Lymphatic malformations: clinical course and management in 64 cases

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 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.