Lemierre’s Syndrome Caused by MRSA Infection in an Infant
1Evelyne Kalyoussef, MD; 1Chirag Rajan Patel, MD, and 1Huma Quraishi, MD
Contact Information: Evelyne Kalyoussef, MD [email protected]
1Department of Otolaryngology – Head & Neck Surgery, University of Medicine and Dentistry of New
Jersey – New Jersey Medical School, Newark, NJ
Objective:
1. Describe an infant with Lemierre’s Syndrome caused by MRSA Sepsis
2. Discuss treatment options and management of IJV Thrombosis and
Lemierre’s Syndrome in Children
Methods:
Lemierre’s Syndrome is usually characterized by recent oropharyngeal infection
complicated by internal jugular vein thrombosis. The main pathogen is usually
Fusobacterium necrophorum. The vast majority of patients affected are young
adults. We present a case of community acquired Methicillin-resistant
Staphylococcus aureus (MRSA) in an eight week old male who was being
breastfed by his mother, who was a MRSA carrier. He presented with a two day
history of fever and left neck swelling and was found to have a deep neck
abscess extending into the mediastinum with internal jugular thrombosis
extending retrograde to involve the sigmoid sinus.
Results:
The infant was taken to the operating room multiple times for incision and
drainage and washout procedures. The internal jugular vein was not ligated;
however, he was treated with anticoagulation with demonstration of
recannulation of the sigmoid sinus. The patient was treated with long term IV
antibiotics. In addition, his mother was treated with Chlorhexidine baths for
MRSA decolonization.
Conclusion:
Classically described in older patients with Fusobacterium infections, there is a
growing body of literature reporting Lemierre’s syndrome secondary to MRSA
infections, particularly in a younger patient population suggesting a changing
demographic as well as a changing microbial pattern. We present the
youngest case of Lemierre’s Syndrome secondary to community acquired
MRSA.
Abstract
Introduction Lemierre’s syndrome (LS) is characterized by septic
thrombophlebitis of the internal jugular vein after an oropharyngeal
infection. Septic emboli can subsequently develop and, in the
preantibiotic era, fatality ensued within a few weeks of onset. This
entity was first described as “post anginal sepsis” in 1900 by
Courmont and Cade but gained it’s moniker after Andre Lemierre’s
extensive description in 19361. It was initially described as the
development of septic thrombophlebitis of the tonsillar and
peritonsillar veins followed by involvement of the parapharyngeal
space and internal jugular vein caused by Bacillus funduliformis
(now known as Fusiformis necrophorus) 2. While Lemierre‘s
syndrome was a frequent complication of deep neck infections in the
pre-antibiotic era, there was a steady decline in the incidence after
the advent of antibiotics and it became known as the “forgotten
disease.”3,4 Today, the incidence is estimated at between 0.6 and 2.3
million.5 However, over the past two decades, there has been an
increase in the number of cases of Lemierre’s being reported in the
literature. Lemierre’s is typically associated with anaerobic
infections in otherwise healthy young adults.2
We present a rare case of Methicillin-resistant Staphylococcus
aureus (MRSA) associated Lemierre’s in an eight week old male.
Case Presentation An eight week old male infant presented to an outside hospital with
a two day history of fever and left neck swelling. He was started on
beta-lactamase stable antibiotics for twenty four hours but was
transferred to our hospital after failing to improve. Prenatal and
medical history was only significant for being breastfed by his
mother who was a MRSA carrier. At presentation to our hospital, the
patient was noted to be very irritable with left sided neck swelling
and erythema, fevers (up to 102.3 F), leukocystosis (white blood
count 25) and decreased urine output. However, the patient was
hemodynamically stable with no evidence of airway instability. A
Computed tomography (CT) Scan of the neck with IV contrast was
obtained and demonstrated a low attenuation fluid collection
involving the retropharyngeal space and a rim enhancing abscess of
the left parapharyngeal space. The patient was taken emergently to
the operating room on Hospital Day #1 for incision and drainage of
the left deep neck abscess. Approximately 5ml of frank pus was
drained from the left parapharyngeal space and left internal jugular
vein thrombosis was identified. A penrose drain was left in place and
the patient was transferred to the Pediatric Intensive Care Unit in
critical condition. Given the patient’s maternal history of multiple
MRSA infections, the infant was empirically started on Vancomycin
and Clindamycin.
