Lymphatic malformations

Malformations of lymphatic vessels result from pathological embryonal development of the lymphatic system. The current classification of the International Society for the Study of Vascular Anomalies (ISSVA) differentiates common (cystic) lymphatic malformation (LM), generalized lymphatic anomalies (GLA), LM in Gorham-Stout disease (GSD), channel type LM, primary lymphedema and lymphangioma. Diagnostic procedures include ultrasound and magnetic resonance imaging (MRI) after complete physical examination with registration of clinical signs, such as the Stemmer's sign. Lymphoscintigraphy can give further information on the function of lymphatic vessels. Treatment concepts have to be elaborated in an interdisciplinary team: surgery and interventional radiology including sclerotherapy and laser therapy can be administered. In GSD and GLA as well as in other syndromes with LM, medicinal therapy is possible. Clinical trials are currently being planned to find out which patients could benefit from which drugs.