Presentation and Management of Klippel-Trenaunay Syndrome: A Review of Available Data

Abstract

Klippel-Trenaunay Syndrome (KTS) is a rare and sporadic congenital disorder, characterized by the classical triad of port-wine stains, varicosities along with bone and soft tissue hypertrophy. Symptoms of Klippel-Trenaunay Syndrome include pain, swelling, lymphedema, bleeding, superficial thrombophlebitis, and deep vein thrombosis. The etiology remains indistinct and has been attributed to both genetic and environmental factors. In most cases, a thorough history and clinical examination is enough for the diagnosis of Klippel Trenaunay Syndrome. However, when certain complications are present, noninvasive imaging techniques are used for the diagnosis and evaluation of the disease in patients. Due to the diversity of presentation, a multidisciplinary approach is essential for the proper management of such patients. At present, there is no cure for the disease; rather, symptomatic treatment is employed in order to improve the patients' quality of life. In this review, we provide a brief overview of the clinicopathological profile and management of Klippel-Trenaunay Syndrome.

Keywords: bone hypertrophy; hemangioma; klippel-trenaunay syndrome; laser treatment; port-wine stain; rare disease; varicose veins.