Korean Journal of Gastrointestinal Endoscopy 2003;27(1): 21-25.
S자결장 정맥류를 동반한 Klippel-Trenaunay-Weber 증후군 1예
정용우·한동수·백창희·박윤경·김종표· 이항락·김진배·손주현·함준수·박훤겸*
한양대학교 의과대학 내과학교실, *외과학교실
A Case of Klippel-Trenaunay-Weber Syndrome with Sigmoid Varices
Yong Woo Chung, M.D., Dong Soo Han, M.D., Chang Hee Paik, M.D., Yoon Kyung Park, M.D., Jong Pyo Kim, M.D., Hang Lak Lee, M.D., Jin Bae Kim, M.D., Joo Hyun Sohn, M.D., Joon Soo Hahm, M.D. and Hwon Kyum Park, M.D.*
Departments of Medicine and *General Surgery, Hanyang University Kuri Hospital, Kuri, Korea
Abstract
Colonic varix, which is a rare cause of lower gastrointestinal bleeding, can be developed by various diseases. Colonic variceal bleeding is more fatal than esophageal variceal bleeding. Klippel-Trenaunay-Weber syndrome is a rare congenital vascular malformation characterized by the clinical triad of bony or soft tissue hypertrophy, hemangioma and varicosities or vascular malformations. We report a 42-year-old female patient with fatal sigmoid variceal bleeding. She had dilated vessels over her body and face, and both superior and inferior vena cava were obstructed. She underwent an emergent operation due to uncontrolled massive bleeding from sigmoid varices. We report a case of Klippel-Trenaunay-Weber syndrome with literature review. (Korean J Gastrointest Endosc 2003;27:2125)
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