Management of Lymphatic Vascular Malformations: A Systematic Review of the Literature.

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Abstract

Lymphatic malformations (LM) are common congenital vascular lesions, most often diagnosed at birth. They deform local anatomy and can be life-threatening if they compress the aerodigestive tract or other vital structures. Significant progress has been made in the treatment of LM in the past twenty years. We conducted a systematic review of the literature on the management of LM.On September 21, 2020, we searched PubMed/MEDLINE for studies published from 2000 to 2020 reporting outcomes of invasive and pharmacologic treatment of LM.A total of 251 studies met eligibility criteria. Surgery continues to be a mainstay in the management of LM, especially in the treatment of microcystic and mixed lesions. Sclerotherapy has emerged as a first-line treatment for macrocystic LM and as an adjunctive therapy used in combination with surgery for other lesions. Sirolimus, a strong inhibitor of mTOR, has shown tremendous promise in the treatment of LM, both as an oral and a topical agent. Recent investigations have shown the potential of targeted small molecule modulators of cellular pathways in the treatment of LM.Multiple invasive and pharmacologic therapies have been shown to be effective in the treatment of LM. Future ÌÇÐÄ´«Ã½ should focus on rigorous, prospective comparisons of these treatment modalities.

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