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).
1. Lemierre A. On certain septicaemias due to anaerobic organisms. Lancet. 1936;1:701–703.
2. Vargiami EG, Zefeiriou DI. Eponym: the Lemierre syndrome. Eur J Pediatr. 2010;169:411–414.
3. Chirinos JA, Lichtstein DM, Garcia J, Tamariz LLJ. The evolution of Lemierre syndrome: report of 2 cases and review of the literature. Medicine (Baltimore). 2002;81:458–465.
4. Westhout F, Hasso A, Jalili M, Afghani B, Armstrong W, Nwagwu C, Ackerman LL. Lemierre syndrome complicated by cavernous sinus thrombosis, the development of subdural empyemas, and internal artery narrowing without cerebral infarction. J Neurosurg. 2007;106(1 suppl):53–56.
5. Figueras Nadal C, Creus A, Beatobe S, Moraga F, Pujol M, Vazquez E. Lemierre syndrome in a previously healthy young girl. Acta Paediatr. 2003;92:631–633.
6. Ahad MA, Gaber K, Freegard T. Endogenous endophthalmitis secondary to Lemierre's syndrome. Eye (Lond). 2004;18:860–862.
7. Olson JL, Mandava N. Bilateral intraocular involvement in Lemierre's syndrome. Br J Ophthalmol. 2006;90:249–250.
8. Dua P, Gutman J, Shinder R. Re: Orbital dissemination of Lemierre syndrome from gram-positive septic emboli. Ophthal Plast Reconstr Surg. 2011;27:466–467.
9. Kahn J, Baharestani S, Beck HC, Ng D, Aoumalan CI, Warren FA, Palu RN. Orbital disseminiation of Lemierre syndrome from gram-positive septic emboli. Ophthal Plast Reconstr Surg. 2011;27:e67–e68.
10. Lee S, Rutar T, Velaz FG, Rosenbaum AL. Lemierre's syndrome with fourth nerve palsy. J AAPOS. 2009;13:107–108.
11. Jones C, Siva TM, Seymour FK, O Reilly BJ. Lemierre's syndrome presenting with peritonsillar abscess and VIth cranial nerve palsy. J Laryngol Otol. 2006;120:502–504.
© 2012 Lippincott Williams & Wilkins, Inc.
12. Holm K, Frick I-M, Björk L, Rasmussen M. Activation of the contact system at the surface of Fusobacterium necrophorum
represents a possible virulence mechanism in Lemièrre syndrome. Infect Immun. 2011;79:3284–3290.