MedPix Medical Image Database COW - Case of the Week Case Contributor: Brendon G Tillman Affiliation: Uniformed Services University

MedPix No: History Pt Demographics: Age = 26 y.o. Gender = woman 26 y.o. obese woman with a Hx of persistent and disabling (8/10) global headache and a recent onset of blurred vision Downloaded by (-1)

MedPix No: EXAM & LABS Ocular Exam: Papilledema & Ocular pressure of 46 cmH2OLP (spinal tap) Opening Pressure of 20.5 cmH2OR Transverse sinus pressure gradient 13 mmHg (normal <4mmHg)

3D Reconstruction of Cerebral Sinus Venogram MRV showing right transverse sinus stenosis proximal to the sigmoid sinus/IJV junction. Downloaded by (-1)

3D Reconstruction of Cerebral Sinus Venogram MRV showing right transverse sinus stenosis proximal to the sigmoid sinus/IJV junction. Downloaded by (-1)

Sagittal Venogram of the Cranial Venous Sinuses Cerebral arteriogram & venography demonstrate a high-grade right transverse/sigmoid sinus stenosis distal to vein of Labbe connection. The stenosis was estimated to be at least 80% narrowing to approx diameter of 1mm and a pressure gradient accross the steneosis of 13 mmHg. Downloaded by (-1)

Sagittal Venogram of the Cerebral Venous Sinuses w/ arrow on transverse sinus stenosis Cerebral arteriogram & venography demonstrate a high-grade right transverse/sigmoid sinus stenosis distal to vein of Labbe connection. The stenosis was estimated to be at least 80% narrowing to approx diameter of 1mm and a pressure gradient accross the steneosis of 13 mmHg. Downloaded by (-1)

Coronal Venogram of the Cerebral Venous Sinuses w/ R carotid injection site Cerebral arteriogram & venography demonstrate a high-grade right transverse/sigmoid sinus stenosis distal to vein of Labbe connection. The stenosis was estimated to be at least 80% narrowing to approx diameter of 1mm and a pressure gradient accross the steneosis of 13 mmHg. Downloaded by (-1)

Coronal Venogram of the Cerebral Venous Sinuses w/ L carotid injection site Cerebral arteriogram & venography demonstrate a high-grade right transverse/sigmoid sinus stenosis distal to vein of Labbe connection. The stenosis was estimated to be at least 80% narrowing to approx diameter of 1mm and a pressure gradient accross the steneosis of 13 mmHg. Downloaded by (-1)

Coronal Venogram w/ balloon angioplasty of transverse sinus stenosis. Neurointerventional radiology endovascular procedure had successful balloon angioplasty and stent placement across the right transverse sinus. Downloaded by (-1)

Coronal Venogram w/ balloon angioplasty of transverse sinus stenosis. Neurointerventional radiology endovascular procedure had successful balloon angioplasty and stent placement across the right transverse sinus. Downloaded by (-1)

Coronal Venogram w/ stent placed along transverse sinus stenosis. Neurointerventional radiology endovascular procedure had successful balloon angioplasty and stent placement across the right transverse sinus. Downloaded by (-1)

Sagittal Venogram w/ stent placed along transverse sinus stenosis. Neurointerventional radiology endovascular procedure had successful balloon angioplasty and stent placement across the right transverse sinus. Downloaded by (-1)

FINDINGS MR Venogram (MRV) showing right transverse sinus stenosis, proximal to the sigmoid sinus and interntal jugular vein (IJV) junction. Cerebral catheter arteriogram & venography demonstrate a high-grade right transverse-sigmoid sinus stenosis, distal to the vein of Labbe connection. The stenosis was estimated as at least 80% narrowing, to an approximate diameter of 1mm with a pressure gradient across the stenosis of 13 mmHg. Neurointerventional radiology endovascular procedure was successful with a balloon angioplasty and stent placement across the stenosis in the right transverse sinus.

DIFFERENTIAL DIAGNOSIS What is your Differential Diagnosis? Obstruction of venous outflow, eg, venous sinus stenosis/thrombosis, jugular vein compression - Pseudotumor Cerebri (idiopathic intracranial hypertension) - Decreased CSF absorption (i.e. arachnoid granulation) - Increased cerebrospinal fluid (CSF) production (i.e. choroid plexus papilloma) - Intracranial mass lesions (tumor, abscess) - Obstructive hydrocephalus

Diagnosis: Pseudotumor Cerebri w/ right transverse sinus stenosis Dx Confirmed by: Clinical presentation, MRV, & Cerebral Venogram

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: McCarthy, KD, Viernstein, L. Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Pharmacol Exp Ther 1974; 189: Corbett, JJ, Thompson, HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46: 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.