Download Wong, A

Title: A Rare Emergent Case: Periorbital Swelling, Proptosis, and Ophthalmoplegia Secondary
to Fungal Sinusitis and Septic Cavernous Sinus Thrombosis
Authors: Anna Wong OD(1), Heema Desai OD (2), Tybee Eleff OD MS(3)
(1,2) Bronx Lebanon Hospital Center, Optometry Resident
(3) Bronx Lebanon Hospital Center, Optometry Attending
Abstract: A patient presents with sudden-onset proptosis and ophthalmoplegia secondary to
fungal sinusitis and septic cavernous sinus thrombosis. In this rare condition, prompt diagnosis
and treatment is critical to decrease morbidity and mortality.
I.
Case History
1. 48 year old African male
2. Seen as an in-patient consult for sudden-onset right eyelid swelling and binocular
vertical diplopia
3. One day prior, the patient presented to the emergency room for a recent severe
right-sided headache x 2 weeks. He was febrile with subjective neck stiffness,
and was admitted to the hospital to rule out meningitis.
4. Patient denies any previous ocular or medical history.
5. No ocular or oral medications
6. Prior to hospital admission:
i. CT: Possible sinusitis
ii. Lumbar puncture was unremarkable with negative CSF cultures
II. Pertinent findings
1. Clinical
i. VA at near OD 20/30, OS 20/50 PH 20/30
ii. PERRL(-) APD
iii. EOMs
a. OD limited in all gazes, especially downgaze and adduction
b. OS full
iv. Color vision
a. Ishihara 11/11 OD/OS
b. Hardy Rand and Rittler (HRR) 5/6 OD/OS
c. No red cap desaturation OD/OS
v. Mild proptosis OD
a. Hertel ophthalmometry OD 22, OS 20, Base 104
vi. Ptosis OD with fullness of superior lid, but no palpable mass.
vii. Slit lamp examination unremarkable OD/OS
viii. TAP OD 20 mm Hg, OS 16 mm Hg
ix. Dilated fundus exam unremarkable. Optic disc distinct margins OU.
2. Laboratory
i. Elevated serum and CSF glucose; elevated hemoglobin A1c
ii. Elevated homocysteine levels
3. Radiology
i. CT
a. Head/Facial bones: Pansinusitis
b. Orbit with contrast
a. Thrombosis of right superior ophthalmic vein
b. Partial occlusion of left superior ophthalmic vein
c. Suspicious for cavernous sinus thrombosis bilaterally
d. Extensive paranasal sinus disease
ii. MRI brain/orbit
a. Bilaterally enlarged superior ophthalmic veins
b. Partial thrombosis of right cavernous sinus
c. Recanalizing thrombosis of superior ophthalmic vein
iii. MRV brain: No dural venous sinus thrombosis
4. Pathology
i. CSF bacterial antigen negative
ii. Blood culture positive for H. Influenza
iii. Sinus culture suggestive of Aspergillus
III. Differentials
1. Orbital complications of sinusitis
i. Cavernous sinus thrombosis (CST), cellulitis, abscesses
a. Bacterial
b. Fungal
2. Inflammatory disease: Sarcoidosis, thyroid, Wegener’s granulomatosis
3. Neoplasm
4. Trauma
IV. Diagnosis and Discussion
1. Diagnosed with fungal paranasal sinusitis with septic CST.
2. Septic cavernous sinus thrombosis (CST) secondary to paranasal sinusitis is a
rare disorder
i. Common signs/symptoms:
a. Proptosis and orbital swelling can occur secondary to retrobulbar
congestion and/or fungal infiltration of the orbit (1, 5).
b. Headaches, facial pain, and blurred vision (5, 7, 8).
ii. Greater prevalence in the immunocompromised or uncontrolled diabetics
(1, 5, 7).
a. The patient was diagnosed with diabetes mellitus type II on
admission due to glucose levels and HbA1C
iii. Fungal etiologies must be considered in paranasal sinus infections
a. Sinusitis most commonly caused by Staphylococcus.
a. May also be due to fungi (1, 2)
b. Most fungal sinusitis is caused by Aspergillus and Mucor (3)
a. Mucormycosis progresses rapidly
b. Aspergillosis develops more slowly but may lead to CST (1)
c. Sinus culture in this patient verified aspergillosis
iv. Diagnostic features on radiological imaging
a. Filling defects and irregular contours of the cavernous sinus (3, 8)
b. Dilatation of the superior ophthalmic vein (8)
v. Bilaterality, trigeminal nerve involvement, and more severe ocular findings,
such as papilledema and APD, are suggestive of progressive CST (1, 8).
a. Indicates disseminated disease
b. Can ultimately result in mortality despite treatment
c. This patient had bilateral thrombosis, but did not progress to
bilateral ocular involvement due to prompt diagnosis and
treatment.
