Coronary Artery Aneurysms and Thrombosis in Kawasaki Disease

A 7-month-old male infant was brought to the hospital after being found by his foster parents to be inconsolable and crying. He was up-to-date on his immunizations and met all his developmental milestones. Significant birth history included intrauterine exposure to methamphetamine, with a positive methamphetamine drug screen at birth, and subsequent removal from his mother and placement with a foster family.

A month earlier, he presented to an emergency department with symptoms of diarrhea for one week, loss of appetite, fever, pink eye, and rash on his entire body. A pediatrician diagnosed him with gastroenteritis secondary to adenovirus, he was treated with intravenous fluids, and he was released three days later with instructions to continue with oral rehydration with Pedialyte.

He continued to have intermittent loss of appetite and diarrhea, change of personality from being a happy baby to one with low energy, and significant weight loss. Worried, his foster parents brought him to the emergency department.

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Image 1A:
Enlarged heart with giant coronary artery aneurysms of the right coronary and left anterior descending arteries.

At the emergency department, he became tachycardic, displayed decreased capillary refill, and had episodes of apnea necessitating bag-mask ventilation. A complete blood count showed the white blood cell count was elevated, but without profound left-shift, and he had mild anemia. While in the pediatric intensive care unit, an emergency bedside echocardiogram was done that showed markedly dilated coronary arteries consistent with Kawasaki disease. Despite aggressive management, he had multiple episodes of respiratory arrest without loss of pulse and was pronounced dead.

Autopsy examination revealed a pale infant with pale organs and an enlarged heart with thrombosed giant coronary artery aneurysms involving the proximal portions of the right coronary and the entire left anterior descending arteries (Image 1A). Both kidneys had multiple wedge-shaped acute cortical infarcts.

Histopathologically, there were thrombosed aneurysms of the coronary arteries along with panvasculitis of the coronary arterial wall, lymphocytic epicarditis, lymphocytic myocarditis, and multifocal contraction band necrosis. An interlobular aneurysm with thrombus formation was identified with panarteritis was seen in one of the kidneys.