Lymphatic Malformation: Risk of Progression During Childhood and Adolescence
Lymphatic Malformation: Risk of Progression During Childhood and Adolescence
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DOI：
10.1097/scs.0b013e3182413ea8
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发表时间：
2012-01-01
影响因子：
0.9
通讯作者：
Greene, Arin K.
中科院分区：
文献类型：
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作者：
Hassanein, Aladdin H.;Mulliken, John B.;Greene, Arin K.
随着时间的推移，软组织中的淋巴畸形（LMs）往往会扩大，导致扭曲、阻塞和功能问题。本研究的目的是确定LMs的自然进展，以便于患者咨询，了解病理生理，指导治疗。我们的血管异常中心数据库回顾了皮肤和软组织LMs患者；排除合并或内脏病变。预测变量为年龄、通道类型（大囊、微囊、合并）、性别、病变大小（局部、弥漫性）和位置（头颈部、四肢、躯干）。结果变量是由扩张或体征和症状的发生/恶化定义的畸形的自然进展。共纳入441例患者，其中女性234例（53.1%），男性207例（46.9%）。淋巴畸形位于头颈部（61.2%）、四肢（17.5%）、躯干（16.1%）和多处（5.2%）。儿童在青春期前有42.2%的风险，成年前有84.7%的风险，在其一生中有95.3%的风险。青春期（63.8%）比儿童期（40.8%）更容易发生进展；优势比为2.6 （P = 0.003）。弥漫性LMs比局限性病变更容易恶化（P = 0.001），而通道类型（P = 0.63）、性别（P = 0.42）和位置（P = 0.28）对进展没有影响。淋巴畸形在青春期发展的风险大于儿童期；青春期荷尔蒙可能有助于扩张。由于这种高进展率，应该考虑早期治疗无症状的LMs。
Lymphatic malformations (LMs) in soft tissues tend to enlarge over time, causing distortion, obstruction, and functional problems. The purpose of this study was to determine the natural progression of LMs to facilitate patient counseling, gain insight into pathophysiology, and guide therapy.Our Vascular Anomalies Center database was reviewed for patients with cutaneous and soft tissue LMs; combined or visceral lesions were excluded. Predictive variables were age, channel type (macrocystic, microcystic, combined), sex, lesion size (localized, diffuse), and location (head/neck, extremities, trunk). The outcome variable was natural progression of the malformation defined by expansion or the onset/worsening of signs and symptoms.The study included 441 patients: 234 females (53.1%) and 207 males (46.9%). Lymphatic malformations were located in the head/neck (61.2%), extremities (17.5%), trunk (16.1%), or multiple sites (5.2%). Children had a 42.2% risk of progression before adolescence, 84.7% before adulthood, and 95.3% during their lifetime. Progression was more likely in adolescence (63.8%) than in childhood (40.8%); the odds ratio was 2.6 (P = 0.003). Diffuse LMs worsened more often than localized lesions (P = 0.001), whereas channel type (P = 0.63), sex (P = 0.42), and location (P = 0.28) did not influence progression.Lymphatic malformations have a greater risk of progression in adolescence than in childhood; pubertal hormones may contribute to expansion. Because of this high rate of progression, early treatment of asymptomatic LMs should be considered.