Klippel-Trenaunay Syndrome: A Case Report and Literature Review

Abstract

The eponymously named Klippel-Trenaunay Syndrome (KTS) is a rare cutaneous vascular condition that was first described in 1900 by two French physicians Klippel and Trenaunay, who first noticed this syndrome in two patients who presented with a port wine stain and varicosities of an extremity associated with hypertrophy of soft and bony tissue. They termed the syndrome “naevus vasculosus osteohypertrophicus”. In 1907, Frederick Parkes Weber, unaware of Klippel and Trenaunay's report, described a patient with the three aforementioned symptoms as well as an arteriovenous malformation of the affected extremity. He termed the process hemangiectatic hypertrophy and more commonly known today as Parkes Weber Syndrome (PWS). KTS must be distinguished from PWS which has a hallmark feature of AVFs [1].