Cerebral venous sinus thrombosis Hammad ghanchi

Anatomy - Sinuses Venous collection of blood between the periosteal and meningeal layers of the Dura, lined with endothelium Lack typical vessel layers & valves Drain: Blood CSF Ultimately drain into Internal Jugular Vein

Anatomy

Cerebral Venous Sinus Thrombosis Thrombosis within the venous system of the Brain ~ “DVT in the Brain” 3 types Dural Sinus Thrombosis Cortical Venous Thrombosis Deep Venous Thrombosis

Pathophysiology Closed vascular system Restriction of blood flow into brain due to the clot in the cerebral sinus blocking outflow (venous congestion) Cerebral Edema and Increased intracranial pressure Decreased inflow of oxygenated blood Infarction (termed ‘venous infarction’)

etiology Infection Pregnancy/Puerperium Oral Contraceptive Pills Dehydration Iatrogenic (post-cranictomy, transvenous pacemaker placement) Trauma Malignancy Inherited Thrombophilias Diabetes Mellitus Homocystinuria Behcet’s Syndrome Lumbar Punctures (rarely)

Saposnik G et al. Stroke. 2011;42:1158-1192

Age and sex distribution of cerebral venous and sinus thrombosis (CVT) in adults. Age and sex distribution of cerebral venous and sinus thrombosis (CVT) in adults. Bars represent the number of patients with CVT for the specific age/sex category. Data provided by Dr Jose Ferro from the International Study on Cerebral Venous and Dural Sinuses Thrombosis. Saposnik G et al. Stroke. 2011;42:1158-1192

Magnetic resonance venogram showing the cerebral venous system and most frequent (%) location of cerebral venous and sinus thrombosis, as reported in the International Study on Cerebral Venous and Dural Sinuses Thrombosis (n=624).44. Magnetic resonance venogram showing the cerebral venous system and most frequent (%) location of cerebral venous and sinus thrombosis, as reported in the International Study on Cerebral Venous and Dural Sinuses Thrombosis (n=624).44 Saposnik G et al. Stroke. 2011;42:1158-1192

Clinical presentation - history Headache (>90%) – no association between location and site of thrombosis Throbbing (9%) Band-like (20%) Thunderclap (5%) Other (pounding, exploding, stabbing, etc) (20%) Abnormal Vision Nausea/Vomiting Seizure Altered mental status Stroke-like symptoms (hemiplegia, hemiparesis, aphasia,etc) Ataxia Dizziness/Vertigo Wasay M et all, Headache in Cerebral Venous Thrombosis: incidence, pattern, and location in 200 consecutive patients. J Headache Pain. Apr 2010; 11(2):137-9

Clinical presentation – physical exam Mental Status Changes variable (no change to coma) Papilledema Cranial Nerve Deficits Cavernous sinus thrombosis -> extraocular motor dysfunction Thrombosis extending into jugular vein may develop jugular foramen syndrome (CN 9, 10, 11) Hemiparesis with Superior Sagittal Sinus involvement

Workup Non-Contrast Head CT – normal in 10-20 % of cases Hyperdense sinuses and veins; cord sign (right) is pathognomic Flame hemorrhages Small ventricles White matter edema

Workup Contrast-Enhanced Head CT Dura may enhance Gyral enhancement Dense deep white matter veins (collateral flow) Tentorial enhancement Empty Delta Sign (Right) Filling defect of superior sagittal sinus (pathognomonic)

Workup Head MRI Clot is acutely isointese on T1 and hypointense on T2 Subacute becomes hyperintense in T1 and T2 Late (>10 days) becomes black from flow void CT findings present on MRI

WorkUp Magnetic Resonance Venogram (MRV) Will demonstrate lack of blood flow Magnetic resonance venography confirmed thrombosis (black arrows) of right transverse and sigmoid sinuses and jugular vein. Saposnik G et al. Stroke. 2011;42:1158-c1192

