Vaillant Loïc, Tauveron Valérie
Université François-Rabelais de Tours, CHRU de Tours, 37044 Tours cedex 01, France.
Presse Med. 2010 Dec;39(12):1279-86. doi: 10.1016/j.lpm.2010.09.010. Epub 2010 Oct 28.
Limb lymphedema is frequent and not well-known. Clinical classification distinguishes primary lymphedemas due to developmental disorders of the lymphatic system (hereditary or not, sometimes associated with other malformations) and secondary lymphedemas. Primary lymphedema is a lymphedema without a cause to explain lymphatic impairment. It is due to an abnormal lymphangiogenesis in utero. It is often associated with mutation in a gene involved in lymphangiogenesis (FOX C2, VEGFR 3, SOX18, PROX 1…). To assess clinical diagnosis, non-invasive techniques are able to study structure and function of the lymphatic system (mainly isotopic lymphography). Treatment is the complex decongestive therapy which associates manual lymphatic drainage and bandage. Predisposing or precipitating factors have to be treated (particularly streptococcal infections). Surgical treatment has precise and rare indication.
肢体淋巴水肿很常见但却鲜为人知。临床分类将因淋巴系统发育障碍（遗传性或非遗传性，有时与其他畸形相关）导致的原发性淋巴水肿和继发性淋巴水肿区分开来。原发性淋巴水肿是一种没有可解释淋巴损伤原因的淋巴水肿。它是由于子宫内淋巴管生成异常所致。它常与参与淋巴管生成的基因（FOX C2、VEGFR 3、SOX18、PROX 1等）突变相关。为评估临床诊断，非侵入性技术能够研究淋巴系统的结构和功能（主要是同位素淋巴造影）。治疗方法是综合消肿疗法，该疗法结合了手法淋巴引流和绷带包扎。诱发或促发因素必须加以治疗（尤其是链球菌感染）。手术治疗有精确且罕见的适应症。
