Phlegmasia Alba Dolens
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Phlegmasia alba dolens (also colloquially known as milk leg or white leg; not to be confused with
phlegmasia cerulea dolens Phlegmasia cerulea dolens (PCD) (literally: 'painful blue inflammation'), not to be confused with preceding phlegmasia alba dolens, is an uncommon severe form of lower extremity deep venous thrombosis (DVT) that obstructs blood outflow from a vein ...
) is part of a spectrum of diseases related to
deep vein thrombosis Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and enl ...
. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. In cases of pregnancy, it is most often seen during the third trimester, resulting from a compression of the left
common iliac vein In human anatomy, the common iliac veins are formed by the external iliac veins and internal iliac veins. The left and right common iliac veins come together in the abdomen at the level of the lumbar vertebrae, fifth lumbar vertebra, forming the ...
against the pelvic rim by the enlarged
uterus The uterus (from Latin ''uterus'', : uteri or uteruses) or womb () is the hollow organ, organ in the reproductive system of most female mammals, including humans, that accommodates the embryonic development, embryonic and prenatal development, f ...
. Today, this disease is most commonly (40% of the time) related to some form of underlying malignancy.
Hypercoagulability Thrombophilia (sometimes called hypercoagulability or a prothrombotic state) is an abnormality of blood coagulation that increases the risk of thrombosis (blood clots in blood vessels). Such abnormalities can be identified in 50% of people who ...
(a propensity to clot formation) is a well-known state that occurs in many cancer states. The incidence of this disease is not well reported.


Cause

The disease presumably begins with a
deep vein thrombosis Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and enl ...
that progresses to total occlusion of the deep venous system. It is at this stage that it is called phlegmasia alba dolens. It is a sudden (acute) process. The leg, then, must rely on the superficial venous system for drainage. The superficial system is not adequate to handle the large volume of blood being delivered to the leg via the arterial system. The result is edema, pain and a white appearance (''alba'') of the leg. The next step in the disease progression is occlusion of the superficial venous system, thereby preventing all venous outflow from the extremity. At this stage it is called
phlegmasia cerulea dolens Phlegmasia cerulea dolens (PCD) (literally: 'painful blue inflammation'), not to be confused with preceding phlegmasia alba dolens, is an uncommon severe form of lower extremity deep venous thrombosis (DVT) that obstructs blood outflow from a vein ...
. The leg becomes more swollen and increasingly more painful. Additionally, the edema and loss of venous outflow impedes the arterial inflow. Ischemia with progression to gangrene are potential consequences. Phlegmasia alba dolens is distinguished, clinically, from
phlegmasia cerulea dolens Phlegmasia cerulea dolens (PCD) (literally: 'painful blue inflammation'), not to be confused with preceding phlegmasia alba dolens, is an uncommon severe form of lower extremity deep venous thrombosis (DVT) that obstructs blood outflow from a vein ...
in that there is no ischemia and congestion. In severe cases of venous obstruction the arterial pulse may gradually disappear and venous gangrene may ensue.


Diagnosis

The condition can be identified clinically & as early as possible to prevent further deterioration & complications like
embolism An embolism is the lodging of an embolus, a blockage-causing piece of material, inside a blood vessel. The embolus may be a blood clot (thrombus), a fat globule (fat embolism), a bubble of air or other gas (air embolism, gas embolism), amniotic ...
,
ischaemic necrosis Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism (to keep tissue alive). Ischemia is generally caused by problems wit ...
, and
gangrene Gangrene is a type of tissue death caused by a lack of blood supply. Symptoms may include a change in skin color to red or black, numbness, swelling, pain, skin breakdown, and coolness. The feet and hands are most commonly affected. If the ga ...
. The diagnosis is confirmed with Doppler ultrasound of the veins of the affected leg, and occasionally other imaging modalities.


Treatment

The mainstay of the treatment of deep vein thrombosis is with
anticoagulation An anticoagulant, commonly known as a blood thinner, is a chemical substance that prevents or reduces the coagulation of blood, prolonging the clotting time. Some occur naturally in blood-eating animals, such as leeches and mosquitoes, which h ...
, but in very large DVTs various other modalities may be used to reduce the risk of complications. This may be surgical
thrombectomy Mechanical thrombectomy, or simply thrombectomy, is the removal of a blood clot (thrombus) from a blood vessel, often and especially endovascularly as an interventional radiology procedure called endovascular thrombectomy (EVT). It thus contr ...
or catheter-directed
thrombolysis Thrombolysis, also called fibrinolytic therapy, is the breakdown (lysis) of thrombus, blood clots formed in blood vessels, using medication. It is used in ST elevation myocardial infarction, stroke, and in cases of severe venous thromboembolism ( ...
.


Etymology

Phlegmasia alba dolens literally means "painful white
edema Edema (American English), also spelled oedema (British English), and also known as fluid retention, swelling, dropsy and hydropsy, is the build-up of fluid in the body's tissue (biology), tissue. Most commonly, the legs or arms are affected. S ...
". It received the name "milk leg" because it was once thought to be caused by the
metastasis Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
of milk.


References


External links

{{Medical resources , ICD10 = {{ICD10, I, 80, 1, i, 80, 1 , ICD9 = {{ICD9, 451.11 , ICDO = , OMIM = , DiseasesDB = 32484 , MedlinePlus = , eMedicineSubj = article , eMedicineTopic = 461809 , MeshID = Vascular diseases