain areas. Typically, large dilation of PVS represents an incidental imaging finding that is not associated with clinical

Fig. 1. Pre-operative T2w MRI of the head showing massively dilated perivascular spaces – or Virchow-Robin spaces – (5 ⫻ 3.5 cm) of the right thalamo-mesencephalic region extending into the right basal ganglia. Obstructive hydrocephalus due to compression of the third ventricle and the aqueduct of Sylvius is present.

Correspondence: Dr. Roland Roelz, Department of Stereotactic and Functional Neurosurgery, University Medical Center Freiburg, Breisacher Str. 64, 79106 Freiburg, Germany. Tel: ⫹ 49-761-270-50010. E-mail: [email protected] Received for publication 20 May 2014; accepted 16 August 2014

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Fig. 2. Post-operative cranial imaging. (a) Sagittal T2w MRI following introduction of cystoventricular catheter. Hydrocephalus appears unchanged. (b) Axial T2w MRI 2 weeks after surgery showing fluid egress from the ventricles along the catheter into the subdural space over the right hemisphere causing hygroma. (c) Axial CT after connection of the cystoventricular catheter to a peritoneal catheter and evacuation of the right-sided subdural hygroma by a 3.5 mm burr hole drainage. Ventricles are collapsed and subdural hygroma has resolved.

diametre) of the right thalamomesencephalic region extending into the right basal ganglia, leading to compression of the third ventricle and the aqueduct of Sylvius with consecutive hydrocephalus. Flow void along the aqueduct was absent on sagittal T2w imaging. The cyst fluid was isointense to the signal of the cerebrospinal fluid (CSF) and there was no pathological enhancement upon gadolinium administration. Arguing that a size reduction of the lesion could reestablish patency of the aqueduct and thus relieve hydrocephalus, we first performed a cystoventriculostomy by inserting a ventricular catheter with openings both within the cysts and the right lateral ventricle via a right frontal trajectory using a stereotaxy system under general anaesthesia. The catheter was connected to a subcutaneus punction chamber to allow for ventricular access. Simultaneously, a biopsy was taken from a cyst wall. Histopathology revealed normal central nervous system (CNS) tissue without signs of inflammation or neoplasia (not shown). Immediately after the procedure, the patient experienced a near-complete resolution of his left hemiparesis. MRI of the brain 2 days after surgery (Fig. 2a) showed a slight reduction in the polycystic lesion and unchanged hydrocephalus. Obstruction of the aqueduct persisted as a T2w flow void signal was absent. Because a significant neurological improvement was present, the patient was discharged despite imaging findings of persisting hydrocephalus. At a scheduled clinical examination 2 weeks later, the patient was doing well without neurological deficits and complaints. Repeat MRI now demonstrated a slightly decreased size of the cystic lesion and ventricles, but T2w hyperintensity along the ventricular catheter and a subdural CSF isointense fluid collection (Fig. 2b and c) implying fluid egress from the ventricle into the frontal brain parenchyma and the subdural compartment through the trajectory of the catheter. Obviously, hydrocephalus persisted as the normal route of liquor circulation via the aqueduct had not been restored despite shrinkage of the cystic lesion. In a second operation, the cystoventricular

catheter was connected to a shunt valve (miniNAV 10, Miehtke-Aesculap, Germany) – the catheter was subcutaneously tunnelled to the abdomen and inserted in the peritoneal cavity. At the same time, a right frontal 3.5 mm burr hole was drilled, a catheter was inserted into the subdural space and the subdural hygroma was released. Three days after surgery, collapse of the ventricles and resolution of the subdural hygroma were documented by computed tomography of the head (Fig. 2d). The patient was discharged and remained without problems upon regular outpatient visits at 6 months after diagnosis and surgery.

Discussion Dilated PVS are common benign lesions and have consistently been associcated with aging and vascular pathologies.1 However, the precise mechanisms underlying expansion of PVS are poorly understood. Typical MRI features help to distinguish dPVS from other cystic brain lesions like low-grade glioma and parasitic infections, in order to avoid unnecessary invasive diagnostics and treatments.1 Symptomatic massive expansion of PVS, then termed giant or tumefactive PVS, is extremely rare and warrants neurosurgical intervention. The procedures performed to treat symptomatic giant PVS are either directed to treat hydrocephalus or to reduce cyst volume by drainage or a combination of both depending on the symptoms and the type of the lesion.2 Frequently applied methods comprise low-risk endoscopic and stereotactic surgeries, such as ventriculo-cisternostomy, thirdventriculostomy and cystostomy or cystoventriculo- and cystocisternostomy.3 We assume that a very slow expansion over years accounts for the slowly progressive hemiparesis in our patient supporting the rarely reported finding of growing giant PVS. A stereotactic procedure was chosen as the lesion consisted of multiple small cysts poorly accessible by endoscopy. The two-step procedure performed in this case demonstrates that cystoventriculostomy alone was sufficient to revert hemipa-

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resis, but not to treat obstructive hydrocephalus as aqueduct compression persisted. Peritoneal drainage resulted in definitive treatment in our patient.

Conclusion

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Dilated perivascular spaces are normal anatomical findings and need to be differentiated from morphologically similar lesions to avoid unnecessary invasive diagnostic and treatment. Massive expansion very rarely leads to hydrocephalus and/or various neurological symptoms due to local mass effect. In these cases, neurosurgery is indicated. The type of intervention needs to be tailored according to the location, extension and configuration of the lesion.

Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

References 1. Zhu YC, Dufouil C, Mazoyer B, et al. Frequency and location of dilated Virchow-Robin spaces in elderly people: a populationbased 3D MR imaging study. Am J Neuroradiol 2011;32: 709–13. 2. Sturiale CL, Albanese A , Lofrese G, et al. Pathological enlargement of midbrain Virchow-Robin spaces: a rare cause of obstructive hydrocephalus. Br J Neurosurg 2011;25:130–31. 3. House P, Salzman KL, Osborn AG, et al. Surgical considerations regarding giant dilations of the perivascular spaces. J Neurosurg 2004;100:820–4.

Giant perivascular spaces causing hemiparesis successfully treated by cystoventriculoperitoneal shunt.

We report a 55-year-old man affected by a slowly progressive hemiparesis. Imaging revealed giant dilations of brain perivascular spaces, or Virchow-Ro...
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