Retropharyngeal Edema: An Unusual Manifestation of Kawasaki Disease

Background: Kawasaki disease (KD) is an acute multisystem vasculitis of unknown etiology that typically affects young children. KD presenting as a retropharyngeal inflammatory process is very rare. Objectives: To report a case of KD initially presenting as a retropharyngeal edema mimicking a deep neck infection, and to review previously published reports in the literature. Case Report: We report a case of KD in a previously healthy 3-year-old child who presented with acute onset of fever and cervical adenitis, along with computed tomography scan findings of retropharyngeal edema and inflammation. KD was suspected due to persistent fever and no improvement in the patient's condition despite appropriate antibiotic therapy; and other classic findings of KD eventually developed. An echocardiogram obtained on the 10th day of illness revealed pericardial effusion but no coronary ectasia or aneurysm. Treatment with high-dose intravenous immunoglobulin resulted in dramatic clinical improvement. Follow-up echocardiograms obtained 2 and 8 weeks after discharge revealed a small left coronary artery aneurysm (CAA). Nine months later, the CAA had resolved and the patient was well with no sequelae. Conclusions: Although unusual, physicians should be aware of retropharyngeal edema as an atypical presentation of KD.