- Email: [email protected]
Lemierre syndrome complicated by cerebral abscess
American Journal of Emergency Medicine (2013) 31, 458.e1–458.e3
www.elsevier.com/locate/ajem
Case Report Lemierre syndrome complicated by cerebral abscess Abstract Often referred to as the “forgotten disease,” the incidence of Lemierre syndrome has seen a resurgence over the last 20 to 30 years. Cerebral abscesses are a rare complication of Lemierre syndrome, with only a few cases reported in the medical literature. We describe a case of Lemierre syndrome diagnosed in an 18-year-old man with complications of cerebral abscess and disseminated intravascular coagulation with a complicated course. Discussion will include causes for the resurgence of this disease and its initial management. We present this case to increase awareness of the potential for Lemierre syndrome in the young adult and adolescent population. In 1936, Andre Lemierre [1] described a condition in adolescents beginning with tonsillitis and developing into a suppurative thrombophlebitis of the internal jugular vein, leading to bacteremia with hematogenous spread to other organs. Lemierre syndrome is caused by Fusobacterium necrophorum, a gram-negative anaerobe, in approximately 90% of cases. However, this disease can be caused by anaerobic streptococci and other gram-negative anaerobes in approximately 10% of individuals [2]. Although the original case series reported a mortality of 90% in the preantibiotic era, there is still a mortality risk of 4% to 12% in patients with this syndrome [1,3]. In this case report, we describe a case of Lemierre syndrome in an 18-year-old man complicated by septic shock with metastatic spread to the lungs and brain and abscess formation requiring neurosurgical intervention. An 18-year-old man presented to the emergency department (ED) with a chief complaint of fever. His symptoms were described as 5 days of cough, neck pain, headache, and decreased fluid intake. His mother described a progression of his symptoms with breathing difficulty and a change in mental status during this time. The patient immigrated from Haiti 2 years previously to live with his mother; otherwise, no recent travel history was reported. His medical history was unremarkable, all developmental milestones were met appropriately, and his family history was not remarkable. A review of systems was notable for epistaxis, back pain and 0735-6757/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
neck pain, and decreased vision out of his left eye. No diarrhea, rashes, or sick contacts were reported. Physical examination revealed a young man in severe distress who was confused, disoriented, and sleepy but rousable. His triage vital signs showed a blood pressure of 114/73 mm Hg, a heart rate of 124 beats/min, a respiratory rate of 32 breaths/min, and a temperature of 102.7°F rectally. He had mild neck lymphadenopathy and tenderness, normal heart sounds, and normal breath sounds. His abdomen was soft and nontender with no organomegaly. His skin examination showed no rashes or petechia and was warm and dry. A complete neurological examination was unobtainable because of the lack of patient cooperation as a result of his altered mental status. Laboratory analysis revealed a white blood cell (WBC) count of 16.3109 /L with 26 x 109 /L platelets. The differential showed 23% band cells. Blood cultures were sent. He was hyponatremic with a sodium of 131 mEq/L, hypokalemic with a potassium of 3.3 mEq/L, and in acute renal failure with a blood urea nitrogen of 53 mg/dL, and a creatinine of 1.49 mg/dL. An arterial blood gas was normal; however, the shock panel revealed a lactate level of 3.4 mg/dL. A lumbar puncture was performed, revealing an opening pressure of 18.5 cm H20. The cell count for the cerebrospinal fluid (CSF) showed a WBC of 21/uL, with 88% neutrophils, 7% lymphocytes, and no bands; cerebrospinal fluid and blood cultures returned negative after 72 hours. Meningitic doses of ceftriaxone and vancomycin were started in the ED, and laboratory studies were repeated. Liver function tests were elevated with an aspartate aminotransferase 235 units/L and an alanine transaminase of 132 units/L, and his total bilirubin was 6.0 mg/dL, with a direct bilirubin of 5.1 mg/dL. Further tests showed a C-reactive protein level of 327.6 mg/L and an erythrocyte sedimentation rate of 60 mm/hr. A chest x-ray showed a right lower lobe consolidation, with the patient's urinalysis demonstrating 50 to 75 red blood cells/high-power field, 0 to 2 WBCs/high-power field, and loaded bacteria. A bedside ultrasound was also performed in the ED. The echocardiogram showed adequate filling, and a sonogram of the gallbladder revealed no cholelithiasis or cholecystitis. Further coagulation studies were drawn revealing a D-dimer level of 26.34 mg/L (reference range, 0.5-2.0 mg/L), a fibrin degradation product level of higher than 20 μg/mL