3. The use of anticoagulants for CST are controversial (3, 8)
i. Few studies due to rarity of the disease
a. May exacerbate an existing intracranial hemorrhage (1)
b. Possible benefit in absence of existing cerebral hemorrhage (1)
c. No difference in mortality in patients on anticoagulants (6)
d. Potential benefits of preventing progression and facilitating
recanalization of the thrombosis are in favor of therapy (6)
4. Elevated homocysteine levels (4)
i. Independent risk factor for heart disease, myocardial infarctions, and
stroke
ii. Exact mechanism in thrombosis is not well understood. Possible causes:
a. Vascular endothelial damage
b. Effect on coagulation pathway
c. Increased platelet aggregation
V. Treatment, management
1. Day 1
i. Admission to hospital to rule out meningitis
ii. Radiology: CT: Sinusitis
iii. Labs: Diagnosed with diabetes mellitus
iv. Pathology
a. CSF culture negative; Meningitis was ruled out
b. Blood culture positive for H.Influenzae
v. Medical: IV Cefepime and vancomycin initiated
2. Day 2
i. Initial ophthalmology consult: Findings per above
a. Recommend CT and MRI of brain, orbits, and sinuses for new
onset proptosis and ophthalmoplegia
ii. Medical: continue IV antibiotics
3. Day 3
i. Radiology: MRV: No dural venous thrombosis
ii. Surgical: Bilateral endoscopic ethmoidetcomies and sphenoidotomies
4. Day 4
i. Radiology: CT facial bones: pansinusitis
ii. Medical: continue IV antibiotics
iii. Headaches improved; improving proptosis
5. Day 5:
i. Radiology: MRI
a. Partial thrombosis of right cavernous sinus
b. Recanalizing thrombosis of superior ophthalmic vein
ii. Pathology: sinus culture positive for Aspergillus
iii. Medical: Continue IV antibiotics; Start oral metronidazole
6. Day 6:
i. Radiology: CT : Suspicious for CST bilaterally
ii. Hypercoagulable work-up initiated: Elevated homocysteine
iii. Medical
a. Continue IV and oral antibiotics
b. Lovenox and coumadin initiated for anticoagulant therapy
iv. Extraocular motilities full and resolved proptosis
7. Day 7
i. Medical
a. Continue anticoagulants and IV and oral antibiotics
b. IV Voriconazole initiated for fungal coverage
8. Day 10
i. Discharge from hospital
ii. Continue antibiotics, antifungal, and anticoagulants
iii. Dietary changes for diabetic treatment
9. Day 21: infectious disease follow-up
i. Start oral voriconazole and oral ceftriazone
10. 1 month: hematology follow-up
i. Start folic acid for elevated homocysteine levels
11. 4 month follow-up
i. VA OD 20/20, OS 20/20
ii. PERRL(-)APD
iii. EOMs full without restriction OD/OS
iv. Color vision Ishihara 11/11 OD/OS
v. No proptosis
a. Hertel ophthalmometry OD 19, OS 19, Base 107
b. Palpebral aperture size OD 10mm, OS 10mm
vi. TAP OD/OS 17 mm Hg
vii. Completed antifungal and antibacterials
viii. Currently still on Lovenox and folic acid
12. 6 month follow-up: MRI
i. Bilateral recanalization of the cavernous sinuses
ii. Decrease in size of the superior ophthalmic veins
VI. Conclusions
1. Careful investigation with imaging, blood work, and cultures is crucial in
diagnosing septic cavernous sinus thrombosis associated with fungal sinusitis.
i. Repeated radiological testing may be warranted
ii. Risk factors, such as hypercoagulopathies, should be tested for and
treated
2. Prompt diagnosis and treatment will minimize the risk of sight- and lifethreatening complications of this condition
3. The role of the optometrist :
i. Appropriate and timely referrals for testing
ii. Close follow-up, particularly initially with recent onset symptoms
iii. Patient must be monitored closely for progression/resolution
iv. Co-management with a team of practitioners, including ENT, neurology,
hematology, and radiology
VII. References
1. Barahimi B, Murchison AP, Bilyk JR. Forget me not. Surv Ophthalmol. 2010;
55(5):467-480.
2. Chen HW, Su CP, Su DH, Chen HW, Chen YC. Septic cavernous sinus
thrombosis: an unusual and fatal disease. J Formos Med Assoc. 2006;
105(3):203-209.
3. Cheung EJ, Scurry WC, Isaacson JE, McGuinn JD. Cavernous sinus thrombosis
secondary to allergic fungal sinusitis. Rhinology. 2009; 47: 105-108.
4. D’Angelo A, Selhub J. Homocysteine and thrombotic disease. Blood. 1997; 90(1):
1-11.
5. Dyken ME, Biller J, Yuh WTC, Fincham R, Moore SA, Justin. Carotid-cavernous
sinus thrombosis caused by aspergillus fumigatus: magnetic resonance imaging
with pathologic correlation – a case report. Angiology. 1990;652-657.
6. Levine SR, Twyman RE, Gilman S. The role of anticoagulation in cavernous
sinus thrombosis. Neurology. 1988; 38: 517-522.
7. Munjal M, Khurana AS. Fungal infections and cavernous sinus thrombosis Indian
J Otolaryngol Head Neck Surg. 2004; 56(3): 235-237.
8. Pavlovich P, Looi A, Rootman J. Septic thrombosis of the cavernous sinus: two
different mechanisms. Orbit 2006; 25: 39-43.