Hindawi Publishing Corporation Case Reports in Gastrointestinal Medicine Volume 2013, Article ID 813653, 6 pages http://dx.doi.org/10.1155/2013/813653 Case Report Klippel-Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature Salih Samo, 1 Muhammed Sherid, 2 Husein Husein, 3 Samian Sulaiman, 1 Margaret Yungbluth, 4 and John A. Vainder 1 1 Department of Internal Medicine, Division of Gastroenterology, Saint Francis Hospital Evanston Program, University of Illinois at Chicago, 355 Ridge Avenue, Evanston, IL 60202, USA 2 Department of Internal Medicine, Division of Gastroenterology, CGH Medical Center, 100 East LeFevre Road, Sterling, IL 61081, USA 3 Department of Internal Medicine, Division of Gastroenterology, University of Tishreen, Aleppo Street, P.O. Box 2230, Latakia, Syria 4 Department of Pathology, Saint Francis Hospital Evanston Program, University of Illinois at Chicago, 355 Ridge Avenue, Evanston, IL 60202, USA Correspondence should be addressed to Salih Samo; salihsamo@hotmail.com Received 25 April 2013; Accepted 16 May 2013 Academic Editors: T. Hirata, V. Lorenzo-Z´ u˜ niga, S. Nomura, and S. Van Biervliet Copyright © 2013 Salih Samo et al. his is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome of vascular malformations and sot tissue and bone hypertrophy. Vascular malformations can afect multiple organ systems. Involvement of the gastrointestinal (GI) tract is uncommon in KTS, but it can be a source of life-threatening bleeding. We report a case of a 32-year-old male with a known diagnosis of KTS who presented with a life-threatening rectal bleeding and was treated with proctosigmoidectomy and massive blood products transfusion. He expired ater a long hospitalization. We then review the literature on KTS and management of some of its complications. 1. Introduction Klippel-Trenaunay syndrome (KTS) was irst described by two French physicians, Klippel and Trenaunay, in 1900 [1]. he term describes a rare congenital syndrome of venous, lymphatic, and capillary malformations and sot tissue and bone hypertrophy of usually one limb [2] (lower limb is involved more frequently with extension to the trunk). It seems that the right lower limb is afected more frequently than the let lower limb. Patients can be diagnosed with KTS with only one or more of the abovementioned features since patient might not have all the features [3, 4]. Vascular malformations have been reported to afect the gastrointestinal (GI) tract, liver, spleen, and heart and follow a progressive course [2, 5]. Let-sided inferior vena cava (IVC), secondary to persistence of the let and regression of the right supracardinal vein, has been reported [5, 6]. Vascular malformations can also afect genitourinary tract (kidney, bladder, penis, scrotum, vagina, and vulva) and manifest as intrapelvic or retroperitoneal vascular masses [7]. In this paper, we present a case of a 32-year-old male with KTS who presented with a life-threatening rectal bleeding. 2. Case Presentation A 32-year-old male presented with a sudden onset of massive rectal bleeding. He had a history of KTS since childhood. He had multiple admissions for bleeding, presenting mainly as hematuria that started at the age of 11. He also had one episode of self-limited rectal bleeding four years prior to this presentation. His medications were multivitamins with iron supplement. At the time of admission, physical examination was remarkable for blood pressure of 55/29 mm Hg, HR of 135 per minute, respiratory rate of 22 per minute, and normal temper- ature. He appeared pale and diaphoretic. Cardiopulmonary examination was unremarkable except for tachycardia and tachypnea. His abdomen was sot and nontender, with active red blood oozing from his rectum. He was noted to have