What is Phlegmasia Alba and Cerulea Dolens (Milk Leg)?
Being able to correctly and efficiently diagnose acute venous thromboembolism (VTE), a blood clot that starts in a vein, is especially important for patients in the hospital. This is because VTE and its complications, which may include deep vein thrombosis (DVT), a blood clot in a deep vein usually in the leg, and pulmonary embolism (PE), a blood clot in the lungs, are the top preventable reasons for hospital deaths. They can also cause long-term health problems.[1]
Two less common complications of DVT are Phlegmasia alba dolens (PAD) and phlegmasia cerulea dolens (PCD). They carry a high risk of death and losing a limb[2]. Phlegmasia refers to severe cases of DVT in the lower leg, which could progress to a situation where the limb does not get enough blood supply, leading to potential loss of the limb.
Fabricius Hildanius was the first to describe this condition in the 16th century. Later, in 1938, Gregoire gave the name phlegmasia cerulea dolens, which translates to “painful blue inflammation”. This is different from phlegmasia alba dolens, or “painful white inflammation,” which is seen more often.[3] PAD, which is also called “milk leg,” is an early stage of this condition, where the clotting blocks the regular flow of blood. PCD is a further development of this condition and can lead to venous gangrene, a serious condition where tissues decay due to a lack of blood flow.
What Causes Phlegmasia Alba and Cerulea Dolens (Milk Leg)?
Phlegmasia dolens is a medical condition in which a sudden large blood clot (also known as a Venous Thromboembolism or VTE) blocks the flow of blood from a limb, commonly a leg. Basically, a VTE is a fancy term for a blood clot that forms in a vein. This can be particularly serious if it occurs in a vein that’s deep inside the body, impacting the iliofemoral segments (the veins in the groin area).
Studies have shown that in 20% to 40% of cases, phlegmasia is linked to cancer. However, there are other things that can increase the risk of developing VTE; these are known as risk factors. Some of these risk factors include disorders that make your blood clot more easily, issues with vein flow or function, a condition known as May Thurner syndrome where the left major vein in the pelvis is compressed, surgery, injury, pregnancy, the placement of filters in the largest vein in the body (the inferior vena cava or IVC), using hormonal therapy or birth control pills, being immobilized for a long time, inflammatory bowel disease, heart failure, and having a central venous catheter (a tube placed in a large vein to give medications or fluids). In about 10% of cases, we don’t know why the phlegmasia happened.
Risk Factors and Frequency for Phlegmasia Alba and Cerulea Dolens (Milk Leg)
PAD and PCD are diseases linked with severe VTE, a kind of blood clot. These diseases are rare, so it’s hard to say exactly how many people get them. They are most common in people in their 50s and 60s, but they can happen at any age, even in babies as young as 6 months old. They are slightly more common in men than women.
- Most cases of PAD and PCD affect the lower limbs, especially the left leg because of its close proximity to the right iliac artery.
- It’s also not rare for both legs to be affected.
- These conditions can cause significant problems if the iliofemoral segment (the part of the body where the leg and the trunk meet) is involved.
- If not treated properly, PAD and PCD can keep coming back and cause serious health problems.
- If the conditions worsen to the point of vein gangrene, there’s a 20%-50% chance of needing an amputation and a 20%-40% chance of death.
Signs and Symptoms of Phlegmasia Alba and Cerulea Dolens (Milk Leg)
Phlegmasia Alba Dolens (PAD) and Phlegmasia Cerulea Dolens (PCD) are conditions that are commonly known as “milk leg”. Symptoms can be unpredictable and could develop gradually over several days or suddenly, within a few hours. Typical symptoms include swelling, pain, and pale skin without blue discoloration or tissue damage. In about half to more than half of the patients, PAD often comes before PCD.
PCD shares similar symptoms with PAD, such as pain and significant swelling due to the accumulation of fluid. However, a distinguishing feature of PCD is the presence of cyanosis, or blue skin due to poor blood circulation. As the condition worsens, patients may notice changes to their skin like blisters and tissue death. In severe cases, they may also experience numbness and weakness, particularly if the swelling leads to blocked arteries and compartment syndrome, a painful condition caused by pressure build-up. The blue discoloration usually starts at the extremities but may spread to affect the entire limb. Gangrene, or tissue death, may also occur in about half of the PCD patients. In 10% to 20% of these cases, the gangrene remains superficial with the blood flow in the artery still intact. In severe instances, the deep muscles are affected, and arterial pulses may be absent.
