Klippel-Trenaunay Syndrome Complicated with Scrotal Lymphedema: A Rare Entity Not to be Ignored

Abstract

Klippel-Trenaunay syndrome is an extremely rare congenital angiodysplasia of undetermined aetiology, characterised by venous and lymphatic malformations, bone and soft tissue hypertrophy [1]. Its management is multidisciplinary. In some cases, surgery can improve the quality of life of patients. We report the case of a 59-year-old Moroccan patient, known to have Klippel-Trendaunay syndrome for 20 years, who consulted us for scrotal lymphedema that had been evolving for 3 years. The patient had never travelled abroad, particularly to filarial endemic countries. The clinical examination revealed a large, enlarged scrotum with local haemangiomas [Figure 1] and haemangiomas of the lower limbs. The biological work-up revealed no abnormalities and no imaging was performed.