US Case of the Month - Lurking Lemierre's
/The Case
Presentation: The patient is a middle aged female with a history of intravenous drug use and hepatitis C who presents with a chief complaint of difficulty breathing and throat swelling. She endorses right sided neck pain and sore throat which started the day prior to presentation and rapidly worsened over the course of 24 hours. The swelling has progressed to involve the entire right side of her neck and throat to the point where she feels as though she cannot swallow or breathe. She had multiple uncomplicated dental extractions approximately three weeks prior but recovered from these well. She now denies fevers, new medications, rashes, or cough.
Vitals: T 101.4, BP 126/100, HR 114, RR 23, SPO2 100%
Physical Exam: The patient was resting in bed, tearful, but nontoxic in appearance. HEENT exam is notable for lingular elevation and sublingual swelling. The posterior oropharynx is unable to be visualized due to significant right sided tonsillar hypertrophy with no visible exudates. She is also noted to have left sided uvular deviation and right sided submandibular fullness that is tender but not fluctuant. There is pooling of secretions and she is gurgling. Her neck exam shows significant right sided anterior cervical lymphadenopathy that is tender to palpation with overlying erythema. She has no nuchal rigidity. Cardiac exam shows a tachycardic rate with good perfusion. Pulmonary, abdominal, skin, and extremity exams are all unremarkable.
Workup: Given fever and neck/oropharyngeal swelling, labs were ordered, and the patient was given methylprednisolone, glycopyrrolate, and amoxicillin / clavulonic acid. A bedside ultrasound was performed to evaluate the soft tissue of the neck.
What we see on ultrasound: The soft tissue ultrasound of the neck reveals increased tissue thickness as well as a non-compressible right internal jugular (IJ) concerning for Lemierre’s Syndrome. A CT of the neck was performed to further characterize her pathology. This scan showed three linear metallic foreign bodies at the right neck base with associated inflammation and thrombosis of the right IJ, right external jugular (EJ), and right retromandibular veins as well as a small right parapharyngeal fluid collection.
Non-Compressible Internal Jugular Vein
non-Compressible Internal Jugular vein with absence of blood flow
Visualized clot in Internal jugular vein
Lemierre’s Syndrome
Lemierre’s syndrome is a rare infectious thrombophlebitis of the internal jugular vein. The incidence is around 0.8-3.6 cases per million in the general population (Valerio et al). Patients are typically otherwise healthy with a head or neck infection such as pharyngeal or retropharyngeal abscess. Historically, three criteria have been used to diagnose this syndrome. There must be a primary site of infection in the head or neck, thrombosis or thrombophlebitis of the IJ or other vein of the head or neck, and isolation of Fusobacterium necrophorum from the blood or throat. It has further been divided into typical vs. atypical depending on isolation of F. necrophorum in the bloodstream.
PRESENTATION:
The typical presentation is a young male or female with a sore throat followed by painful ipsilateral neck swelling along the anterior border of the sternocleidomastoid with muscle pain, fever, odynophagia, dysphagia, and trismus. You may also see signs and symptoms of metastatic foci including dyspnea, hemoptysis, and pleuritic chest pain (Van Wissen et al).
PATHOPHYSIOLOGY
The bacteria F. Necrophorum causes a primary pharyngitis which invades the pharyngeal space and internal jugular vein causing septic thrombophlebitis. While F. Necrophorum is the most common causative agent, other pathogens cited are other fusobacterium species, Bacteroides species, and group A, B, and C Strep (Chirinos et al). Other primary sites of infection include odontogenic sources, otitis media, sinusitis, and parotitis.
DIAGNOSIS
The diagnosis of this syndrome is based on physical exam, positive throat and/or blood cultures, and an internal jugular vein thrombosis. Ultrasound can be used to look at the IJ but may miss clots below the clavicle and at the level of the mandible. You can overcome this by using color doppler. Loss of color flow doppler may indicate the presence of a more proximal clot. Typically, CT is the imaging of choice as it also allows you to look for complications of Lemierre’s syndrome such as pulmonary septic emboli. Ultrasound use prior to going for chest CT may guide the addition of a neck CT and may help with the initiation of early antimicrobial therapy (Castro-Marin et al).
COMPLICATIONS
Mortality for these patients in the preantibiotic era could approach 30% but is now estimated to be in the 2-5% range (Johannesen). Septic pulmonary emboli are common and sometimes the first finding that will lead you down the path to diagnosis.Septic embolization to large joints and other soft tissue locations can also be seen.
