Rethinking the Typical Diagnostic and Therapeutic Challenges of Kawasaki Disease

Abstract

This report presents a case of a 9-month-old male subject, with an initial presentation of a viral-like illness progressing rapidly to multiple deep neck abscesses involving the bilateral submandibular, parapharyngeal, retropharyngeal, and anterior mediastinal spaces subsequently confirmed with contrast-enhanced computed tomography of the neck. Additionally, the subject developed mucocutaneous signs of generalized erythematous rash, with red, fissured lips along with edema of the extremities. Initial lab parameters were suggestive of high inflammatory markers, low albumin, and deranged coagulation profile. Treatment with broad-spectrum intravenous antibiotics, albumin, and fresh frozen plasma infusions was commenced. Echocardiography revealed dilatation of the left main coronary artery with mild mitral valvular regurgitation. Based on the clinical criteria, a diagnosis of typical Kawasaki disease (KD) on day 5 of illness was made, and the subject received intravenous immunoglobulin (IVIg) and aspirin. The clinical course was complicated by IVIg resistance, for which a second dose of IVIg and pulse systemic corticosteroid therapy was required. Post defervescence, an isolated elevation of activated thromboplastin time, partially corrected with mixing studies and a positive circulating lupus anticoagulant was noted. The patient did not develop any bleeding or thromboembolic complications during his hospital stay. Surgical drainage of the neck abscesses did not yield any pus or fluid collection. After a stormy course of 31 days, the subject was discharged with an improving trend of inflammatory markers and no progression of coronary artery dilatation. Gradual resolution to baseline health was noted upon follow-up, 18 months postdischarge from the hospital. Despite being one of the most common cause of childhood acquired cardiac disease, KD still poses a significant diagnostic and treatment challenge to physicians. This requires physicians to be vigilant for timely diagnosis and appropriate management to prevent significant cardiac comorbidities and be aware of the possible complications that might arise during the treatment.