ABSTRACT
Kawasaki disease (KD), an acute febrile childhood vasculitis of unknown etiology, preferentially involves the coronary arteries. Diagnosis typically rests on strict clinical criteria. If untreated, KD may be complicated by coronary arteritis and progress to aneurysm formation, thereby predisposing the child to a small but significant risk of death. We report a case of atypical KD causing death due to rupture of a coronary artery aneurysm with massive cardiac tamponade. The clinical challenge to recognize KD during the acute phase--especially in atypical cases when the diagnostic criteria are incomplete--is critical. Therapeutic intervention with intravenous gamma-globulin (IVIG) and aspirin during the first 10 days of onset is highly effective not only in reducing nearly tenfold such potentially fatal cardiac complications by arresting the immune-mediated necrotizing arteritis, but also in alleviating the acute symptoms related to systemic inflammation.