TREATMENT
The treatment for Lemierre’s syndrome consists of Piperacillin/Tazobactam IV q6 hours, a carbapenem (meropenem 1g IV q8 hours), or ceftriaxone 1g IV q24 hours plus metronidazole 500mg IV q8 hours. Vancomycin is included if the patient has risk factors for MRSA or there is hemodynamic instability.
A systematic review of 84 cases was performed by Karkos et al. which concluded that the role of anticoagulation is unclear. Another retrospective chart review by Cupit-Link et al showed that there may be no benefit to anticoagulation. Gore et al. also performed a meta-analysis comparing anticoagulation vs. no anticoagulation in Lemierre’s syndrome and found no mortality benefit or effect on vessel recanalization. Ultimately, anticoagulation does not have a clear role.
The need for surgical debridement of the IJ is determined by the patient’s clinical course on antibiotics. If the patient has continued septic emboli despite appropriate therapy, there may be a role for surgical excision; however, this is rarely required so there is no significant data to support a mortality benefit.
CASE RESOLUTION
The patient was admitted to ENT and subsequently went to the operating room for the removal of the foreign bodies as well as irrigation and debridement of the abscess. She was started on antibiotics and anticoagulation and was ultimately discharged four days later with no apparent complications.
Post by Dr. Payton Thode
Dr. Thode is a PGY-3 in Emergency Medicine at the University of Cincinnati
Editing by Dr. Jessica Baez
Dr. Baez is a Ultrasound trained Attending and Assistant Program Director in Emergency Medicine at the University of Cincinnati.
References
Castro-Marin F, Kendall J. Diagnosis of Lemierre Syndrome by Bedside Emergency Department Ultrasound. The Journal of Emergency Medicine. 2010 Oct 34;39:436-439.
Chaker K, Berrada O, Lyoubi M, Oukessou Y, Abada RA, Rouadi S, Roubal M, Mahtar M. Lemierre's syndrome or re-emerging disease: Case report and literature review. Int J Surg Case Rep. 2020 Dec 9;78:151-154. doi: 10.1016/j.ijscr.2020.12.015. Epub ahead of print. PMID: 33352443; PMCID: PMC7753231.
Chirinos JA, Lichtstein DM, Garcia J, Tamariz LJ. The evolution of Lemierre syndrome: report of 2 cases and review of the literature. Medicine (Baltimore) 2002; 81:458-465.
Gore MR. Lemierre Syndrome: A Meta-analysis. Int Arch Otorhinolaryngol 2020; 24:e379.
Karkos PD, Asrani S, Karkos CD, Leong SC, Theochari EG, Alexopoulou TD, Assimakopoulos AD: Lemierre's syndrome: a systematic review. Laryngoscope 2009;119:1552-1559. 10.1002/lary.20542 19554637
M.C. Cupit-Link, A.N. Rao, D.M. Warad, V. Rodriguez, Lemierre syndrome: a retrospective study of the role of anticoagulation and thrombosis outcomes, Acta Haematol. 137 (2) (2017) 59–65.
Valerio L, Corsi G, Sebastian T, Barco S. Lemierre syndrome: Current evidence and rationale of the Bacteria-Associated Thrombosis, Thrombophlebitis and LEmierre syndrome (BATTLE) registry. Thromb Res. 2020 Dec;196:494-499. doi: 10.1016/j.thromres.2020.10.002. Epub 2020 Oct 8. PMID: 33091703.
Valerio L, Zane F, Sacco C, Granziera S, Nicoletti T, Russo M, Corsi G, Holm K, Hotz MA, Righini C, Karkos PD, Mahmoudpour SH, Kucher N, Verhamme P, Di Nisio M, Centor RM, Konstantinides SV, Pecci A, Barco S. Patients with Lemierre syndrome have a high risk of new thromboembolic complications, clinical sequelae and death: an analysis of 712 cases. J Intern Med. 2020 May 22. doi: 10.1111/joim.13114. Epub ahead of print. PMID: 32445216.
Van Wissen, Matthijs, et al. "Unusual presentation of Lemierre's syndrome: two cases and a review." Blood Coagulation & Fibrinolysis 20.6 (2009): 466-469. Journals@Ovid Full Text. Web. 28 December. 2020.
Johannesen, K. M., & Bodtger, U. (2016). Lemierre’s syndrome: current perspectives on diagnosis and management. Infection and drug resistance, 9, 221.
