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A 10-month-old with Lemierre syndrome complicated by purulent pericarditis
American Journal of Emergency Medicine (2013) 31, 274.e5–274.e7
www.elsevier.com/locate/ajem
Case Report A 10-month-old with Lemierre syndrome complicated by purulent pericarditis
Abstract Lemierre syndrome is a potentially life-threatening septic thrombophlebitis associated with a neck infection. We present a case of a 10-month-old female infant with Lemierre syndrome complicated by thrombotic strokes and purulent pericarditis. A healthy 10-month-old female infant presented to the pediatric emergency department of our tertiary care center complaining of 5 days of fever to 105°F and 1 day of neck stiffness and decreased oral intake. In the pediatric emergency department, she developed septic shock, requiring vasopressor support, endotracheal intubation, and broad-spectrum antibiotics. A computed tomographic scan demonstrated a neck abscess associated with a right internal jugular thrombus and septic emboli to her lungs and brain. This constellation was consistent with Lemierre syndrome. Further studies demonstrated the thrombus extended into her left ventricular outflow tract. She was emergently taken to the operating room for incision and drainage of her neck abscess, started on anticoagulation with heparin, and eventually transitioned to enoxaparin. Her hospital course was complicated by a middle cerebral artery distribution infarction and subsequent hemorrhagic conversion with generalized tonic clonic seizures managed by levetiracetam. Ten days into her hospital stay, she developed pericardial tamponade, and cardiac surgery performed a pericardial window for loculated, purulent pericardial effusion. Initial blood cultures were positive for methicillin-sensitive Staphylococcus aureus, and the patient was treated with 6 weeks of nafcillin. She has recovered with minimal permanent sequelae. This is one of the youngest cases of Lemierre syndrome documented. To our knowledge, it is also the first case complicated by purulent pericarditis reported in the literature. A 10-month-old, previously healthy girl presented to the Pediatric Emergency Department (PED) of our tertiary care center with a 5-day history of intermittent fever as high as 105°F, upper respiratory illness symptoms, and decreased oral intake. She had been evaluated 4 days previously in the 0735-6757/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
same PED for the same complaints and diagnosed with a viral illness and discharged. The day of the second presentation to the PED, she was seen by her pediatrician, where she appeared ill and was sent to the PED for further evaluation and treatment. Her family noticed a worsening of her fever, despite antipyretics, and a refusal to move her head to the right for 24 hours. She had no significant medical history, no family history of thrombophilia or immunodeficiency, and no allergies, and her immunizations were up to date. She had recently been on amoxicillin for otitis media. Her initial vitals were heart rate of 167 beats per minute, blood pressure of 93/68 mm Hg, respiratory rate of 40 breaths per minute, oxygen saturation of 96% on room air, and a temperature of 102.5°F orally. She initially was alert and irritable, but consolable. Her tympanic membranes were normal bilaterally; her neck had tender anterior cervical lymphadenopathy; and her tonsils were symmetric, without exudate bilaterally. She displayed significant discomfort when moving her neck to the right. Given the concern for a retropharyngeal abscess, a contrasted computed tomographic (CT) study of her neck was obtained along with a complete blood count and blood cultures. The CT demonstrated a right posterior neck abscess with corresponding thrombosis of the right internal jugular vein extending to the right sigmoid sinus (Fig. 1). She also had numerous pulmonary nodules consistent with septic emboli. This constellation of symptoms was consistent with Lemierre syndrome. Upon return from the CT, the patient quickly decompensated and became more lethargic, tachycardic, tachypneic, and hypoxic, with increasing capillary refill. She was emergently intubated. Direct laryngoscopy demonstrated swollen vocal cords and tracheal deviation to the left. She received fluid resuscitation with multiple 20 mL/kg normal saline boluses. She was given broad-spectrum antibiotics including vancomycin, cefuroxime, and clindamycin. Despite fluid resuscitation of more than 60 mL/kg of normal saline, she became progressively more hypotensive to a blood pressure of 80/45 mm Hg, requiring a dopamine infusion for vasopressor support. An otolaryngology consultation was obtained emergently, and the patient was taken immediately to the operating room for incision and drainage of her neck
274.e6
Case Report
Fig. 1 Axial image of right posterior neck abscess and thrombosis of internal jugular vein.
