Lymphatic Malformations

Paediatric Lymphoedema and Primary Lymphoedema is caused by alterations (mutations) in genes responsible for the development of the lymphatic system and therefore leads to the lymphatic system breaking down. The "faulty" genes cause the parts of the lymphatic system responsible for draining fluid to not develop properly or not work as they should which can then cause swelling and tissue changes. The swelling will appear as a mass or lumps these growths are benign and not cancerous. In the case of some lymphatic malformations the lymphatic vessels did not develop properly in the womb. Primary lymphoedema often runs in families, although not every child born to someone with this condition will develop lymphoedema themselves.

Lymph Fluid is a clear pale-yellow colour

The lymphatic system forms part of the immune system that identifies and fights off invaders, such as bacteria and viruses consisting of a network of vessels within the body through which lymph circulates, and many lymph nodes filter the lymph fluid that helps the body fight infection. Often the lymphatic vessels do not develop properly in the womb. Nobody knows why this happened, nobody is to blame, and this could not have been prevented. These malformations grow because the lymph in the vessels do not drain well, they then swell causing a flare-up. They often look red, swollen and can feel tender if touched.

Two main types of LM

macrocystic lymphatic malformations (cystic hygromas or lymphangiomas) have large stretched vessels and cysts filled with lymph, blood from internal bleeding or both
microcystic lymphatic malformations are spongy have have small vessels and tiny cysts