Phlegmasia Cerulea Dolens

What is phlegmasia cerulea dolens?

Phlegmasia cerulea dolens (fleg MAY zee uh suh ROO lee uh DOH lenz) is a severe blockage from deep vein thrombosis or DVT (blood clot) in multiple veins. This usually happens in one or both legs, but can happen in your arms.

When a blockage affects your larger and smaller veins as this condition does, your leg can’t get the blood flow it needs. Because the blockages keep blood from leaving your veins, blood backs up. The swelling this causes increases the back pressure on your arteries as well, keeping them from bringing in oxygen-rich blood. This is like a sponge that can’t absorb any more liquid because no one’s wringing it out.

This is an emergency. You need immediate diagnosis and treatment to save your leg and your life.

How common is phlegmasia cerulea dolens?

Phlegmasia cerulea dolens is so rare, there’s little data on the number of cases. Healthcare providers may only see it once or twice in their careers.

Symptoms of phlegmasia cerulea dolens

The translated name, “painful blue inflammation,” actually describes symptoms.

Phlegmasia cerulea dolens symptoms in your leg or arm include:

• Red, blue or purple tint to your skin (this can spread to your whole leg).
• Severe pain.
• Swelling.
• Blisters on your skin.
• Pricking or tingling feeling.

Because of the anatomy of your circulatory system, this condition is much more likely to happen in your left leg.

What causes phlegmasia cerulea dolens?

A large venous thromboembolism (blood clot), usually a DVT in your leg, causes this complication.

What are the risk factors for phlegmasia cerulea dolens?

Risk factors for phlegmasia cerulea dolens are similar to those for having a blood clot. They include:

• Cancer (this is the most common risk factor).
• Surgery.
• Injury.
• Pregnancy.
• Birth control or hormone therapy you swallow.
• Inflammatory bowel disease (IBD).
• A BMI (body mass index) higher than 30.
• Blood clotting disorders.
• Venous insufficiency.
• May-Thurner syndrome.
• Heart failure.
• Inability to move around (for example, prolonged bed rest).

This condition happens most often to people in their 40s and 50s.

What are the complications of phlegmasia cerulea dolens?

About 50% of people with phlegmasia cerulea dolens get gangrene, or a severe infection. Sometimes, a provider has to remove a limb that has gangrene.

People who have phlegmasia cerulea dolens may also get a pulmonary embolism.

How is phlegmasia cerulea dolens diagnosed?

A healthcare provider will review your medical history and give you a physical exam. They may ask you questions like:

• When did your symptoms start?
• What do your symptoms keep you from doing?
• Have you had treatments before for blood vessel issues?
• Have you or someone in your family had issues with blood clotting too much?

What tests will be done to diagnose phlegmasia cerulea dolens?

When considering how to diagnose phlegmasia cerulea dolens, a provider may need to do tests. These may include:

• Ultrasound.
• Blood tests.
• Computed tomography angiogram.
• Magnetic resonance angiogram.

How do you treat phlegmasia cerulea dolens?

The first thing a provider will do is bring your leg up higher than 60 degrees above your heart level. This helps your blood drain out of your leg veins, improves circulation and reduces swelling. They also may give you fluids, wrap your whole leg in a compression bandage and ask you to walk around.

You’ll need medicine and, most likely, a procedure. If you have blisters or gangrene, your leg is in danger. A provider will remove your blood clot as soon as possible to reverse phlegmasia cerulea dolens before gangrene starts.

The goals of phlegmasia cerulea dolens treatment are to remove your blood clots, open your veins so blood can get through and keep your condition from getting worse.

Specific medicines/procedures used

Phlegmasia cerulea dolens treatment may include:

• Anticoagulant medicines, such as heparin.
• Thrombolysis (using a catheter to deliver medicines that dissolve the clot).
• Thrombectomy (using a minimally invasive or open procedure to remove your clot).
• Angioplasty and/or a stent for people with May-Thurner syndrome.

Complications/side effects of the treatment

Open surgery to remove your blood clot can have complications, such as:

• A high chance of a blood clot happening again.
• Damage to your blood vessel.

Anticoagulants and thrombolysis carry a risk of bleeding. Thrombolysis also has a risk of pulmonary embolism. A provider may recommend an inferior vena cava (IVC) filter to prevent a pulmonary embolism.

How soon after treatment will I feel better?

Some people can feel better as quickly as one hour after they start to receive anticoagulants. It’s more common for people to feel better several hours after starting the medicine. If your condition doesn’t get better in six to 12 hours, a provider may recommend a procedure in addition to the medicine.

An operation to remove a blood clot can get rid of the swelling or abnormal color in your leg. You may be able to see and feel the difference right after surgery.

Can phlegmasia cerulea dolens be prevented?

Yes, you may be able to avoid it by preventing DVT or getting treatment for DVT before it leads to phlegmasia cerulea dolens.

What can I expect if I have phlegmasia cerulea dolens?

Phlegmasia cerulea dolens puts you at risk of losing a leg or even your life. Healthcare providers will work quickly to give you treatment to keep your condition from getting worse. It may feel like they’re rushing to give you anticoagulants or get you to surgery, but that’s because they want to give you the best outcome. Quick treatment is the best way to do that.

You may be in the hospital for a few days, and a provider may do imaging tests again to make sure they’ve gotten rid of your blood clots.

Outlook for phlegmasia cerulea dolens

Thrombolysis has success rates from 85% to 95%.

Untreated phlegmasia cerulea dolens can lead to gangrene. Once you have gangrene, you have a 20% to 50% risk of needing an amputation. With or without gangrene, your risk of the condition becoming fatal is 20% to 40%.

Without proper treatment, phlegmasia cerulea dolens can happen again.

Even with quick treatment, many people who have phlegmasia cerulea dolens will get issues with their veins’ valves and with post-thrombotic syndrome.

How do I take care of myself?

After your initial phlegmasia cerulea dolens treatment, you’ll need to keep taking anticoagulants to keep from getting the condition again. You’ll have to take them for several months or longer, depending on your situation.

When should I see my healthcare provider?

A healthcare provider may need to check your blood regularly to make sure it doesn’t clot too quickly or too slowly. You’ll also need regular visits to make sure you don’t have any complications later.

When should I go to the ER?

Seek medical care right away if you have symptoms of phlegmasia cerulea dolens or if symptoms return after you’ve had it. You should also get medical attention if you get symptoms of another blood clot or symptoms of pulmonary embolism.

What questions should I ask my doctor?

Questions to ask your provider may include:

• How long do I need to take anticoagulants?
• How often do I need follow-up appointments for this?
• What caused my DVT?

What is the difference between phlegmasia alba and cerulea dolens?

Phlegmasia alba dolens is a complication of a blood clot, but it doesn’t stop your blood vessels from supplying blood to your tissues. The “alba” in the name means “white,” which describes how your leg looks. You may have this before it becomes phlegmasia cerulea dolens.

Phlegmasia cerulea dolens, which is worse, affects your circulation more. It keeps your blood vessels from getting blood to your tissues near the clot.

A note from Cleveland Clinic

Time is critical when you have phlegmasia cerulea dolens. Don’t be afraid to advocate for prompt care for yourself or a loved one with this condition. Quick treatment is important to save your leg and possibly your life. It can be overwhelming to be the subject of quick decisions and swift action. Ask questions about anything you don’t understand about your situation.