Division of Plastic and Reconstructive Surgery, Department of Surgery, Oregon Health and Science University, Portland, Oregon, USA.
Department of Undergraduate Medical Education, Oregon Health and Science University School of Medicine, Portland, Oregon, USA.
Lymphat Res Biol. 2021 Feb;19(1):41-50. doi: 10.1089/lrb.2020.0100. Epub 2021 Jan 25.
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.
淋巴和混合畸形较为罕见,临床表现多变。它们是由于胚胎早期血管和淋巴管形成过程中的错误而产生的。这导致淋巴液持续渗透到软组织中,并引起局部浸润性肿块,伴有病理后遗症。淋巴畸形现在不再采用过去描述性的术语,如“囊状水瘤”,而是根据大小分为巨囊型、微囊型或混合病变。超声和磁共振成像等影像学技术的进步使这些病变的准确特征成为可能,并最终可以根据淋巴管畸形的形态进行早期诊断和实施适当的治疗。淋巴管畸形的管理极具挑战性,多学科方法对于获得最佳的美学和功能结果最为有效。淋巴管畸形的分子生物学新发现为治疗靶点提供了依据,并确立了药物治疗的作用。硬化疗法、激光和射频消融已被证明是治疗淋巴管畸形的有效微创治疗选择。手术干预在治疗对这些微创技术有抗性的局灶性病变中具有作用。手术规划取决于临床目标、大小、解剖位置、特征和浸润程度。