Hassanein Aladdin H, Mulliken John B, Fishman Steven J, Quatrano Nicola A, Zurakowski David, Greene Arin K
Department of Plastic and Oral Surgery, Vascular Anomalies Center, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
J Craniofac Surg. 2012 Jan;23(1):149-52. doi: 10.1097/SCS.0b013e3182413ea8.
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.
软组织中的淋巴管畸形（LMs）往往会随着时间的推移而扩大，导致畸形、梗阻及功能问题。本研究的目的是确定淋巴管畸形的自然进展情况，以便为患者咨询提供便利，深入了解病理生理学，并指导治疗。我们查阅了血管异常中心数据库中患有皮肤及软组织淋巴管畸形的患者；排除了合并或内脏病变的患者。预测变量包括年龄、通道类型（大囊型、微囊型、混合型）、性别、病变大小（局限性、弥漫性）及部位（头颈部、四肢、躯干）。结局变量是由畸形扩大或体征和症状的出现/加重所定义的畸形自然进展情况。该研究纳入了441例患者：女性234例（53.1%），男性207例（46.9%）。淋巴管畸形位于头颈部（61.2%）、四肢（17.5%）、躯干（16.1%）或多个部位（5.2%）。儿童在青春期前进展的风险为42.2%，成年前为84.7%，一生中有95.3%的风险。青春期进展的可能性（占63.8%）高于儿童期（占40.8%）；优势比为2.6（P = 0.003）。弥漫性淋巴管畸形比局限性病变恶化更频繁（P = 0.001），而通道类型（P = 0.63）、性别（P = 0.42）及部位（P = 0.28）不影响进展。淋巴管畸形在青春期比儿童期有更高的进展风险；青春期激素可能促使其扩大。鉴于这种高进展率，对于无症状的淋巴管畸形应考虑早期治疗。
