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Lymphatic and Mixed Malformations
Lymphatic Research and Biology ( IF 1.6 ) Pub Date : 2021-02-24 , DOI: 10.1089/lrb.2020.0100
Brynn A Hathaway 1 , Stephanie Radu 2 , Johanna Wilson 2 , Allison C Nauta 1
Affiliation  

Lymphatic and mixed malformations are rare and variable in presentation. They arise due to errors in vascular and lymphatic formation during early embryonic development. This leads to persistent infiltration of lymph fluid into soft tissues and causes a locally invasive mass with pathologic sequelae. Departing from historically descriptive terminology, such as “cystic hygroma,” lymphatic malformations are now categorized as macrocystic, microcystic, or mixed lesions, based on size. Advances in imaging modalities, such as ultrasonography and magnetic resonance imaging, have made accurate characterization of these lesions possible and ultimately allow for early diagnosis and implementation of appropriate treatment based on the morphology of the lymphatic malformation. Management of lymphatic malformations can be quite challenging, and a multidisciplinary approach is most effective for optimum aesthetic and functional outcomes. New discoveries in the molecular biology of lymphatic malformations have provided treatment targets and established a role for pharmacotherapy. Sclerotherapy, laser, and radiofrequency ablation have all proven to be effective as minimally invasive treatment options for lymphatic malformations. Surgical intervention has a role in the treatment of focal lesions recalcitrant to these less invasive techniques. Operative planning is dictated by clinical goals, size, anatomic location, characteristics, and extent of infiltration.

中文翻译:

淋巴和混合畸形

淋巴和混合畸形罕见且表现多样。它们是由于早期胚胎发育过程中血管和淋巴管形成的错误而产生的。这导致淋巴液持续浸润到软组织中,并导致具有病理后遗症的局部浸润性肿块。不同于历史上的描述性术语,例如“囊性湿疹”,淋巴管畸形现在根据大小分为大囊性、微囊性或混合性病变。超声检查和磁共振成像等成像方式的进步使这些病变的准确表征成为可能,并最终允许根据淋巴管畸形的形态进行早期诊断和实施适当的治疗。淋巴管畸形的管理可能非常具有挑战性,多学科方法对于最佳美学和功能结果最有效。淋巴管畸形分子生物学的新发现提供了治疗靶点并确立了药物治疗的作用。硬化疗法、激光和射频消融都已被证明是有效的淋巴管畸形微创治疗选择。手术干预在治疗这些微创技术难以治疗的局灶性病变方面发挥着作用。手术计划由临床目标、大小、解剖位置、特征和浸润程度决定。硬化疗法、激光和射频消融都已被证明是有效的淋巴管畸形微创治疗选择。手术干预在治疗这些微创技术难以治疗的局灶性病变方面发挥着作用。手术计划由临床目标、大小、解剖位置、特征和浸润程度决定。硬化疗法、激光和射频消融都已被证明是有效的淋巴管畸形微创治疗选择。手术干预在治疗这些微创技术难以治疗的局灶性病变方面发挥着作用。手术计划由临床目标、大小、解剖位置、特征和浸润程度决定。
更新日期:2021-02-25
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