Acute Proptosis

Mark Soontornvachrin, MDRaghu Mudumbai, MD

Ophthalmology Grand RoundsAugust 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 Tono-

Pen): 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 +APD OD TP (5%) 28 12

EOM Frozen Full

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

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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