
Definition
A venous malformation (VM) is a collection of abnormally formed veins. Unlike
normal veins that have well developed muscle in the wall, a venous malformation
has a very poorly formed wall with less muscle than normal. The weakness
of the wall of the veins is believed to cause them to gradually stretch
or enlarge over time. A VM can develop in any part of the body.
Symptoms And Signs
Venous malformations typically cause swelling and enlargement of the affected
area, often associated with pain. Swelling occurs when the VM fills with
blood, and the blood remains in the abnormal veins. The swelling worsens
when the effected area is lower than the rest of the body (dependency)
or when the pressure in the veins rises (for example, during a Valsalva
maneuver or breath-holding). VMs are soft. Usually, they can be indented
when you press on them and get smaller when the effected area is raised.
When the skin is involved with the venous malformation, it typically looks
blue or purple. Because the blood in a VM circulates very slowly, blood
clots form and may calcify, causing "phleboliths" or vein stones. The
formation of blood clots is often painful, and can cause the malformation
to become swollen and firm. Symptoms vary according to the location of
the VM. Those involving the tongue or other structures around the airway
may cause problems with breathing or speaking, while those in the arms
and legs typically cause painful swelling. Rarely, blood clots that form
in a VM can travel to the lungs, causing a pulmonary embolus. An extremely
large VM can consume blood clotting proteins, causing localized intravascular
coagulation, where the body loses some of its ability to form blood clots.
Diagnosis
A VM that involves the skin and superficial tissue can often be diagnosed
by physical examination, if the physician is familiar with vascular anomalies.
Magnetic resonance imaging (MRI) is the best imaging test to diagnose
a VM, and to determine the extent. Ultrasonography is also useful to make
the diagnosis when the VM is near the surface. Angiography is usually
not needed to make the diagnosis.
Treatment Of Venous Malformations
A VM that is not causing symptoms does not need to be treated. Basic treatment
consists of the use of graded elastic compression stockings or sleeves
(for a VM of the leg or arm) to prevent swelling and low-dose aspirin
to minimize the formation of painful blood clots. When these measures
are not adequate, techniques are used to close or remove the enlarged
venous spaces:
Prognosis
Venous malformations are believed to be the result of a genetic abnormality
in the affected tissue. Therefore, except for small lesions, VM are not
curable. Regardless of the treatment used, they will probably re-occur.
In spite of this fact, it has been shown that treatment is helpful in
the long term to control the growth and the symptoms. Extensive VMs often
require a series of ablation procedures, and then additional treatment
a few years later. Most patients live relatively normal lives, and many
are high achievers in school, physical activities and careers.
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