Do I need to wear a compression garment for an arteriovenous malformation and why?

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Local compression is an integral part of the medical treatment of AVMs. It should be introduced from the earliest
age when the diagnosis is made and when the location allows.
Compression garments must be made to measure, particularly for children, and must be well adapted to the patient's
morphotype to avoid being deleterious (tourniquet effect, for example). It can be made in all
modes: ankle brace, knee brace, sock, stocking, tights, sleeve.
Local compression plays a protective role vis-à-vis̀-trauma on fragile skin or a dilated vessel
, but also compresses dilated subcutaneous vascular networks (reducing the flow of MAVs
potentially slowing their development). It is in no way curative. The class is type 2
minimum with equipment similar to that used in burn patients.
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Is sport advisable if I have an arteriovenous malformation?

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Lifestyle must be adapted to the pathology and its location. Any local trauma must be avoided́:
contact sports, certain high-risk professions. There are no prohibitions, but wishes must be discussed in
multidisciplinary consultation so that we can help tò guide the patient in his or her life choices. For children,
they should be guided as soon as possible towards a trauma-free sport (swimming, for example).
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What precautions should be taken in the event of pregnancy?
Pregnancy is not contraindicated, but requires careful anticipation and monitoring by the specialized multidisciplinary
team. Progressive flare-ups of AVMs have beeń observed during/after pregnancy
in some patients.

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What is coagulopathy in venous malformation?

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In venous malformations, blood tends to stagnate in the blood pockets and this contributes to the
formation of thrombi (blood clots), which will gradually be "digested" by enzymes.
This is reflected by an elevation on the blood test of D-Dimers (> 500ng/ml). We consider
pathological only if the D-Dimer level is elevated (> 1,500 ng/ml), and associated with pain.
You will be asked to take a blood sample as an initial check-up to determine your baseline level. It is
usual to find slightly elevated D-dimer levels in venous malformations, without this being
worrying or dangerous for the patient. It is therefore neither phlebitis nor pulmonary embolism, and there is
no need to consult an emergency room apart from specific symptoms such as unusual shortness of breath and calf pain.
Furthermore, thrombosis is intrinsic to the malformation, and in the absence of communication with the deep venous network, there is no risk of
migration or pulmonary embolism.

What do I need to know if I'm traveling?

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In the event of a long trip (> 4H), and if your baseline D-dimer level is >500 ng/mL, an injection of anticoagulant is recommended on the morning of the trip. In the case of prolonged travel (over 2 days), an injection in the morning before departure and on arrival may be recommended.
Anticoagulation is achieved by an injection of low-molecular-weight heparin (LMWH), e.g. Lovenox
0.4 mL.
This is done to prevent the risk of thrombosis within the venous malformation, and
thus prevent painful episodes.
Class 2 compression garments are also recommended for venous malformations of the lower
limbs.

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What to do in the event of an acute pain crisis?

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In the event of a painful episode, simple analgesic treatment with Paracetamol or anti-inflammatories may
suffice. If these episodes are punctuated by strss, climatic changes, cycles, etc. throughout the year, or when a triggering element is identified, an anti-platelet aggregation treatment can be instituted
with Kardégic 75 mg or 160 mg or Résitune 75 mg or 100 mg (in
accordance with the patient's weight) per day, to be taken in the middle of meals, in order to fluidify the blood in the
venous pockets. This treatment is taken in courses of 2-3 months, and can be renewed by the attending physician.
If the acute pain crisis does not subside with simple analgesics (Paracetamol or anti-inflammatories), it may be necessary to introduce anticoagulation for a few days
.
The anticoagulant treatment consists of 1 injection/day of low-molecular-weight heparin (LMWH), by
e.g. Lovenox 0.4 ml for 7 to 10 days, followed by Kardégic or Résitune.
What precautions should be taken in the event of surgery?
In the event of a scheduled invasive procedure (surgery, removal of wisdom teeth, childbirth, etc.), and if D-
Dimers are greater than 1.500 ng/ml, we recommend starting anticoagulation with HPBM 10
days before and after the procedure.
In the event of an emergency procedure, and if the D-Dimer is greater than 1,500 ng/ml, this treatment can be carried out 6 hours
beforehand, using Heparin IVSE (electric syringe).

What precautions should I take if I'm pregnant?

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Pregnancy is not contraindicated, but requires careful anticipation and monitoring by the specialized multidisciplinary
team.
Pregnancy alone is a condition that can physiologically increase D-dimer levels. It is therefore
advisable to know your baseline D-dimer level prior to pregnancy. There are two possible situations:
1. Your D-dimer level is below 1,500 ng/mL: monitoring by D-dimer assay
can be scheduled for the 2nd trimester, and if it is stable, no special precautions are required.
2. Your D-dimer level is above 1,500 ng/mL: monitoring by D-dimer assay
is recommended every month.
- If they are > 2,500 ng/mL AND you are symptomatic, treatment with LMWH should be
initiated to thin the blood and relieve pain.
- If they are > 2,500 ng/mL, even in the absence of symptoms, treatment with LMWH should
be initiated systematically 10 days before a planned delivery and continued
at least 3 weeks after delivery.

Is sport advisable if I have a venous malformation?
For children and adults alike, physical activity and sport are important for maintaining
good health. There are no particular contraindications linked to venous malformation, but
sporting or professional activity must be adapted in the event of pain or significant discomfort.

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What to do in the event of a lymphatic malformation flare-up?
A lymphatic malformation flare-up can cause swelling of the cysts, pain, red inflammatory skin
around the cysts, and sometimes fever or feverishness.
Treatment with a combination of antibiotics and a short course of corticosteroids should be started as soon as possible. Most often
, this will be a 7-day course of broad-spectrum penicillin antibiotics, but may be
modified if there is a history of allergy. Corticosteroids are adapted to the patient's weight and should be taken as 1
morning dose.
A prescription will be given to you at the consultation and can be used during these
flare-ups. This prescription can also be renewed by your GP.
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What are the risks during pregnancy?
Pregnancy does not appear to have a significant impact on the evolution of
lymphatic malformations. However, cases of inflammatory flare-ups and increased volume have been reported
after delivery.
As far as possible, background treatments should be avoided during pregnancy. Sirolimus, for example,
should be suspended a few weeks before conception is scheduled.
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Is it advisable to take part in sports if I have a lymphatic malformation?
For children and adults alike, physical activity and sport are important for maintaining
good health. Depending on the location of their
lymphatic malformation and the discomfort it causes, patients should be guided towards the most appropriate sports. In cases of major functional disability
, adapted physical activity should be provided (physiotherapy, adapted sports).

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