RESUMO
OBJECTIVE: To describe the clinical presentation and treatment of two patients with the Klippel-Trenaunay syndrome referred to our hospital because of rectal bleeding and to review the literature concerning the diagnosis and treatment of this complication. CASE 1: Fifteen year old male with the Klippel-Trenaunay syndrome and chronic anemia who presented with severe rectal bleeding. CASE 2: Nineteen year old female with the same syndrome and a two year history of intermittent rectal bleeding, anemia and thrombocytopenia. In both cases the study protocol revealed varicose lesions in the colon as the cause of bleeding and other vascular malformations related to their syndrome. TREATMENT: The first patient was treated with partial colectomy and colorectal anastomosis. Four years after surgery he presented with new episodes of bleeding and was treated with sclerosis of the residual rectal varices using formaldehyde. The second patient was treated with partial colectomy and colostomy. She has received to sessions of sclerosis with absolute alcohol of the residual varices in the rectal stump. Colostomy closure is soon to be performed. CONCLUSION: Klippel-Trenaunay syndrome is a rare clinical entity with vascular alterations at different levels. A small percentage of cases may present rectal bleeding due to colonic varices and can lead to chronic anemia or severe hemorrhage with hemodynamic implications. Treatment of this complication involves resection of the affected colonic segment combined with a secondary procedure to control bleeding of the residual rectal varices.