A 59-year-old man with a history of ESRD on dialysis presented with neck swelling and sore throat and was found to have Lemierre's syndrome based on CT findings of retropharyngeal fluid and internal jugular vein thrombosis, and was treated with IV antibiotics and underwent a prolonged hospital course complicated by hypotension, coagulopathy, and metastatic adenocarcinoma before passing away.

1. ICU Morning Report
Block 10
March 2011
Angie Sprigle
Avnee Shah
Kian Eftekhari
Ahad Lodhi

2. HPI

3. HPI
 59 y/o M
 CC: sore throat and neck swelling
 HPI: Pt presented for sore throat and per history was
supposed to have been taking antibiotics at home, but had
not been compliant. He also presented with lethargy that had
been present since 2 days prior to admission. Pt had also
been unable to eat due to significant facial and neck swelling.
Pt was also SOB. 2 dialysis sessions of his were skipped
secondary to his not feeling well.
 Pt was taken to OR for nasal intubation for airway
protection. His BP acutely dropped and the pt was started on
dopamine with limited fluid boluses secondary to ESRD.

4. Course on recent Admission
 Pt had been discharged from CCMC on 3/4 after being
admitted for complaints of dry cough and facial swelling –
during this admission, he was initially started on Unasyn
and clindamycin for possible cellulitis although it seemed
less likely
 An MRI was requested. The patient was unable to obtain
it because of his pacemaker.
 ENT was consulted and after the denial of MRI. A CT was
done. The first CT done was without contrast on
admission that showed extensive stranding of the
subcutaneous fat and the scalp and facial region was
nonspecific due to cellulitis and edema.
Retropharyngeal/prevertebral soft tissue swelling is noted
as well.

5. Course on recent Admission
 Repeat CT done with contrast prior to that also showed multiple
abnormalities in the soft tissue of the neck, a thrombus in the left
jugular vein, complex bilateral pleural effusions.
 The retropharyngeal fluid was decreasing in size. It was a concern that
maybe this retropharyngeal fluid initially was infectious.
 ENT was possibly going to take him to the OR to drain it. However,
after speaking with colleagues, the team decided to just watch clinically
since he was on antibiotics.
 His white blood cell count was elevated and he was not clinically
unstable.
 After this repeat CT with IV contrast was done and showing that the
retropharyngeal fluid was draining, it was decided the patient would
not be taken to the OR.
 Instead basal laryngoscopy was done at bedside, which just found
erythema. The patient was started on IV Decadron for the swelling,
and the swelling improved.
 The patient's clindamycin was stopped and he was maintained on
Unasyn for antibiotic. D/c on Augmentin as OP.

6. Review of Systems
Pt was intubated and sedated prior to
exam so ROS unable to be obtained.

7. Medications
 Abilify20mg PO daily  Minoxidil 5mg PO BID
 Zaroxolyn 2.5mg PO daily  ASA 81mg PO daily
 Renagel  Bidil 20/37.5mg PO TID
 Calcium  B complex c B12 150mg PO
 Lantus 14U SQ qHS daily
 Albuterol  Coumadin 4mg PO qHS
 Aranesp 60mg SQ  Carvedilol 25mg PO BID
q2weeks  Lisinopril 20mg PO daily
 Advair Diskus 1 puff daily  Augmentin (not taking)
 Bumex 5mg PO BID
 Simvastatin 20mg PO daily  Allergies: PCN

8. Past Medical History
 DM  Secondary
 Asthma, COPD hyperparathyroidism
 CAD  Bipolar
 CHF  Depression
with MR, TR, LVH
 Pulm HTN  Psychosis
 Hep C  Schizophrenia
 ESRD on HD
 Sleep apnea  Past Surgical Hx:
 Pernicious anemia Pacemaker, Splenectomy
 Social Hx: lives alone, no
 Proteinuria
tobacco/alcohol/drugs
 Diabetic neuropathy

9. Physical Exam
 Initial ED vitals-
◦ T 97.0, P 74, R 20, SpO2 95%RA, BP 112/49
 General exam: intubated and sedated. Moving with
stimulation/pain
 HEENT: PERRLA, neck swelling B/L, tongue protruding
 Chest: Coarse breath sounds anteriorly
 Heart: Tachycardia, no murmurs
 Abdomen: soft, NTTP, BS present
 Neurological: sedated
 Extremities: R groin a-line/femoral line, intact distal pulses, no
edema

10. LAB STUDIES
WBC-12.4, Hgb-12.2, Plt-225
Electrolytes WNL except for BUN-33,
Cr-6.5, Glc- 45
ALP-190, Albumin-2.8, Lipase-714
pBNP-1444
CK- 292, Trop (-)
ABG: 7.38/41/75/23 on AC
500/12/5/100%

11. CT Scan
•When compared to the prior study there has been
interval increase in soft tissue swelling that is most
pronounced along the left side of the face the does
extend into the sublingual region. Additionally there
has been some interval enlargement of extensive
lymphadenopathy particularly on the left.
•Mild increased propagation of the left internal jugular
vein thrombus
•Enlargement of a large left-sided pleural effusion 5.
Increased mediastinal lymphadenopathy

12. CT Scan
Since the prior study there has been
thickening of the retropharyngeal tissue
with suggestion of linear fluid tracking along
the length of the retropharynx without
evidence of a discrete loculated fluid
collection. This is consistent with
edematous phlegmonous change of the
retropharyngeal tissue and a developing
abscess cannot be completely excluded.