458.e2
Case Report
(b5 μg/mL), and a prothrombin time and partial thromboplastin time both mildly elevated at 14.0 seconds and 31.7 seconds, respectively. Clindamycin was later added for broader antibiotic coverage. A computed tomography scan of the head revealed a low attenuation subdural collection along the left frontal lobe that likely represented empyema formation and opacification of the left maxillary sinus. Carotid Dopplers revealed extensive thrombus in the right internal jugular vein extending from the intracranial segment (Fig. 1). A magnetic resonance angiogram performed later that day further confirmed the thrombosis seen on carotid Dopplers with possible thrombotic involvement of the cavernous sinus and evidence of cerebritis. Diffuse disease was also noted of the bilateral frontal and ethmoid sinuses and left maxillary sinus with possible extension into the left orbit. The patient was admitted to the pediatric intensive care unit for acute renal failure, disseminated intravascular coagulation, septic shock, infected venous thrombus, bacterial meningitis, and bacterial pneumonia probably representing septic emboli. Immediate interventions included broad-spectrum antibiotics with piperacillin/tazobactam and vancomycin and aggressive hydration with hypertonic saline mixed with 5% dextrose in normal saline. Platelets were transfused, and after consultation with hematology, lowmolecular-weight heparin was started. Dopamine was started later in day 1 of hospital admission because patient was hypotensive despite 5 L of intravenous fluids. ENT, neurosurgical, and neurology consult obtained later that day did not feel that patient would benefit from surgical intervention and recommended continued medical management with Fosphenytoin for seizure prophylaxis. On day 2 of hospital admission, the patient was intubated due to respiratory distress and worsening mental status, with the patient's chest x-ray showing worsening infiltrates and increased pulmonary edema (Fig. 2). Norepinephrine was later added for blood pressure maintenance. The patient's overall clinical status continued to improve over the next few days. When finally deemed stable enough
Fig. 1
Carotid Dopplers of the right internal jugular vein.
Fig. 2
Chest x-ray showing infiltrates and pulmonary edema.
for a repeat computed tomographic scan (Fig. 3), an enlarging left front epidural abscess measuring 4.4 × 0.8 cm with minimal midline shift and extensive sinus disease was observed. ENT and neurosurgery both recommended surgical intervention in light of these new findings. Subsequently, the patient underwent endoscopic sinus drainage and craniotomy with resolution of abscess (Fig. 4), without complication on hospital days 12 and 13, respectively. On hospital day 15, the patient was transferred to the pediatric floor from the pediatric intensive care unit. The patient remained hospitalized for 21 days, and his condition improved without any major adverse events. The patient was evaluated for possible immunocompromising disease, but human immunodeficiency virus load was
Fig. 3 Computed tomography of the head shows an enlarging left front epidural abscess.
Case Report
Fig. 4 Computed tomography of the head after endoscopic sinus drainage and craniotomy.
negative, with less than 48 copies/mL. A peripheral inserted central catheter line was placed on hospital day 26 to receive 6 weeks of intravenous antibiotics. Skilled visiting nursing services were obtained, and on hospital day 35, the patient was discharged. Although classically described to most medical students as “the forgotten disease,” the incidence of Lemierre syndrome has seen a resurgence over the last 20 to 30 years. Multiple theories have been entertained for the explanation to this increased prevalence, with one theory being better proficiency in diagnosis of this disease. However, more plausible reasons for the increase in Lemierre syndrome and Fusobacterium infections probably lie in the decreased use of the Fusobacterium-sensitive antibiotics, penicillin and amoxicillin, for oropharyngeal infections and their replacement with macrolides and second- and third-generation cephalosporins, with less frequent dosing schedules that lack activity against Fusobacterium species [4]. New literature has mandated that our vigilance be increased in assessing for these infections because it has been shown that 10% of sore throats in adolescents and young adults may be caused by F necrophorum [5]. In addition, a study in Denmark has noted a rate of F necrophorum infections of 21% complicating peritonsilar abscesses in adolescents [6]. These same authors calculate that approximately 1 in 400 cases of F necrophorum will progress to Lemierre syndrome [5]. If Lemierre syndrome is suspected, treatment includes aggressive management with early recognition; adequate antibiotic coverage; appropriate surgical management, such
