Unusual age at presentation of klippel-trenaunay syndrome

Abstract

Klippel-Trenaunay (KT) syndrome is a congenital vascular disorder which is rare and affects one or both limbs. It has incidence of about 2-5 in 100,000. French physicians Klippel and Trenaunay first described this syndrome in the year 1900. They named the syndrome as nevus vasculosus osteohypertrophicus. In 1907, Park Weber named the same condition hemangiectatic hypertrophy. The etiology is unknown. Though it is a sporadic condition, paradominant inheritance pattern has also been suggested. Patients generally present in the first decade of life. It affects males more than females. KT syndrome is a congenital circulatory disorder typically comprising of the triad cutaneous capillary angioma, bone and soft tissue hypertrophy and varicose veins. There are several theories about its pathogenesis. The management of this syndrome consists mainly of early diagnosis, prevention and treatment of complications. We report the case of a 30-year-old male patient with KT syndrome showing the classical triad.