About us
Lymphatic malformations are spaces also known as fluid pockets, because they contain lymphatic fluid, most of which is lemon-yellow in color.
MLs probably appear during the formation of the lymphatic network, which is a system of vessels, pockets and tubes. It is satellite to the veins and is distributed throughout the body, with the exception of the brain.
These may be large pockets (macrocysts), multiple small pockets (microcysts or tissue) or a mixed form (macro and microcysts).
ML is very often superficial, located directly under the skin. The skin may be dotted with small vesicles, which can blacken with bleeding.
Some MLs may also be located deeper down, in the muscles for example, and localized in all parts of the body. Episodes of swelling or enlargement can occur in the body parts where it is located: neck, cheek, lips, tongue, etc.
They may also be associated with combined syndromes or venous malformations (veno-lymphatic malformations).
The development of lymphatic malformations is often sporadic (without cause), but sometimes somatic mutations are found, notably in the PIK3CA gene. These are congenital malformations, developing in the embryo between the 7th and 8th week of pregnancy (1st trimester).
Parents are in no way responsible. No food, medication or activity ingested during pregnancy is responsible for the appearance of a lymphatic malformation.
In the vast majority of cases, lymphatic malformations are annoying, sometimes unsightly, but benign, i.e. not life-threatening. Symptoms are related to inflammatory flare-ups complicating :
- bleeding inside the lymphatic pouch
- superinfection of the lymphatic sac with an increase in the size of the lesion, secondary to a locoregional infection (e.g. angina, dental infection, all infections that develop in the region of the lymphatic sac).
- acute infections (often manifested by a discharge of liquid from the skin surface)
Lymphatic malformations can increase during infectious episodes.
The aim is to limit these infectious episodes:
- For ML in the head and neck region, good oral hygiene is essential. It's important to monitor children's health and treat them for ear infections as soon as the first symptoms appear.
- In the event of a lymphatic malformation flare-up, antibiotics are combined with short-term corticosteroids for 7-10 days. In order to treat the cause of the flare-up, a search is made for the entry point.
Depending on their location, size and appearance, different treatments can be envisaged: sclerosis, a procedure to reduce the size of the pockets; or surgery if sclerosis is difficult or ineffective.
If there are few or no symptoms, management may consist of simple monitoring.
Further information is available on the PNDS :
