A Systematic Review of Lemierre’s Syndrome With a Focus on Ophthalmologic Complications

Abstract

Lemierre's syndrome (LS), once known as "the forgotten disease," is a rare and potentially lifethreatening condition that has had a gain in incidence over the last 30 years due to a variety of factors that could include changes in antibody prescription patterns, particularly in regard to the treatment of pharyngitis/tonsillitis. Due to its low incidence and broad spectrum of symptoms, LS does not have an obvious clinical diagnosis and can confuse the clinician managing the patient. Furthermore, it is equally difficult to treat patients suffering from LS as it requires a multidisciplinary approach from multiple subspecialties. Thus, communication between hospitalists, radiologists, otolaryngologists, neurologists, and ophthalmologists is critical towards quickly diagnosing the disease condition so that prompt antibiotics, anticoagulation, and surgical intervention can occur. Atypical presentations can also exist, making the diagnosis and management exponentially more challenging. Ophthalmologic symptoms are a particularly rare and atypical presentation of LS. These rare symptoms in LS can be terrifying for patients and providers alike; yet, there does not seem to be any modern medical literature that summarizes ophthalmologic complications for LS patients. To our knowledge, this is the first systematic review of LS with a focus on ophthalmologic complications that has been done. The main objective of this review paper is to provide an up-to-date literature review of LS epidemiology, pathophysiology, diagnosis, and treatment while also performing a novel systematic review of reported cases of LS with ophthalmological complications. We hope to bring more awareness towards LS and its atypical presentations so that physicians will be better able to rapidly diagnose and treat their patients in order to minimize long-term morbidity and mortality.

A Challenging Case of Lemierre’s Syndrome With Central Nervous System Involvement and a Comprehensive Review

“…As a comparison, the previously mentioned 2016 review by Johannesen and Bodtger identified 137 LS patients over only five years (2010-2015), and they had a very similar inclusion criterion as we did [3]. Our 2020 review found that impaired extraocular motility, which would present as diplopia and cranial nerve III, IV, and VI palsies, was the most common presenting symptom, with 88.89% of LS cases (24 cases) presenting with that ophthalmological complication: abducens nerve palsies specifically occurred in 50% of LS cases (12 cases) with extraocular motility impairment and 44.44% of all LS cases with ophthalmological complications as defined by that criteria [20]. Our patient in this case had many symptoms that would classify her as having LS with ophthalmological complications including diplopia, bilateral abducens nerve palsies, blurred vision, and proptosis.…”

“…Blurred vision or decreased visual acuity was specifically one of her earliest presenting symptoms along with diplopia. She also specifically had cavernous sinus thrombosis, which occurred in 70.37% (19 cases) of cases in our systematic review, and decreased visual acuity, which occurred in 29.63% (8 cases) of those cases [20]. The majority of cases in our review had imaging that confirmed IJV thrombosis (81.48%; 22 cases) but did not have culture confirming F. necrophorum from a sterile site (only 33.33%; 9 cases) [20].…”

“…She also specifically had cavernous sinus thrombosis, which occurred in 70.37% (19 cases) of cases in our systematic review, and decreased visual acuity, which occurred in 29.63% (8 cases) of those cases [20]. The majority of cases in our review had imaging that confirmed IJV thrombosis (81.48%; 22 cases) but did not have culture confirming F. necrophorum from a sterile site (only 33.33%; 9 cases) [20]. Our patient did not follow this pattern in the literature we reviewed as she actually had positive cultures with negative imaging.…”

“…Our patient did not follow this pattern in the literature we reviewed as she actually had positive cultures with negative imaging. Moreover, our patient was treated with both anticoagulation and surgical intervention, which was also used in 51.85% of those cases (14 cases) [20]. Furthermore, the antibiotics used to treat our patient with LS were among the two most commonly prescribed in our review of the literature.…”

“…We recently published a systematic literature review on LS with a focus on ophthalmological complications. In that review, we identified a total of 27 patients between 2009 and 2019 who met our criteria for LS and who presented with "any symptom involving the orbit, the extraocular muscles, the optic nerve, the oculomotor nerve, the trochlear nerve, the abducens nerve, or the cavernous sinus" [20]. As a comparison, the previously mentioned 2016 review by Johannesen and Bodtger identified 137 LS patients over only five years (2010-2015), and they had a very similar inclusion criterion as we did [3].…”