abscess in an effort to obtain source control. The otolaryngology team successfully drained her posterior neck abscess and was able to get pus from the left subglottic region. Noted was an abscess cavity containing approximately 4 mL of pus adjacent to the right internal jugular vein just below the skull base. Postoperatively, she was started on a heparin infusion for her thrombosis. The pediatric intensive care unit physicians identified a new, harsh systolic heart murmur on her admission physical examination after surgery. Cardiology was consulted, and a transthoracic echocardiogram was obtained that demonstrated a linear echodensity adhered to the mitral valve, measuring 3.5 mm long and protruding into the left ventricular outflow tract. This was consistent with thrombus and endocarditis. She had an associated small pericardial effusion. The blood cultures grew gram-positive cocci in clusters within 24 hours of collection, which later speciated to methicillin-sensitive Staphylococcus aureus, and antibiotics were subsequently narrowed to nafcillin. Serial echocardiograms showed resolution of her intracardiac echodensity after receiving heparin. Her vasopressor support was quickly weaned, and on hospital day 3, she was successfully extubated. On hospital day 3, the patient developed generalized tonic clonic seizures, which were treated with benzodiazepines and levetiracetam. An emergent magnetic resonance imaging (MRI) study with venogram demonstrated 2 large areas of infarct in the middle cerebral artery (MCA) distribution, consistent with septic emboli, with 2 punctate foci of microhemorrhage within the areas of infarct (Figs. 2 and 3). An MRV (venogram) demonstrated thrombosis of the distal right transverse sinus and sigmoid sinus as well. Repeat imaging throughout her hospital stay demonstrated no further hemorrhage.
Fig. 2 Axial MRI image of MRI demonstrating left MCA distribution cerebral infarction.
On hospital day 10, she became hypotensive to 68/43 with muffled heart sounds. Emergent echocardiogram demonstrated a loculated pericardial effusion and early pericardial tamponade. Cardiac surgery was consulted, and she underwent emergent pericardial window and placement of mediastinal drains for loculated purulent pericarditis. One drain was removed after 5 days; the second was removed 2 days later. The pericardial fluid was cultured but did not grow any bacteria.
Fig. 3 Axial MRI image of MRI demonstrating left MCA distribution cerebral infarction.
Case Report The patient continued to steadily improve and was ultimately discharged on hospital day 19, with 6 weeks of home intravenous nafcillin, 1 mg/kg of enoxaparin subcutaneously twice daily, and levetiracetam for seizure prophylaxis. Since discharge, the patient has done remarkably well. She was readmitted briefly 2 weeks postdischarge after she developed a fever of 103°F and seizure activity. However, pan-cultures were no growth, and she was discharged home to continue her nafcillin. Although she does have some verbal delay, she has had rare seizure activity. She has been evaluated by cardiology with subsequent normal echocardiograms. The patient completed her 8-week course of enoxaparin and requires no current anticoagulants. Her growth and development have had no significant issues with minimal long-term sequelae from her Lemierre syndrome. Lemierre syndrome is septic thrombophlebitis associated with a throat or neck infection. The syndrome was first described in 1918 but is named after Andre Lemierre, who, in 1936, published a series of 20 cases of anaerobic bacteremia stemming from pharyngitis [1]. Eighteen (98%) of those patients died in the 1930s; however, the associated mortality is currently estimated at 5% [2]. Although still a rare diagnosis in the emergency department, the documented reports of Lemierre syndrome are increasingly frequent [2-4]. Whether this is due to a true increase in incidence or increased awareness and diagnosis is not yet known. Regardless, Lemierre syndrome remains a