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Daily bibliographic review of the Neurosurgery Department. La Fe University Hospital. Valencia, Spain

Hypertensive slit ventricle syndrome: pseudotumor cerebri with a malfunctioning shunt?

slit ventricles

J Neurosurg 119:1503–1510, 2013

Symptomatic shunt malfunction without ventricular enlargement is known as slit ventricle syndrome (SVS). Patients presenting with this syndrome are not a homogeneous group. Of the 5 different types classified by Rekate, Type 1 is caused by CSF overdrainage and is associated with low pressures; Types 2 and 3 are associated with shunt blockage and elevated CSF pressures; Type 4 is cephalocranial disproportion that increases brain parenchymal pressure but not CSF pressure; and Type 5 is headache unrelated to shunt function. The low and normal CSF pressure types are relatively well understood, but the high-pressure forms are more problematic. In the high-pressure forms of SVS it is said that the lack of ventricular dilation is related to a reduction in brain compliance analogous to idiopathic intracranial hypertension or pseudotumor cerebri. Despite this, there is little evidence in the literature to support this conjecture.

With this in mind, 3 cases of SVS associated with elevated CSF pressure are presented. The MR venogram findings and hemodynamics of these 3 cases are shown to be identical to those of pseudotumor cerebri. A literature review indicates that an underlying venous impairment may be functioning in the patients who re-present with small ventricles following shunt malfunction.

Category: hydrocephalus

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