Rectal bleeding, deep venous thrombosis, and coagulopathy in a patient with Klippel-Trénaunay syndrome

Klippel-Trénaunay syndrome (KTS) is a rare noninheritable congenital disorder comprising vascular malformations that predispose patients to a paradoxical condition where bleeding occurs in a hypercoagulable state. A 16-year-old boy with a complicated lifetime course of KTS presented with massive rectal bleeding. He underwent a partial colectomy, endorectal resection, and splenectomy. Postoperatively, he developed a saddle pulmonary embolus, ultimately requiring placement of an inferior vena cava filter. He eventually did well and has since had his diverting ostomy reversed and resumed his usual activities. This report validates the use of endorectal resection for venous malformation of the rectum in patients with KTS and highlights the difficult balance of controlling bleeding by correction of a consumptive coagulopathy and the increased risk of thromboembolic complications.