serious, life-threatening complication of neck infections. This infection is most commonly caused by the anaerobic bacteria, Fusobacterium necrophorum; however, we demonstrate a case caused by methicillin-sensitive S aureus, a far less common pathogen. Complications can be wide ranging, including abscess, septic arthritis, meningitis, and stroke [2]. This is one of the youngest cases of Lemierre syndrome documented [2-7]. To our knowledge, it is also the first documented case that was complicated by purulent pericarditis. There has been one documented case of hemorrhagic pericarditis and cardiac tamponade as a complication of anticoagulation for a Lemierre-like syndrome [7]. However, the purulent pericardial tamponade in this patient was from extension of her septic thrombus and part of her original disease process, rather than a treatment complication. Purulent pericarditis is also a rare disease process. It is frequently the result of hematogenous spread and is commonly associated with an upper respiratory infection. Patients most frequently present with fever and cardiac tamponade. Echocardiography is the diagnostic test of choice. The most common causative organism in the antibiotic era is S aureus. A combination of antibiotics and surgical drainage is the mainstay of treatment [8-10]. Surgical options include subxiphoid tube placement and pericardiectomy, both of which have been proven effective. Without optimal treatment, mortality can approach 100%. The mortality with optimal treatment, however, is generally less than 20% [9,10]. Long-term complications,
274.e7 although rare, include constrictive pericarditis. Fortunately, recurrence is also rare. This young girl had a normal appearing posterior oropharynx and did not have frank torticollis. Therefore, in young children who cannot give a history, simply fever and neck pain or odynophagia should raise the suspicion for Lemierre syndrome. This case demonstrates the need to maintain a high index of suspicion for this condition, even at a young age, and to be prepared to evaluate and treat a wide host of associated complications. Rachel W. Root MD Tyler W. Barrett MD, MSCI Department of Emergency Medicine Vanderbilt University Medical Center Nashville 37232-4700, TN, USA E-mail address: [email protected] Thomas J. Abramo MD Department of Pediatrics Division of Pediatric Emergency Medicine Monroe Carell Jr. Children's Hospital at Vanderbilt Vanderbilt University Medical Center Nashville 37232-4700, TN, USA http://dx.doi.org/10.1016/j.ajem.2012.05.019
References [1] Lemierre A. On certain septicemias due to anaerobic organisms. Lancet 1936;1:701-3. [2] Karkos P, Asrani S, Karkos C, et al. Lemierre's syndrome: a systematic review. Laryngoscope 2009;119(8):1552-9. [3] Ramirez S, Hild T, Rudolph C, et al. Increased diagnosis of Lemierre's syndrome and other Fusobacterium necrophorum infections at a children's hospital. Pediatrics 2003;115(5):380. [4] Golderberg N, Knapp-Clevenger R, Hays T, et al. Lemierre's and Lemierre's-like syndromes in children: survival and thromboembolic outcomes. Pediatrics 2005;116(4):543-8. [5] Golderberg N, Knapp-Clevenger R, Hays T, et al. Respiratory failure and hypercoagulability in a toddler with Lemierre's syndrome. Pediatrics 2005;115(5):620-2. [6] Aspesberro F, Sibler T, Van Niewenhuyse JP, et al. Lemierre syndrome in a 5-month-old male infant: case report and review of the pediatric literature. Pediatr Crit Care Med 2008;9(5):35-7. [7] Hoehn K, Capouya J, Daum R, et al. Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus complicated by hemorrhagic pericarditis. Pediatr Crit Care Med 2010;11(3):32-5. [8] Bhaduri-McIntosh S, Prasad M, Moltedo J, et al. Purulent pericarditis caused by group 0041 Streptococcus. Tex Heart Inst J 2006;33(4): 519-22. [9] Roodpeyma S, Sadeghian N. Acute pericarditis in childhood: a 10-year experience. Pediatr Cardiol 2000;21(4):363-7. [10] Cakir O, Gurkan F, Balci AE, et al. Purulent pericarditis in childhood: ten years of experience. J Pediatr Surg 2002;37(10):1404-8.
