Periorbital vasculitis complicating Kawasaki syndrome in an infant

Periorbital vasculitis is a previously unreported complication of Kawasaki syndrome (KS). We describe an infant with severe KS refractory to initial management with salicylate and intravenous immunoglobulin (IVIG). Retreatment with IVIG and high-dose pulsed steroids was required for persistent fever and inflammatory manifestations. Despite aggressive medical therapy, a large left coronary artery aneurysm developed. After apparent complete KS remission, acute periorbital vasculitis developed in the left upper eyelid and orbit, requiring operative intervention for diagnosis and high-dose pulsed steroids for therapy. The significance of this previously unreported ophthalmic complication of KS is reviewed, along with a discussion of the role of steroids in KS manifestations refractory to IVIG.