Atypical Kawasaki Disease presentation in two infants

Introduction: Kawasaki disease KD is an acute, self-limited vasculitis, with clear clinical criteria. The vessels involved are medium sized arteries and particularly coronary arteries. The clinical presentation is in most of the cases clear, but atypical presentations are more and more reported, particularly in infants. We present two infants less than two months old with the diagnosis of Kawasaki disease, one having a severe evolution.

Cases: 7 week old infant with diminished general state transferred to PICU because of fever for 48 hours. After complete septic work-up, broad spectrum antibiotics were begun. Fever persisted with high inflammatory parameters (CRP 235mg/l) and a maculo-papular exanthema appeared. The infant needed volume expansion and non-invasive ventilatory support. At day 8 of hospitalization thrombocytosis appeared with 700G/l. An echocardiography was done at that time, showing bilateral coronary aneurysms of 4.8mm on the right coronary artery and 4mm on the left coronary. Standard treatment with intravenous immuno globulins (IVIG), followed by aspirin 100mg/kg/d was given with rapid resolution of inflammatory signs and partial regression of aneurysms.

7 week old infant with persistent fever, exanthema and slightly elevated inflammatory parameters (CRP 61mg/l). After septic work-up without bacterial focus discharged in good general state. Was readmitted 6 weeks later to PICU because of cardiogenic shock, multiorgan failure and ischemia of all four extremities. Inflammatory parameters were high with CRP 146mg/l, white cell count 27.8 G/l and thrombocytes 753G/l. ECG revealed ST segment changes with troponin I of 19µ/l. Echocardiography demonstrated global left ventricular dysfunction with akinetic apical segment and a giant aneurysm of the left coronary artery with maximum diameter of 14mm. The infant needed mechanical ventilation, cardiac support with milrinone and nitroglycerine iv and aggressive hypertension therapy for several weeks. IVIG were given twice without effect and corticosteroids were added with regression of inflammatory parameters. Evolution was difficult and marked by necrotizing lesions of four extremities.

Conclusion: These two cases illustrate that Kawasaki disease has to be suspected in infants with persisting fever without a clear etiology and elevated inflammatory parameters. In these atypical presentations there is normally a good response to standard treatment, but if unrecognized, Kawasaki disease can lead to severe consequences such as myocardial infarction and necrotizing vasculopathy.