Chapter: Lymphatic malformations
Article: 1 of 14
Update: Mar 15, 2021
Author(s): Meyer, Lutz
Lymphatic malformations (LM) are congenital malformations of the lymphatic vascular system. A distinction must be made between circumscribed (micro- and macrocystic; sponge-like or vesicular) lesions and primary lymphedema, which appears as diffuse swelling.
While cystic LM is based on a localized vascular malformation and drainage into the lymphatic system is usually not a factor, primary lymphedema is a malformation of the lymphatic drainage system involving limited absorption and transport capacity of the lymphatic system with fluid congestion.
Lymphatic malformations can become visible at different times and cause symptoms of varying severity, depending on their extent and localization.