- Swelling
- Pain
- Pale skin
- Blue discoloration
- Blisters and tissue death
- Numbness and weakness
Phlegmasia can also manifest in the upper extremities, although it’s less common. This usually happens when there is ischemic venous thrombosis, a type of blood clot, and gangrene. There are two indicators for upper extremity involvement: a possible cardiac output issue due to occlusion or blood clotting of the central veins, usually linked with central venous catheterization, and the blockage of the peripheral veins.
Another possible sign of Phlegmasia is Pulmonary Embolism. This condition is highly likely to cause emboli, or blood clots that travel through the bloodstreams, and the incident is even higher when tissue death is present. A high degree of caution should be given to patients who show signs of Deep Vein Thrombosis (DVT) and rapid heart rate or substantial oxygen needs.
Testing for Phlegmasia Alba and Cerulea Dolens (Milk Leg)
To diagnose deep vein thrombosis (DVT) and phlegmasia (swelling and redness due to blood clot), your doctor will start with a detailed check-up. They may ask you about your symptoms, when they started, how severe they are and if you have any other medical conditions. They may also ask about any past procedures related to veins or arteries and if there’s any family history of abnormal blood clotting.
After the discussion, your doctor will conduct a physical exam. This will include checking the blood flow to and from your arms and legs by examining the pulse at different points. This can be a bit tricky if you have a lot of swelling, so they might use a special device called a Doppler to make sure the signals are clear.
In severe cases, if you have skin that’s blistered or dead due to lack of blood, or if there are severe nerve effects or gangrene (when tissues die due to lack of blood), immediate efforts will be made to remove the blood clot and increase blood flow back to the heart.
Next, your doctor may ask for some blood tests like a complete blood count, and tests to check how your blood is clotting. They may also want to check the health of your kidneys and hydration status.
To confirm the diagnosis, imaging is often used. In the past, doctors used a test called contrast venography. However, this test is not practical in most situations. Now, most doctors use an ultrasound of the veins (venous duplex ultrasonography) for diagnosis.
In some cases, when they need to see the blood clot in larger veins like the iliac veins (near the pelvis) or the IVC (a large vein that carries blood from lower body to heart), they might use magnetic resonance venography or a computed tomography (CT) scan.
These tests can also help identify anything in the pelvis that might be pressing down on an iliac vein. However, these imaging tests do have their own issues. Magnetic resonance imaging can take more time and might not give clear results if you’re in a lot of pain and can’t stay still. CT scans expose you to radiation and use a contrast agent, which may affect your kidneys, especially if you are already unwell and dehydrated.
Alternatively, if you’re going to have a procedure done where a catheter (thin tube) is inserted to help with the treatment, the doctors can use this opportunity to take images and fill in any gaps in their understanding of the situation.
Your doctor will be looking at certain features on the ultrasound to suggest DVT, such as a vein that they can’t easily squish with the probe, an absence of normal blood flow in the vein, an unusually large vein diameter, or increased echoes (indicating a possible clot) inside the vein’s lumen (the space within a tube where blood flows).
Treatment Options for Phlegmasia Alba and Cerulea Dolens (Milk Leg)
The treatment for acute deep vein thrombosis (DVT), a condition where a blood clot forms in a deep vein, combined with swelling or gangrene (tissue death due to lack of blood flow) can vary. Overall, the main goals are to stop the blood clot from getting bigger, reduce the high pressure on your veins, prevent shock, avoid the development of severe gangrene, and treat the underlying condition causing these problems.
Initial treatments include quickly elevating the affected limb more than 60 degrees higher than the level of your heart. This helps prevent venous stasis, a condition where blood stops moving in the veins, and it helps blood return to the heart. If the limb isn’t elevated effectively, this could lead to venous gangrene. Elevation also decreases swelling and reduces pressure on your arteries, avoiding circulatory collapse and shock. Previously, treatments like hot packs, drugs to reduce nerve responses or blood vessel constriction, and steroids were used, but they are now not recommended, as they show little to no benefit.
The best treatment options include anticoagulation (blood-thinning) therapy, catheter-directed thrombolysis (a procedure to break up blood clots), thrombectomy (a clot removal surgery), or a combination of these, depending on how severe your condition is. Most patients respond well to fluid replacement, aggressive limb elevation, and anticoagulants. In more advanced cases or in patients whose condition doesn’t improve with anticoagulation, doctors may consider catheter-directed thrombolysis, percutaneous mechanical thrombectomy (a clot removal procedure using a catheter), or open surgical thrombectomy.
In the past, open surgery was the go-to treatment, but that was before endovascular intervention (procedures done inside the blood vessels) was introduced. Open surgery was associated with high repetition rates and complications like damage to the vessel lining, rupture, and excessive growth of inner layers of blood vessels. Nowadays, catheter-directed thrombolysis is preferable in patients who are good candidates for clot-dissolving treatments. This method prevents damage to the vessels and can potentially clear clots from smaller veins unreachable by open surgery.
