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This is a vascular anomaly consisting of "extra" veins, most often localized at a specific site. At this point, the veins have lost their "tube" shape, which allows normal blood flow, and form pockets in which the blood stagnates. These pockets will gradually grow with the patient over time, but the location will remain the same. When these pockets are located near the surface of the skin or mucous membranes, they take on a bluish color.
Venous malformations are congenital, meaning that they are present at birth, although they become symptomatic at different ages.
In the vast majority of cases, these malformations are not hereditary, i.e. they affect only one individual in a family, and this individual will not transmit the malformation to his or her children. Recently, somatic mutations have been identified, notably in the TEK/Tie2 gene.
In the vast majority of cases, venous malformations are annoying and sometimes unsightly, but benign, i.e. not life-threatening. However, there are some exceptional cases that fall outside this rule. A coagulation disorder is frequently found in venous malformations, responsible for intra-lesional thrombosis and, very rarely, bleeding. This is also the case with venous malformations of the digestive tract, which can be complicated by digestive haemorrhage.
The most frequent symptoms are swelling of the malformation and painful episodes. Venous pockets can become painful because of their size, but also because of thrombosis episodes (clot formation in the malformation). All everyday situations that increase the pressure inside the veins will lead to a temporary increase in the volume of the pockets and can trigger pain: this is the case for all pushing efforts, prolonged standing, carrying heavy loads, sporting efforts, but also climatic changes or the hormonal cycle.
Venous malformations may be associated with localized intravascular coagulopathy (LIVC), reflecting thrombosis within the venous pockets. This thrombosis can be the cause of painful episodes.
This is reflected on the blood test by an increase in D-dimer levels (> 500 ng/ml). This value is usually normal in patients with no malformation (except in cases of pulmonary embolism, phlebitis, etc.). However, in patients with venous malformations, it is usual to find slightly elevated D-dimer levels, without this being worrying or dangerous. We consider as pathological only an elevation of D-Dimer (> 1,500 ng/ml), associated with pain.
In the event of severe pain, anticoagulant treatment with Low Molecular Weight Heparin (LMWH) can be initiated, e.g. a 7-10 day course of Lovenox 0.4 mL/day.
This can be followed by a course of Kardégic 75 mg or 160 mg (depending on the patient's weight) per day, for a 2-3 month course.
In the case of scheduled invasive procedures (surgery, removal of wisdom teeth, childbirth, etc.), if D-dimer levels exceed 1500 ng/mL, we recommend that you start taking LMWH 10 days before and after the procedure.
In the event of an emergency procedure and if D-dimer levels are > 1500 ng/mL, subcutaneous anticoagulant treatment with Low Molecular Weight Heparin (LMWH) can be initiated 6 hours beforehand.
Venous malformations are rare diseases. Treatment depends on symptomatology and location, and may vary from one team to another.
In our center, venous malformations are initially treated by medical therapy, combining :
- Wearing a compression garment whenever possible, to facilitate venous drainage and reduce pocket swelling. This is usually a Class II compression garment, which should be worn as often as possible and renewed regularly.
- Raising the limb, especially at night.
- Taking low-dose Aspirin (2 mg/kg/day, i.e. Kardégic 75mg or 160mg) every day for 1 to 3 months. This treatment thins the blood and prevents thrombosis.
- Massage the venous malformation during painful episodes with Helichrysum oil.
If this treatment is not sufficient, we can propose treatment with either sclerotherapylaser treatment, surgery or a combination of these techniques.
These techniques are not always offered, and when the venous malformation is not very troublesome or too difficult to access, we are content with simple monitoring.