Figure 2. CT Neck with IV contrast obtained on Hospital Day #4 demonstrating progression of the fluid collections surrounding the left carotid sheath
Figure 1. CT Neck with IV contrast obtained on Hospital Day #1 demonstrating left paraphyargeal abscess, retropharygeal phelgmon and left internal jugular vein thrombosis (blue arrow).
Table 1. Cases of Staphylococcus aureus related Lemierre’s Syndrome. MRSA= methicillin resistant Staphylococcus aureus ; MSSA= methicillin sensitive Staphylococcus aureus
Conclusions
References
Lemierre syndrome is a rare but potentially life threatening infection which is on
the rise today, with a changing demographic as well as a changing microbial
pattern. Without awareness of MRSA as a causative agent, appropriate antibiotic
coverage may be delayed. Early diagnosis and intervention is essential. We
present the youngest cases of Lemierre’s Syndrome secondary to community
acquired MRSA.
Discussion Lemierre’s syndrome typically occurs in otherwise healthy young adults secondary to an
oropharyngeal infection. Internal jugular vein septic thrombosis develops and septic emboli
can lead to pulmonary and joint involvement.4 LS is typically associated with anaerobic
organisms: Fusobacterium necrphorum (57%), Fusobacterium species (30%) and
Fusobacterium nucleatum (3%) followed by anaerobic streptococci and other miscellaneous
Gram-negative anaerobes (10%).6 Staphylococcus aureus is not usually a cause of
Lemierre’s syndrome.
Classically described in older patients with Fusobacterium infections, there is a growing
body of literature reporting Lemierre’s syndrome secondary to MRSA infections, particularly
in a younger patient population suggesting a changing demographic as well as a changing
microbial pattern7,8. Since 2002, there have been 13 cases of septic internal jugular vein
thrombophlebitis secondary to staphylococcus aureus unrelated to catheter placement
reported in the literature.9-19 No reports were identified in the preceding 20 years. Nine of
these cases were caused by methicillin resistant staph aureus.9-13, 16-17 Five of these cases
occurred in pediatric patients (age range 3 months to 16 yrs), and three of these pts were
less than one year of age.3,9,10 The clinical presentation varied among the patients. Most
presented with facial or neck swelling and fever. All three children under 12 months of age,
presented with rapid onset neck swelling, fever and deep neck abscess formation.3,9,10 All
three of these patients underwent incision and drainage and prolonged treatment with IV
antibiotics and did well.3,9,10
Staphylococcus aureus is the most commonly isolated human bacterial pathogen. 6,20,21
Methicillin- resistant Staphylococcus aureus (MRSA) is increasingly prevalent in the United
States. There has been a dramatic increase in the number of both hospital acquired and
community acquired MRSA infections.20,21 MRSA related Lemierre’s Syndrome appears to
be more virulent and affecting a younger population of patients.13 Early diagnosis with
aggressive antimicrobial therapy and surgical intervention is key to effective treatment of
Lemierre syndrome and prevention of septic emboli.
Internal jugular vein thrombophelbitis usually manifests as unilateral swelling and pain at the
angle of the mandible and along the sternocleidomastoid muscle with associated trismus.
Although rare, Lemierre syndrome has the potential for significant morbidity and possibly
mortality. Early recognition and aggressive treatment are important in preventing severe
systemic manifestations. We recommend CT scan imaging with IV contrast or ultrasound
imaging for diagnosis. Empiric broad spectrum antibiotics should be started as soon as
possible. MRSA should be considered in all patients not responding to beta lactamase
stable conventional antibiotic therapy. While the effectiveness of anticoagulation is still
unclear, anticoagulation to prevent progression of thrombus or development of septic emboli
may be considered in the treatment algorithm of Lemierre’s. Anticoagulation therapy is thus
based on individual patient cases and physician preferences rather than firm evidence.
