Tx and Followup:
In this patient had stenosis of her right transverse with a pressure gradient of 13mm Hg across the lesion. Right and left cerebral angiography followed by venogram was performed to better identify the location of the right transverse stenosis and surrounding vessels in preparation for stenting. After seeing the patient several times before in clinic for an adequate pre-operation work-up she was scheduled for right transverse sinus stenosis. Access was obtained from the left common femoral vein and angioplasty of the right transverse sinus was performed and a Zilver 7 x 60 mm stent was placed. Status post surgery the patient had improved symptoms to include improved vision and significant decrease in pain from headaches. Hope to continue with close follow-up with patient.
Additional Discussion:
Pseudotumor cerebri also known as idiopathic intracranial hypertension (IIH) is defined by a class clinical symptoms that occur in the setting of elevated intracranial pressure and normal composition of cerebrospinal fluid. Classic clinical symptoms include headaches, vision changes (including vision loss), hearing changes (tinnitus), and the disease is typically seen in obese females of childbearing age. Papilledema is the most common physical exam finding, but visual field loss and sixth nerve palsy may also be seen as well. Symptoms may become severe to include severe disabling headaches and complete vision loss. Brain computed tomography (CT) and magnetic resonance imaging (MRI) are typically normal, however, the following findings may be seen, but are currently not diagnostic:
– Cerebral venous sinus stenosis
– Flattening of the posterior sclera
– Contrast enhancement of the prelaminar optic nerve
– Empty sella
– Distension of perioptic subarachnoid space
– Intraocular protrusion of the prelaminar optic nerve
– Orbital optic nerve vertical tortuosity
Treatment for pseudotumor cerebri typically includes medical management with acetazolamide and pain control for headaches. Furosemide (Lasix®) and corticosteroids can be used as well. Surgical interventions include lumbo-peritoneal shunt (LPS) and ventriculo-peritoneal shunt (VPS), which often produce immediate results. However, about 50% there is a regression back to pseudotumor symptoms, more often with LPS than VPS.
Optic nerve sheath fenestration is also sometimes used to treat visual changes or loss of vision. Venous stenting of the stenosis of the cerebral sinuses is a relatively new way of treating pseudotumor. Current articles show mixed results, but some studies show that patients with pseudotumor may have some cerebral sinus stenosis approximately 30 – 90% of the time. Whether the stenosis is a primary or secondary cause is unknown.
References:
Friedman, DI, Jacobson, DM. “Diagnostic criteria for idiopathic intracranial hypertension.” Neurology 2002; 59:1492.
McCarthy, KD, Viernstein, L. “Long-term intracranial pressure recording in the management of pseudotumor cerebri.” J Pharmacol Exp Ther 1974; 189:194.
Corbett, JJ, Thompson, HS. “The rational management of idiopathic intracranial hypertension.” Arch Neurol 1989; 46:1049.
Donnet, A, Metellus, P, Levrier, O, et al. “Endovascular treatment of idiopathic intracranial hypertension: clinical and radiologic outcome of 10 consecutive patients.” Neurology 2008; 70:641.
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- Pseudotumor Cerebri w/ right transverse sinus stenosis
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