458.e3 as drainage, if indicated; and intensive care unit monitoring. Penicillin and metronidazole for several weeks are a widely accepted treatment method, with clindamycin recommended for those with penicillin allergy. In metastatic cases, such as ours, abscess or empyema drainage may be necessary [3]. Although heparin was initiated in our patient, the use of anticoagulation is an area of continuing debate [3,7,8]. Hyperbaric oxygen therapy has been described in the literature in case reports but is currently not the standard of care [9]. Although relatively unheard of after widespread antibiotic use, Lemierre syndrome is on the rise because of multiple factors. The primary causative agent, F necrophorum, is much more prevalent than once thought, and high suspicion must be maintained when confronted with an adolescent or a young adult with sore throat or peritonsilar abscess. If suspected, aggressive management is needed with penicillin and metronidizole or clindamycin with intensive care unit evaluation and abscess drainage, where indicated. Water Valesky MD Konstantinos Agoritsas MD Downstate Medical Center Kings County Hospital/State University of New York, Brooklyn, NY E-mail address: [email protected] http://dx.doi.org/10.1016/j.ajem.2012.08.004
References [1] Lemierre A. Septicaemias and anaerobic organisms. Lancet 1936;1: 701-3. [2] Karkos PD, Asrani S, Karkos CD, et al. Lemierre's syndrome: a systematic review. Laryngoscope 2009;119:1552-9. [3] Hagelskjaer K, 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. [4] Ramirez S, Hild TG, Rudolph CN, Sty JR, Kehl SC, Havens P, et al. Increased diagnosis of Lemierre syndrome and other Fusobacterium necrophorum infections at a children's hospital. Pediatrics 2003; 112(5):e380. [5] Centor RM. Expand the pharyngitis paradigm for adolescents and young adults. Ann Intern Med 2009;151(11):812-5. [6] Hagelskjaer KL, Prag J. Localised Fusobacterium necrophorum infections: a prospective laboratory-based Danish study. Eur J Clin Microbiol Dis 2008;27(8):733-9. [7] Hoehn KS. Lemierre's syndrome: the controversy of anticoagulation. Pediatrics 2005;115(5):1415-6. [8] Goldenberg NA, Knapp-Clevenger R, Hays T, Manco-Johnson MJ. Lemierre's and Lemierre's-like syndromes in children: survival and thromboembolic outcomes. Pediatrics 2005;116(4):e543-8. [9] Hodgson R, Emig M, Pisarello J. Hyperbaric oxygen (HBO2) in the treatment of Lemierre syndrome. Undersea Hyperb Med 2003;30(2): 87-91.
www.elsevier.com/locate/ajem
Case Report Lemierre syndrome complicated by cerebral abscess Abstract Often referred to as the “forgotten disease,” the incidence of Lemierre syndrome has seen a resurgence over the last 20 to 30 years. Cerebral abscesses are a rare complication of Lemierre syndrome, with only a few cases reported in the medical literature. We describe a case of Lemierre syndrome diagnosed in an 18-year-old man with complications of cerebral abscess and disseminated intravascular coagulation with a complicated course. Discussion will include causes for the resurgence of this disease and its initial management. We present this case to increase awareness of the potential for Lemierre syndrome in the young adult and adolescent population. In 1936, Andre Lemierre [1] described a condition in adolescents beginning with tonsillitis and developing into a suppurative thrombophlebitis of the internal jugular vein, leading to bacteremia with hematogenous spread to other organs. Lemierre syndrome is caused by Fusobacterium necrophorum, a gram-negative anaerobe, in approximately 90% of cases. However, this disease can be caused by anaerobic streptococci and other gram-negative anaerobes in approximately 10% of individuals [2]. Although the original case series reported a mortality of 90% in the preantibiotic era, there is still a mortality risk of 4% to 12% in patients with this syndrome [1,3]. In this case report, we describe a case of Lemierre syndrome in an 18-year-old man complicated by septic shock with metastatic spread to the lungs and brain and abscess formation requiring neurosurgical intervention. An 18-year-old man presented to the emergency department (ED) with a chief complaint of fever. His symptoms were described as 5 days of cough, neck pain, headache, and decreased fluid intake. His mother described a progression of his symptoms with breathing difficulty and a change in mental status during this time. The patient immigrated from Haiti 2 years previously to live with his mother; otherwise, no recent travel history was reported. His medical history was unremarkable, all developmental milestones were met appropriately, and his family history was not remarkable. A review of systems was notable for epistaxis, back pain and 0735-6757/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
