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C.M. Aderman, HMSIII G. Lieberman, MD Cavernous Sinus Thrombosis Christopher M. Aderman, HMS year III Dr. Gillian Lieberman, MD April 2011 C.M. Aderman, HMSIII G. Lieberman, MD Agenda • Patient presentation • Relevant anatomy • Cavernous sinus thrombosis – Classic signs and symptoms – Pathogenesis • Differential diagnosis • Case resolution • Menu of radiologic tests • Companion cases • Radiologic imaging 2 C.M. Aderman, HMSIII G. Lieberman, MD Objectives 1. Learn the clinical presentation and differential diagnosis for cavernous sinus thrombosis 2. Understand the menu of radiologic tests available 3. Review orbital anatomy 3 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: HPI • 18 year old woman presented to university health center with fever, headache, cough, neck lymphadenopathy – Diagnosed with “infectious mononucleosis” • Several days later, she became lethargic and started to have rigors, admitted to OSH • At OSH, WBC: 17.7 (4% bands), creatinine: 2.5, BP: 80/40's, and GNR in blood • Antibiotics were initiated • The following morning, awoke with right facial numbness, double vision, and inability to open right eye • She was transferred to BIDMC for further care 4 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Medical History • • • • PMH: No medical history or hospitalizations Allergies: NKDA Medications: Ibuprofen as needed, no OCP Social Hx: College student, lives in the dorm; denies smoking, alcohol or illicit drug use. • Family Hx: No history of strokes or hypercoagulability 5 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Physical Exam • • • • • • • • • Vitals: T:99°F BP:99/50 HR:102 RR:36 O2 sat:99% Gen: Lying in bed, fatigued, NAD HEENT: NC/AT, moist oral mucosa Neck: Supple, no tenderness to palpation, normal ROM, no carotid or vertebral bruit; neck lymphadenopathy Back: No focal tenderness or erythema CV: RRR, normal S1 and S2, no murmurs/gallops/rubs Lung: Clear to auscultation bilaterally Abd: Soft, nontender , nondistended, normoactive bowel sounds Ext: No edema 6 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Neurologic Exam • Neuro: – Left cranial nerves II‐XII intact – Right eye papilledema, blurred disc margin – Right pupil 6 mm sluggish, cannot adduct or move eye superiorly or inferiorly – Right eye ptosis – Pain with eye movements – Diminished sensation in V1 and V2, sensation normal in V3 on right – Sensation, strength, reflexes, coordination normal 7 C.M. Aderman, HMSIII G. Lieberman, MD It appears that multiple cranial nerves are involved. Can we localize these abnormalities to one lesion in the brain? Let’s review the relevant anatomy. 8 C.M. Aderman, HMSIII G. Lieberman, MD Orbital Apex Anatomy 9 Drake, et al, Fig 8.83 C.M. Aderman, HMSIII G. Lieberman, MD Muscles at the Orbital Apex 10 Drake, et al, Fig 8.90 C.M. Aderman, HMSIII G. Lieberman, MD Orbital Anatomy Let’s review some anatomy on this coronal C+ CT at the level of the orbits. CNII (optic nerve) CNIII (oculomotor) Medial rectus Superior rectus Inferior rectus CNIV (trochlear) Superior oblique CNVI (abducens) Lateral rectus Superior ophthalmic vein PACS, BIDMC 11 C.M. Aderman, HMSIII G. Lieberman, MD Orbital Venous Drainage 12 Drake, et al, Fig 8.93 C.M. Aderman, HMSIII G. Lieberman, MD Venous Drainage of the Skull 13 Drake, et al, Fig 8.43 C.M. Aderman, HMSIII G. Lieberman, MD Cavernous Sinus Anatomy The cavernous sinus extends from the superior orbital fissure to the petrous portion of temporal bone. Cavernous sinus blood supply arises from the superior ophthalmic veins, cerebral veins, sphenoparietal sinuses, deep facial muscles, and inferior ophthalmic veins. 14 Drake, et al, Fig 8.44 C.M. Aderman, HMSIII G. Lieberman, MD With a better understanding of the orbital and cavernous sinus anatomy, we can now form a differential diagnosis for our patient’s ophthalmoplegia and cranial nerve findings. 