Magnetic resonance venogram showing thrombosis (black arrows) of the superior sagittal sinus and sigmoid sinuses. Magnetic resonance venogram showing thrombosis (black arrows) of the superior sagittal sinus and sigmoid sinuses. A, 2 days after symptom onset. B, 1 year follow-up after oral anticoagulation therapy (OAC). Saposnik G et al. Stroke. 2011;42:1158-c1192

Workup IV Digital Subtraction Angiography Gold standard for diagnosis Rarely used due to invasive nature

Medical Management Correct underlying etiology Systemic Heparin Remains best treatment, even in the presence of intracerebral hemorrhage Avoid steroids (reduces fibrinolysis, increases coagulation) Control HTN Anticonvulsants if seizures present Hydrate aggressively as ICP tolerates

Anticoagulation First reported use in 1942 by Stansfield Supported by a small clinical trial of 20 patients 0 patients died on Heparin vs 3 deaths on placebo Trial terminated after 3 weeks Repeated again on 59 patients in 1999 Low molecular weight heparin vs placebo (for 3 weeks followed by oral warfarin for 3 months vs nothing) Favorable outcomes but not statistically significant Recent study compared Unfractionated Heparin vs Low Molecular Weight Heparin (2010) Showed better functionality at 6 months and decreased incidence of intracerebal hemorrhage with LMW Heparin No difference in complete recovery or mortality Randomized, placebo-controlled trial of anticoagulant treatment with low-molecular-weight heparin for cerebral sinus thrombosis. Stroke. 1999 Mar;30(3):484-8. PMID: 10066840 Anticoagulation for cerebral venous sinus thrombosis. Cochrane Database Syst Rev. 2011 Aug 10;(8):CD002005. doi: 10.1002/14651858.CD002005.pub2. PMID: 21833941 Unfractionated or low-molecular weight heparin for the treatment of cerebral venous thrombosis. Coutinho JM Stroke. 2010 Nov;41(11):2575-80. doi: 10.1161/STROKEAHA.110.588822. Epub 2010 Oct 7. PMID 20930161

Surgical intervention Monitor ICP if patient continues to deteriorate Ventriculostomy Thrombolytic therapy, sometimes directly into the sinus TPA Urokinase Streptokinase Decompressive craniectomy Decreases ICP but no proven improvement in outcome

Interventional Neuroradiology Penumbra system – sucks out the clot in small amounts MERCI retriever – corkscrew-shaped wire that pulls out clot Angiojet – efficacious for venous clots, but not arterial clots experimental . Used in severe cases as a last resort Penumbra - https://www.youtube.com/watch?v=ajcgsAr6K2A MERCI Retreiver - https://www.youtube.com/watch?v=P2TNz-TniIA Angiojet - http://vimeo.com/85367047

Follow-up/prognosis Visual loss with papilledema may be treated with optic nerve fenestrations Long term treatment after resolution of acute phase with heparin and/or warfarin for 3-12 months No clear guidelines Indicators of poor prognosis: Deep sinus involvement Extension into cortical veins Presenting Low GCS (Coma) Rapid deterioration (no time for collateral compensation) 10-day delay in diagnosis Hemorrhage on CT Papilledema http://www.medscape.com/viewarticle/514553_1

References Handbook of Neurosurgery 7th Edition, Mark S. Greenburg Wasay M et al, Headache in Cerebral Venous Thrombosis: incidence, pattern, and location in 200 consecutive patients. J Headache Pain. Apr 2010; 11(2):137-9 Denise, M. Lemke, Loffti. Cerebral Venous Sinus Thrombosis. Journal of Neuroscience Nursing. 2005. 37(5) p258-264 Layon, J et al. Textbook of Neurointensive Care 1st edition. December 2003. p417-419 Saposnik G et al.Diagnosis and Management of Cerebral Venous Thrombosis. Stroke. 2011;42:1158- 1192