Int J Colorectal Dis DOI 10.1007/s00384-015-2141-x
LETTER TO THE EDITOR
Acute appendicitis from the shadows of Lemierre disease Diwakar Ryali Sarma & Mubashir Mulla & Ioannis Panagopoulos
Accepted: 21 January 2015 # Springer-Verlag Berlin Heidelberg 2015
Dear Editor:
Introduction Lemierre’s syndrome is a rare disorder of young adults caused by the anaerobic bacterium, Fusobacterium necrophorum. The condition is characterized by a primary oropharyngeal infection with evidence of septic thrombophlebitis, exhibited by positive blood cultures, clinical or radiographic evidence of internal jugular vein thrombosis and at least one metastatic focus. The incidence of Lemierre’s syndrome is reported to be nearly one in a million. We describe a case of Lemierre’s syndrome initially presenting as acute appendicitis and evolving into full-blown severe sepsis.
Case report An 11-year-old Caucasian male, with no prior medical history, was evaluated in the emergency department for a 2-day history of sore throat, right iliac fossa pain, loss of appetite and fever. Physical exam revealed localized peritonism in the right iliac fossa and biochemical parameters showed very high inflammatory markers. He was D. R. Sarma (*) : I. Panagopoulos Darent Valley Hospital, Dartford and Gravesham NHS Trust, Darenth Wood Road, DA28DA Dartford, UK e-mail: [email protected]
taken up for a laparoscopic appendicectomy with the provisional diagnosis of acute appendicitis. Intra-operatively, an inflamed appendix with surrounding mesenteric adenitis and turbid fluid was noted in the pelvis. Appendicectomy was done in the standard manner and the specimen sent for histological examination. Immediate post operatively, the child developed severe sepsis with impending organ failure and had to be shifted to the intensive care unit. Over the next 24 hours, his clinical condition worsened with acute kidney injury, bilateral lung abscess and hypoxic brain injury. He was aggressively resuscitated in intensive care and multidisciplinary approach advocated in his ongoing management. He was on broad spectrum antibiotics with moderate response. Fortyeight-hour cultures from the blood, urine, sputum and peritoneal fluid showed mixed growth of aerobes and anaerobes with a remarkable isolation of F. necrophorum in the serum culture. The histology of the appendix showed acute suppurative appendicitis. Emergent antibiotic regimen was advocated according to the sensitivity and there was remarkable improvement of the patient. At this point, clear thrombophlebitis was noted in bilateral internal jugular veins (confirmed by Doppler ultrasound), erythema and congestion noted in the oropharynx. A clinical diagnosis of Lemierre’s syndrome presenting as acute appendicitis was made. Serial blood examination and axial imaging of the chest and abdomen showed resolving sepsis and lung abscess. Antibiotics and high dependency unit care was continued for a further 48 hours. There was significant improvement in the vital parameters and patient was discharged to ward-based management and home soon after. There was complete resolution of all the organ failures.
I. Panagopoulos e-mail: [email protected]
Discussion
M. Mulla Royal Gwent Hospital, Newport, UK e-mail: [email protected]
One of the forerunners in killer infectious disease in the preantibiotic era was Lemierre’s disease. As Alexander Fleming
Int J Colorectal Dis
discovered penicillin and its use became widespread to treat oropharyngeal infections, Lemierre’s syndrome was rarely reported. We present a case report of an 11-year-old presenting with acute appendicitis, masking a worsening pharyngitis and internal jugular vein thrombophlebitis (part of the severe sepsis of Lemierre’s disease) which illustrates that subsequent re-emergence of this often forgotten disease. Fusobacteria are nonmotile, sporulating, obligate anaerobic gram-negative rods that are normal flora in the human upper respiratory tract, gastrointestinal tract and female genital tract. Unlike other anaerobic bacteria, fusobacteria produce lipopolysaccharide endotoxin, leukocidin and hemolysin. Hemagglutinin production augments the fulminant nature of the disease, causing platelet aggregation and septic thrombus formation. The exact mechanism of invasion and penetration of the pharyngeal mucosa with superficial thrombophlebitis and metastatic septic emboli has not been determined. Literature review suggests that, in Fusobacterium pharyngitis, there is extension of septic focus from the peritonsillar space to the internal jugular vein. Once invasion of the internal jugular vein is achieved, the resultant bacteremia triggers platelet aggregation and thrombus formation. Thrombus formation and rapid bacterial growth result in a nidus for metastatic septic embolization. In our case report, septic embolization would have been into the appendicular end artery, leading to the presentation with an acute surgical abdomen. Emboli from the jugular veins metastasize to the pulmonary vasculature in
up to 85 % of patients resulting in complicated pleural effusions and pulmonary abscesses, both of which were noted in the course of the disease, post operatively. Our case highlights the usefulness of having anaerobic bottles in blood culture sets. For a syndrome that is so characteristic, it is notable how often the diagnosis is missed until an anaerobic gram-negative rod is isolated from blood culture. In addition, this case illustrates how Lemierre’s disease can present to a wide variety of specialties, including general medicine, otorhinolaryngology, pulmonology, orthopaedics, and general surgery.
Conclusion The incidence of Lemierre’s disease, once believed to be one in a million, has increased incidence in the Western world. This case highlights how it can mimic an acute surgical abdomen and often manifest from the shadows to bring impending doom, hence the need of further epidemiological studies and investigational research to make prompt diagnosis and effective treatment. Conflict of interest All authors are in agreement with the content of the submission and have no conflict of interest. Funding source None Ethical approval Ethical approval is not required.
