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Au Manongas, MD PGY-2 December 9, 2009 16 y/o male with chest pain Patient presented to PMD ten days ago for sore throat and dysphagia. Strep test done was negative and he was discharged home. His symptoms continued and became associated with fever, neck pain and swelling and so he went back to PMD after three days and was placed on Azithromycin. One day prior, he developed chills, 2 episodes of NBNB vomiting, progressive shortness of breathing and pleuritic chest pain. Past Medical History: Unremarkable Medications: Azithromycin Allergy: NKDA Immunizations: UTD Family History: Unremarkable HEADS: Non-smoker; non-alcoholic beverage drinker; no illicit drug use; no pets; no animal exposure; no recent travel; sexually active T: 103.5F HR: 155 RR: 30 BP: 90/53 O2 sat: 85% Gen: ill-appearing; well-developed, in moderate respiratory distress HEENT: PERRL, clear conjunctiva, anicteric sclera, dry mucus membranes, mild diffuse erythema over posterior pharynx, no tonsillar hypertrophy or exudates, no mucosal lesions, no dental caries or abscesses Neck: no meningismus; B/L shotty ACLAD, tenderness to palpation with distinct nodularity along anterior aspect of right SCM with distinct nodularity, no erythema or warmth C/L : SCE, subcostal retractions, tenderness to palpation over sternal and parasternal areas, bibasilar crackles Heart: tachycardic, S1S2 normal, no murmur, rub or gallop Abdomen: soft, NT, ND, + BS, no hepatosplenomegaly Extremities: FROM, cap refill 2 seconds Skin: no rash, warm, diaphoretic Neuro: awake, alert, no focal deficits DIFFERENTIALS CBC : WBC – 20.5 N - 93% L – 2.8 BMP : HCO3 – 18 LFTs : WNL Coags: WNL CRP : 150 ESR: 180 HIV ELISA, Monospot test, Hepatitis panel, Cardiac enzymes: WNL Blood Culture : Fusobacterium necrophorum EKG : Sinus tachycardia LEMIERRE’S SYNDROME ( A Forgotten Disease) CLASSIC L.S.: 1. primary infection in the oropharynx (pharyngitis, sinusitis, OM, mastoiditis, odontogenic infection) ANDRE LEMIERRE 2. septicemia 3. evidence of internal jugular vein thrombophlebitis 4. metastatic focus NECROBACILLOSIS POSTANGINAL SEPTICEMIA Common in preantibiotic era Incidence rate: 1/1 million Increase incidence in the last 10 years Affects healthy adolescents and young adults Both sexes equally affected Majority of reports from Europe and North America Mortality rate: 90% in preantibiotic era; now 6% FUSOBACTERIUM NECROPHORUM FUSOBACTERIUM NECROPHORUM Gram negative anaerobic bacillus Part of normal flora Endotoxins, Leukocidin & Hemagglutinin, Platelet aggregation OTHERS: Streptococcus Bacteroides Lactobacillus Staphylococcus Eikinella 1. Primary infection – Pharyngitis 2. IJV septic thrombophlebitis – swollen &/or tender neck 3. Metastatic complications – Lungs, bone, joint, soft tissue, CNS, liver, spleen, kidney “For a syndrome that is so characteristic, it is remarkable how often the diagnosis is missed until an anaerobic gramnegative rod is isolated from blood culture.” • Clinicians are unaware of the condition • Manifestation of septic emboli distract clinicians from the initial oropharyngeal infection • Cases present to a wide variety of specialists Previously fit adolescent or young adult History of sore throat in preceding week “Be not deceived by a comparatively innocent appearing pharynx as the veins of the tonsil may be carrying the death sentence of your patient.” Onset of high fever and rigors Signs of internal jugular venous thrombosis : neck pain, stiffness, swelling, trismus, cord sign Pulmonary involvement • Pleuritic chest pain, dry cough, hemoptysis, ARDS Bone and joint manifestations • Septic arthritis, osteomyelitis Soft tissue lesions • Gluteal & abdominal wall abscesses Intra-abdominal sepsis • Abdominal pain, Jaundice, liver & splenic abscess, Peritonitis CNS complications • Meningitis, cerebral abscess, cavernous sinus & sigmoid sinus thrombosis Renal complications • Renal abscess, glomerulonephritis, hemolytic- uremic syndrome Hematological complications • DIC, peripheral ischemia & gangrene Viral Pharyngitis Infectious Mononucleosis Pneumonia Tuberculosis Endocarditis Blood Culture Culture from involved site CBC BMP LFT’s CRP & ESR Coagulation profile Chest X-ray Ultrasound of Neck CT scan of neck CT scan of chest MRI of neck/chest MULTIPLE PERIPHERAL NODULES/PLEURAL EFFUSIONS FEEDING VESSEL ANTIBIOTIC THERAPY • Mainstay of treatment • Prolonged therapy : 4 to 6 weeks • Most commonly used agents: Metronidazole; Penicillin/B- lactamase inhibitor; Imipinem • Antibiotic of choice: Metronidazole 1. Excellent activity against Fusobacterium 2. Good tissue penetration 3. Bactericidal activity 4. Excellent oral bioavailability • Mixed infection: Metronidazole + Penicillin ANTICOAGULANT Controversial • Probably decreases risk of clot extension • Possibly shortened the course of the disease • Likely helped avoid surgical drainage Indications: • Cerebral infarct or sinus venous thrombosis • Persistence of septic emboli despite antibiotic therapy SURGERY • Drainage of any abscess • Ligation/excision of Internal Jugular vein ADJUNCTIVE THERAPY • Hyperbaric Oxygen • Activated Protein C Exclusion of streptococcal infection does not exclude a bacterial cause in a patient with severe tonsillar infection Key to diagnosis is AWARENESS & HIGH INDEX OF SUSPICION Patient started on Ceftriaxone and Metronidazole Thoracentesis done Chest pain persisted on D4 of antibiotics Echocardiography R/O endocarditis Started on Heparin Plan: Continue antibiotics for total of 6 weeks and heparin indefinitely; repeat CT scans after one week THANK YOU!