Abstract
Klippel–Trenaunay–Weber syndrome is a congenital condition characterized by a failure of blood or lymph vessels to form properly. There is ongoing discussion about the classification of this disease as it has clinical overlap with several other diseases.
Gastrointestinal (GI) symptoms include:
Hematochezia: the most common GI complaint
Occult to severe and fatal bleeding; recurrent episodes
Visceral organ involvement can occur
Splenic hemangioma can spontaneously rupture, leading to internal bleeding.
GI clinical signs and findings are:
Klippel–Trenaunay–Weber syndrome consists of a triad of
Varicose veins
Port-wine stain
Bony and soft tissue hypertrophy all tissues of the involved limb
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Feldman M, Friedman LS, Brandt LJ, editors. Sleisenger and Fordtran’s gastrointestinal and liver disease. 9th ed. Philadelphia: Saunders; 2010.
Servelle M, Bastin R, Loygue J, et al. Hematuria and rectal bleeding in the child with Klippel and Trenaunay syndrome. Ann Surg. 1976;183:418–28.
Ceballos-Quintal JM, Pinto-Escalante D, Castillo-Zapata I. A new case of Klippel-Trenaunay-Weber (KTW) syndrome: evidence of autosomal dominant inheritance. Am J Med Genet. 1996;63:426–7.
Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. Klippel-Trénaunay syndrome: spectrum and management. Mayo Clin Proc. 1998;73:28–36.
Wilson CL, Song LM, Chua H, et al. Bleeding from cavernous angiomatosis of the rectum in Klippel-Trenaunay syndrome: report of three cases and literature review. Am J Gastroenterol. 2001;96:2783–8.
Wang ZK, Wang FY. Klippel-Trénaunay syndrome with gastrointestinal bleeding, splenic hemangiomas and left inferior vena cava. World J Gastroentero. 2010;16:1548.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2013 Springer Science+Business Media New York
About this chapter
Cite this chapter
Tadros, M. (2013). Klippel–Trenaunay–Weber Syndrome: Gastrointestinal Features. In: Wu, G., Selsky, N., Grant-Kels, J. (eds) Atlas of Dermatological Manifestations of Gastrointestinal Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6191-3_43
Download citation
DOI: https://doi.org/10.1007/978-1-4614-6191-3_43
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4614-6190-6
Online ISBN: 978-1-4614-6191-3
eBook Packages: MedicineMedicine (R0)