LETTER TO THE EDITOR
Acute appendicitis from the shadows of Lemierre disease Diwakar Ryali Sarma & Mubashir Mulla & Ioannis Panagopoulos
Accepted: 21 January 2015 # Springer-Verlag Berlin Heidelberg 2015
Dear Editor:
Introduction Lemierre’s syndrome is a rare disorder of young adults caused by the anaerobic bacterium, Fusobacterium necrophorum. The condition is characterized by a primary oropharyngeal infection with evidence of septic thrombophlebitis, exhibited by positive blood cultures, clinical or radiographic evidence of internal jugular vein thrombosis and at least one metastatic focus. The incidence of Lemierre’s syndrome is reported to be nearly one in a million. We describe a case of Lemierre’s syndrome initially presenting as acute appendicitis and evolving into full-blown severe sepsis.
Case report An 11-year-old Caucasian male, with no prior medical history, was evaluated in the emergency department for a 2-day history of sore throat, right iliac fossa pain, loss of appetite and fever. Physical exam revealed localized peritonism in the right iliac fossa and biochemical parameters showed very high inflammatory markers. He was D. R. Sarma (*) : I. Panagopoulos Darent Valley Hospital, Dartford and Gravesham NHS Trust, Darenth Wood Road, DA28DA Dartford, UK e-mail: [email protected]
taken up for a laparoscopic appendicectomy with the provisional diagnosis of acute appendicitis. Intra-operatively, an inflamed appendix with surrounding mesenteric adenitis and turbid fluid was noted in the pelvis. Appendicectomy was done in the standard manner and the specimen sent for histological examination. Immediate post operatively, the child developed severe sepsis with impending organ failure and had to be shifted to the intensive care unit. Over the next 24 hours, his clinical condition worsened with acute kidney injury, bilateral lung abscess and hypoxic brain injury. He was aggressively resuscitated in intensive care and multidisciplinary approach advocated in his ongoing management. He was on broad spectrum antibiotics with moderate response. Fortyeight-hour cultures from the blood, urine, sputum and peritoneal fluid showed mixed growth of aerobes and anaerobes with a remarkable isolation of F. necrophorum in the serum culture. The histology of the appendix showed acute suppurative appendicitis. Emergent antibiotic regimen was advocated according to the sensitivity and there was remarkable improvement of the patient. At this point, clear thrombophlebitis was noted in bilateral internal jugular veins (confirmed by Doppler ultrasound), erythema and congestion noted in the oropharynx. A clinical diagnosis of Lemierre’s syndrome presenting as acute appendicitis was made. Serial blood examination and axial imaging of the chest and abdomen showed resolving sepsis and lung abscess. Antibiotics and high dependency unit care was continued for a further 48 hours. There was significant improvement in the vital parameters and patient was discharged to ward-based management and home soon after. There was complete resolution of all the organ failures.
I. Panagopoulos e-mail: [email protected]
Discussion
M. Mulla Royal Gwent Hospital, Newport, UK e-mail: [email protected]
One of the forerunners in killer infectious disease in the preantibiotic era was Lemierre’s disease. As Alexander Fleming
Int J Colorectal Dis
discovered penicillin and its use became widespread to treat oropharyngeal infections, Lemierre’s syndrome was rarely reported. We present a case report of an 11-year-old presenting with acute appendicitis, masking a worsening pharyngitis and internal jugular vein thrombophlebitis (part of the severe sepsis of Lemierre’s disease) which illustrates that subsequent re-emergence of this often forgotten disease. Fusobacteria are nonmotile, sporulating, obligate anaerobic gram-negative rods that are normal flora in the human upper respiratory tract, gastrointestinal tract and female genital tract. Unlike other anaerobic bacteria, fusobacteria produce lipopolysaccharide endotoxin, leukocidin and hemolysin. Hemagglutinin production augments the fulminant nature of the disease, causing platelet aggregation and septic thrombus formation. The exact mechanism of invasion and penetration of the pharyngeal mucosa with superficial thrombophlebitis and metastatic septic emboli has not been determined. Literature review suggests that, in Fusobacterium pharyngitis, there is extension of septic focus from the peritonsillar space to the internal jugular vein. Once invasion of the internal jugular vein is achieved, the resultant bacteremia triggers platelet aggregation and thrombus formation. Thrombus formation and rapid bacterial growth result in a nidus for metastatic septic embolization. In our case report, septic embolization would have been into the appendicular end artery, leading to the presentation with an acute surgical abdomen. Emboli from the jugular veins metastasize to the pulmonary vasculature in
up to 85 % of patients resulting in complicated pleural effusions and pulmonary abscesses, both of which were noted in the course of the disease, post operatively. Our case highlights the usefulness of having anaerobic bottles in blood culture sets. For a syndrome that is so characteristic, it is notable how often the diagnosis is missed until an anaerobic gram-negative rod is isolated from blood culture. In addition, this case illustrates how Lemierre’s disease can present to a wide variety of specialties, including general medicine, otorhinolaryngology, pulmonology, orthopaedics, and general surgery.
Conclusion The incidence of Lemierre’s disease, once believed to be one in a million, has increased incidence in the Western world. This case highlights how it can mimic an acute surgical abdomen and often manifest from the shadows to bring impending doom, hence the need of further epidemiological studies and investigational research to make prompt diagnosis and effective treatment. Conflict of interest All authors are in agreement with the content of the submission and have no conflict of interest. Funding source None Ethical approval Ethical approval is not required.
