It features angiomatous artero-venous, hyperoximia-inducing osteohypertrophy, often observed with underlying dysplasia of lymphatic collectors of the affected segments. Its picture includes disproportionate gygantism of the affected extremity and multiple angiomas. Other organic malformations may be associated with this syndrome (see also Angiodysplasia).
Figure: Klippel Trénaunay Syndrome: a disproportionate hypertrophy of the affected (left) extremity is evident.