Wren presented a patient who was admitted for work up of neck swelling.
The patient was a 54 year old woman with a history ESRD. She had been having swelling in the left submandibular region of the neck for about 1 week associated with throat pain and subjective fevers.
History is important and can help you create a good differential. Be sure and ask about chronicity, with more acute neck swelling likely due to viral lymphadenopathy, abscess, anaphylaxis, and angioedema and more chronic things concerning for lymphoma or metastasis. Ask about associated symptoms of sore throat, tooth pain, fevers, URI symptoms, weight loss, and B symptoms. It’s good to know about instrumentation. Did the patient have a recent procedure like a central line or tunneled catheter, or were they recently intubated? Are they on any new medications?
The differential for neck swelling can be broad and we approached it by thinking about the neck anatomy and how different structures can lead to swelling in the neck.
Approach to Neck Swelling in Adults
Think about the structures in the neck. It is a tight compartment. If there is any form of inflammation this can lead to swelling. Think about location on the neck where the swelling is and whether or not it is diffuse, localized, unilateral, or bilateral. Unilateral swelling is more concerning for a solitary lymph node enlargement, from parotitis, sialadenitis from an obstructing salivary gland stone, an abscess, lymphoma, metastasis, swelling within the carotid sheath. More diffuse swelling is concerning for IVC syndrome, cellulitis.
Lymphadenopathy from a viral etiology usually affects multiple nodes in the neck. It is important to factor in the character of the swelling. If lymph nodes are mobile and tender, it is more likely infectious. Static and painless are concerning for malignancy.
Here is a helpful mnemonic for remembering different causes of neck swelling:
TRAP N’ SWELL
T – Thyroid mass/Tonsillitis
R – Retropharyngeal abscess
A – Anaphylaxis
P – Parotitis (mumps, bacterial infections)
N’ – Neoplastic spread
S – Sialadenitis (stone or bacterial infection of the salivary glands)
VV – Viral lymphadenopathy (EBV, Rhino, Entero, Adenovirus)
E – Evasive (unusual infections: Bartonella Henselae (cat scratch Fever), Tularemia, Non-TB Mycobacterial infections (MAC), TB, actinomyces (sulfur granules, draining tracts), toxoplasmosis gondii (kitty litter)
L – Lymphoma (Burkitt’s Lymphoma, Hodgkin’s lymphoma)
L – Large vessel aka Thrombophlebitis of the IJ or IVC syndrome
Back to our patient. The patient was admitted for further work-up and monitoring of her airway and diffuse neck swelling. On exam, she was afebrile and otherwise hemodynamically stable. She did not have any voice changes, salivation, or difficulty breathing. Her throat appeared erythematous but no exudates were noted. Her uvula was midline and there was no posterior oropharynx swelling noted. Diffuse lymphadenopathy was palpated in the neck, with the left sided being more prominent than the right. Patient did not have a leukocytosis and other labs were stable.
She has a Centor Score of 2, (based on absence of cough, +fever (at home), +LDN, -1 for age ≥45) where testing for strep is based on provider judgement and is optional. A rapid strep test was performed.
ENT was consulted because of her and diffuse swelling and initial concern for airway monitoring. They recommended a CT with contrast. Her CT showed a left internal jugular thrombus and diffused cervical lymphadenopathy.
Her rapid strep test came back positive for group A strep.
The IJ thrombus, lymphadenopathy and positive group A strep was concerning for an early form of Lemierre’s syndrome. Now we will take a deeper dive into what this long-time described syndrome is.
Lemierre’s Syndrome – Described in 1936 by Lemierre. It was a dreaded deadly outcome of pharyngitis in the pre-antibiotics era (fatal in 7-15 days). There are no official criteria and it is a clinical diagnosis including a combination of: fever, neck pain, swelling, thrombus in the IJ with sequela of septic emboli. It is more commonly described in younger adults and in men, although still very rare. Around 10/million.
The most common pathogen is Fusobacterium necropherum. Others include fusobacterium nucleatum, streptococci, staphylococcus, and klebsiella pneumonie.
85% of the time it originates from an infection in the pharynx or tonsils. The causative bacteria invades the pharyngeal mucosa and lateral neck spaces which is often weakened after viral or bacterial pharyngitis. The lipopolysaccharides of fusobacterium have endotoxic properties that play a role in infectious spread. It can seed the internal jugular vein through the tonsillar vein or spread through fascial planes into the right IJ causing septic thrombophlebitis.
Part of the syndrome is the manifestation of septic emboli. This sequel is most commonly found in the lungs in the form of pneumonia or pleural empyema (85% of time lungs are affected in Lemierre’s syndrome). Patients can also have septic joints of the knee or hip, septic emboli to the brain, splenic abscesses, endocarditis, and pericarditis.
Presentation is most commonly pharyngitis in young people and older people are more likely to present with the complications of septic emboli.
Get blood cultures and sample an exudate in the pharynx. Fusobacterium can take >7 days to grow in cultures. If you suspect Lemierre’s syndrome and do not yet have a pathogen, treat with a B-lactam and metronidazole and narrow once you have a know the causative agent. Metronidazole is bactericidal and has good deep tissue penetration. Patients require a prolonged treatment course of 4-6 weeks. There are no large prospective studies examining whether or not to anticoagulate. The evidence we have is equivocal, therefore we do not generally anticoagulate unless thrombus causes continued infection or has excessive extension. Rarely has it been described to intervene with excision or thrombolysis. Standard treatment is antibiotic therapy with monitoring for resolution.
Our patient had group A strep pharyngitis with lymphadenopathy that led to spread of inflammation into the neck causing internal jugular thrombophlebitis. She was fortunate in that she did not have any septic embolic sequela. She was discharged with a prolonged course of amoxicillin-clavulanate and was not given anticoagulation after a risk-benefit discussion (she had had a significant GI bleed in the recent past).
Reference:
Lee WS, Jean SS, Chen FL, Hsieh SM, Hsueh PR. Lemierre’s syndrome: A forgotten and re-emerging infection. J Microbiol Immunol Infect. 2020;53(4):513-517. doi:10.1016/j.jmii.2020.03.027