Gram-negative bacteremia in four patients with Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome (KTWS) is a sporadically occurring congenital malformation syndrome consisting of hemangiomata, venous varicosities, and hypertrophy of soft tissues and/or overgrowth of bone.1-3 Although the list of potential complications is long, KTWS is generally a nonprogressive condition. The problems, which include edema, stasis dermatitis, skin ulceration, cellulitis, anemia, thrombosis, phleboliths, phlebitis, bone and joint abnormalities, scoliosis, and paresthesias, tend to be chronic in nature.4 Recognized life-threatening complications include disseminated intravascular coagulation (DIC)5 and gastrointestinal bleeding as a result of hemangiomata in the intestine.6,7 Systemic infection has not been reported as a major cause of morbidity and mortality. The purpose of this report is to present 4 patients with KTWS who had invasive infection with gram-negative organisms to document another life-threatening complication of this syndrome.