A case of Klippel-Trenaunay-Weber syndrome with severe varices from the descending colon to the rectum

Abstract

A 72-year-old female was referred to our hospital because of melena. She had been diagnosed as having the Klippel-Trenaunay-Weber syndrome at the age of 55. Amputation of the right lower extremity, and a wide resection of the stomach for duodenal ulcer had been carried out at the age of 61.
A contrast-enhanced abdominal CT showed obstruction of the main portal vein, prominent dilatation of the inferior mesenteric vein, and varices of the rectum. Colonoscopy revealed severe varices extending from the descending colon to the rectum.
We considered that the blood flow was changed course via the internal iliac vein from the inferior mesenteric vein into the inferior vena cava. This hemodynamic change caused dilatation of the inferior mesenteric vein and the development of collateral vessels as varices around the rectum.