Lymphangiomas Emmanuel A . Ameh Louise Caouette - Laberge

Combined/complex malformations Klippel-Trenaunay syndrome (capillary-lymphatico-venous malformation) Proteus syndrome Maffucci syndrome Table 110.1: Classification of lymphatic malformations. Introduction Lymphangiomas are developmental defects of the lymphatic channels that belong to a large spectrum of vascular malformations. They are most commonly located in the head and neck region, and to a lesser extent on the axilla and trunk, but can occur anywhere there are lymphatic vessels. Even though they are congenital defects, they may not become apparent until several years after birth. Although benign, lymphangiomas frequently present surgical difficulties and challenges due to their propensity to infiltrate and extend around neighbouring structures. Lymphatic malformations encompass entities other than lymphangiomas. The goal of this chapter is to address the lesions known as lymphangiomas or hygromas. This chapter does not discuss pulmonary and intestinal lymphangiectasia and peripheral lymphoedema, which may be congenital or acquired, or lymphangiomatosis, an ill-defined disease usually implying the coexistence of lymphangiectasia and multiple lymphangiomas in several sites.