PERIORBITAL VASCULITIS COMPLICATING KAWASAKI-SYNDROME IN AN INFANT

Periorbital vasculitis is a previously unreported complication of Kawa saki syndrome (KS). We describe an infant with severe KS refractory to initial management with salicylate and intravenous immunoglobulin (IV IG). Retreatment with MG and high-dose pulsed steroids was required fo r persistent fever and inflammatory manifestations. Despite aggressive medical therapy, a large left coronary artery aneurysm developed. Aft er apparent complete KS remission, acute periorbital vasculitis develo ped in the left upper eyelid and orbit, requiring operative interventi on for diagnosis and high-dose pulsed steroids for therapy. The signif icance of this previously unreported ophthalmic complication of KS is reviewed, along with a discussion of the role of steroids in KS manife stations refractory to IVIG.