Catheter-directed thrombolysis has proven to be effective in various studies. Patients with deep vein thrombosis showed significant improvements: their clots were reduced quickly, their veins’ openness was restored, and they had less risk of valve issues or post-thrombotic syndrome (a long-term complication of DVT). However, like any clot-dissolving treatment, it comes bearing the risk of bleeding complications. It’s also less successful in patients with long-lasting or chronic symptoms.
Percutaneous mechanical thrombectomy has also shown to be an efficient alternative or additional treatment to catheter-directed thrombolysis. It uses a catheter to remove or break apart the clot. Comparing it to catheter-directed thrombolysis, it requires less time to infuse clot-dissolving agents and carries a lower risk of bleeding. Additionally, it results in shorter stays in the intensive care unit and the hospital overall.
However, these treatments do come with some risks, such as a chance for pulmonary embolus (a blood clot in the lungs). This can occur because dissolving the clot can fragment it and the use of wires inside the vein can displace the clot. Thus, the consideration should be made for installing a filter in the inferior vena cava (the large vein that carries deoxygenated blood from the lower body to the heart) in certain patients who have extensive clot burden.
Currently, open surgery is used relatively rarely. Even though it lowers the risk of serious and non-fatal pulmonary emboli, the procedure itself can be very intense.
Finally, if the limb develops compartment syndrome (a dangerous condition where pressure builds up and affects blood flow), consideration should be made for a surgery to relieve this pressure. If amputation is required because other treatments have failed, it’s usually delayed to allow the area to clearly define itself and for the swelling to decrease.
What else can Phlegmasia Alba and Cerulea Dolens (Milk Leg) be?
Doctors may consider a number of different conditions when diagnosing phlegmasia alba and cerulea dolens, which are serious vein diseases. These conditions might include:
- Arterial embolism (when a blood clot gets stuck in the arteries)
- Deep vein thrombosis (a blood clot in a deep vein, usually the leg)
- Cellulitis (a skin infection)
- Lymphedema (swelling normally on the arms or legs)
- Venous valvular insufficiency (vein issues that affect blood flow)
- Superficial thrombophlebitis (inflammation of a vein just under the skin)
What to expect with Phlegmasia Alba and Cerulea Dolens (Milk Leg)
The general outlook for patients is unfortunately not great and can worsen as symptoms advance. In fact, the overall death rate lies between 20% to 40%, especially in cases where gangrene, a type of tissue death caused by lack of blood flow, has formed.
Even with immediate treatment for those who develop a serious condition called acute DVT (Deep Vein Thrombosis, where blood clots form in the deep veins of your body) or phlegmasia (a severe form of deep vein thrombosis), many patients unfortunately develop complications. These include venous valvular insufficiency, a condition where the veins’ valves don’t function properly, and post-thrombotic syndrome, a long-term complication causing pain, swelling, and skin changes in the affected part of the body.
Even after treatment, 20% of patients after 5 years and 44% after 10 years have reported that the valves in their veins are not working as they should be.
Possible Complications When Diagnosed with Phlegmasia Alba and Cerulea Dolens (Milk Leg)
The possible complications associated with phlegmasia alba and cerulea dolens might include:
- Formation of venous gangrene
- Potential limb loss
- Formation of a clot in the lung, also known as a Pulmonary embolus
- Compartment syndrome, which is an increased pressure in a muscle compartment that can affect blood flow
- Post-thrombotic syndrome, which are long-term complications of deep vein thrombosis
- Death, in severe cases
Preventing Phlegmasia Alba and Cerulea Dolens (Milk Leg)
Knowing the risk factors for developing Deep Vein Thrombosis (DVT) – a condition where a blood clot forms in a deep vein, usually in the leg – is very important. This helps in early detection and stop the symptoms from getting worse. Risk factors include having a past record of DVT or Pulmonary Embolism (PE), which is a blood clot in the lungs. Having a family history of these conditions, or blood disorders making clots more likely also increases the risk.
Other risk factors are venous stasis or insufficiency (poor blood flow or pooling of blood in the veins), May Thurner syndrome (a condition where a vein in the left leg is compressed by an artery), recent major surgery or physical trauma, pregnancy, being overweight, hormonal replacement therapy or birth control pills, not being able to move for a long time (like on long plane rides), inflammatory bowel disease, heart failure, or having a central venous catheter (a tube used to deliver medicine directly to the larger veins).
If people have any of these risk factors and notice pain, swelling of a limb, skin changes or discoloration, or loss of motor (movement) and/or sensory (feeling) abilities, they should quickly seek medical attention. These symptoms could indicate that they have Deep Vein Thrombosis.
