Clinical Program for Cerebrovascular Disorders Mount Sinai Medical Center Superior Sagittal Sinus and Transverse Sinus Thrombosis Clinical Case Presentation Clara Raquel Epstein, MD Fellow Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Dural Sinus Thrombosis Involvement of Dural Sinuses and Other Veins in Descending Order of Frequency: Sinuses Superior Sagittal Sinus and Left Transverse Sinus (70% each) Multiple Sinuses in 71% Superficial Cortical Veins Deep Venous System (e.g. Internal Cerebral Vein) Cavernous Sinuses (Rare involvement) Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Pathophysiology Venous thrombosis reduces venous outflow from the brain. This venous engorgement causes white matter edema. The increased venous pressure may also lead to hemorrhage. Both hemorrhage and edema raise ICP. Thus, clinical findings may be due to elevated ICP, and focal findings may be due to edema and /or hemorrhage. SSS occlusion alone won’t cause cranial nerve findings except perhaps for visual obscuration and abducens (VI) nerve palsy from elevated ICP. Thrombosis in the jugular bulb may compress the nerves in the jugular foramen pars nervosa causing hoarseness, aphonia, difficulty swallowing and breathlessness. AKA Vernet’s Syndrome – or syndrome of the Jugular Foramen Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Common Diseases Associated with Dural Sinus Thrombosis Infection (usually local-otitis media, sinusitis, peritonsilar abscess) Pregnancy and Peurperium (Highest risk in first 2 weeks post-partum, Incidence 1/10,000 births) Oral Contraceptives Dehydration, Cachexia, Burns Cardiac Disease (CHF) Ulcerative Colitis Periarteritis Nodosa Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Common Diseases Associated with Dural Sinus Thrombosis Sickle Cell Trait Trauma (Seen in 10% of combat injuries involving the brain, may occur in the absence of skull fracture) Iatrogenic (e.g. s/p radical neck surgery, transvenous pacemaker placement, s/p craniotomy) Protein C Deficiency Diabetes Mellitus Homocystinuria Behcet’s Syndrome Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Clinical Presentation No pathognomonic findings; many signs and symptoms are due to elevated ICP May present as a syndrome clinically indistinguishable from pseudotumor cerebri High Incidence of Concurrent Thromboembolic Disease in Other Organs Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Clinical Presentation SSS Anterior 1/3 may occlude without sequelae. Posterior to this, venous infarction more likely to develop Mid-portion ->increased muscle tone ranging from spastic hemi- or quadraparesis Posterior ->field cuts or cortical blindness or massive CVA with cerebral edema and death Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Clinical Presentation TS TS may occur without deficit unless contralateral TS is hypoplastic, in which case presentation is similar to posterior SSS occlusion Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Clinical Presentation of Dural Sinus Thrombosis Sign/Symptom Series A Series B H/A 100% 74% N/V 75% - Seizures 70% 29% Hemiparesis 34% Papilledema 45% Blurred Vision 60% Altered Consciousness 35% 26% Series A: 20 Young Females- Estanol B, Rodriguez A, Conte G, et al.: Intracranial Venous Thrombosis in Young Women. Stroke 10:680-4, 1979. Series B: 38 cases from France- Bousser M G, Chiras J, Bories J, et al.: Cerebral Venous Thrombosis – A Review of 38 Cases. Stroke 16: 199-213, 1985. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Diagnosis CT Scan – Non-Contrast (may be normal in 20%) Findings include: Hyperdense sinuses and veins (high density clots in cortical veins produce the cord sign which is pathognomonic for cerebral venous thrombosis; seen in 2/30 patients) Petechial “flame” hemorrhages in unusual location for aneurysm or “hypertensive” hemorrhage) Small ventricles seen in 50% Thrombosis of SSS may produce high density in the region of Tocular herophili (AKA the Delta Sign) White matter edema Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Diagnosis CT Scan – Contrast Findings include: The dura around the sinus may enhance and become denser than clot in 35% of cases (AKA the empty delta sign) Gyral enhancement occurs in 32% Dense deep (white matter) veins (collateral flow) Intense tentorial enhancement (common Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Diagnosis Angiography Findings include: Non-filling of segments of sinuses, or filling defects in segments that are visualized Prolonged circulation time: present in 50% of cases Stumps and abnormal collateral pathways MRI/MRA/MRV LP – OP usually increased, CSF bloody or xanthochromic Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Treatment Should be aggressive-Recoverability of the brain is greater than with arterial occlusive stroke Complicated management because measures that counteract thrombosis (e.g. anticoagulation) tend to increase the risk of hemorrhagic infarct and measures that tend to lower ICP tend also to increase blood viscosity leading to increased coagulability Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Medical Management Heparin Control hypertension Anticonvulsant to control seizures Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Medical Management Correct underlying abnormality (antibiotics for infection, etc.) Monitor ICP with ventriculostomy Hydrate aggressively as ICP tolerates Measures to lower ICP: Elevate HOB