Korean J Gastroenterol.  2004 Feb;43(2):137-141.

A Case of Klippel-Trenaunay-Weber Syndrome Presenting with Esophageal and Gastric Varices Bleeding

• Kim JH
• Kim CW
• Son DK
• Chung BW
• Yang SE
• Kim JI
• Kim JK
• Chung IS

Abstract

Klippel-Trenaunay-Weber syndrome is a congenital vascular disorder consisted of a variety of vascular malformations, enlargement of the involved limb, and varicose veins. We report a case of Klippel-Trenaunay-Weber syndrome presenting portal hypertension and varices bleeding caused by hypoplasia of the portal vein. Portal hypertension was caused by portal vein hypoplasia associated with Klippel-Trenaunay-Weber syndrome. There were three episodes of variceal bleeding, and hemostasis were achieved by endoscopic band ligation, Sugiura operation, and splenic artery embolization respectively. Although successful hemostasis was achieved, an additional procedures to reduce portal hypertension were needed to prevent repeated episodes of variceal bleeding.

Keyword

Klippel-Trenaunay-Weber syndrome; Hemangioma; Hypertension; portal; Esophageal and Gastric Varices

MeSH Terms

Adult
English Abstract
Esophageal and Gastric Varices/*complications
Gastrointestinal Hemorrhage/*etiology
Humans
Hypertension, Portal/complications
Klippel-Trenaunay-Weber Syndrome/*complications
Male
Portal Vein/abnormalities