13. Hospital Course

14. Hospital Course (Day 1)
- After presentation with increasing airway swelling,
pt was nasally intubated in OR.
Initial CT showed increasing retropharyngeal fluid,
with abscess not able to be excluded
-CT also showed increasing left internal jugular vein
thrombus
-Pt was seen by ENT, who felt that
Lemierre’s syndrome was the most likely
diagnosis
-Recommended 4-6 weeks of IV antibiotics
(coverage of Fusobacterium necrophorum) and
extubation as soon as possible

15. Hospital Course (Day 1)
Enlargement of large left-sided pleural
effusion on CT
Increased mediastinal lymphadenopathy
on CT
US guided thoracentesis of effusion done

16. Hospital Course (Day 1)
Supportive care
Nasal intubation for airway protection
◦ Dexamethasone to reduce swelling
Hypotension likely secondary to septic shock
◦ Fluids
◦ Dopamine
◦ Antibiotics (initially Clinda, Aztreonam, Vanco)
Followed by renal for HD (pt with ESRD)
Also found to be coagulopathic (Coumadin held)

17. Hospital Course (Day 2-5)
Got PICC line for prolonged access for
antibiotics, etc. (INR had to be corrected
with Vitamin K)
HD continued as tolerated
Sputum and blood showed no growth
◦ Per ID, Aztreonam and Vanco discontinued
◦ Clindamycin continued

18. Hospital Course (Days 2-5)
 Attempts made to wean off dopamine, but blood
pressure variable
 Wean from vent also unsuccessful
 Pleural fluid that was initially sent came back
showing adenocarcinoma vs. mesothelioma
◦ Heme/Onc consulted
◦ PanCT showed extensive lymphadenopathy, with ?? of
primary cancer
◦ Immunohistochemistry pending
◦ Unlikely candidate for any chemotherapy
◦ Palliative care consult placed

19. Hospital Course (Days 6-10)
Pt found to have adrenal insufficiency,
likely contributing to hypotension
◦ Hydrocortisone added
◦ Pressures stopped but had to be restarted
due to hypotension
Pt able to be extubated
◦ Puree diet tolerated

20. Hospital Course (Days 6-10)
CEA elevated at 202
Immunohistochemistry showed that
colorectal CA was the likely primary
Heme/onc continued to recommend
palliation only
Palliative care following patient, with
family members in moderate
disagreement of what LOC should be

21. Hospital Course (Days 11-18)
Hypotension
◦ Fludrocortisone added to hydrocortisone
◦ Dopamine drip weaned off
Pt continued to do well off vent, get abx
(Flagyl and Levaquin added due to
possible GI source), and continue dialysis

22. Hospital Course (Days 11-18)
Pt made LOCII (all no)
Stable for transfer to floor, where remained for
several days
Pt then became unresponsive, hypotensive
Family contacted and changed LOC (yes to
intubation, no to CPR)
Pt was intubated, stabalized, but later that night
developed asystole and passed away (no code
called)

23. Lemierre’s Syndrome

24. Lemierre’s Syndrome
Lemierre credited with describing the disease in
1936 although reports of similar entities had
occurred earlier
He called this disease “postanginal septicemia”
which referred to a series of patients who
developed:
◦ Acute oropharyngeal infection
◦ Suppurative jugular vein thrombosis
◦ Sepsis
◦ Septic embolization of the lungs with abscess

25. Changing epidemiology of Lemierre’s
syndrome
 1900-1940s
◦ Many reports of post anginal sepsis
◦ Collections of cases, Lemierre, Alston etc
 1940s-1970s
◦ Reduction of cases of post anginal sepsis in antibiotic era
◦ Disappearance of anaerobic bacteraemia 2ary to tonsillitis
◦ Loss of clinical awareness
 1980s- 2007
◦ Re-emergence of awareness
◦ ? Changing incidence
25

26. Published cases of F. necrophorum
90
80
70
No of cases
60
50
40
30
20
10
0
1970-4 1975-9 1980-4 1985-9 1990-4 1995-9 2000-4
Period

