Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement

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Object. Pseudotumor cerebri, or benign intracranial hypertension, is a condition of raised intracranial pressure in the absence of a mass lesion or cerebral edema. It is characterized by headache and visual deterioration that may culminate in blindness. Pseudotumor cerebri is caused by venous sinus obstruction in an unknown percentage of cases. The purpose of this study was to investigate the role of cerebral venous sinus disease in pseudotumor cerebri and the potential of endoluminal venous sinus stent placement as a new treatment.

Methods. Nine consecutive patients in whom diagnoses of pseudotumor cerebri had been made underwent examination with direct retrograde cerebral venography (DRCV) and manometry to characterize the morphological features and venous pressures in their cerebral venous sinuses. The cerebrospinal fluid (CSF) pressure was measured simultaneously in two patients. If patients had an amenable lesion they were treated using an endoluminal venous sinus stent. Five patients demonstrated morphological obstruction of the venous transverse sinuses (TSs). All lesions were associated with a distinct pressure gradient and raised proximal venous sinus pressures. Four patients underwent stent insertion in the venous sinuses and reported that their headaches improved immediately after the procedure and remained so at 6 months. Vision was improved in three patients, whereas it remained poor in one despite normalized CSF pressures.

Conclusions. Patients with pseudotumor cerebri should be evaluated with DRCV and manometry because venous TS obstruction is probably more common than is currently appreciated. In patients with a lesion of the venous sinuses, treatment with an endoluminal venous sinus stent is a viable alternative for amenable lesions.

Article Information

Address reprint requests to: Brian K. Owler, M.B., B.S., Department of Neurosurgery, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Missenden Road, Camperdown New South Wales 2050, Sydney, Australia. email: brianowl@bigpond.com.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Upper: Simultaneous CSF pressure (measured through a Rickham reservoir) and SSS venous pressure recordings obtained during DRCV, demonstrating the close correlation between the SSS venous pressure and the CSF pressure as described by Davson, et al. Center Left: Right TS obstruction on DRCV (arrow). Center Right: Right TS (Tx) venous pressure recordings demonstrating the pressure gradient across the point of stenosis (center left) with simultaneous CSF pressure recording. Lower Left: Left TS obstruction on DRCV (arrow). Lower Right: Left TS venous pressure recordings demonstrating the pressure gradient across the point of stenosis (lower left) with simultaneous CSF pressure recording. T = torcular herophili.

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    Case 1. Pre- (left) and post- (right) stent DRCV of the left TS demonstrating its filling after placement of the stent (S).

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    Case 2. Upper: Right TS filling defect on DRCV (arrow). This stenosis was associated with a pressure gradient of 13 mm Hg. In the left TS a similar filling defect was seen that was associated with a pressure gradient of 12 mm Hg. Lower: Axial postcontrast CT scan and T2-weighted MR image demonstrating a small lesion in the left TS (arrows). An almost identical lesion was seen on the right side. The lesion had characteristics similar to those of fat.

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    Case 3. Anteroposterior DRCV study demonstrating the right venous sinus filling defect (arrows), which was associated with a pressure gradient of 25 mm Hg. A similar filling defect with a pressure gradient of 25 mm Hg pressure gradient was visible on the left side. JB = jugular bulb.

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    Case 4. A DRCV study of the right TS demonstrating a filling defect (arrows). There was a pressure gradient of 12 mm Hg across this obstruction. The other TS was hypoplastic.

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    Case 5. Left: An MR venogram demonstrating bilateral TS obstruction (arrows). Center: Cerebral arteriogram demonstrating bilateral TS narrowing (arrows). Right: Lateral DRCV demonstrating right and left venous sinus obstructions (arrows). These less discrete TS narrowings were associated with pressure gradients of 7 and 9 mm Hg, respectively.

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    Photomicrograph of a histological cross section of TS demonstrating a small brain hernia. This was associated with the development of a dural arteriovenous fistula. Similar brain hernias may be involved in the pathophysiological mechanisms of pseudotumor cerebri and may be responsible for some of the filling defects of the TSs that are evident in the cases presented.

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