The Klippel-Trenauny Weber Syndrome: An Unusual Presentation of Hematochezia and a Review of the Literature

Bhavesh B. Shah, Anthony Lupetin, Joseph Young, Rad Agrawal, Katie Farah


A 27-year-old male who presented to our clinic with a nine year history of painless hematochezia. Examination was remarkable for right thigh hypertrophy and large varicose veins in the right lateral thigh. His medical history was significant for a port-wine stain on his right thigh noted early in childhood, a right leg venectomy and iron deficiency anemia. Colonoscopy revealed grade I internal hemorrhoids.  Prominent rectal veins were also visualized from 10cm to the anal verge. Vascular surgery was consulted and a right leg venogram was obtained demonstrating extensive pelvic venous collaterals and an aberrant sciatic vein. There was also communication from the common femoral vein to the venous collaterals in the knee and thigh. Pathology review from his previous venectomy revealed arteriovenous fistulas. These findings were consistent with the Klippel-Trenauny Weber Syndrome (KTWS). Our patient subsequently underwent endovenous ablation of his right sciatic vein. This was followed by ultrasound-guided foam sclerotherapy. Follow-up sigmoidoscopy revealed complete ablation of his rectal varices. The Klippel-Trenauny syndrome was described in 1900 as a rare, non-hereditary congenital abnormality of obscure etiology characterized by the clinical triad of soft tissue hypertrophy of an extremity, varicosities, and cutaneous hemangiomas or lymphangiomas. Increased venous pressure during defecation and direct mucosal trauma caused by feces can aggravate recurrent hematochezia. Endoscopy may show visible vessels or nodules and bluish angiomatous lesions. Endoscopic recognition of rectal varices, and determination of the etiology of hematochezia is important in guiding appropriate therapy and management.

Gastroenterol Res. 2009;2(3):188-189
Publish ahead of print May 22, 2009


Hematochezia; Endoscopy; Rectal Varices; Klippel-Trenauny Weber Syndrome

Full Text: HTML PDF

Browse  Journals  


Journal of clinical Medicine Research

Journal of Endocrinology and Metabolism

Journal of Clinical Gynecology and Obstetrics

World Journal of Oncology

Gastroenterology Research

Journal of Hematology

Journal of Medical Cases

Journal of Current Surgery

Clinical Infection and Immunity

Cardiology Research

World Journal of Nephrology and Urology

Cellular and Molecular Medicine Research

Journal of Neurology Research

International Journal of Clinical Pediatrics






Gastroenterology Research, bimonthly, ISSN 1918-2805 (print), 1918-2813 (online), published by Elmer Press Inc.            
The content of this site is intended for health care professionals.

This is an open-access journal distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, which permits unrestricted
non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Creative Commons Attribution license (Attribution-NonCommercial 4.0 International CC-BY-NC 4.0)

This journal follows the International Committee of Medical Journal Editors (ICMJE) recommendations for manuscripts submitted to biomedical journals,
the Committee on Publication Ethics (COPE) guidelines, and the Principles of Transparency and Best Practice in Scholarly Publishing.

website:   editorial contact:
Address: 9225 Leslie Street, Suite 201, Richmond Hill, Ontario, L4B 3H6, Canada

© Elmer Press Inc. All Rights Reserved.