Case presentation
A 7-year-old girl presented by isolated cervical adenitis with no fever, conjunctivitis or edema. She had no history of previous disease, and had appropriate development. She was treated by suspicious to infection disease with cephalexin. Because of no suitable response, cefotaxime and clindamycin was started. Pain and swelling over the right side of her neck with torticollis was remained. There was no history of trauma to head/neck. Due to prolonged fever (39-40 C), fatigue, malaise, ankle, hand and elbow pain and arthritis, she was admitted in our hospital
The child was immunized for her age as per the national vaccination program. After 7 days, she deteriorated and was admitted in hospital by torticollis, evidence of systemic inflammation and treatment failure. She was ill and her head was tilted to the right with chin rotation to the left. Her vital signs at time of admission were: heart rate: 85/min, respiratory rate: 25/min, blood pressure: 110/80 mmHg and temperature: 37.2°C. After 10 days, other classic signs of KD were revealed. Physical examination found erythematous and congested throat, normal breath sounds with palpable lymph nodes, no audible heart murmur, and no skin rash. Initial laboratory data demonstrated pyuria, white blood cell count of 23.4 x109/L, and an elevated CRP level of 103 mg/L and platelet count 620000/mm³. She was put on intravenous antibiotic (cefotaxime and vancomycin). She had received steroid, because of lacking IVIG in pediatric infectious services and aspirin was started at an anti-inflammatory dose at 80 mg/Kg in four divided doses. 2-dimensional echocardiography in day 14 showed giant coronary aneurysms, left ventricular (LV) dyskinesia and mitral regurgitation (MR) beside 60 percent EF. Catheterization and selective coronary angiography at day 16 of disease showed giant aneurismal dilatation of both coronary arteries, clot formation in coronary arteries and LV dysfunction (Figure1). Coronary artery bypass graft surgery (CABG; LIMA to LAD and venous graft to RCA), aneurysmectomy and aneurysmorrhaphy was done by cardiac surgeon at the second month of disease. Although LV function improved and she was not any complaint but warfarin therapy continued due to aneurismal LAD. Recent angiography was done also (Figure2). She is followed up until now (2 years, every 6 months) and has no sequel.