Stanford Center for Lymphatic and Venous Disorders, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, Calif.
Stanford Center for Lymphatic and Venous Disorders, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, Calif.
J Vasc Surg Venous Lymphat Disord. 2021 Jul;9(4):1077-1082. doi: 10.1016/j.jvsv.2021.01.013. Epub 2021 Feb 1.
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.
On September 21, 2020, we searched PubMed/MEDLINE for studies published from 2000 to 2020 reporting outcomes of invasive and pharmacologic treatment of LMs.
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.
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.
淋巴管畸形（LM）是常见的先天性血管病变，多数在出生时被诊断。它们会使局部解剖结构变形，如果压迫呼吸道或其他重要结构，可能会危及生命。在过去的 20 年中，LM 的治疗取得了显著进展。我们对 LM 治疗的文献进行了系统评价。
2020 年 9 月 21 日，我们在 PubMed/MEDLINE 上搜索了 2000 年至 2020 年发表的报告 LM 侵袭性和药物治疗结果的研究。
共有 251 项研究符合入选标准。手术一直是 LM 治疗的主要方法，尤其是在治疗微囊型和混合性病变方面。硬化疗法已成为巨囊型 LM 的一线治疗方法，也作为一种辅助疗法与手术联合用于治疗其他病变。西罗莫司（mTOR 的强效抑制剂）作为口服和局部制剂，在 LM 的治疗中显示出巨大的潜力。最近的研究表明，靶向细胞通路的小分子调节剂在 LM 的治疗中具有潜力。
多种侵袭性和药物治疗方法已被证明对 LM 的治疗有效。未来的研究应集中在对这些治疗方式进行严格的前瞻性比较。
