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 Apr;127(4):1571-1581. doi: 10.1097/PRS.0b013e31820a64f3.
There are many causes for a large lower limb in the pediatric age group. These children are often mislabeled as having lymphedema, and incorrect diagnosis can lead to improper treatment. The purpose of this study was to determine the differential diagnosis in pediatric patients referred for lower extremity "lymphedema" and to clarify management.
The authors' Vascular Anomalies Center database was reviewed between 1999 and 2010 for patients referred with a diagnosis of lymphedema of the lower extremity. Records were studied to determine the correct cause for the enlarged extremity. Alternative diagnoses, sex, age of onset, and imaging studies were also analyzed.
A referral diagnosis of lower extremity lymphedema was given to 170 children; however, the condition was confirmed in only 72.9 percent of patients. Forty-six children (27.1 percent) had another disorder: microcystic/macrocystic lymphatic malformation (19.6 percent), noneponymous combined vascular malformation (13.0 percent), capillary malformation (10.9 percent), Klippel-Trenaunay syndrome (10.9 percent), hemihypertrophy (8.7 percent), posttraumatic swelling (8.7 percent), Parkes Weber syndrome (6.5 percent), lipedema (6.5 percent), venous malformation (4.3 percent), rheumatologic disorder (4.3 percent), infantile hemangioma (2.2 percent), kaposiform hemangioendothelioma (2.2 percent), or lipofibromatosis (2.2 percent). Age of onset in children with lymphedema was older than in patients with another diagnosis (p = 0.027).
"Lymphedema" is not a generic term. Approximately one-fourth of pediatric patients with a large lower extremity are misdiagnosed as having lymphedema; the most commonly confused causes are other types of vascular anomalies. History, physical examination, and often radiographic studies are required to differentiate lymphedema from other conditions to ensure the child is managed appropriately.
在儿科年龄组中，导致下肢巨大的原因有很多。这些儿童经常被误诊为淋巴水肿，如果诊断不正确，可能会导致治疗不当。本研究的目的是确定因下肢“淋巴水肿”就诊的儿科患者的鉴别诊断，并阐明治疗方法。
作者的血管异常中心数据库在 1999 年至 2010 年期间对以下肢淋巴水肿诊断就诊的患者进行了回顾。对记录进行了研究，以确定增大肢体的正确原因。还分析了其他诊断、性别、发病年龄和影像学研究。
170 名儿童被诊断为下肢淋巴水肿，但只有 72.9%的患者得到了确诊。46 名儿童（27.1%）存在另一种疾病：微囊/大囊淋巴管畸形（19.6%）、非特定联合血管畸形（13.0%）、毛细血管畸形（10.9%）、Klippel-Trenaunay 综合征（10.9%）、半侧肥大（8.7%）、创伤后肿胀（8.7%）、Parkes Weber 综合征（6.5%）、脂肪营养不良（6.5%）、静脉畸形（4.3%）、风湿性疾病（4.3%）、婴儿血管瘤（2.2%）、kaposiform 血管内皮细胞瘤（2.2%）或脂肪纤维瘤病（2.2%）。淋巴水肿患儿的发病年龄大于其他诊断患儿（p = 0.027）。
“淋巴水肿”不是一个通用术语。大约四分之一的下肢巨大的儿科患者被误诊为淋巴水肿；最常被混淆的原因是其他类型的血管异常。为了区分淋巴水肿与其他疾病，确保儿童得到适当的治疗，需要进行病史、体格检查和影像学研究。
