Miller, Brooke MD; Khalifa, Yousuf MD; Feldon, Steven E. MD, MBA; Friedman, Deborah I. MD, MPH
Flaum Eye Institute (BM, YK, SEF, DIF), University of Rochester School of Medicine and Dentistry, Rochester, New York.
Address correspondence to Deborah I. Friedman, MD, MPH, Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390; E-mail: email@example.com
Supported by a Research to Prevent Blindness Unrestricted Grant to Department of Ophthalmology, University of Rochester.
The authors report no conflicts of interest.
Abstract: Lemierre syndrome is an uncommon septic thrombophlebitis of the veins of the head and the neck usually occurring after a severe oropharyngeal infection. Although subsequent septic emboli most commonly affect distant sites, such as the lungs and joints, the authors present a case of Lemierre syndrome causing bilateral cavernous sinus syndrome.
In 1936, André-Alfred Lemierre, a French bacteriologist, described anaerobic septicemia following throat infections in 20 patients, 18 of whom died (1). Lemierre syndrome, an uncommon septic thrombophlebitis affecting the veins of the head and neck, generally follows a throat infection. It usually affects otherwise healthy individuals and is not contagious. Although it occurred more frequently and was often fatal before the antibiotic era, Lemierre syndrome still may be seen, often affecting otherwise healthy individuals. Our case demonstrates bilateral cavernous sinus involvement in Lemierre syndrome.
A previously healthy 35-year-old man presented to the emergency department with a 3-week history of sore throat, malaise, and fever. Outpatient treatment consisted of 3 days of 60 mg of prednisone for a positive monospot test. Several days before presentation, the patient developed acute binocular diplopia and right proptosis which, associated with progressive dyspnea, prompted initial evaluation in a local emergency room.
Ophthalmologic examination revealed a visual acuity of 20/30, right eye, and 20/25, left eye. The right eye was proptotic with chemosis and limited movements in all directions.
Magnetic resonance venography (MRV) revealed right transverse and sigmoid sinus thrombosis (Fig. 1), and computed tomography (CT) showed right internal jugular vein thrombosis (Fig. 2). Magnetic resonance imaging (MRI) of the brain was consistent with bilateral cavernous sinus thrombosis (Fig. 3). This was confirmed with diffusion-weighted imaging (Fig. 4A), which also demonstrated thrombosis of the right superior ophthalmic vein (Fig. 4B).
Laboratory testing was significant for leukocytosis of 24,600 cells per microliter and an elevated erythrocyte sedimentation rate of 65 mm/h. Blood cultures were negative, likely related to previous outpatient treatment with broad-spectrum antibiotics. Empiric treatment was initiated with vancomycin and meropenem, as well as intravenous heparin. The patient's respiratory status deteriorated, presumably from septic emboli to the lungs causing acute respiratory distress syndrome, and he was intubated and transferred to our medical center.
The patient was intubated and sedated upon arrival, although he could follow commands. Visual acuity was 20/400, right eye, and 20/200, left eye. Pupils measured 3.5mm, right eye, and 3.0 mm, left eye, and there was no relative afferent pupillary defect. Eye movements were limited in all directions (Fig. 5). There was bilateral proptosis, greater on the right, and increased resistance to retropulsion was also greater on the right. External examination revealed bilateral periorbital edema with chemosis, with intraocular pressures of 24 mm Hg, right eye, and 22 mg Hg, left eye. The only funduscopic abnormality was dilated retinal veins in the right eye.
The patient steadily improved during 2 weeks of hospitalization. He was discharged on anticoagulation and 4 more weeks of intravenous antibiotics. Six months later, he had 20/20 vision in both eyes with a residual mild right sixth nerve palsy.
Lemierre syndrome is a potentially fatal disorder that may complicate bacterial pharyngeal infection in an otherwise healthy individual and is characterized by thrombophlebitis of the veins of the head and the neck. Because of anatomic proximity, infection is thought to spread from peritonsillar tissues to the adjacent pharyngeal space, with subsequent thrombophlebitis of the internal jugular vein (2). Septic emboli arising from the internal jugular vein most commonly affect distant sites, such as the lungs and joints (2). Although the most commonly identified organism in infected patients is Fusobacterium necrophorum, other causative organisms include Bacteroides species, Streptococcus and Enterococcus species, and Proteus mirabilis (3). In the antibiotic era, Lemierre syndrome may be dismissed erroneously as a disease of the past (2). However, recent reports indicate that the incidence of Lemierre syndrome is increasing, possibly related to reduced use of antibiotic therapy for pharyngitis (4).
Ophthalmic involvement in Lemierre syndrome is rare with few reports of proptosis (5), endogenous endophthalmitis (6), vitreous hemorrhage (7), and orbitopathy (8,9). There are reports of isolated fourth and sixth nerve palsies resulting from Lemierre syndrome (10,11).
Cavernous sinus syndrome has been associated with Lemierre syndrome, but, to our knowledge, there are no cases of severe bilateral ophthalmoplegia as an early manifestation. One report described a patient with MRI and MRA findings of cavernous sinus involvement but the patient did not have ophthalmoplegia (4). Another described a patient with extraocular muscle enlargement, chemosis, proptosis, and ophthalmoparesis (9). Neuroimaging demonstrated thrombosis of both superior ophthalmic veins and a left intracranial dural venous sinus, with thrombophlebitis of the left internal jugular vein and bilateral orbital abscesses. Despite anticoagulation and aggressive treatment with intravenous antibiotics, the patient's vision declined to bare light perception and he developed complete ophthalmoplegia with fixed and nonreactive pupils. MRI showed a right carotid-cavernous fistula, and the patient died less than 3 weeks after presentation.
Our report highlights an unusual cause of cavernous sinus syndrome, although the mechanism for cavernous sinus involvement remains speculative. It is possible that our patient had a heightened hypercoagulable state, related both to endothelial damage from microorganism invasion and a proinflammatory immune response to systemic infection. Supporting this notion, one study found that the surface of the F. necrophorum bacterium activates branches of the contact system, the system that links inflammation and coagulation (12). In Lemierre syndrome, bacteria may migrate through the vessel wall of veins of the head and neck, causing platelet activation, inflammation, and activation of the extrinsic coagulation pathway, enhancing local coagulation (12).
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