Man develops acute blurry vision, diplopia
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A 42-year-old man with no significant medical history presented initially in the emergency department of an outside hospital with a 2-week history of progressive bilateral nasal congestion, facial pain and pressure.
He was started on Augmentin (amoxicillin clavulanate, USAntibiotics) the day prior. He was later transferred due to acute development of blurry vision in the left eye and binocular diplopia accompanied by nausea and vomiting.
Source: Julia Ernst, MD, PhD, and Kendra Klein-Mascia, MD
Examination
On bedside exam, best corrected near visual acuity was 20/20 in the right eye and 20/25 in the left eye with no dyschromatopsia. Pupils were equal, round and briskly reactive to light bilaterally with no relative afferent pupillary defect. IOP was normal in the right eye and elevated to 34 mm Hg in the left eye. Extraocular motility was full in the right eye but limited in all directions of gaze in the left eye (Figure 1). Confrontation visual fields were full bilaterally.
Anterior segment exam was unremarkable in the right eye, whereas the left eye demonstrated relative proptosis of 3 mm, periorbital edema, erythema, lagophthalmos and significant chemosis (Figure 1). Dilated fundus exam was unremarkable in the right eye. In the left eye, it was notable for dilated retinal vessels. There was no disc edema in either eye.
Imaging
Initially, the patient underwent CT of the sinus with contrast, which revealed sphenoethmoidal predominant sinus disease with associated heterogeneous mucosal enhancement, suggesting acute sinusitis affecting the left sinus greater than the right sinus and nonspecific mild asymmetric enlargement of the left superior ophthalmic vein.
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Blurry vision, diplopia
The patient’s symptoms of acute decreased vision in the left eye, unilateral periorbital pain, proptosis, chemosis, dilated retinal vessels and initial imaging showing dilated superior ophthalmic vein indicate obstruction of venous drainage from the left orbit. Additionally, significant limitation in the motility of all extraocular muscles on the left, suggestive of multiple cranial nerve palsies without signs of optic nerve compromise, localizes the site of pathology to the cavernous sinus, with cavernous sinus thrombosis (CST) as the likely etiology.
Differential diagnosis of cavernous sinus pathology is broad and includes venous, neoplastic, inflammatory and infectious causes. The acute nature of the patient’s condition was concerning for carotid-cavernous fistula; however, in a young patient without history of trauma, it was unlikely. Other causes such as lytic bone lesions near the sphenoid or sella turcica, primary or metastatic tumors, sarcoidosis and Tolosa-Hunt syndrome were lower on the differential due to the acute nature and septic presentation of our patient. Therefore, in the setting of progressive sinus infection in a septic patient and grave prognosis if timely treatment is not instituted, septic cavernous sinus thrombosis (SCST) was highest on the differential.
Workup and management
The patient underwent prompt MRI of the brain and orbits with contrast (Figure 2). It demonstrated thrombosis of the left cavernous sinus greater than the right, with associated intraorbital inflammation, and left orbital proptosis greater than right. It showed asymmetric enhancement of the left optic nerve sheath but without expansion or frank restricted diffusion and reactive dural thickening. It was notable for sinus disease and opacification of posterior ethmoid and sphenoid sinus with possible inspissated secretions or pus.
The patient was managed by the otolaryngology team. He was started on IV ampicillin/sulbactam and vancomycin, anticoagulation with heparin, IV dexamethasone, Cosopt (dorzolamide-timolol, Théa) and Alphagan (brimonidine tartrate, AbbVie) eye drops in the left eye, and oral acetazolamide. Blood workup included blood cultures.
The patient underwent functional endoscopic sinus surgery the next day with bilateral sphenoidotomies. He was closely followed by ophthalmology, neurology and infectious disease. His ocular symptoms improved the following day with better motility, reduced proptosis and chemosis of the left eye, and reduction of IOP to 17 mm Hg in the left eye. The right eye remained stable without symptoms. The sinus culture came back positive for Streptococcus anginosus whereas the blood cultures remained negative. He continued to clinically improve with resolution of systemic symptoms on day 3 and was transitioned to amoxycillin clavulanate on day 5. He was discharged home in stable condition after 5 days of hospitalization. The patient was seen 4 weeks later in the ophthalmology clinic. His IOP remained low, and his eye drops were discontinued. He reported residual diplopia with abduction deficit of the left eye on exam. He was subsequently followed locally.
Discussion
Cavernous sinus thrombosis is a rare disorder with an estimated annual incidence of 0.2 to 1.6 per 100,000 people per year. It can affect individuals of all ages. Septic cavernous sinus thrombosis is a severe, life-threatening disorder that is typically caused by sinus infections and less commonly by pharyngitis, cellulitis, otitis or dental infections or following traumatic injury or surgery. It develops as a result of the local spread of infection, thrombophlebitis or septic emboli that become trapped in the valveless cavernous sinus. Aseptic cavernous sinus thrombosis can occur in the setting of trauma, tumor, thrombophilic conditions, oral contraceptive use, malignancy or pregnancy.