www.elsevier.com/locate/ajem
Case Report A 10-month-old with Lemierre syndrome complicated by purulent pericarditis
Abstract Lemierre syndrome is a potentially life-threatening septic thrombophlebitis associated with a neck infection. We present a case of a 10-month-old female infant with Lemierre syndrome complicated by thrombotic strokes and purulent pericarditis. A healthy 10-month-old female infant presented to the pediatric emergency department of our tertiary care center complaining of 5 days of fever to 105°F and 1 day of neck stiffness and decreased oral intake. In the pediatric emergency department, she developed septic shock, requiring vasopressor support, endotracheal intubation, and broad-spectrum antibiotics. A computed tomographic scan demonstrated a neck abscess associated with a right internal jugular thrombus and septic emboli to her lungs and brain. This constellation was consistent with Lemierre syndrome. Further studies demonstrated the thrombus extended into her left ventricular outflow tract. She was emergently taken to the operating room for incision and drainage of her neck abscess, started on anticoagulation with heparin, and eventually transitioned to enoxaparin. Her hospital course was complicated by a middle cerebral artery distribution infarction and subsequent hemorrhagic conversion with generalized tonic clonic seizures managed by levetiracetam. Ten days into her hospital stay, she developed pericardial tamponade, and cardiac surgery performed a pericardial window for loculated, purulent pericardial effusion. Initial blood cultures were positive for methicillin-sensitive Staphylococcus aureus, and the patient was treated with 6 weeks of nafcillin. She has recovered with minimal permanent sequelae. This is one of the youngest cases of Lemierre syndrome documented. To our knowledge, it is also the first case complicated by purulent pericarditis reported in the literature. A 10-month-old, previously healthy girl presented to the Pediatric Emergency Department (PED) of our tertiary care center with a 5-day history of intermittent fever as high as 105°F, upper respiratory illness symptoms, and decreased oral intake. She had been evaluated 4 days previously in the 0735-6757/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
same PED for the same complaints and diagnosed with a viral illness and discharged. The day of the second presentation to the PED, she was seen by her pediatrician, where she appeared ill and was sent to the PED for further evaluation and treatment. Her family noticed a worsening of her fever, despite antipyretics, and a refusal to move her head to the right for 24 hours. She had no significant medical history, no family history of thrombophilia or immunodeficiency, and no allergies, and her immunizations were up to date. She had recently been on amoxicillin for otitis media. Her initial vitals were heart rate of 167 beats per minute, blood pressure of 93/68 mm Hg, respiratory rate of 40 breaths per minute, oxygen saturation of 96% on room air, and a temperature of 102.5°F orally. She initially was alert and irritable, but consolable. Her tympanic membranes were normal bilaterally; her neck had tender anterior cervical lymphadenopathy; and her tonsils were symmetric, without exudate bilaterally. She displayed significant discomfort when moving her neck to the right. Given the concern for a retropharyngeal abscess, a contrasted computed tomographic (CT) study of her neck was obtained along with a complete blood count and blood cultures. The CT demonstrated a right posterior neck abscess with corresponding thrombosis of the right internal jugular vein extending to the right sigmoid sinus (Fig. 1). She also had numerous pulmonary nodules consistent with septic emboli. This constellation of symptoms was consistent with Lemierre syndrome. Upon return from the CT, the patient quickly decompensated and became more lethargic, tachycardic, tachypneic, and hypoxic, with increasing capillary refill. She was emergently intubated. Direct laryngoscopy demonstrated swollen vocal cords and tracheal deviation to the left. She received fluid resuscitation with multiple 20 mL/kg normal saline boluses. She was given broad-spectrum antibiotics including vancomycin, cefuroxime, and clindamycin. Despite fluid resuscitation of more than 60 mL/kg of normal saline, she became progressively more hypotensive to a blood pressure of 80/45 mm Hg, requiring a dopamine infusion for vasopressor support. An otolaryngology consultation was obtained emergently, and the patient was taken immediately to the operating room for incision and drainage of her neck
274.e6
Case Report
Fig. 1 Axial image of right posterior neck abscess and thrombosis of internal jugular vein.