Hyperventilation Drain CSF Pentobarbital Coma Hyperosmotic and/or loop diuretics last and replace fluid loss with isotonic IV fluids to prevent dehydration (I.e. goal is hypertonic euvolemia) Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Aggressive Treatment When medical management fails or in the setting of deteriorating presentation with progressive deficits Direct “attack” on clotted sinus Urokinase, streptokinase, or tPA either systemically or infused directly into clotted sinus Direct surgical treatment-thrombectomy and sinus reconstruction Decompressive craniectomy (+/-decompressive lobectomy) this decreases ICP but may not improve outcome Visual loss with papilledema may be treated with optic nerve sheath decompression Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Prognosis Mortality Approximately 30% (Range 5-70% reported in the literature) Poor prognisticators include Coma Extremes of age (infancy or elderly) Rapid neurologic deterioration Focal signs Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Sagittal Sinus and Transverse Sinus Thrombosis Clinical Case Presentation A 41 year old right handed female (G2P2) without significant past medical history, and not on oral contraceptives, was transferred to Mount Sinai Medical Center on 9/15/99 from Long Island for evaluation of Sagittal Sinus and Transverse Sinus Thrombosis and papilledema. One week prior to admission the patient was diagnosed with bronchitis and sinusitis with questionable otitis media and was on Levaquin for four days. The patient reported that on Sunday, 9/12/99 prior to admission, while playing tennis, she developed a severe occipital headache associated with nausea and vomiting. She went to the emergency department and was administered toradol (IM) with significant relief and was sent home with a diagnosis of migraine headaches. The next day the headaches worsened and she progressively developed difficulty writing and difficulty moving her right upper and lower extremities. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Hospital Course On 9/15/99 an MRI was obtained which showed evidence of a Sagital Sinus and Left Transverse Sinus Thrombosis. The patient was transferred to Mount Sinai Medical Center and admitted to the NSICU for further evaluation and medical management. On admission the patient presented with evidence of bilateral papilledema R>L, and slight right upper extremity pronator drift. She had obvious impairment of her handwriting but was otherwise neurologically intact. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Hospital Course Neurosurgery was consulted and it was determined on arrival that the patient’s condition was stable with minimal neurological deficits and would benefit from medical management. In light of the patient’s presentation and findings, she did not require direct intrasinus thrombolysis via interventional radiologic procedures. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Hospital Course The patient was started on heparin per protocol and Diamox. Cautious hemodynamic and neurologic monitoring was continued. The patient was further evaluated for hypercoagulability. In addition, PT/OT was consulted to address the handwriting deficits. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Hospital Course On hospital day # 3 the patient was started on coumadin. The heparin was continued until the INR was within a therapeutic range, at which time the heparin was discontinued. Ophthalmology was consulted to further evaluate the patient. Throughout the hospital course the patient remained stable. On hospital day #7 the patient was deemed stable for discharge to home. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Hospital Course On 10/17/99 the patient was seen for follow up and MRI/MRA/MRV were obtained for further evaluation on 10/19/99. The patient will continue coumadin for a duration of one year with repeat studies to be performed in six months. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Discussion Superior Sagittal Sinus thrombosis is often accompanied or precipitated by thrombosis of the transverse sinus. Propagation of infection from the petrous bone to cause thrombophlebitis of the sigmoid and transverse sinuses was a common cause of increased intracranial pressure (“otitic hydrocephalus”) often seen in children prior to the widespread use of antibiotics. This etiology of dural sinus thrombosis is still observed on occasion. It is often seen in the case of chronic otitis media, with evidence of inflammatory thickening of the mucosa in the mastoid region which can be seen on CT scans. Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Sagittal Sinus and Transverse Sinus Thrombosis Clinical Case Adam Davis, MD Interventional Neuroradiology Louis Aledort, MD Hematology Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Diagnostic Studies 9/15/99 CT Head 9/16/99 MRA/V 9/20/99 MRI Brain 10/20/99 MRI/MRA/MRV Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. Literature Review Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. HCT 9-15-99 Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. MRI 9-16-99 Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
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Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved. MRI/A/V 10-8-99 Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.
Copyright 1999, Clara Raquel Epstein, MD All Rights Reserved.