Lymphatic malformations: Clinical course and management in 64 cases
Lymphatic malformations: Clinical course and management in 64 cases
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DOI：
10.1111/j.1440-0960.2011.00777.x
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发表时间：
2011-08-01
影响因子：
2
通讯作者：
Wargon, Orli
中科院分区：
文献类型：
--
作者：
Hogeling, Marcia;Adams, Susan;Wargon, Orli
背景：淋巴畸形（LM）是罕见的血管畸形。目的：明确 LM 儿童的临床特征及其治疗。方法：我们对 LM 儿童进行回顾性图表审查并对家长进行电话访谈。记录人口统计学和临床特征、诊断影像、治疗和并发症。结果：确定了 30 名男性和 34 名女性 LM 患者。 LM 的解剖位置最常见的是头部和颈部 (48%)。 LM 更频繁地累及身体左侧 (62%)。儿童最常见的表现为皮肤颜色肿瘤或囊肿的肿胀，较少见的是偏侧肥大、瘀伤和浅表假性水疱。大多数LM是大囊性的(60%)，其次是微囊性的(24%)和混合性的(16%)。诊断时的平均年龄为 37 个月，51% 的 LM 在出生时就已明显。 LM 导致 70% 的病例发病并导致一名儿童死亡。治疗方法为硬化疗法，使用 3% 十四烷基硫酸钠 (30.5%)、OK-432 (17%) 和多西环素 (10%)； 27%接受了手术； 34% 未接受治疗。结论：LM 是异质性血管畸形，可能导致显着的发病率。诊断常常被延误。包括皮肤病学、诊断和介入放射学以及小儿外科在内的多学科治疗方法非常重要。
Background: Lymphatic malformations (LM) are rare vascular malformations.Objectives: To define the clinical characteristics of children with LM and their management.Methods: We performed a retrospective chart review of children with LM and telephone interviews with parents. Demographic and clinical features, diagnostic imaging, treatments and complications were recorded.Results: Thirty male and 34 female patients with LM were identified. The anatomic location of the LM was most frequently the head and neck (48%). LM involved the left side of the body more frequently (62%). Children presented most commonly with swelling as skin coloured tumours or cysts, and less frequently with hemihypertrophy, bruising, and superficial pseudo-vesicles. Most LM were macrocystic (60%), followed by microcystic (24%) and mixed (16%). The mean age at diagnosis was 37 months, with 51% of LM obvious at birth. LM caused morbidity in 70% of cases and death in one child. Treatments were sclerotherapy with sodium tetradecyl sulfate 3% (30.5%), OK-432 (17%) and doxycycline (10%); 27% received surgery; 34 % had no treatment.Conclusions: LM are heterogenous vascular malformations that may result in significant morbidity. Diagnosis is often delayed. A multidisciplinary approach to management including dermatology, diagnostic and interventional radiology and paediatric surgery is important.