neck pain, and decreased vision out of his left eye. No diarrhea, rashes, or sick contacts were reported. Physical examination revealed a young man in severe distress who was confused, disoriented, and sleepy but rousable. His triage vital signs showed a blood pressure of 114/73 mm Hg, a heart rate of 124 beats/min, a respiratory rate of 32 breaths/min, and a temperature of 102.7°F rectally. He had mild neck lymphadenopathy and tenderness, normal heart sounds, and normal breath sounds. His abdomen was soft and nontender with no organomegaly. His skin examination showed no rashes or petechia and was warm and dry. A complete neurological examination was unobtainable because of the lack of patient cooperation as a result of his altered mental status. Laboratory analysis revealed a white blood cell (WBC) count of 16.3109 /L with 26 x 109 /L platelets. The differential showed 23% band cells. Blood cultures were sent. He was hyponatremic with a sodium of 131 mEq/L, hypokalemic with a potassium of 3.3 mEq/L, and in acute renal failure with a blood urea nitrogen of 53 mg/dL, and a creatinine of 1.49 mg/dL. An arterial blood gas was normal; however, the shock panel revealed a lactate level of 3.4 mg/dL. A lumbar puncture was performed, revealing an opening pressure of 18.5 cm H20. The cell count for the cerebrospinal fluid (CSF) showed a WBC of 21/uL, with 88% neutrophils, 7% lymphocytes, and no bands; cerebrospinal fluid and blood cultures returned negative after 72 hours. Meningitic doses of ceftriaxone and vancomycin were started in the ED, and laboratory studies were repeated. Liver function tests were elevated with an aspartate aminotransferase 235 units/L and an alanine transaminase of 132 units/L, and his total bilirubin was 6.0 mg/dL, with a direct bilirubin of 5.1 mg/dL. Further tests showed a C-reactive protein level of 327.6 mg/L and an erythrocyte sedimentation rate of 60 mm/hr. A chest x-ray showed a right lower lobe consolidation, with the patient's urinalysis demonstrating 50 to 75 red blood cells/high-power field, 0 to 2 WBCs/high-power field, and loaded bacteria. A bedside ultrasound was also performed in the ED. The echocardiogram showed adequate filling, and a sonogram of the gallbladder revealed no cholelithiasis or cholecystitis. Further coagulation studies were drawn revealing a D-dimer level of 26.34 mg/L (reference range, 0.5-2.0 mg/L), a fibrin degradation product level of higher than 20 μg/mL
458.e2
Case Report
(b5 μg/mL), and a prothrombin time and partial thromboplastin time both mildly elevated at 14.0 seconds and 31.7 seconds, respectively. Clindamycin was later added for broader antibiotic coverage. A computed tomography scan of the head revealed a low attenuation subdural collection along the left frontal lobe that likely represented empyema formation and opacification of the left maxillary sinus. Carotid Dopplers revealed extensive thrombus in the right internal jugular vein extending from the intracranial segment (Fig. 1). A magnetic resonance angiogram performed later that day further confirmed the thrombosis seen on carotid Dopplers with possible thrombotic involvement of the cavernous sinus and evidence of cerebritis. Diffuse disease was also noted of the bilateral frontal and ethmoid sinuses and left maxillary sinus with possible extension into the left orbit. The patient was admitted to the pediatric intensive care unit for acute renal failure, disseminated intravascular coagulation, septic shock, infected venous thrombus, bacterial meningitis, and bacterial pneumonia probably representing septic emboli. Immediate interventions included broad-spectrum antibiotics with piperacillin/tazobactam and vancomycin and aggressive hydration with hypertonic saline mixed with 5% dextrose in normal saline. Platelets were transfused, and after consultation with hematology, lowmolecular-weight heparin was started. Dopamine was started later in day 1 of hospital admission because patient was hypotensive despite 5 L of intravenous fluids. ENT, neurosurgical, and neurology consult obtained later that day did not feel that patient would benefit from surgical intervention and recommended continued medical management with Fosphenytoin for seizure prophylaxis. On day 2 of hospital admission, the patient was intubated due to respiratory distress and worsening mental status, with the patient's chest x-ray showing worsening infiltrates and increased pulmonary edema (Fig. 2). Norepinephrine was later added for blood pressure maintenance. The patient's overall clinical status continued to improve over the next few days. When finally deemed stable enough
Fig. 1
Carotid Dopplers of the right internal jugular vein.
