Greives Matthew R, Aldrich Melissa B, Sevick-Muraca Eva M, Rasmussen John C
Vascular Anomalies Clinic, Division of Pediatric Plastic Surgery, Department of Pediatric Surgery and.
Brown Foundation Institute of Molecular Medicine for the Prevention of Human Diseases, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, Texas.
Pediatrics. 2017 Apr;139(4). doi: 10.1542/peds.2015-4456.
Primary lymphedema in the pediatric population remains poorly diagnosed and misunderstood due to a lack of information on the causation and underlying anatomy of the lymphatic system. Consequently, therapeutic protocols for pediatric patients remain sparse and with little evidence to support them. In an effort to better understand the causation of primary pediatric lymphedema and to better inform clinical care, we report the use of near-infrared fluorescence lymphatic imaging on the extremities of an alert, 21-month-old boy who presented with unilateral right arm and hand lymphedema at birth. The imaging results indicated an intact, apparently normal lymphatic anatomy with no obvious malformation, but with decreased lymphatic contractile function of the affected upper extremity relative to the contralateral and lower extremities. We hypothesized that the lack of contraction of the lymphatic vessels rather than an anatomic malformation was the source of the unilateral extremity swelling, and that compression and manual lymphatic drainage could be effective treatments.
由于缺乏关于淋巴系统病因及基础解剖结构的信息，儿科人群中的原发性淋巴水肿仍然难以诊断且易被误解。因此，针对儿科患者的治疗方案仍然稀少且缺乏有力证据支持。为了更好地理解原发性儿科淋巴水肿的病因并为临床护理提供更充分的信息，我们报告了对一名21个月大、意识清醒的男孩进行近红外荧光淋巴成像的情况，该男孩出生时即出现右侧手臂和手部单侧淋巴水肿。成像结果显示淋巴解剖结构完整、看似正常，无明显畸形，但患侧上肢相对于对侧及下肢的淋巴收缩功能下降。我们推测淋巴管缺乏收缩而非解剖畸形是单侧肢体肿胀的原因，并且压迫和手动淋巴引流可能是有效的治疗方法。
