Introduction: Kawasaki disease (KD) is one of the commonest pediatric vasculitides and is associated with a significant risk of development of coronary artery abnormalities if left untreated. Areas covered: In this review, we have highlighted the incomplete and unusual presentations of KD and also emphasize the controversies pertaining to 2D echocardiography in KD. A PubMed search was performed regarding diagnosis and unusual presentations of KD. Expert opinion: Diagnosis of KD is essentially clinical and based on recognition of typical clinical features that may appear sequentially and all signs and symptoms may not be present at one point of time. There is no confirmatory laboratory test for diagnosis of this condition. Further complicating the picture is the fact that incomplete and atypical forms KD may be seen in up to 50% patients. Although 2D echocardiography continues to be the preferred imaging modality for cardiac assessment in patients with KD, it has its limitations.
Keywords: Kawasaki disease; coronary artery abnormalities; diagnosis; echocardiography; unusual presentations.