Clinical diagnosis
Case 81
【Progress】He was admitted to the pediatrics-expert hospital. He received esophagus endoscopic examination which revealed no evidence of double esophagus and perforation. Laboratory test revealed no immune disorder. He was given antibiotics and anticoagulants for approximately one month and a half, gradually improving and finally leading to disappearance of swollen lymphnodes and mediatstinitis, but remained thrombus in the left jugular vein. He was discharged the hospital because of no evidence of active inflammation.
【Discussion】
Lemierre syndrome is an active inflammatory neck disease with internal jugular vein thrombosis (1, 2). Lemierre syndrome criteria is originally as follows; acute oropharyngeal infection; thrombo-phlebitis of the internal jugular vein; septicemia: secondary metastatic lung and/or joint septic abscess (3). The typical course of Lemierre syndrome is that it initially occurs oropharyngitis with peritonsillar abscess and several days later, it develops thrombophlebitis and septicemia associated with neck pain and swelling.
Before the time of prevailed usage of antibiotics, this syndrome was commonly encountered. However, the widespread of antibiotics after 1970’s brought about the dramatic decrease in the incidence of Lemierre syndrome, Lemierre syndrome is called as a “forgotten disease” in the 1980’s and the 1990’s. However, the incidence of Lemierre syndrome is getting increased after the 21 century (1, 2, 4, 5).
The inflammatory source is now described to occur from palatin tonsils and peri-tonsiller tissue, such as pharyngitis, parotitis, sinusitis, osteitis media and mastoiditis. Bacterial endotoxin induces platelet aggregation, phlebitis, and septic thrombus formation.
Oral pharyngitis is most caused by gram-positive aerobic streptococcus, while Lemierre syndrome is caused by gram-negative anaerobic bacillus of Fusobacterium necrophorum (6). Fusobacterium necrophorum is one of the most common normal flora in the oral cavity and the gastrointestinal tract. It is not known why this typically non-invasive bacteria alter its character to penetrate mucosa and invade to the internal jugular vein. The precede viral or bacterial infection might play a role for the alteration. Our patient had repeatedly croup, swollen face and swollen lymphnods before the present episode. He was given corticosteroids for repeated croup, which might contribute to hide the typical symptom of oral pharyngitis.
It affects adolescence and young adults. The most cases are found in the 2nd decade of life (51%), followed by the 3rd decade (20%) and the 1st decade (8%)(2, 7). As one of the typical symptoms, a cervical mass along the anterior margin of the stermocleidomastoid muscle at the submandibular angle might be found (2). A mass is usually mistaken for enlarged lymphnodes. Our patient age was two years and six months-old infant with enlarged mass at the jaw level (Fig. 1), corresponded to the invasion site to right internal jugular vein.
As the treatment, the appropriate antibiotics for gram-negative anaerobic rod bacillus is favorable and delayed response is associated with poor prognosis. Prolonged therapy of antibiotics is recommended with reports getting good outcome with a mean of 6 weeks (2 to 9 weeks)(2, 8). Anticoagulants for internal jugular vein occlusion are reasonable but controversial about effectiveness on recanalization (2). In our case, he was given antibiotics for one month and a half, leading to disappearance of the right neck mass and good response on laboratory inflammatory data but remaining occlusion of right jugular vein.
【Summary】
We present a two years and six months-old infant for right cervical mass, dyspnea and edematous face. Enhanced neck CT showed a right-sided mass which invades to right jugular vein associated with thrombus, swollen lymphnodes and invasive mediastinitis. He was prescribed steroid hormone in advance due to mimicking croup. He had no fever and no laboratory data indicating active inflammation. However, eventually he was diagnosed as Lemierre syndrome. Steroid administration given for croup might hide the latent inflammation, and contribute to activate Fusobacterium necrophrum. We should keep in mind that Lemierre syndrome is active neck disease (mainly oral pharyngitis) with internal jugular vein thrombosis caused by platelet aggregation due to endotoxin of Fusobacterium necrophorum which is one of normal flora. The preceding oral pharyngitis is considered to alter the character of Fusobacterium to invade mucosa and venous wall.
【References】
1.Alherabi A. A case of Lemierre syndrome. Ann Saudi Med. 2009; 29(1): 58–60.
2.Eilbert W, et al. Lemierre’s syndrome. Int J Emerg Med. 2013; 6: 40. Published online 2013 Oct 23. doi: 10.1186/1865-1380-6-40.
3.Lemierre A. On certain septicaemia due to anaerobic organisms. Lancet. 1936;I:701–703.
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6.Ianniello F, Ferri E, Pinzani A. Septic thrombophlebitis of the internal jugular vein due to Fusobacterium necrophorum (Lemierre's syndrome): case report and review of literature. Acta Otorhinolaryngol Ital. 1998;18(5):332–7. [PubMed]
7.Karkos PD, et al. Lemierre’s syndrome: a systematic review. Laryngoscope. 2009;6(8):1552–1559. doi: 10.1002/lary.20542. [PubMed] [Cross Ref]
8.Wright WF, et al.. Lemierre syndrome. South Med J. 2012;6(5):283–288. doi:10.1097/SMJ.0b013e31825581ef. [PubMed] [Cross Ref]
2017.11.22