Clinical Pathological Case presentation on a patient with Lemierre's Syndrome presenting with flank pain

1. Part 1
Farooq Ali Khan MDCM
PGY2 FRCP-EM
April 12th 2011

2. Patient in ambulatory care
 26 y.o. Male
 Moroccan, in Canada x2 y
 Married, works as manager at a wireless
phone company
 CC: Right flank pain
 PMH: none
 Meds: none
 Allergies: none

3. HPI
 Day 1: headache
 Day 2: nausea/vomiting, chills
 Day 3: watery diarrhea. Chills.
Decreased urine output.
 Day 4: goes to GP, given oral
rehydration and pepto bismol. Takes
tylenol and loratadine for symptoms.
Subsequently develops fever and
hematuria. No dysuria, frequency or
urgency

4. HPI
 Day 5: presents to ED with R flank pain
radiating to back, chest pain, abdominal
pain, vomiting, diarrhea and frank
hematuria
 No recent antibiotics, travel or exposure
to sick contacts
 Self-limited sore throat with swollen
tonsils for 2 days that occurred 10 days
prior to ED visit. No rhinorrhea, no
hemoptysis

5. Physical exam
 Triage vitals
 HR 120, BP 103/47, RR 38, T 38.3
 Looks unwell, dry mucus membranes, cracked
lips
 GCS 15, no nuchal rigidity
 No cervical lymphadenopathy
 Chest clear
 Normal S1 S2, no JVD, no murmur
 Abdo diffusely tender maximally in lower
quadrants with some guarding. Bowel sounds
positive. Frank hematuria
 Vitals after 2L bolus – HR 90, BP 96/56

6. Initial labs
 CBC
 Hgb 168, WBC 8.7, Plat 62
 SMA7
 Na+ 127, K+ 3.7, Cl- 96, HC03
- 16
 Gl 6.1, urea 23.7, creat 399
 LFTs
 T. Bili 22.7, AST 87, ALT 100, ALP 136, GGT 66
 Amylase 59
 LDH 259
 VBG
 pH 7.4, pCO2 21.4, HC03
-15.2, BE -8.2, lactate 2.1

7. 1 shift and 4L of NS later...
 CBC
 Hgb 145, WBC 25, Plat 71
 Man. Diff. Neut 12.2, bands 3.9, echinocytes 2+, decreased
platelets
 SMA7
 Na+ 129, K+ 4.3, Cl- 97, HC03
- 20
 Gl 5.9, urea 26.7, creat 503
 LFTs
 T. Bili 25.2, AST 92, ALT 98, ALP 108, GGT 55
 Lipase 161, CRP 178, Haptoglobin 3.78
 Coags
 INR 1.26, PTT 47.7, D-dimer 3.28, fibrinogen 4.01
 UA
 Blood +++, protein +++, Bili +++, ketones +, glucose –
 Leukocyte esterase Ca 125, nitrite +
 pH 5.5, turbid, red, spec grav 1.015

8. Microbiology
 Blood gram stain: gram -ve rods
 Antistreptolysin O 569
 Stool C+S, O+P, C.diff negative
 Urine culture negative
 Urine legionella negative
 Sputum culture negative
 NPA for influenza negative
 HIV negative
 Hep A, B, C negative

9. Initial CXR

11. CXR after 2L of fluid

12. Abdo US report
 CONCLUSION:
 Multiple hepatic cysts with a hypoechoic
liver lesion with cystic changes. This
should be further evaluated by MRI/CT
scan with contrast (if dialysis
considered).
 Slightly enlarged spleen.
 Enlarged and very echogenic kidneys,
consistent with renal medical disease,
consider acute glomerulonephritis.

13. Course in ED
 Given total 6L of fluid
 Consulted Medicine, ID, Nephro, GI
 Patient on his way to the ICU

14. Part 2

15. Course in hospital
 Admitted to the ICU
 Started on Timentin
 Received hemodialysis
 Clinical improvement
 Discharge from ICU after 4 days

16. Differential diagnoses for renal
failure in this patient
 Hemolytic Uremic Syndrome
 Post-Streptococcal Glomerulonephritis
 Sepsis-induced Acute Tubular Necrosis

17. HUS?
 In favor
 Diarrheal illness
 Gram -ve rods in
blood
○ (? E. Coli 0157:H7)
 Thrombocytopenia
 Renal dysfunction
 Fever, high WBC and
CRP
 Not in favor
 No anemia
 No hemolysis
 No other
microangiopathic
changes on smear
○ Teardrop cells
○ Helmet cells
○ Microspherocytes

