It is important to recognize the signs and symptoms of deep vein thrombosis (DVT), especially because certain forms of the disease, though rare, can be potentially life-threatening. Phlegmasia cerulea dolens is a severe form of DVT that requires medical care right away.
What Is Phlegmasia Cerulea Dolens?
It’s quite a mouthful, but the name phlegmasia cerulea dolens (PCD) is Greek, meaning “painful blue inflammation.” It is a massive DVT that most often occurs in the upper leg. If it’s untreated, PCD can lead to venous gangrene, which causes massive tissue death.
What Causes Phlegmasia Cerulea Dolens?
PCD stems from a condition called phlegmasia alba dolens. This translates to “painful white inflammation,” which comes from a clot in a deep leg vein that blocks blood flow. As a result, the leg is swollen, white, and painful. This condition is sometimes known as “milk leg,” since it affects women in the third trimester of pregnancy or after they give birth.
If this isn’t caught and treated, it can progress to phlegmasia cerulea dolens. When this happens, the blood clot completely blocks the vein. This cuts off circulation, leads to the buildup of fluid, and worsens swelling.
How Common Is Phlegmasia Cerulea Dolens?
It’s hard to know for sure, since it’s so rare. Deep vein thrombosis itself affects about 2 million Americans each year. Some research suggests that about 20% of people who are treated for severe DVT have phlegmasia. It’s also thought to be slightly more common in men than in women, with a male-to-female ratio of 1.5:1.
Who’s Most at Risk for Phlegmasia Cerulea Dolens?
Risk factors include:
What Are the Symptoms of Phlegmasia Cerulea Dolens?
If you have PCD, you will have severe pain, rapid swelling, and bluish skin around the area below the blocked vein. Symptoms occur 3 to 4 times more often in the left leg compared to the right leg. The symptoms can come on slowly or suddenly. As the swelling and pain worsen, the white blanching of the skin becomes bluish as the blood supply (and oxygen) is cut off from the area. This can cause massive fluid loss and fluid-filled skin blisters.
How Is Phlegmasia Cerulea Dolens Diagnosed?
To diagnose PCD, a doctor will ask you about your symptoms. They will also do a physical exam to check for areas of swelling, tenderness, and changes in skin color. If they think you might have PCD, they will order a series of imaging tests. This includes:
- Duplex ultrasound. This will let the doctor know if you have a deep vein thrombosis, or blood clot, that is triggering these symptoms. This noninvasive test uses sound waves to create a picture of how blood flows through your veins.
- Point-of-care ultrasound (POCUS). You may get this ultrasound if you go to the emergency room. It’s faster than a duplex ultrasound, and most ER doctors can do it. Research suggests that it’s about as accurate as a duplex ultrasound.
- CT venography. This is a special type of X-ray where contrast material (dye) is injected into a large vein in the foot or ankle so that the doctor can see the deep veins in the leg and hip. It’s the most accurate test for diagnosing blood clots, but it’s not always available at all hospitals.
How Is Phlegmasia Cerulea Dolens Treated?
Since PCD is very serious and can lead to life-threatening complications, it needs to be treated right away. This includes:
- Leg elevation. Raise the leg to an angle greater than 60 degrees above the level of your heart. This will help ease swelling and put less stress on the arteries so they are less likely to collapse.
- Anticoagulation. Anticoagulant medicine prevents blood clots. They are called blood thinners, but they don’t actually thin your blood. They reduce its ability to clot. This prevents the clot from becoming larger while it’s reabsorbed by your body. It also reduces the chances that more clots will develop. The most common treatment is a drug called heparin, which is given to you intravenously (through your veins).
- Catheter-directed thrombolysis (CDT). If the PCD is more advanced, it may require a procedure known as CDT. This uses X-ray imaging and a long, thin, hollow tube known as a catheter to guide medications such as heparin to the site of the blood clot to dissolve it.
- Percutaneous thrombectomy. A doctor guides a catheter to the site of the embolism via X-ray, where it is used to either break up the clot or suck it out.
- Open surgical thrombectomy. During this procedure, a surgeon cuts into a blood vessel to remove the clot, repair the blood vessel, and restore blood flow. Sometimes, a balloon is put into the blood vessel to help keep it open. This treatment isn’t used as often as other treatments like CDT or percutaneous thrombectomy because it has higher recurrence and complication rates.
What Are the Complications of Phlegmasia Cerulea Dolens?
If left untreated, PCD may lead to tissue death (venous gangrene) and the need for amputation. Some patients may even go into shock because they’ve lost so much fluid and their body’s inflammatory response has gone into overdrive.
What’s the Prognosis for Phlegmasia Cerulea Dolens?
Even where there are effective therapies, phlegmasia cerulea dolens and venous gangrene remain life- and limb-threatening. In fact, deaths rates range between 20% and 40%, with pulmonary embolism (PE) making up about a third of the cause. Amputation rates of anywhere from 12% to 50% have also been reported among survivors. Even those who keep their leg have long-term damage: 60% to 94% report persistent pain and swelling, although treatments like compression stockings can help.