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Acute Proptosis Mark Soontornvachrin, MD Raghu Mudumbai, MD Ophthalmology Grand Rounds August 9, 2007 History CC: Right eye swelling HPI: 19 y/o F with acutely progressive swelling and decreased vision OD x 4 days Poor historian Since swimming 5 days PTA, increasing facial pain, and RE swelling “Incoherent” per grandfather 1 day PTA Noted by family to hit herself in the RE several times while asleep 1 day PTA Taken to OSH morning of admission and seen by outside ophthalmology consult Started on treatment for high IOP OD (90s by TonoPen): Diamox IV, Cosopt, Alphagan Transferred to HMC for definitive care History POH: No surgery/trauma PMH: ADHD, developmental delay Meds: Zyprexa, Klonipin All: NKDA SH: Denies T/E/D; from Arlington, WA FH: No ocular disease ROS: Subjective fever, HA, malaise Exam Vitals: T 101.2 (at OSH); other VS stable External: Prominent R proptosis with RUL/RLL edema, RUL ptosis OD OS VA NLP 20/30 P 3→2 3→2 TP (5%) 28 12 EOM Frozen Full +APD OD Exam SLE LLL: See previous → WNL S/C: Hemorrhagic chemosis → WNL K: Clear OU AC: D&Q OU I: WNL OU L: WNL OU NDFE No disc swelling/pallor OU Exam Questions? Differential Diagnosis Differential Diagnosis Orbital cellulitis Orbital subperiosteal abscess Orbital apex syndrome Cavernous sinus thrombosis Idiopathic orbital inflammation Orbital mass/tumor Thyroid-associated orbitopathy Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Imaging Summary 19 y/o F with acute onset unilateral proptosis R NLP, APD, frozen globe R pansinusitis R orbital cellulitis R medial orbital subperiosteal abscess R cavernous sinus thrombosis Differential Diagnosis Orbital cellulitis Orbital subperiosteal abscess Orbital apex syndrome Cavernous sinus thrombosis Idiopathic orbital inflammation Orbital mass/tumor Thyroid-associated orbitopathy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy Cavernous Sinus: Anatomy A: ICA B: CN III C: CN IV D: CN VI E: CN V1 F: CN V2 Cavernous Sinus: Tributaries Cavernous Sinus: Drainage Cavernous Sinus: Neuroimaging Cavernous Sinus Thrombosis (CST) Thrombophlebitic process affecting the cavernous sinus Most commonly infectious etiology Occurs as sequelae of local infection (often concurrently) Orbital cellulitis Subperiosteal abscess Orbital abscess Sinusitis CST: Pathogenesis Cavernous sinuses lack valves; allows bi- directional spread of infection Local spread from infectious source (ie. infected sinus) via draining veins as contiguous phlebitis Septic emboli from distant source Bacterial growth induces thrombosis Thrombus acts as good growth medium for more bacterial growth CST: Sources of Infection Paranasal sinusitis Ethmoid Sphenoid Nasal furunculosis Oral/dental infections Middle ear infections Organisms Staphylococcus aureus (70%) Streptococcus sp. (20%) Gram negatives (5%) Rarely fungal (immunocompromised) CST: Epidemiology Typically young adults Uncommon, no incidence data Fatal prior to antibiotic era (pre-1940s) Mortality estimate: 14-79% Morbidity estimate: 50% Cranial neuropathies Visual loss CST: Clinical Presentation Time from initial infection to presentation usually between 1-21 days (average 5-6 days) Systemic features (sepsis) Headache Fever Tachycardia Hypotension Mental status changes CST: Ocular Findings Classically unilateral, then bilateral within days Venous congestion Chemosis Proptosis Retinal vein dilatation External ophthalmoplegia Restriction from orbital venous congestion Cranial nerve palsies (CN III, VI, IV) Ophthalmic anesthesia / maxillary anesthesia Horner’s syndrome Visual loss (rare in isolated CST) Occlusion of ICA, ophthalmic artery, CRA Ischemic optic neuropathy CST: Complications Intracranial infection Meningitis Encephalitis Abscess Pituitary insufficiency Hemorrhagic infarction Death CST: Work-Up CBC Blood cultures Lumbar puncture Neuroimaging (CT, MRI) Expansion of cavernous sinuses Convex bowing of lateral wall Abnormal filling defects Dilation of superior ophthalmic vein Dural enhancement of cavernous sinus border CST: Neuroimaging CST: Treatment Empiric high dose IV antibiotics Third generation cephalosporin Anti-staphylococcal penicillin Metronidazole Continued treatment with IV abx for at least two weeks after apparent clinical resolution Surgical drainage of primary infection sites Steroids controversial (except if pituitary insufficiency) CST: Treatment Anticoagulation No consensus for use despite theoretical rationale Risks include systemic and intracranial bleeding 2 cases of intracranial hemorrhage and 2 cases of systemic hemorrhage reported in literature No prospective randomized trials due to rarity of CST and risk of hemorrhage CST: Anticoagulation Southwick (1986): Retrospective review of 86 case reports of infectious CST from 1940-1984 Heparinized patients (n=28) Death: 4 (14%) Full recovery: 10 (36%) Recovery with sequelae: 14 (50%) Non-heparinized patients (n=58) Death 23: (40%) Full recovery 15: (26%) Recovery with sequelae: 20 (34%) Differences were statistically significant Probably confounded by reporting bias Case Follow-Up Ceftriaxone, vancomycin, metronidazole started LP and blood cultures did not grow any organisms Otolaryngology drained sinuses endoscopically IOP remained elevated in mid-30s throughout hospitalization; VA remained NLP Otolaryngology revised previous sinus surgery and decompressed orbit (medial wall, floor) Surgical cultures grew MSSA; abx changed to nafcillin IV and metronidazole Patient discharged with home IV abx for 6 weeks Case Follow-Up Ophthalmology follow-up 1 week after d/c Comfortable right eye NLP Pupil 6 mm, non-reactive, +APD OD TA 16 (on Diamox, Alphagan, Cosopt) Markedly improved proptosis Severely motility restriction in all directions Complete RUL ptosis Decreased corneal sensation Normal anterior and posterior segment exam Summary Suspect cavernous sinus thrombosis in the setting of acute unilateral proptosis Frequent etiologies include sinus and facial infections Concurrent orbital cellulitis and/or orbital apex syndrome may occur IV antibiotics clearly reduce mortality and need to be started immediately Anticoagulation is controversial, but can consider in cases of clot expansion References Bhatia K, Jones NS. Septic cavernous sinus thrombosis secondary to sinusitis: Are anticoagulants indicated? A review of the literature. J laryngol Otol. 2002;16:667-676 Bilyk JR and Jakobiec FA. Chapter 32: Embryology and anatomy of the orbit and lacrimal system in Duane’s Ophthalmology (2007) Cannon ML et al. Cavernous sinus thrombosis complicating sinusitis. Pediatr Crit Care Med 2004;5(1):86-88 Enbright JR et al. Septic thrombosis of the cavernous sinuses. Arch Intern Med 2001;161:2671-2676 Pavlovich P et al. Septic thrombosis of the cavernous sinus: Two different mechanisms. Orbit 2006;25:39-43 Southwick FS et al. Septic thrombosis of the venous dural sinuses. Medicine. 1986;65:82-106 Watkins LM et al. Bilateral cavernous sinus thromboses and intraorbital abscesses secondary to Streptococcus milleri. Ophthalmology 2003;110:569-574