Lemierre s Syndrome: A Rare Complication of Acute Bacterial Pharyngotonsillitis. Authors: John Cecconi, MD; Nadine Khouzam, MD

Transcription

1 Lemierre s Syndrome: A Rare Complication of Acute Bacterial Pharyngotonsillitis Authors: John Cecconi, MD; Nadine Khouzam, MD

2 Clinical Case Demographics: 18 year old Caucasian male with negative PMH CC: Fever, cough HPI: Presented to the ED with worsening fever, cough and SOB. College student home for the weekend. He has been feeling unwell for almost one month with the following symptoms: URI-like symptoms of cough and intermittent fevers as well as sore throat, difficulty swallowing and fatigue. During illness had Rapid Strep testing that was negative, and testing for EBV mononucleosis was positive. Was started on a tapering dose of oral prednisone which he is currently still taking. Intermittent periods of improvement but continued to have decreased oral intake and persistent fevers. He has had a 20 pound weight loss during the illness. He came to the ED today due to new right upper thoracic back pain with deep inspiration and left sided jaw pain.

3 Case Continued PMH: No significant PSH: No prior surgeries FH: SH: Mother: Hypertension. Father: Hypertension, diabetes Paternal grandmother: Pancreatic cancer Denies tobacco or drug use. Alcohol use occasionally on the weekends. Freshman at College, lives in a dormitory. Home on break currently.

4 Case Continued Temperature:[99.5 F F] 99.5 F Heart Rate: [ ] 100 Respiratory Rate: [18-22] 18 Blood Pressure: (98-133)/(57-63) 118/57 HEENT: Tonsillar edema and erythema, tenderness to palpation of Left jaw, difficulty fully opening mouth. Cardiovascular: Tachycardic, no murmur. Respiratory: Lungs are clear to auscultation, tachypneic.

5 Case Continued Labs, Imaging CBC: WBC 11.1 (H), Hematocrit 40.9 (L), Platelet 129 (L) CMP: BUN 26 (H), Creatinine 1.01, BUN/CR 25.7 (H), glucose (133), calcium 8.3 (L), albumin 2.8 (L), alkaline phosphatase 167 (H), AST 49 (H) Blood Cultures: In process Rapid strep testing: negative Chest X-ray: Pleural density on the right consistent with focal atelectasis or small infiltrate.

6 Case Continued Sequence of Events: 11/24: Admitted with diagnosis of sepsis secondary to CAP 11/25: Discharged home in morning to complete course of Augmentin Called to return to ED as blood cultures growing gram negative rods 11/26: Blood cultures grow Fusobacterium necrophorum, ID consulted 11/27: Patient seen by ID, Dr. Wong: recommended CT neck, antibiotics transition to Unasyn and clindamycin 11/28: Patient transferred to Upstate for ENT evaluation as CT neck showed 1.2 cm x 1.2 cm hypodensity in lingular tonsil, thrombophlebitis internal jugular vein. Prior to transfer CT chest showed patchy areas of air space disease with no focal lung abscess

7 Background Also known as post-anginal sepsis or human necrobacillosis Complication of acute bacterial pharyngotonsillitis Affects primarily young, previously healthy people Secondary septic thrombophlebitis of the internal jugular vein Septic embolization may cause metastatic abscesses Most often a result of Fusobacterium necrophorum, an obligate anaerobic, pleomorphic, gram-negative rod Rarely caused by other gram-negative bacteria, such as Bacteroides or Peptostreptococcus

8 Epidemiology Lemierre s Syndrome is extremely rare A Danish retrospective study put the incidence at 1 case per million per year A French study from 2004 reported the prevalence in the general population to be 0.8 cases per million More common before the introduction of antibiotics Likely underreported and under recognized

9 Pathophysiology Fusobacterium necrophorum colonizes the infection site Anaerobic bacteria invade surrounding soft tissue and parapharyngeal space Formation of peritonsillar abscess Bacteria travel through peritonsillar vessels to internal jugular vein Thrombus forms within internal jugular vein Inflammation of IJV cause septic thrombophlebitis Microemboli disseminate causing abscesses and septic infarctions Pulmonary capillaries are first encountered by emboli Fusobacterium produces hemagglutinin -> DIC

10 Clinical presentation Acute pharyngitis primarily Or secondary to any infection of head and neck region High fever with rigors and malaise 3-10 days later Nonspecific pain and tenderness along the sternocleidomastoid, with trismus Hoarseness, dysphagia Cough, SOB, pleuritic chest pain Arthralgias Signs of sepsis or septic shock

11 Diagnosis Primarily a clinical diagnosis Leukocytosis, neutrophilia Subclinical hyperbilirubinemia or abnormal LFTs Elevated CRP and ESR Ultrasound or CT to evaluate internal jugular vein Chest X-ray or chest CT Bacterial cultures Joint aspirates

12 Treatment Optimal treatment regiment not known due to disease rarity Intravenous antibiotics Susceptible to beta-lactams, metronidazole, clindamycin, and third generation cephalosporins Avoid monotherapy; co-infection with additional bacteria common High dose penicillin combined with metronidazole or clindamycin Treatment duration: 2-6 weeks Transition to oral antibiotics when infection well controlled Drainage of abscesses and empyemas may be necessary, as well as debridement of necrotic tissue Anticoagulation with IV heparin -> Coumadin for 3 months for thrombosis retrograde to the cavernous sinus

13 Prognosis In pre antibiotic era mortality rate was 32-90% When properly diagnosed, mortality is around 4.6% Some studies have reported current mortality as high as 17% If meningitis is present, the mortality reaches 30% and half of survivors have permanent sequelae, such as cranial nerve palsies Mortality may be much higher as disease is not well known and often remains undiagnosed

14 Take home points Consider in young previously healthy patients with prolonged symptoms of pharyngitis followed by symptoms of severe sepsis or pneumonia, or with an atypical lateral neck pain. Diagnosis is confirmed by identification of thrombophlebitis of the internal jugular vein and blood cultures growing anaerobic bacteria. Contrast-enhanced CT is useful in the diagnosis of thrombophlebitis of the IJV; and characteristic CT findings may be the main clue to the diagnosis. In the appropriate clinical setting early imaging with CT Neck is indicated. Does this mean that we can prescribe antibiotics for every sore throat guilt free?

15 References 1. Eilbert W, Singla N. Lemierre's syndrome. Int J Emerg Med. 2013;6 (1): 40. doi: / Kim BY, Yoon DY, Kim HC et-al. Thrombophlebitis of the internal jugular vein (Lemierre syndrome): clinical and CT findings. Acta Radiol. 2013;54 (6): doi: / Screaton NJ, Ravenel JG, Lehner PJ, Heitzman ER, Flower CD (November 1999). "Lemierre Syndrome: Forgotten but Not Extinct-Report of Four Cases". Radiology. Radiological Society of North America. 213 (2): doi: /radiology r99nv PMID Sibai K, Sarasin F (2004). "[Lemierre syndrome: a diagnosis to keep in mind]". Revue médicale de la Suisse romande (in French). 124 (11): PMID Weesner CL, Cisek JE (1993). "Lemierre syndrome: the forgotten disease". Annals of Emergency Medicine. 22 (2): doi: /s (05) PMID