Department of Paediatric Dermatology, UNSW School of Women's and Children's Health, Sydney Children's Hospital, Sydney, NSW, Australia.
Australas J Dermatol. 2011 Aug;52(3):186-90. doi: 10.1111/j.1440-0960.2011.00777.x. Epub 2011 Jun 9.
Lymphatic malformations (LM) are rare vascular malformations.
To define the clinical characteristics of children with LM and their management.
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.
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.
LM are heterogeneous 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.
淋巴管畸形（LM）是一种罕见的血管畸形。
明确儿童 LM 的临床特征及其治疗方法。
我们对患有 LM 的儿童进行了回顾性病历分析，并对其父母进行了电话访谈。记录了人口统计学和临床特征、诊断性影像学检查、治疗方法和并发症。
共发现 30 名男性和 34 名女性 LM 患儿。LM 的解剖位置最常见于头颈部（48%）。LM 更常累及身体左侧（62%）。患儿最常表现为皮肤颜色的肿块或囊肿样肿胀，其次为单侧肥大、瘀斑和浅表假性水疱。大多数 LM 为大囊型（60%），其次为微囊型（24%）和混合型（16%）。诊断时的平均年龄为 37 个月，51%的 LM 在出生时即已明显。70%的 LM 导致发病，1 例患儿死亡。治疗方法包括 3%十四烷基硫酸钠（30.5%）、OK-432（17%）和多西环素（10%）硬化治疗；27%接受了手术；34%未接受治疗。
LM 是一种异质性的血管畸形，可能导致严重的发病。诊断往往被延误。多学科管理方法，包括皮肤科、诊断和介入放射学以及小儿外科学，非常重要。