A B
A B
A B
B
Reference for case (Year)
Age of Patient
Bacterium
Complications
Intervention for Thrombosis
Kalyoussef et al (2012) 2 month old MRSA Medistinal abscess,sigmoid sinus thrombosis Heparin/Enoxaparin
Fleish et al. (2007) 3 month old MRSA Mediastinal abscess Enoxaparin
Hoehn et al. (2010) 7 month old MRSA Septic pulmonary emboili, epyema, pneumothorax, hemorrhagic pericardial effuusion Enoxaparin/Heparin
Fong and Watson (2002) 7month old MRSA Septic pulmonary emboili, cervical abscess, mastoiditis None Bently and Brennan (2009) 8yr MRSA Multiple pulmonary nodules ASA Kadhiravan et al. (2008) 16 yr MRSA
Septic pulmonary emboil, cavitation, pleural effusion, hemoptysis None
Chanin et al (2011) 22yr MRSA Necrotizing pneumonia and cerebral infarcts None Puymirat et al. (2008) 22yr MSSA
Multiple pulmonary nodules, cavitation, cavernous sinus thrombosis Heparin, ligation of IJ/EJ
Boga et al. (2007) 22yr MRSA Multiple small nodular lung densities, pleural effusion Heparin
Bilal et al. (2009) 30yr MRSA Multiple pulmonary nodules, pleural effusion, cavernous sinus thrombosis, cranial nerve palsies Heparin/Warfarin
Shivashakar et al. (2008) 32yr MSSA
Pulmonary nodules/abscesses, cavernous sinus thrombosis, cerebral infarcts
Heparin/Warfarin/Activated Protein C
Herek et al. (2010) 33yr MRSA Multiple septic pulmonary emboli, necrotizing pneumonia None
Ceylan et al. (2009) 80yr MSSA Pulmonary nodular infiltrates, pleural effusions None
Lemierre A: On certain septicemias due to anaerobic organisms. Lancet. 1936; 1: 701-703.
Ridgway JM, Parikh DA, Wright R, et al. Lemierre syndrome: a pediatric case series and review of literature. American Journal of Otolaryngology-Head and Neck Medicine and Surgery. 2010; 31:
38-45.
Hoehn KS, Capouya JD, Daum RS, et al: Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus complicated by hemorrhagic pericarditis. Pediatr
Crit Care Med. 2010; 11:e32-e35.
Mohamed BP, Carr L. Neurological complications in two children with Lemierre syndrome. Developmental Medicine & Child Neurology 2010; 52; 779-781.
Syed MI, Baring D, Addibdle M, et al. Lemierre syndrome: two cases and a review. Laryngoscope. 2007; 117:1605-10.
Karkos PD, Asrani S, Karkos CD, et al. Lemierre’s Syndrome: A Systematic Review. Laryngoscope. 2009; 119: 1552-1559.
Hagelskajaer LH, Prag J, Malczynski J, et al. Incidence and clinical epidemiology of necrobacillosis, including Lemierre’s syndrome, in Denmark 1990-1995. Eur J Clin Microbiol Infect Dis. 1998;
17: 561-565.
Chirinos JA, Lichstein DM, Garcia J, et al. The evolution of Lemierre syndrome: report of 2 cases and review of literature. Medicine. 2002; 81: 458-465.
Fleisch AF, Nolan S, Gerber JG, et al. Methicillin-Resistant Staphylococcus aureus as a cause of extensive retropharyngeal abscess in two infants. The Pediatric Infectious Disease Journal. 2007;
26 (12): 1161-1163.
Fong SM, Watson M. Lemierre syndrome due to non-multiresistant methicillin-resistant Staphylococcus aureus. J Paediatr Child Health. 2002; 38: 305-307.
