Lymphatic malformations: clinical course and management in 64 cases

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.