Vignes S, Vidal F, Arrault M, Boccara O
Unité de lymphologie, Centre national de référence des maladies vasculaires rares (lymphœdèmes primaires), hôpital Cognacq-Jay, 15, rue Eugène-Millon, 75015 Paris, France.
Unité de lymphologie, Centre national de référence des maladies vasculaires rares (lymphœdèmes primaires), hôpital Cognacq-Jay, 15, rue Eugène-Millon, 75015 Paris, France.
Arch Pediatr. 2017 Aug;24(8):766-776. doi: 10.1016/j.arcped.2017.05.002. Epub 2017 Jun 23.
Lymphedema results from impaired lymphatic transport with increased limb volume and is divided into primary and secondary forms. In children, primary lymphedema is the most frequent, with a sporadic, rarely familial form or associated with complex malformative or genetic disorders. Diagnosis of lymphedema is mainly clinical and lymphoscintigraphy is useful to assess the lymphatic function of both limbs precisely. The main differential diagnosis is overgrowth syndrome. Erysipelas (cellulitis) is the main complication, but psychological or functional discomfort may occur throughout the course of lymphedema. Lymphedema management is based on multilayer low-stretch bandage, skin care, and eventually manual lymph drainage. The objective of treatment is to reduce lymphedema volume and then stabilize it. Multilayer low-stretch bandage and elastic compression are the cornerstone of treatment. Parent's motivation, including self-management, is required to ensure the child's compliance and improve quality of life.
淋巴水肿是由于淋巴运输受损导致肢体体积增加，分为原发性和继发性两种形式。在儿童中，原发性淋巴水肿最为常见，呈散发性，很少有家族性形式，或与复杂的畸形或遗传疾病相关。淋巴水肿的诊断主要依靠临床检查，淋巴闪烁造影有助于精确评估双下肢的淋巴功能。主要的鉴别诊断是过度生长综合征。丹毒（蜂窝织炎）是主要并发症，但在淋巴水肿的整个病程中可能会出现心理或功能不适。淋巴水肿的治疗基于多层低弹性绷带、皮肤护理，最终进行手法淋巴引流。治疗的目标是减少淋巴水肿的体积，然后使其稳定。多层低弹性绷带和弹性压迫是治疗的基石。需要家长的积极性，包括自我管理，以确保孩子的依从性并提高生活质量。
