International Journal of Pediatric Otorhinolaryngology Extra
Volume 5, Issue 4 , Pages 152-154, December 2010

Lemierre's syndrome after modified radical mastoidectomy: An unusual variant caused by Citrobacter freneli

Department of ENT and Head and Neck Surgery, TU Teaching Hospital, Maharajgunj, Kathmandu, Nepal

Received 18 July 2009; received in revised form 23 August 2009; accepted 26 August 2009. published online 25 September 2009.

Article Outline

Abstract 

Lemierre's syndrome is characterised by an oropharyngeal infection leading to secondary septic thrombophlebitis of the internal jugular vein. Most of the cases are caused by Fusobacterium necrophorum. Here, we report an unusual variant of Lemierre's syndrome developed after modified radical mastoidectomy. There was right sigmoid sinus and right internal jugular vein thrombosis along with multiple abscess foci seen in bilateral lung with right pyopneumothorax. The organism isolated from chest foci was Citrobacter freneli. We also briefly review the literature of Lemierre's syndrome.

Keywords: Lemierre's syndrome, Modified radical mastoidectomy, Otitis media

 

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1. Introduction 

Lemierre's syndrome is characterised by an oropharyngeal infection which leads to secondary septic thrombophlebitis of the internal jugular vein. It typically occurs in healthy young adults. In most of the cases the syndrome is caused by the anaerobic bacterium known as Fusobacterium necrophorum. Andre Lemierre first described it in 1936 [1]. The syndrome is thought to be uncommon now-a-days due to widespread use of antibiotics in the primary setting, but if it does occur, its rarity may cause diagnostic difficulty [2]. Moreover, if not detected and treated early, the condition can become fatal and mortality rates as high as 10% have been reported [3]. Here, we report a case of Lemierre's syndrome and source of infection being the middle ear.

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2. Case report 

A 4 years old boy presented to our hospital with the complaints of right ear discharge for 6 months along with postauricular swelling and high grade fever for 3 days. There was associated history of vomiting. On examination the child was febrile and other vitals sign were normal. There was obliterated right retroauricular groove, 2cm×3cm fluctuant swelling in right postauricular region and tenderness over mastoid prominence. Otoscopic examination revealed posterior superior bony wall sagging along with mucopurulent, foul smelling discharge. Tympanic membrane could not be visualised properly except a margin of pars tensa perforation. So, an initial diagnosis of right chronic otitis media with acute mastoiditis was made. The child underwent right modified radical mastoidectomy under general anaesthesia. There was cholesteatoma and granulation tissue in various sites including attic, aditus and antrum. Patient received intravenous antibiotics which included ceftriaxone, gentamycin and vancomycin.

Despite our treatment, patient progressively worsened and developed high grade fever up to 104°F. Examination revealed tenderness along right internal jugular vein. Per abdominal examination revealed hepatosplenomegaly. His haemoglobin level was also decreasing from 10.2 to 5.4Gm%, platelets – 14,800/cmm, total count – 19,420/cmm, differential count neutrophil – 88, lymphocytes – 9, bands – 3. Prothrombin time was 22s control being 13. Pus culture revealed no growth.

So, we suspected an internal jugular vein thrombosis which was confirmed by ultrasonography (USG) neck revealed the same. USG abdomen revealed hepatosplenomegaly and chest X-ray showed right lung basal consolidation with minimal effusion (Fig. 1). CT scan head and neck revealed right lateral sinus thrombosis with extension into jugular vein. CT cerebral venogram showed hypodense thrombus in right sigmoid sinus, right internal jugular vein and empty right jugular foramen (Fig. 2, Fig. 3). There were also multiple small cavitating lesions in apical region of bilateral lungs.

Patient was diagnosed as a variant of Lemierre's syndrome and was treated with multiple drug therapy which includes – metronidazole, clindamycin, vancomycin and low molecular weight heparin. Despite treatment patient develop multiple abscess foci seen in bilateral lung with right pyopneumothorax. USG guided chest tube was inserted and 20ml pus was drained. Pus culture showed Citrobacter freneli. Chest tube was removed after 17 days. After 2 months of hospital stay, patient improved and he was discharged from the hospital.

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3. Discussion 

Several papers in the 1980s highlighted that this “forgotten disease” (Lemierre's syndrome) had not gone away [3]. Although it remains a rare disease with an incidence of approximately one per million persons per year [3], [4] there has been a remarkable resurgence of publications in the cited literature since 1990. Recent papers have suggested that the incidence of this condition is rising [3], [4]. The pathogenesis of Lemierre's syndrome is progressive dissemination of septic emboli from tonsillar and peritonsillar veins to the internal jugular vein, resulting is bactermia [5].

Lateral sinus thrombophlebitis formation may result either from direct spread of the infection from the mastoid or from thrombophlebitis of small veins of the mastoid, which communicate with the lateral sinus [6], [7]. Extension or propagation of the thrombus upwards may reach the confluence of the sinuses – the torcula Herophili – and even the superior sagittal sinus beyond. Invasion of superior or inferior petrosal sinuses may lead the disease to cavernous sinus. Venous thrombophlebitis extending into brain substance accounts for the very high association of this complication with brain abscesses. Downward propagation of thrombus into and through the internal jugular vein can reach the subclavian vein [8]. So, a case of acute mastoiditis by the direct extension or from thrombophlebitis can lead to lateral sinus thromophlebitis. The clinical picture along with use of high technology imaging can rule out lateral sinus thrombophlebitis in patients with acute bacterial mastoiditis.

Though Bacteroids melaninogenicus, Eikenella corrodens, and non-group A streptococcus has been isolated from patient with this syndrome, F. necrophorum is overwhelming the most common organism responsible for Lemierre's syndrome [9]. The aetiological agent can be cultured from blood and sites of infection.

In most cases, the infection originates in the palatine tonsils and adjacent tissues. However, it can also initiate as pharyngitis, parotitis, otitis media, sinusitis, odontogenic infection or mastoiditis. Suggestive features of Lemierre's syndrome includes: previously fit adolescent or young adult, history of sore throat in preceding 7 days, onset of high fever and rigors, signs of internal jugular vein thrombosis, dry cough and pleuritic chest pain, chest radiograph shows multiple nodular lesions, bilateral pleural effusions, other features of metastatic abscess – for example, empyema or septic arthritis or skin/soft tissue abscess, release of foul smelling pus from abscess or empyema [10].

Internal jugular venous thrombophlebitis can only be confirmed by imaging techniques. Ultrasonography is often used as the initial modality for demonstration of internal jugular vein thrombosis, being less expensive and not requiring exposure to radiation. However, it provides poor imaging beneath the clavicle and mandible and can miss a fresh thrombus with low echogenecity [9]. Gallium scintiscans, retrograde venogarphy, contrast enhanced computerized tomography (CT) or magnetic resonance imaging (MRI) has been used [11]. Contrast enhanced CT or MRI is an ideal study when considering the diagnosis of internal jugular vein thrombosis, since the extent of thrombus, the anatomy and any localised infection can be identified [11]. MRI has been suggested as the study of choice in venous thrombosis because of greater soft tissue contrast, easier identification of thrombus and avoidance of intravenous contrast agent and radiation. In our case, CT cerebral venogram was done which revealed hypodense thrombus in right sigmoid sinus and right internal jugular foramen. Chest X-ray typically shows multiple bilateral opacities and small pleural effusions, empyema, cavitation and pneumothorax. Blood investigation reveals leucocytosis with a left shift, thrombocytopenia and increased prothrombin time. Liver function test abnormalities can be seen in up to 50% of cases [12]. Elevated blood urea nitrogen and creatinine levels, elevated erythrocyte sedimentation rate, and transient haematuria have also been reported. Blood cultures should confirm on anaerobic septicaemia caused by Fusobacterium, but may be negative because of the use of antibiotics prior to hospitalization. In this case report, pus culture from the foci of chest showed C. freneli while culture of ear discharge revealed no growth.

The diagnosis of lateral sinus thrombophlebitis is confirmed by surgical exploration of the sinus plate. The surgical protocol consists of needling the sinus on completion of the mastoidectomy and, if free flow of blood does not occur, the sinus is incised and the thrombus removed to the extent possible [6], [7].

Principles of treatment of Lemierre's syndrome or any similar variant of this condition remain the same. It consists of adequate fluid resuscitation of the septic patient, followed by prompt surgical drainage of the purulent collections under broad spectrum intravenous antibiotic cover. Because of the frequent occurrence of mixed infection, monotherapy with metronidazole is considered to be inadvisable and penicillin and metronidazole is commonly recommended [13]. Armstrong et al. commented “only a minority of patients receive anticoagulation in most studies and yet do well [14] with vigorous antibiotic therapy and surgical drainage, anticoagulation should probably be reserved for patients with evidence of retrograde progression to the cavernous sinus [15]”. This is a very rare case report because the child developed Lemierre's syndrome after modified radical mastoidectomy. Despite treatment patient developed multiple abscess foci on bilateral lung and right pyopneumothorax.

Ligation and resection of the thrombosed internal jugular vein is usually unnecessary and should be considered in patients with refractory sepsis or severe respiratory compromise from repeated pulmonary emboli. The use of anticoagulation remains controversial and no controlled trials have been performed on its efficacy in septic thrombosis [9].

In the pre-antibiotic era, the prognosis of this syndrome was poor. With antibiotic treatment, despite the severity of sepsis, complete recovery is common, and mortality rates range from 0% to 18% [16].

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4. Conclusion 

Lemierre syndrome is a rare but serious complication of otitis media. It is a disease that requires high index of suspicion for diagnosis. It is important for clinicians to recognize it early and provide adequate treatment to reduce morbidity and prevent mortality.

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References 

  1. Lemierre A. On certain septicaemias due to anaerobic organisms. Lancet. 1936;1:701–703
  2. Venkateswaran S, Sze FKH. A case of Lemierre's syndrome presenting with multiple pulmonary abscesses associated with a tension hydropneumothorax resulting in a mediastinal shift. Ann. Acad. Med. Singapore. 2005;34:450–453
  3. Hagelskjaer LH, Prag J, Maclzynski J, Kristensen JH. Incidence and clinical epidemiology of necrobacillosis, including Lemierre's syndrome, in Denmark 1990–1995. Eur. J. Clin. Microbiol. Infect. Dis. 1998;17:561–565
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  12. Carlson ER, Bergamo DF, Coccia CT. Lemierre's syndrome: two cases of a forgotten disease. J. Oral. Maxillofac. Surg. 1994;52:74–78
  13. Sinave CP, Glenna JH, Fardy PW. The Lemierre syndrome: suppurative thrombophlebitis of the internal jugular vein secondary to oropharyngeal infection. Medicine. 1989;68:85–94
  14. Eykyn SJ. Necrobacillosis. Scand. J. Infect. Dis. (Suppl.). 1989;62:41–46
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PII: S1871-4048(09)00051-3

doi:10.1016/j.pedex.2009.08.003

International Journal of Pediatric Otorhinolaryngology Extra
Volume 5, Issue 4 , Pages 152-154, December 2010

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