Management of lymphatic vascular malformations: A systematic review of the literature.

ABSTRACT

OBJECTIVE: 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 LMs in the past 20 years. We conducted a systematic review of the literature on the management of LMs. METHODS: On September 21, 2020, we searched PubMed/MEDLINE for studies published from 2000 to 2020 reporting outcomes of invasive and pharmacologic treatment of LMs. RESULTS: A total of 251 studies met the eligibility criteria. Surgery has continued to be a mainstay in the management of LMs, especially in the treatment of microcystic and mixed lesions. Sclerotherapy has emerged as a first-line treatment of macrocystic LMs and as an adjunctive therapy used in combination with surgery for other lesions. Sirolimus, a strong inhibitor of mTOR (mechanistic target of rapamycin), has shown tremendous promise in the treatment of LMs, as both an oral and a topical agent. Recent investigations have shown the potential of targeted small molecule modulators of cellular pathways in the treatment of LMs. CONCLUSIONS: Multiple invasive and pharmacologic therapies have been shown to be effective in the treatment of LMs. Future research should focus on rigorous, prospective comparisons of these treatment modalities.