18. PSGN?
 In favor
 Sore throat prior to
present illness
 Decreased urine
output and fluid
overload
 Gross hematuria and
proteinuria
 Elevated ASOT
 Renal ultrasound
consistent
 Not in favor
 No red cell casts on
microscopy
 C3 and C4 levels
were later found to be
normal
 Does not explain
other bacteremia and
GI symptoms
 Renal biopsy
inconsistent

20. Sepsis
 In favor
 SIRS
 Metabolic acidosis
 Multisystem organ
dysfunction
○ GI symptoms
○ Elevated LFTs
○ Consumptive
coagulopathy
 Gram -ve rods in blood
culture
 Renal biopsy consistent
with ATN
 A few unanswered
questions..
 Why did someone so
young get sick so
quickly?
 Why were so many
systems involved
despite no evidence of
immune suppression?
 What was the
organism?
 Was the sore throat a
red herring?

22. Today on grand rounds reruns...

23. Fusobacterium
necrophorum
 Anaerobe primarily responsible for
oropharyngeal infections
 Associated with Lemierre’s syndrome

24. Epidemiology
 F. Necrophorum causes endemic
pharyngitis
 Primarily adolescents and young adults (16-30)
 incidence of 10% (equal to group A strep)
 ~1 in 400 cases of F. Necrophorum
pharyngitis can lead to systemic
necrobacillosis and Lemierre’s syndrome
 Mortality 4.6%
 Resurgence in last 15 years

25. Pathophysiology
 Pharyngitis caused by
 Fusobacterium Necrophorum primarily
 Another organism (e.g. GAS) with
secondary colonization by F. Necrophorum

26. Pathophysiology
 Invades blood vessels in parapharyngeal
space causing bacteremia with a wide
range of presentations
 Classic fulminating Lemierre's syndrome
○ IJ thrombus
○ Rapid dissemination of septic microemboli to
lung, joints, intraabdominal organs, and CNS
 Transient bacteremia which leads weeks
later to a focal abscess
 Toxin production leading to sepsis with
thrombocytopenia and DIC

27. What can be done about it?
 Necrobacillosis and LS are preventable
 Think about it when seeing a young person
with a sore throat
 Consider throat culture for other organisms
besides Strep
 Red flags for pharyngitis
 Symptoms do not resolve in 3-5 days
 Symptoms rapidly worsen
 Unilateral neck swelling
 Systemic symptoms (e.g. Bacteremic)
 Treat with penicillin/flagyl or clindamycin

28. References
 Marx: Rosen's Emergency Medicine, 7th ed. - 2009 - Mosby, An Imprint
of Elsevier.
 Riordan T. Human infection with Fusobacterium necrophorum
(Necrobacillosis), with a focus on Lemierre’s syndrome. Clin
Microbiol Rev. 2007;20:622- 59. [PMID: 17934077]
 Centor RM. Expand the Pharyngitis Paradigm for Adolescents and
Young Adults. Annals of Internal Medicine. 2009;151(11):812-5
 Batty A, Wren MW. Prevalence of Fusobacterium necrophorum and
other upper respiratory tract pathogens isolated from throat swabs.
Br J Biomed Sci. 2005;62:66-70
 Amess JA, O’Neill W, Giollariabhaigh CN, Dytrych JK. A six-month
audit of the isolation of Fusobacterium necrophorum from patients
with sore throat in a district general Hospital. Br J Biomed Sci.
2007
 Hagelskjaer Kristensen L, Prag J. Lemierre’s syndrome and other
disseminated Fusobacterium necrophorum infections in Denmark: a
prospective epidemiological and clinical survey. Eur J Clin Microbiol
Infect Dis. 2008;27:779-89

29. References
 Syed MI, Baring D, Addidle M, Murray C, Adams C
(September 2007). "Lemierre syndrome: two cases
and a review". The Laryngoscope (The American
Laryngological, Rhinological & Otological Society;
Lippincott Williams & Wilkins) 117 (9): 1605–1610
 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): 369–374
 Beldman TF, Teunisse HA, Schouten TJ (November
1997). "Septic arthritis of the hip by Fusobacterium
necrophorum after tonsillectomy: a form of Lemierre
syndrome?". European journal of pediatrics (Springer-
Verlag) 156 (11): 856–857