abscess in an effort to obtain source control. The otolaryngology team successfully drained her posterior neck abscess and was able to get pus from the left subglottic region. Noted was an abscess cavity containing approximately 4 mL of pus adjacent to the right internal jugular vein just below the skull base. Postoperatively, she was started on a heparin infusion for her thrombosis. The pediatric intensive care unit physicians identified a new, harsh systolic heart murmur on her admission physical examination after surgery. Cardiology was consulted, and a transthoracic echocardiogram was obtained that demonstrated a linear echodensity adhered to the mitral valve, measuring 3.5 mm long and protruding into the left ventricular outflow tract. This was consistent with thrombus and endocarditis. She had an associated small pericardial effusion. The blood cultures grew gram-positive cocci in clusters within 24 hours of collection, which later speciated to methicillin-sensitive Staphylococcus aureus, and antibiotics were subsequently narrowed to nafcillin. Serial echocardiograms showed resolution of her intracardiac echodensity after receiving heparin. Her vasopressor support was quickly weaned, and on hospital day 3, she was successfully extubated. On hospital day 3, the patient developed generalized tonic clonic seizures, which were treated with benzodiazepines and levetiracetam. An emergent magnetic resonance imaging (MRI) study with venogram demonstrated 2 large areas of infarct in the middle cerebral artery (MCA) distribution, consistent with septic emboli, with 2 punctate foci of microhemorrhage within the areas of infarct (Figs. 2 and 3). An MRV (venogram) demonstrated thrombosis of the distal right transverse sinus and sigmoid sinus as well. Repeat imaging throughout her hospital stay demonstrated no further hemorrhage.
Fig. 2 Axial MRI image of MRI demonstrating left MCA distribution cerebral infarction.
On hospital day 10, she became hypotensive to 68/43 with muffled heart sounds. Emergent echocardiogram demonstrated a loculated pericardial effusion and early pericardial tamponade. Cardiac surgery was consulted, and she underwent emergent pericardial window and placement of mediastinal drains for loculated purulent pericarditis. One drain was removed after 5 days; the second was removed 2 days later. The pericardial fluid was cultured but did not grow any bacteria.
Fig. 3 Axial MRI image of MRI demonstrating left MCA distribution cerebral infarction.
Case Report The patient continued to steadily improve and was ultimately discharged on hospital day 19, with 6 weeks of home intravenous nafcillin, 1 mg/kg of enoxaparin subcutaneously twice daily, and levetiracetam for seizure prophylaxis. Since discharge, the patient has done remarkably well. She was readmitted briefly 2 weeks postdischarge after she developed a fever of 103°F and seizure activity. However, pan-cultures were no growth, and she was discharged home to continue her nafcillin. Although she does have some verbal delay, she has had rare seizure activity. She has been evaluated by cardiology with subsequent normal echocardiograms. The patient completed her 8-week course of enoxaparin and requires no current anticoagulants. Her growth and development have had no significant issues with minimal long-term sequelae from her Lemierre syndrome. Lemierre syndrome is septic thrombophlebitis associated with a throat or neck infection. The syndrome was first described in 1918 but is named after Andre Lemierre, who, in 1936, published a series of 20 cases of anaerobic bacteremia stemming from pharyngitis [1]. Eighteen (98%) of those patients died in the 1930s; however, the associated mortality is currently estimated at 5% [2]. Although still a rare diagnosis in the emergency department, the documented reports of Lemierre syndrome are increasingly frequent [2-4]. Whether this is due to a true increase in incidence or increased awareness and diagnosis is not yet known. Regardless, Lemierre syndrome remains a