Fig. 2
Chest x-ray showing infiltrates and pulmonary edema.
for a repeat computed tomographic scan (Fig. 3), an enlarging left front epidural abscess measuring 4.4 × 0.8 cm with minimal midline shift and extensive sinus disease was observed. ENT and neurosurgery both recommended surgical intervention in light of these new findings. Subsequently, the patient underwent endoscopic sinus drainage and craniotomy with resolution of abscess (Fig. 4), without complication on hospital days 12 and 13, respectively. On hospital day 15, the patient was transferred to the pediatric floor from the pediatric intensive care unit. The patient remained hospitalized for 21 days, and his condition improved without any major adverse events. The patient was evaluated for possible immunocompromising disease, but human immunodeficiency virus load was
Fig. 3 Computed tomography of the head shows an enlarging left front epidural abscess.
Case Report
Fig. 4 Computed tomography of the head after endoscopic sinus drainage and craniotomy.
negative, with less than 48 copies/mL. A peripheral inserted central catheter line was placed on hospital day 26 to receive 6 weeks of intravenous antibiotics. Skilled visiting nursing services were obtained, and on hospital day 35, the patient was discharged. Although classically described to most medical students as “the forgotten disease,” the incidence of Lemierre syndrome has seen a resurgence over the last 20 to 30 years. Multiple theories have been entertained for the explanation to this increased prevalence, with one theory being better proficiency in diagnosis of this disease. However, more plausible reasons for the increase in Lemierre syndrome and Fusobacterium infections probably lie in the decreased use of the Fusobacterium-sensitive antibiotics, penicillin and amoxicillin, for oropharyngeal infections and their replacement with macrolides and second- and third-generation cephalosporins, with less frequent dosing schedules that lack activity against Fusobacterium species [4]. New literature has mandated that our vigilance be increased in assessing for these infections because it has been shown that 10% of sore throats in adolescents and young adults may be caused by F necrophorum [5]. In addition, a study in Denmark has noted a rate of F necrophorum infections of 21% complicating peritonsilar abscesses in adolescents [6]. These same authors calculate that approximately 1 in 400 cases of F necrophorum will progress to Lemierre syndrome [5]. If Lemierre syndrome is suspected, treatment includes aggressive management with early recognition; adequate antibiotic coverage; appropriate surgical management, such
458.e3 as drainage, if indicated; and intensive care unit monitoring. Penicillin and metronidazole for several weeks are a widely accepted treatment method, with clindamycin recommended for those with penicillin allergy. In metastatic cases, such as ours, abscess or empyema drainage may be necessary [3]. Although heparin was initiated in our patient, the use of anticoagulation is an area of continuing debate [3,7,8]. Hyperbaric oxygen therapy has been described in the literature in case reports but is currently not the standard of care [9]. Although relatively unheard of after widespread antibiotic use, Lemierre syndrome is on the rise because of multiple factors. The primary causative agent, F necrophorum, is much more prevalent than once thought, and high suspicion must be maintained when confronted with an adolescent or a young adult with sore throat or peritonsilar abscess. If suspected, aggressive management is needed with penicillin and metronidizole or clindamycin with intensive care unit evaluation and abscess drainage, where indicated. Water Valesky MD Konstantinos Agoritsas MD Downstate Medical Center Kings County Hospital/State University of New York, Brooklyn, NY E-mail address: [email protected] http://dx.doi.org/10.1016/j.ajem.2012.08.004
References [1] Lemierre A. Septicaemias and anaerobic organisms. Lancet 1936;1: 701-3. [2] Karkos PD, Asrani S, Karkos CD, et al. Lemierre's syndrome: a systematic review. Laryngoscope 2009;119:1552-9. [3] Hagelskjaer K, 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. [4] Ramirez S, Hild TG, Rudolph CN, Sty JR, Kehl SC, Havens P, et al. Increased diagnosis of Lemierre syndrome and other Fusobacterium necrophorum infections at a children's hospital. Pediatrics 2003; 112(5):e380. [5] Centor RM. Expand the pharyngitis paradigm for adolescents and young adults. Ann Intern Med 2009;151(11):812-5. [6] Hagelskjaer KL, Prag J. Localised Fusobacterium necrophorum infections: a prospective laboratory-based Danish study. Eur J Clin Microbiol Dis 2008;27(8):733-9. [7] Hoehn KS. Lemierre's syndrome: the controversy of anticoagulation. Pediatrics 2005;115(5):1415-6. [8] Goldenberg NA, Knapp-Clevenger R, Hays T, Manco-Johnson MJ. Lemierre's and Lemierre's-like syndromes in children: survival and thromboembolic outcomes. Pediatrics 2005;116(4):e543-8. [9] Hodgson R, Emig M, Pisarello J. Hyperbaric oxygen (HBO2) in the treatment of Lemierre syndrome. Undersea Hyperb Med 2003;30(2): 87-91.