Boston, Mass. From the Departments of Plastic and Oral Surgery, Surgery, and Radiology, Vascular Anomalies Center, Children's Hospital Boston, Harvard Medical School.
Plast Reconstr Surg. 2011 Jun;127(6):2419-2431. doi: 10.1097/PRS.0b013e318213a218.
Lymphedema results from maldevelopment of the lymphatic system (primary) or injury to lymphatic vasculature (secondary). Primary lymphedema is far less common than the secondary condition. The purpose of this study was to determine the clinical features of primary lymphedema in the pediatric age group.
The authors' Vascular Anomalies Center database was reviewed for patients evaluated between 1999 and 2010 with onset of lymphedema before 21 years of age. Cause, sex, age of onset, location, and familial/syndromic association were determined. Morbidity, progression, and treatment were analyzed.
Lymphedema was confirmed in 142 children: 138 cases (97.2 percent) were primary and four (2.8 percent) were secondary. Analysis of the primary cohort showed that 58.7 percent of the patients were female. Age of onset was infancy, 49.2 percent; childhood, 9.5 percent; or adolescence, 41.3 percent. Boys most commonly presented in infancy (68.0 percent), whereas girls usually developed swelling in adolescence (55.3 percent). Lymphedema involved an extremity (81.9 percent), genitalia (4.3 percent), or both (13.8 percent). The lower limb was most commonly affected (91.7 percent), and 52.9 percent had bilateral lower extremity disease. Eleven percent of patients had familial or syndromic lymphedema. Cellulitis occurred in 18.8 percent of children; 13.0 percent required hospitalization. The majority of patients (57.9 percent) had progression of their disease. Treatment was compression garments alone (75.4 percent) or in combination with pneumatic compression (19.6 percent); 13.0 percent had operative intervention.
Pediatric primary lymphedema usually involves the lower extremities. Boys typically are affected at birth, and girls most often present during adolescence. Most patients do not have major morbidity, are successfully managed by compression, and do not require surgical treatment.
淋巴水肿是由于淋巴系统发育不良（原发性）或淋巴血管损伤（继发性）引起的。原发性淋巴水肿远比继发性疾病少见。本研究的目的是确定儿科年龄组原发性淋巴水肿的临床特征。
作者的血管异常中心数据库中回顾了 1999 年至 2010 年间就诊的患者，这些患者在 21 岁之前出现淋巴水肿。确定病因、性别、发病年龄、部位以及家族性/综合征相关性。分析发病率、进展和治疗情况。
在 142 名儿童中确诊为淋巴水肿：138 例（97.2%）为原发性，4 例（2.8%）为继发性。对原发性队列的分析表明，58.7%的患者为女性。发病年龄为婴儿期 49.2%，儿童期 9.5%，青春期 41.3%。男孩最常出现在婴儿期（68.0%），而女孩通常在青春期出现肿胀（55.3%）。淋巴水肿累及四肢（81.9%）、生殖器（4.3%）或两者（13.8%）。下肢最常受累（91.7%），52.9%有双侧下肢疾病。11%的患者有家族性或综合征性淋巴水肿。18.8%的儿童发生蜂窝织炎；13.0%需要住院治疗。大多数患者（57.9%）的疾病进展。治疗方法为单独使用压缩服装（75.4%）或联合使用气动压缩（19.6%）；13.0%的患者进行了手术干预。
儿科原发性淋巴水肿通常累及下肢。男孩通常在出生时发病，女孩则通常在青春期发病。大多数患者无严重发病率，通过压缩治疗成功管理，无需手术治疗。
