1. Acute Proptosis Mark Soontornvachrin, MD Raghu Mudumbai, MD
Ophthalmology Grand Rounds
August 9, 2007

2. 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

3. 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

4. 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%)
EOM Frozen Full

5. Exam SLE NDFE 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

6. Exam

7. Questions?

8. Differential Diagnosis

9. 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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42. 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

43. Differential Diagnosis
Orbital cellulitis
Orbital subperiosteal abscess
Orbital apex syndrome
Cavernous sinus thrombosis
Idiopathic orbital inflammation
Orbital mass/tumor
Thyroid-associated orbitopathy

44. Cavernous Sinus: Anatomy

45. Cavernous Sinus: Anatomy

46. Cavernous Sinus: Anatomy

47. Cavernous Sinus: Anatomy

48. Cavernous Sinus: Anatomy

49. Cavernous Sinus: Anatomy

50. Cavernous Sinus: Anatomy

51. Cavernous Sinus: Anatomy

52. Cavernous Sinus: Anatomy
A: ICA
B: CN III
C: CN IV
D: CN VI
E: CN V1
F: CN V2

53. Cavernous Sinus: Tributaries

54. Cavernous Sinus: Drainage

55. Cavernous Sinus: Neuroimaging

56. 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

57. 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

58. 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)

59. 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

60. 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

61. 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

62. CST: Complications Intracranial infection Pituitary insufficiency
Meningitis
Encephalitis
Abscess
Pituitary insufficiency
Hemorrhagic infarction
Death

63. 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

64. CST: Neuroimaging

65. 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)

66. 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

67. CST: Anticoagulation
Southwick (1986): Retrospective review of 86 case reports of infectious CST from
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

68. 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

69. 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

70. 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

71. 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:
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:
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: