Isolated Facial Vein Thrombosis in a Case of Lemierre's Syndrome

Introduction

Lemierre's disease is also called as ‘forgotten disease’ due to its rarity in today’s era.¹ It presents typically after an episode of pharyngitis with septic thrombosis of internal jugular vein and possible septic emboli either in pulmonary or some other distant site. It is caused usually by anaerobic bacteria Fusobacterium necrophorum.² However, other bacteria including Streptococcus viridans have been described as causative organisms.³

Case Presentation

A 13-year old boy presented in the first week of November 2021 with fever (41.1ºC) for 12 hours and change in sensorium. On clinical examination, the body temperature was 39.4ºC, and was making irrelevant talk and was not oriented to time, space and person.

A history of throat pain two days prior was presented. There were no current outbreaks, no animal contact, no history of swimming or walking in accumulated water. The child was infected with COVID-19 five months ago. Our initial impression was febrile encephalopathy or acute encephalitis syndrome. His vitals were stable, temperature was 37.7ºC, and was still delirious and drowsy. Over next two hours, initial reports showed Hb 15 gm%, WBC 2850 cells/cmm. The differential white cell count and platelet count were normal. MP antigen came negative. C-reactive protein (CRP) was 79 mg/dL, ESR was 30 mm first hour.

MRI brain was normal. Over next 6-8 hours, child’s mentation improved, and he became completely conscious and oriented. However, continued fluctuating high grade fever of 41.1ºC was observed on day three of admission.

On fourth day, patient complained of left sided cheek pain and on 6th day, a swelling developed over the area with trismus. Intra-oral examination was normal and on day six the blood culture grew gram-positive cocci. Dental examination revealed no significant abnormalities and Orthopantomogram (OPG) findings were normal. Longitudinal ultrasound and axial colour-Doppler examination of the parotid area showed parotid oedema, subcutaneous tissue oedema and thrombophlebitis of deep facial vein (Figure 1 and 2). In view of parotid oedema, serum amylase levels were checked which came normal.

The growth in the blood culture was identified as Streptococcus intermedius both on Vitek media and by MALDI-TOF MS (Matrix assisted laser desorption ionization-time of flight mass spectrometry). Streptococcus intermedius is a subtype of Streptococcus viridans.

To rule out other causes of thrombosis including post-COVID-19 Multisystem inflammatory syndrome in children (MIS-C), we did some investigations. The RT-PCR report for COVID-19 was negative, COVID-19 IgG was positive, COVID-19 IgM was negative, echocardiogram was normal. No vegetation was observed and Lupus anticoagulant panel was found to be normal.

Presence of thrombosis in facial vein (a tributary of internal jugular vein) and isolation of Streptococcus viridans from blood culture in a previously healthy child lead us to the diagnosis of Lemierre’s syndrome.

The child was started on antibiotics metronidazole and linezolid, following which he was afebrile within 48 hours. The facial swelling decreased over next four days and trismus reduced over next two weeks.

Discussion

Lemierre’s disease, also called ‘anaerobic post-anginal sepsis’ is associated with septic thrombosis of internal jugular vein and isolation of anaerobic bacterium, usually Fusobacterium necrophorum from the blood culture. Some patients show evidence of distant septic emboli presenting as pulmonary infiltrates, CNS emboli or arthritis.

Though Fusobacterium is classically described as the causative organism for the condition, other organisms including Staphylococci and Streptococcus viridans have been described as causative organisms.³ Also isolated facial vein involvement with thrombophlebitis of the internal jugular vein has been described.⁴

The disease was first described in a series of patients who had anaerobic infection and distant septic embolization by Lemierre in 1930.⁵ As it became a rarity in the antibiotic era, it was named as ‘forgotten disease’ in 1980s. However, recently it is being more frequently reported.⁶ It typically occurs in a previously healthy individual, usually in the second decade of life. Fever is almost universal and the illness develops around a week after the presentation of symptoms similar to pharyngitis. Patients can present with painful neck swelling or trismus. There can be septic embolic complications such as pneumonia or arthritis.

There is usually a delay of 5-6 days before the final diagnosis is made. Presence of thrombus in internal jugular vein becomes usually evident on USG or CT scan done to investigate the cause of pain/ swelling.

Isolation of anaerobic bacteria is often seen especially in antibiotic naïve patients. These bacteria are usual commensals in oropharynx. Change in pharyngeal mucosa which causes damage to the mucosal integrity probably allows these bacteria to penetrate deeper tissue planes and become pathogenic.⁷ Prolonged course of antibiotics, usually for two weeks is recommended.⁸ Prognosis is often good.

We report here a less common cause of pharyngitis with potentially serious complications.

Conflict of interest

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