15 C.M. Aderman, HMSIII G. Lieberman, MD Differential Diagnosis: Acute Painful Ophthalmoplegia • Cavernous sinus thrombosis (CN III, IV, VI, V1‐V2, superior ophthalmic vein) • Orbital apex syndrome (superior orbital fissure: CN III, IV, VI, V1, superior ophthalmic vein; optic canal: ophthalmic artery and optic nerve), our patient did not have impaired vision • Superior orbital fissure syndrome (CN III, IV, VI, V1), our patient had V2 involvement • Orbital cellulitis (periorbital swelling, proptosis, chemosis, ophthalmoplegia, fever, decreased vision, pain) • Preseptal cellulitis (no proptosis or ophthalmoplegia) 16 Colson AE, et al, 1999 Ebright JR, et al, 2001 C.M. Aderman, HMSIII G. Lieberman, MD Differential Diagnosis: Chronic Painful Ophthalmoplegia • Local malignancy, metastasis • Aseptic thrombus from trauma, myeloproliferative diseases, dehydration • Granulomatous diseases (TB or fungal, sarcoid, syphilis, Tolosa‐Hunt syndrome) • Aneurysm of internal carotid artery • Carotid‐cavernous fistula • Endocrine exophthalmos • Ophthalmoplegic migraine 17 Colson AE, et al, 1999 Ebright JR, et al, 2001 C.M. Aderman, HMSIII G. Lieberman, MD Given that our patient had acute onset of cranial nerve III, IV, VI, VI and V2 involvement, cavernous sinus thrombosis is the most likely diagnosis. Let’s look at the menu of radiologic tests available for further evaluation. 18 C.M. Aderman, HMSIII G. Lieberman, MD Cavernous Sinus Thrombosis: Menu of Radiologic Tests • MRI with and without contrast, MRV – Sensitive for detection of venous thrombus • CT with and without contrast – Usually the first study, may be normal in 30% • Before CT or MRI were available: – Clinical diagnosis or found at autopsy – Cerebral angiography or orbital venography • Difficult to puncture facial veins with edema • Also risky to inject contrast under pressure (disseminated infection, extension of thrombus) 19 Schuknecht B, et al, 1998 Chu K, et al, 2001 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis on Coronal MRI Coronal C‐ T1 weighted MRI at the level of the cavernous sinus Thickening of the right cavernous sinus (arrow) compared with the left 20 PACS, BIDMC C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis on Axial MRI Axial C‐ T1 weighted MRI at the level of the orbits Thickening of the right cavernous sinus (arrow) PACS, BIDMC 21 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis on MRA Three-dimensional time-offlight MR arteriography Right carotid artery (arrow) remains patent throughout its course through the cavernous sinus 22 PACS, BIDMC C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis on Axial CT The following day Axial C+ CT at the level of the orbits Mild enlargement of superior ophthalmic vein (arrow) Low attenuation region in the right cavernous sinus (arrow) representing thrombus 23 PACS, BIDMC C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis on Coronal CT Coronal C+ CT at the level of the cavernous sinus Areas of low attenuation within the cavernous sinus (arrow) also visible on this view PACS, BIDMC 24 C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis, Six Days Later Six days after presentation Coronal contrast enhanced T1 weighted MRI with fat suppression at the level of the cavernous sinus Right internal carotid narrowing (arrow) compared with left Meningeal thickening (arrow) along inferior aspect of temporal lobe consistent with meningitis 25 PACS, BIDMC C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous Sinus Thrombosis, Twelve Days Later Twelve days after presentation Right ICA narrowing (arrows) secondary to inflammatory changes along the wall MRA Coronal C+ T1 weighted MRI PACS, BIDMC 26 PACS, BIDMC C.M. Aderman, HMSIII G. Lieberman, MD Our Patient: Cavernous sinus thrombosis, MRA Comparison Absent flow through the right ICA compared with MRA at presentation (arrows). On the day of presentation Twelve days after presentation 27 PACS, BIDMC PACS, BIDMC C.M. Aderman, HMSIII