27. Lemierre’s Microbiology
Most commonly anaerobic organisms:
◦ Fusobacterium necrophorum
◦ Fusobacterium nucleatum
◦ Fusobacterium prausnitzii
◦ Fusobacterium naviforme
◦ Fusobacterium gonidiaformans
◦ Bacteroides fragilis
◦ Peptostreptococcus spp
◦ Eikenella corrodens

29. F necrophorum as normal flora?
Jean Hallé
André Lemierre
Gorbach & Bartlett etc etc etc
But……
No published study has cultured F nec from
oropharynx of healthy subjects
Not found in studies of excised tonsils
Most molecular studies found no evidence
1 PCR study 20% carriage in healthy student
nurses and soldiers Jensen 2007 Clin Micro & Inf Dis
Conclusion: F necrophorum is an exogenously acquired infection
29

30. Lemierre’s Proposed Pathogenesis
Known colonizer of oral tract (such as F
necrophorum) gains entry to parapharyngeal
space with antecedent viral or bacterial
infection
Progression of infection to thrombophlebitis
Could be secondary to presence of
hemagglutinin or heparinase with these
organisms

31. Spread via


tonsillar vein

Septicaemia &
Septic emboli
⏎

32. Exposure to F. necrophorum
Tonsillitis
Persistent or
recurrent
Peritonsillar abscess tonsillitis
Meningitis / Cerebral
Deep neck space
abscess Thrombophlebitis of tonsillar infection
vein
Clot Propagation to
Sigmoid / transverse
sinuses Spread through lateral
pharyngeal space
Internal Jugular Vein
Clinical septic
thrombophlebitis “cord
sign”
F. necrophorum bacteraemia
Classic Lemierre’s syndrome / Subacute form progressing over Transient bacteraemia leading to
Postanginal septicaemia with weeks (pre-antibiotic era) deep abscesses weeks or months
metastases in lung etc. later
“Idiopathic” “Benign” bacteraemia
Metastatic lesions with no metastases “bacteraemic
tonsillitis”
32

33. Lemierre’s syndrome epidemiology
•Incidence 1/million popn/year
•Age 16-30 median 19
•Sex M:F 2:1
•Almost invariably previously fit

34. Lemierre’s Typical Presentation
Fever, malaise, cervical & submandibular
lymphadenopathy
Antecedent upper severe pharyngitis
Severe neck pain
Possible tender, palpable “cord” at the
anterior margins of SCM indicative of
thrombus

35. Lemierre’s Diagnosis
Diagnosis usually requires:
◦ Positive culture of F necrophorum or other
anaerobe
◦ Evidence of septic thrombophlebitis
◦ History of preceding oropharyngeal infection
◦ Radiographic imaging (MRI >> CT)
recommended

36. Lemierre’s Diagnosis
• Anginal illness or compatible clinical findings
&
• Metastatic lesions in lungs or remote site
&
• Isolation of Fusobacterium sp from blood cultures or
a normally sterile site.
or
• Evidence of IJV thrombophlebitis

37. ALL CASES
393
F. Necrophorum isolated F. necrophorum
251 not isolated 142
Site of Origin Culture
Other Dental Sinus Unclear Ear Throat Fusobacterium Other Other Negative
24 sp 62 Anaerobe 24 35
4 4 8 32 179 21
Fulfil current criteria for Lemierre’s Syndrome?
152 38 9 9 14
Lemierre’s
Syndrome
222

38. Distribution of extrapulmonary lesions in
cases of Lemierre’s syndrome

39. Lemierre’s Imaging
(A) Neck MRI, T2-weighted sequences showing hyperintensity with mild distention of the right jugular vein (small white arrow) compared with
the lack of signal in the left jugular vein representing normal flow (large white arrow). (B) MR venography showing absence of signal in the right
jugular vein (small white arrow) with normal signal in the left jugular vein (large white arrow). From Habek et al.

40.  Events leading to Lemierre’s
 Acquisition of F necrophorum
 Predisposing viral infection
 Mucosal damage
 Thrombophilia
 Single nucleotide polymorphisms predisposing to severe sepsis
 Progression to Lemierre’s depends on
◦ Mucosal trauma
◦ EBV or other viral infection
◦ Genetic factors such as thrombophilia

41. Therapy
Antibiotics Main causes
◦ Clindamycin, chloramphenicol, penicillins
• Group A strep
◦ Metronidazole best but no rigorous datanecrophorum
• F
Surgical drainage
◦ Empyema
◦ Neck abscess
◦ Bone, joint
Anticoagulants
Adjunctive therapy
41

42. Therapy
 Antibiotics
◦ Clindamycin
◦ penicillin poor
◦ Metronidazole best but no rigorous data
 Surgical drainage
◦ Empyema
◦ Neck abscess
◦ Bone, joint
 Anticoagulants
 Adjunctive therapy
42