Treatment of lymphatic malformations

The development of the Hamburg Classification, along with a better understanding of vascular malformations, has allowed a more concise description of lymphatic malformation (LM). The management of LM has become clear: lymphangioma shou no longer be considered a single LM lesion, but rather one of two different LM lesions belonging to an extratruncular lesion, even though the management of lymphangioma is different from its truncular lesion counterpart that clinically presents as primary lymphedema. Although they often present as discrete clinical entities, the lesions can occur together, making clinical management confusing. Treatment options for lymphangioma should be approached cautiously with appropriate consideration of its embryologic characteristics, as for any other extratruncular lesion, when options such as sclerotherapy and/or surgical excision are being considered. Primary lymphedema should be managed as a truncular lesion, where decongestive physiotherapy remains the mainstay of treatment to improve lymph-transporting function. Additional surgical therapy, either reconstructive or ablative, should only be carried out as a supplemental therapy with justifiable indications.