Chen Wei F, Jou Christopher, Pandey Sonia K, Lo Shih-Lun
From the Department of Plastic Surgery, Center for Lymphedema Research and Reconstruction, Cleveland Clinic, Cleveland, OH.
Department of Plastic Surgery, Cathay General Hospital, Taipei, Taiwan.
Plast Reconstr Surg Glob Open. 2024 Dec 20;12(12):e6328. doi: 10.1097/GOX.0000000000006328. eCollection 2024 Dec.
Primary lymphedema, a condition characterized by impaired lymphatic function, has long remained underexplored. Current diagnostic approaches rely on clinical history and genetic testing, yet the genetic underpinnings remain elusive in many cases. Traditional thinking suggests that primary lymphedema is confined to specific anatomical regions, but our experience challenges this notion. We hypothesize that primary lymphedema is systemic lymphatic dysfunction.
All patients with clinical diagnosis of primary lymphedema from January 2020 to April 2022 were included in our study. Demographic data, medical and surgical history, and indocyanine green (ICG) lymphographic findings were collected.
A total of 152 patients met our inclusion criteria. We observed a predominance of female patients (75%) and a mean age of 43.9 years. The onset of swelling varied, with most patients (82.3%) experiencing it in their lower extremities. Notably, ICG lymphography revealed abnormal lymphatic findings in all symptomatic limbs, affecting multiple extremities in 97.4% of patients. Importantly, even among patients initially presenting with limited symptoms, asymptomatic extremities exhibited lymphatic defects. In addition, the extent of lymphatic disease, assessed through ICG lymphography, surpassed clinical symptoms in 80% of cases, underscoring the systemic nature of primary lymphedema.
Our study suggests that primary lymphedema is a systemic lymphatic insufficiency, affecting the entire lymphatic system. This underscores the importance of comprehensive assessments, even with limited symptoms, to facilitate earlier diagnosis and more effective treatment approaches.
原发性淋巴水肿是一种以淋巴功能受损为特征的疾病，长期以来一直未得到充分研究。目前的诊断方法依赖于临床病史和基因检测，但在许多情况下，其遗传基础仍不明确。传统观点认为原发性淋巴水肿局限于特定的解剖区域，但我们的经验对这一观点提出了挑战。我们假设原发性淋巴水肿是一种全身性淋巴功能障碍。
纳入2020年1月至2022年4月临床诊断为原发性淋巴水肿的所有患者。收集人口统计学数据、医疗和手术史以及吲哚菁绿（ICG）淋巴造影结果。
共有152例患者符合我们的纳入标准。我们观察到女性患者占主导（75%），平均年龄为43.9岁。肿胀的发作情况各不相同，大多数患者（82.3%）的下肢出现肿胀。值得注意的是，ICG淋巴造影显示所有有症状肢体的淋巴均有异常表现，97.4%的患者累及多个肢体。重要的是，即使在最初症状有限的患者中，无症状肢体也表现出淋巴缺陷。此外，通过ICG淋巴造影评估的淋巴疾病程度在80%的病例中超过了临床症状，这突出了原发性淋巴水肿的全身性。
我们的研究表明，原发性淋巴水肿是一种全身性淋巴功能不全，影响整个淋巴系统。这强调了即使症状有限也进行全面评估的重要性，以便于早期诊断和采取更有效的治疗方法。
