Bilateral retinal ischemia in Kawasaki disease. Postmortem findings and electron microscopic observations

A 4-month-old infant died of cardiovascular complications of Kawasaki disease (KD). At autopsy, aneurysmal dilatation with recent thromboses were found in coronary arteries, abdominal aorta, brachial and left common carotid arteries. A vasculitis with thrombosis of a branch of the ophthalmic artery was demonstrated in the right orbital apex. Light microscopy disclosed that the ocular abnormalities were confined to the retinas of both eyes that exhibited inner retinal ischemia. By electron microscopy, the inner retina showed clumping of nuclear chromatin, mitochondrial swelling, rupture of plasma membranes, and increased number of lipid figures. Müller cell processes were intact. Stereologic analysis of the mitochondria in the retinal pigment epithelium, photoreceptor inner segment, and ganglion cells in the KD infant demonstrated that identical mitochondrial changes occurred in the retina from an adult woman who had a recent central retinal artery occlusion.