A case of atypical Kawasaki disease with giant coronary artery aneurysm containing thrombus

Abstract

Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability. Typical mucocutaneous signs of Kawasaki Disease were absent upon presentation. A trans-thoracic echocardiogram identified a giant aneurysm of the left anterior descending artery with thrombus formation in-situ and the child was managed with intravenous immunoglobulin, steroids, high dose aspirin therapy and later warfarinisation. Cardiovascular sequelae of Kawasaki disease include giant coronary artery aneurysms with thrombosis. Enlargement of a coronary aneurysm after the acute phase of Kawasaki disease is uncommon and the outcome of interventional approaches poorly studied.