serious, life-threatening complication of neck infections. This infection is most commonly caused by the anaerobic bacteria, Fusobacterium necrophorum; however, we demonstrate a case caused by methicillin-sensitive S aureus, a far less common pathogen. Complications can be wide ranging, including abscess, septic arthritis, meningitis, and stroke [2]. This is one of the youngest cases of Lemierre syndrome documented [2-7]. To our knowledge, it is also the first documented case that was complicated by purulent pericarditis. There has been one documented case of hemorrhagic pericarditis and cardiac tamponade as a complication of anticoagulation for a Lemierre-like syndrome [7]. However, the purulent pericardial tamponade in this patient was from extension of her septic thrombus and part of her original disease process, rather than a treatment complication. Purulent pericarditis is also a rare disease process. It is frequently the result of hematogenous spread and is commonly associated with an upper respiratory infection. Patients most frequently present with fever and cardiac tamponade. Echocardiography is the diagnostic test of choice. The most common causative organism in the antibiotic era is S aureus. A combination of antibiotics and surgical drainage is the mainstay of treatment [8-10]. Surgical options include subxiphoid tube placement and pericardiectomy, both of which have been proven effective. Without optimal treatment, mortality can approach 100%. The mortality with optimal treatment, however, is generally less than 20% [9,10]. Long-term complications,
274.e7 although rare, include constrictive pericarditis. Fortunately, recurrence is also rare. This young girl had a normal appearing posterior oropharynx and did not have frank torticollis. Therefore, in young children who cannot give a history, simply fever and neck pain or odynophagia should raise the suspicion for Lemierre syndrome. This case demonstrates the need to maintain a high index of suspicion for this condition, even at a young age, and to be prepared to evaluate and treat a wide host of associated complications. Rachel W. Root MD Tyler W. Barrett MD, MSCI Department of Emergency Medicine Vanderbilt University Medical Center Nashville 37232-4700, TN, USA E-mail address: [email protected] Thomas J. Abramo MD Department of Pediatrics Division of Pediatric Emergency Medicine Monroe Carell Jr. Children's Hospital at Vanderbilt Vanderbilt University Medical Center Nashville 37232-4700, TN, USA http://dx.doi.org/10.1016/j.ajem.2012.05.019
References [1] Lemierre A. On certain septicemias due to anaerobic organisms. Lancet 1936;1:701-3. [2] Karkos P, Asrani S, Karkos C, et al. Lemierre's syndrome: a systematic review. Laryngoscope 2009;119(8):1552-9. [3] Ramirez S, Hild T, Rudolph C, et al. Increased diagnosis of Lemierre's syndrome and other Fusobacterium necrophorum infections at a children's hospital. Pediatrics 2003;115(5):380. [4] Golderberg N, Knapp-Clevenger R, Hays T, et al. Lemierre's and Lemierre's-like syndromes in children: survival and thromboembolic outcomes. Pediatrics 2005;116(4):543-8. [5] Golderberg N, Knapp-Clevenger R, Hays T, et al. Respiratory failure and hypercoagulability in a toddler with Lemierre's syndrome. Pediatrics 2005;115(5):620-2. [6] Aspesberro F, Sibler T, Van Niewenhuyse JP, et al. Lemierre syndrome in a 5-month-old male infant: case report and review of the pediatric literature. Pediatr Crit Care Med 2008;9(5):35-7. [7] Hoehn K, Capouya J, Daum R, et al. Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus complicated by hemorrhagic pericarditis. Pediatr Crit Care Med 2010;11(3):32-5. [8] Bhaduri-McIntosh S, Prasad M, Moltedo J, et al. Purulent pericarditis caused by group 0041 Streptococcus. Tex Heart Inst J 2006;33(4): 519-22. [9] Roodpeyma S, Sadeghian N. Acute pericarditis in childhood: a 10-year experience. Pediatr Cardiol 2000;21(4):363-7. [10] Cakir O, Gurkan F, Balci AE, et al. Purulent pericarditis in childhood: ten years of experience. J Pediatr Surg 2002;37(10):1404-8.