Bentley TP, Brennan DF. Lemierre’s syndrome: methicillin- resistant Staphylococcus aureus (MRSA) finds a new home. The Journal of Emergency Medicine. 2009; 37 (2): 131-134.
Kadhiravan T, Piramanayagam P, Banga A, et al. Lemierre’s syndrome due to community acquired methicillin-resistant Staphylococcus aureus infection and presenting with orbital cellulitis: a
case report. Journal of Medical Case Reports. 2008; 3: 374-377.
Chanin JM, Marcos LA, Thompson BM, et al. Methicillin- resistant Staphylococcus aureus USA 300 Clone as a cause of Lemierre’s Syndrome. Journal of Clinical Microbiology. 2011; 49 (5): 2063-
2066.
Boga C, Ozdogu H, Diri B, et al. Lemierre syndrome variant: Staphylococcus aureus associated with thrombosis of both the right internal jugular vein and the splenic vein after the exploration of a
river cave. J Thromb Thrombolysis. 2007; 23: 151-154.
Shivasankar GH, Murukesh N, Varma MPS, et al. Infection by panton-valentine leukocidin-producing Staphylococcus aureus clinically mimicking Lemierre’s syndrome. Journal of Medical
Microbiology. 2008; 57: 118-120.
Bilal M, Cleveland KO, Gelfand MS. Community –acquired methicillin-resistatn Staphylococcus aureus and Lemierre Syndrome. Am J Med Sci. 2009; 338 (4): 326-327.
Herek PA, lewis T, Bailitz JM. An unusual case of Lemierre’s Syndrome due to methicillin- resistant Staphylococcus aureus. The Journal of Emergency Medicine. 2010; 39 (5): 644-646.
Puymirat E, Biais M, Camou F, et al. A Lemierre Syndrome variant caused by Staphylococcus aureus. 2008; 26: 380.e5-380. e7.
Ceylan BG, Yavuz L, Baydar CL, et al. Lemierre Syndrome: a case of rarely isolated microorganism, Staphylococcus aureus. Med Sci MOnit. 2009: 15 (3): CS58-61.
Lowy FD: Staphylococcus aureus infections. N Engl J Med. 1998; 339: 520-532.
David MZ, Glikman D, Crawford SE, et al: What is community-associated methicillin resistant Staphylococcus aureus? J infect Dis. 200l; 197: 1235-1243.
The patient improved slightly but had persistent purulent drainage through the penrose
drain and remained febrile. Repeat magnetic resonance imaging was ordered to
evaluate whether the fluid collections were adequately being drained by the penrose
drain however the patient developed worsening cellulitis with progression to the
contralateral side. The patient became hemodynamically unstable in the MRI suite with
evidence of septic shock. He was taken back to the intensive care unit where he was
stabilized. Repeat CT imaging was obtained the following day when the patient was
hemodynamically stable. Repeat imaging demonstrated some progression of the fluid
collection surrounding the left carotid sheath, peripheral rim enhancement surrounding
the thymus and anterior mediastinum as well as progression of the left internal jugular
thrombosis into the chest and retrograde to involve the sigmoid sinus. The patient was
taken back to the Operating Room on Hospital Day # 4 for neck re-exploration and
drainage of the retropharyngeal abscess as well as drainage of mediastinal abscess by
Pediatric Surgery. Multiple passive drains were placed in the wound and patient was
monitored closely postoperatively. The internal jugular vein was not ligated as the
thrombosis extended beyond the neck. Instead, the patient was started on a heparin
drip after his second operative trip and transitioned to Enoxaparin (1mg/kg per dose
subcutaneously every 12hours). Repeat imaging demonstrated recannulation and
complete resolution of the transverse and sigmoid sinus blood thrombi. Wound cultures
were consistent with MRSA.
The patient was treated with IV antibiotics (Vancomycin and Rifampin) for a total of 15
days. He was discharged home on Bactrim for two additional weeks and left the
hospitable in stable condition without any long term sequelae. The family and his
patient were also treated with daily chlorhexidine baths.