G. Lieberman, MD Cavernous Sinus Thrombosis: Classic Signs and Symptoms Fever Ptosis Proptosis Chemosis Cranial nerve palsies 80-100% Lethargy Headache Periorbital swelling Papilledema Venous engorgement 50-80% Decreased visual acuity Decreased corneal reflex Sluggish or dilated pupil Nuchal rigidity Periorbital sensory loss < 50% Diplopia Seizures < 20% Hemiparesis 28 Southwick FS, et al 1986 C.M. Aderman, HMSIII G. Lieberman, MD Cavernous Sinus Thrombosis: Pathogenesis • Dural sinuses are valveless, susceptible to infection from multiple sites (sphenoids and ethmoids most common, also face, tonsils, soft palate, teeth, ears) • Enlarging infected clots can spread and involve both sides • Can result in sepsis, meningitis, subdural empyema, pituitary necrosis 29 Ebright JR, et al, 2001 C.M. Aderman, HMSIII G. Lieberman, MD Our patient: Case Resolution • Patient ultimately found to have dental infection as source. • Her course was complicated by septic shock, acute renal failure, DIC, septic pulmonary embolism, and respiratory failure necessitating intubation. • She eventually stabilized and was discharged on anticoagulation and antibiotics • Resolution of most cranial nerve symptoms with moderately decreased sensation in V1‐V2 30 C.M. Aderman, HMSIII G. Lieberman, MD Let’s view some companion cases of cavernous sinus thrombosis to further highlight the classic findings on radiologic imaging. 31 C.M. Aderman, HMSIII G. Lieberman, MD CST: Companion Case 1 Periorbital swelling, erythema, proptosis, chemosis (conjunctival edema) Enlarged superior orbital vein (arrows) Axial (C) and coronal (E) C+ CT Pavlovich P, et al, 2006 32 C.M. Aderman, HMSIII G. Lieberman, MD CST: Companion Case 2 Upper lid and periorbital edema, ptosis, chemosis, and conjunctival injection CT C+ axial slice through orbits Small collections of gas in both superior ophthalmic veins, the right cavernous sinus and the right upper lid soft tissue (arrows) 33 Pavlovich P, et al, 2006 C.M. Aderman, HMSIII G. Lieberman, MD CST: Companion Case 3 T1 with contrast showing multiple irregular defects within the enhancing cavernous sinus on the left side (arrowheads). 34 Yoshida T, et al, 2008 C.M. Aderman, HMSIII G. Lieberman, MD CST: Companion Case 4 Enhanced axial CT scan showing engorgement of left superior orbital vein with filling defect within it (arrow), with resultant proptosis of the left eye. 35 Eustis HS, et al, 1998 C.M. Aderman, HMSIII G. Lieberman, MD References • • • • • • • • • Chu K, Kang DW, Yoon BW, Roh JK. Diffusion‐weighted magnetic resonance in cerebral venous thrombosis. Arch Neurol. 2001;58(10):1569. Colson AE, Daily JP. Orbital apex syndrome and cavernous sinus thrombosis due to infection with Staphylococcus aureus and Pseudomonas aeruginosa. Clin Infect Dis. 1999;29(3):701‐702. Drake RL, Vogl W, Mitchell AWM, eds. Gray’s Anatomy for Students. Philadelphia, PA: Elsevier; 2005. Ebright JR, Pace MT, Niazi AF. Septic thrombosis of the cavernous sinuses. Arch Intern Med. 2001;161(22):2671‐2676. Eustis HS, Mafee MF, Walton C, Mondonca J. MR imaging and CT of orbital infections and complications in acute rhinosinusitis. Radiologic Clinics of North America. 1998;36(6):1165‐1183. Pavlovich P, Looi A, Rootman J. Septic Thrombosis of the Cavernous Sinus: Two Different Mechanisms. Orbit. 2006;25:39–43. Schuknecht B, Simmen D, Yüksel C, Valavanis A. Tributary venosinus occlusion and septic cavernous sinus thrombosis: CT and MR findings. AJNR Am J Neuroradiol. 1998;19(4):617‐626. Southwick FS, Richardson EP Jr, Swartz MN. Septic thrombosis of the dural venous sinuses. Medicine (Baltimore). 1986;65(2):82‐106. Yoshida T, Kasai T, Kondo M, et al. Septic cavernous sinus thrombosis caused by penicillin‐resistant Streptococcus. Infections in Medicine. 2008;25(115):20. 36 C.M. Aderman, HMSIII G. Lieberman, MD Acknowledgments • Dr. Gillian Lieberman, MD • Dr. Gul Moonis, MD • Dr. Rafael Rojas, MD • Emily Hanson 37