A Crick in the Neck: Lemierre Syndrome in a 4-year Old Female Presenting as Fever and Torticollis

Introduction: Described by Dr. Andre Lemierre in a 1936 case series of 20 patients, Lemierre Syndrome (LS) is defined as a septic thrombophlebitis of the internal jugular vein (IJV). LS typically begins as an oropharyngeal infection that advances to the IJV through direct extension through the fascial planes of the neck, or indirect lymphatic or hematogenous spread from the peritonsillar vessels. We present a case of LS in a 4-year-old patient who presents much younger than the typical age range of affected individuals, and who recovered well without any long-term sequelae.

Case Description: A 4-year-old ex-27 week female presented with a near 3 week history of intermittent fevers and progressive right-facing torticollis. She had multiple interactions with the health care system over her illness course, and was given diagnoses ranging from general viral syndrome to gingivostomatitis and acute otitis media. Around the 2 week mark, her caretaker described her as having developed a “crick” in her neck while consistently favoring a rightward tilt. On illness day 16, she presented to her pediatrician for routine visit, and was noted to have fever, right tonsillar enlargement, and cervical lymphadenopathy, thereby prompting referral to the emergency department. Her physical exam on admission was additionally significant for a 30 degree rightward head rotation, a swollen and tender right sternocleidomastoid, and submandibular lymphadenopathy. She was resistant to active or passive neck rotation due to discomfort, but was able to traverse the midline with coaxing. Laboratory workup was notable for leukocytosis and thrombocytosis with elevated inflammatory markers, as well as mild transaminitis. Infectious serologic workup was negative for: SARS-CoV-2, Bartonella henselae, Bartonella quintana, EBV, and Mycoplasma. Blood culture showed no growth, but was drawn after antibiotics were given. A CT neck with contrast demonstrated intrinsic occlusion vs compression of the right IJV, and ultrasound and MRI confirmed IJV thrombophlebitis.

Discussion: LS is typically associated with Fusobacterium necrophorum infection, a gram-negative anaerobe, with incidence estimated to be around 1 to 3.6 per million per year and mortality rate around 5 to 9%. Significant morbidity is often present, due to dissemination of septic thromboemboli, potentially affecting the CNS, bones/joints, and lungs. The typical age range for LS in pediatric patients clusters around adolescence, but infants as young as 6 months of age have been reported. As oropharyngeal infections most often precede LS, it is important to keep this rare but serious infection on any differential.

Conclusion: This patient was diagnosed with Lemierre Syndrome. She was treated with an inpatient course of ampicillin/sulbactam before transitioning to oral amoxicillin/clavulanic acid to complete a total of 4 weeks of antibiotic therapy. All elevated laboratory markers normalized prior to hospital discharge, and the patient had complete resolution of symptoms at outpatient follow up.