Unmasking Rarity: An Atypical Presentation of Lemierre's Syndrome

Lemierre's syndrome (LS), otherwise known as postanginal sepsis, is a frequently overlooked condition characterized by septic thrombophlebitis of the internal jugular vein (IJV), usually caused by oropharyngeal infection. However, ear space (otogenic) infections are one of the atypical causes of LS and have been rarely reported. We present a case of a male in his 20s with a history of recurrent acute otitis media (RAOM) who presented with purulent ear discharge, fever, neck pain, and swelling for a week. Blood tests showed a raised white blood cell count with a left shift and increased inflammatory markers. Ear discharge and blood cultures were negative. A contrast-enhanced computed tomography (CT) scan of the head and neck revealed thrombosis of the IJV and opacification of the mastoid air space. The patient was diagnosed with otogenic LS and recurrent acute otitis media and responded well to empiric broad-spectrum antibiotics and supportive care. Anticoagulation therapy was not considered due to the lack of consensus on its effectiveness. After three months of follow-up, symptoms resolved, and venous Doppler ultrasound showed IJV recanalization. This case highlights a rare association and emphasizes the need for timely diagnosis and intervention. Therefore, due to the rare occurrence of the disease, heightened clinical vigilance is needed to avoid the adverse consequences of delayed diagnosis.