Topic Resources
DR P. MARAZZI/SCIENCE PHOTO LIBRARY
Most lymphatic malformations are present at birth or develop within the first 2 years. They occur primarily in the head and neck but also in the axillary and mediastinal regions (1). They may occur sporadically, or occur as part of a syndrome that also includes blood vessel malformations, such as Klippel-Trenaunay, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies/scoliosis) and CLAPO syndromes (capillary vascular malformation of the lower lip, lymphatic malformations of the head and neck, asymmetry, and partial or generalized overgrowth).
Image courtesy of Karen McKoy, MD.
Lesions are usually yellowish tan but occasionally reddish or purple if small blood vessels are intermingled. Puncture of the lesion yields a colorless or blood-tinged fluid.
Reference
1. International Society for the Study of Vascular Anomalies. 2025 ISSVA Classification. Accessed July 15, 2025.
Diagnosis of Lymphatic Malformations
Primarily history and physical examination
Diagnosis of lymphatic malformations is made clinically and by ultrasound or MRI, as the clinical differential diagnosis may be broad.
Treatment of Lymphatic Malformations
Usually unnecessary
Sclerotherapy
Treatment of lymphatic malformations is usually not needed.
Depending on lesion size, extent, and symptoms, Sclerotherapy is first-line treatment for problematic lymphatic malformations.