The cavernous sinuses are located on each side of the sella turcica and above the sphenoid sinuses. They share a close relationship with important anatomic structures. The internal carotid artery, cranial nerve (CN) VI and sympathetic plexus pass through their lumen, whereas the CN III, CN IV, and first and second branches of CN V traverse along the lateral wall. The cavernous sinuses receive blood from the superior and inferior ophthalmic veins and superficial cortical veins and drain into the superior and inferior petrosal sinuses. As a result, the most common signs of CST stem from obstruction of venous drainage of the orbit. That results in periorbital edema, ptosis, proptosis, chemosis, and dilation of conjunctival and retinal vessels. The intimate relationship with cranial nerves can lead to ophthalmoplegia. However, the reason for diplopia is usually multifactorial and can also be caused by restriction due to venous congestion and extraocular muscle inflammation. Patients commonly complain of pain during eye movement, vision changes and diplopia. If sympathetic or parasympathetic nerve fibers are involved, that can lead to internal ophthalmoplegia with resulting mydriasis or myosis, respectively. Thrombophlebitis can also spread via dural sinuses and emissary veins and lead to meningitis, encephalitis or brain abscess or via the jugular vein and cause pulmonary abscess, pneumonia or empyema. Importantly, patients with SCST often present with fever, sepsis and altered mental status.
Diagnosis is made clinically with support of appropriate neuroimaging. MRI with contrast is the most sensitive. However, contrast-enhanced CT can also be valuable due to its speed and cost-effectiveness. Typical findings on imaging include expansion of or filling defects within the cavernous sinus. However, often imaging findings can be subtle and limited to dilation of the superior ophthalmic vein, intraorbital venous congestion, proptosis and increased dural enhancement. Moreover, characteristics of thrombus imaging may vary depending on its stage.
There are no clear guidelines for therapy due to rarity of the disease and hence lack of randomized controlled clinical trials. As a result, management is mostly based on expert opinion. The cornerstone of treatment for SCST is parenteral antibiotics, which commonly involves an anti-staphylococcal agent, a third-generation cephalosporin and metronidazole and is typically continued for 3 to 8 weeks. Additionally, anticoagulation is often used to prevent propagation of thrombus and its anti-inflammatory properties. However, it should not be used in patients who are at an increased risk for intracranial or systemic hemorrhage. Anticoagulation is also hypothesized to contribute to the spread of infection via dissemination of septic thrombi. Other commonly used medications in the setting of SCST are corticosteroids. Similarly, they have not been demonstrated to be effective. However, they are often used to decrease inflammation and vasogenic edema. They are, however, indicated in the setting of hypopituitarism. Surgery of the cavernous sinuses themselves is not indicated. Selected patients can benefit from sphenoidectomy, ethmoidectomy, maxillary antrostomy, mastoidectomy, abscess drainage, craniotomy (subdural empyema), orbital decompression or ventricular shunt placement.
Before the era of antimicrobials, prognosis for SCST was almost universally fatal. The mortality has declined since then, but it still remains as high as 20% to 30%. It is a rapidly progressing, serious condition with average hospitalization reaching 7 weeks. Furthermore, it is not without long-term sequelae including vision decline or loss, cranial nerve palsies, pituitary dysfunction, seizures and other neurological deficits in up to 50% to 75% of patients. Therefore, prompt diagnosis and treatment are crucial.
- References:
- Caranfa JT, et al. Surv Ophthalmol. 2021;doi:10.1016/j.survophthal.2021.03.009.
- Dinkin M, et al. Asia Pac J Ophthalmol (Phila). 2019;doi:10.22608/APO.2018239.
- Frank GS, et al. J AAPOS. 2015;doi:10.1016/j.jaapos.2015.06.001.
- Leach JL, et al. Radiographics. 2006;doi:10.1148/rg.26si055174.
- Munawar K, et al. Clin Imaging. 2020;doi:10.1016/j.clinimag.2020.06.029.
- Plewa MC, et al. Cavernous sinus thrombosis. In: StatPearls [Internet]. StatPearls Publishing; 2023
- van der Poel NA, et al. Orbit. 2019;doi:10.1080/01676830.2018.1497068.
- For more information:
- Edited by Jonathan T. Caranfa, MD, PharmD, and Angell Shi, MD, of New England Eye Center, Tufts University School of Medicine. They can be reached at jcaranfa@tuftsmedicalcenter.org and ashi@tuftsmedicalcenter.org.
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