Lymphatic malformations

Abstract

Lymphatic malformations constitute one subset of a larger group of benign, congenital vascular lesions resulting from embryologic errors in lymphangiogenesis. They range in presentation from small localized masses to large and sometimes debilitating, disfiguring, or invasive lesions with an infiltrative pattern of growth which may complicate treatment. Morbidity includes bleeding, infection, lymphorrhea, obstruction of vital structures, disfigurement, and psychosocial distress. For complex lesions, sclerotherapy and surgical therapy remain the foundation of care with increasing interest in novel agents (e.g., sirolimus) for systemic therapy. There is no single treatment consensus; rather, a management algorithm must be established based upon the experience and strengths of a multidisciplinary team, as well as the specific details of the individual lesion in terms of size, location, and risk profile.