Severe Hemorrhage from Intestinal Hemangiomatosis in Klippel-Trenaunay Syndrome

Summary A child with Klippel-Trenaunay syndrome (KTS) and severe anemia caused by bleeding from diffuse intestinal hemangiomatosis is presented. Hemangiomas of the bowel should be considered in any child with unexplained anemia and coexisting cutaneous hemangiomas. The diagnostic workup of patients with KTS and symptomatic hemangiomatosis is outlined with respect to indications for surgical management.