Klippel-Trenaunay syndrome: a review.

Abstract

The Klippel-Trenaunay syndrome is a triad of congenital anomalies characterized by a vascular nevus, varicose veins and bony and soft-tissue hypertrophy. Although the syndrome is uncommon, initially the condition appears as a skin blemish or varicose veins. To avoid errors in management, it is important to recognize the syndrome. In this paper the authors outline the presentation, investigation and management of Klippel-Trenaunay syndrome and describe four cases of their own. One form of Klippel-Trenaunay syndrome, in which all the anomalies of the triad are important, in which direct signs of a large arteriovenous shunt are present and for which the surgical approach is difficult, is Parkes-Weber syndrome. Lack of reports in the world literature on long-term follow-up leaves many unanswered questions regarding prognosis and management.

• Authors: C K You, J Rees, D A Gillis, J Steeves
• Journal: Canadian journal of surgery. Journal canadien de chirurgie (Can J Surg) Vol. 26 Issue 5 Pg. 399-403 (Sep 1983) ISSN: 0008-428X [Print] Canada
• PMID: 6311389 (Publication Type: Journal Article, Review)

Topics

• Angiomatosis (diagnosis, etiology, pathology)
• Humans
• Klippel-Trenaunay-Weber Syndrome (diagnosis, etiology, pathology, therapy)
• Phlebography
• Prognosis