Phlegmasia Cerulea Dolens- Causes, Clinical Features, Diagnosis, and Treatment

What Is Phlegmasia Cerulea Dolens?

Phlegmasia cerulea dolens is a rare sequela of acute deep vein thrombosis leading to arterial ischemia (reduced blood flow) to the extremities. It has a high risk of amputation and mortality. It results in painful swelling (edema) in the extremities and cyanosis (bluish skin discoloration). If untreated, it leads to gangrene (tissue death), followed by sepsis leading to multiorgan damage and death.

Phlegmasia cerulea dolens is more common in men, particularly those above the age of 50 years. The lower extremities are more commonly affected than the upper extremities. It usually occurs in a hypercoagulable state, with malignancy being the commonest risk factor.

Both Phlegmasia alba dolens and Phlegmasia cerulea dolens (PCD) are rare complications of deep vein thrombosis. Phlegmasia alba dolens means ‘painful white inflammation.’ It occurs when deep vein thrombosis leads to occlusion of the venous system of extremities, but ischemia does not happen as collateral veins are patent. On the other hand, PCD means ‘painful blue inflammation’ and occurs when deep vein thrombosis leads to occlusion of the venous system of extremities, resulting in ischemia. In PCD, collateral circulation is also affected.

What Are the Risk Factors of Phlegmasia Cerulea Dolens?

The most typical risk factor is malignancy, associated with 20 percent to 30 percent of cases. The risk factors include the following:

• Malignancy.

• Hypercoagulable state.

• Pregnancy.

• Surgery.

• Trauma.

• Inferior vena cava filter placement.

• May-Thurner Syndrome (an anatomically variant condition characterized by left iliac vein compression).

• Hormonal therapy.

• Oral contraceptives.

• Heart failure.

• Inflammatory bowel disease.

• Prolonged immobilization(inability to move).

• Venous insufficiency.

• Familial history of deep vein thrombosis.

• Obesity.

What Are the Causes of Phlegmasia Cerulea Dolens?

Both Phlegmasia alba dolens(PAD) and Phlegmasia cerulea dolens(PCD) are rare sequelae of acute deep venous thrombosis with PAD preceding PCD. In about 10 percent of the cases, the cause is unknown. The etiological factors include hypercoagulable states, and malignancy is associated with 20 percent to 30 percent of cases.

The lower extremities are more affected than the upper, with the lower left limb affected the most. Progression of the symptoms leads to gangrene. Such cases result in amputation in 20 percent to 50 percent and can lead to mortality in 20 percent to 40 percent of the cases. In the third trimester of the pregnancy, a large gravid uterus compresses the iliac vein leading to venous stasis, which leads to PCD. In May-Thurner syndrome, the anatomy is such that the left common iliac vein compresses the right common iliac artery leading to venous thrombosis.

What Are the Clinical Features of Phlegmasia Cerulea Dolens?

The clinical features of PCD include:

• Pain.

• Swelling (edema).

• Cyanosis (bluish skin discoloration) begins from the periphery and later progresses to other parts. It is the pathognomonic feature of PCD.

• Gangrene (in about half the patients).

• Tingling feeling.

• Skin blisters.

What Diagnostic Tests Help Detect Phlegmasia Cerulea Dolens?

The Tests That Help Confirm the Diagnosis Are:

• Complete blood count.

• INR (International Normalized Ratio) analysis.

• Analysis of partial thromboplastin time.

• Renal function test.

• Contrast Venography(gold standard test for PCD).

• Venous Duplex Ultrasonography.

• Magnetic Resonance Venography (MRV) (non-invasive imaging technique used to determine vein health).

• Computed Tomography Venogram (CTV).

Suggestive Ultrasound Features Include:

• Increased vein diameter.

• Absence of spontaneous flow through the vessels.

• Increased echogenicity within the vessel lumen.

• Lack of compressibility.

CT is less time-consuming when compared to MRV. But, CT poses a risk of radiation and nephrotoxicity.

What Are the Treatment Options for Phlegmasia Cerulea Dolens?

The management of PCD involves supportive treatment and definitive management.

The Support Measures Are Performed Immediately and Involve the Following:

• Elevation of extremities to an angle greater than 60 percent above heart level increases venous return and reduces venous stasis.

• Elevating the extremities reduces swelling and arterial compression, thus preventing circulatory collapse and shock.

Definite Management Measures Include the Following:

• Most patients respond to anticoagulant therapy, fluid resuscitation, and elevation.

• Intravenous heparin is given as a bolus dose (10 to 15 units/kg) and continued till managed.

• In advanced cases of PCD or refractory venous thrombosis, catheter-directed thrombolysis (CDT) or thrombectomy is done.

• Surgical thrombectomy poses risks like trauma to the vessel, rupture, intimal hyperplasia, low clinical durability, reduced access in case of smaller venues, etc.

• CDT has less risk of vessel trauma and more access in the case of smaller venules.

• These fibrinolytic treatment options possess risks of hemorrhagic complications.

Absolute Contraindications to Lysis Therapy Include the Following:

1. Active bleeding, excluding menses.

2. Coagulopathies.

3. Patients who have undergone neurologic surgery recently.

4. Intracranial malignant lesion surgery.

5. Patients who had prior intracranial hemorrhage.

6. Facial trauma.

Relative Contraindications for Lysis Therapy Include the Following:

1. Pregnancy.

2. Severe liver disease.

3. Active peptic ulcer.

4. Uncontrolled severe hypertension.

5. Patients who had surgery in the last 10 days.

6. Recent trauma.

7. Severe kidney disease.

8. Gastrointestinal bleeding.

In percutaneous mechanical thrombectomy (PMT), a mechanical thrombectomy catheter is used to aspirate the thrombus.

Apart from bleeding complications PMT and CDT possess a risk of pulmonary embolism. Lysis can result in clot fragmentation, and wires may dislodge the thrombus.

What Are the Complications of Phlegmasia Cerulea Dolens?

The Complications of PCD Include the Following:

• Gangrene.

• Amputation of the limb (surgical removal).

• Pulmonary embolism (a condition in which an embolus (blood clot) occludes the arteries in the lung, thus restricting blood flow).

• Death.

• Compartment syndrome (a rare painful condition due to increased pressure build-up in a muscle compartment).

• Post-thrombotic syndrome.

What Is the Differential Diagnosis of Phlegmasia Cerulea Dolens?

The Differential Diagnosis of PCD Includes the Following:

• Cellulitis (a severe bacterial skin infection).

• Deep vein thrombosis.

• Arterial embolism (an embolus (blood clot) gets dislodged from another part of the body, occluding the blood flow in an artery).

• Lymphedema (a chronic condition characterized by increased lymphatic fluid collection in the body resulting in swelling).

• Superficial thrombophlebitis (inflammation of vein near the skin surface).

What Is the Prognosis of Phlegmasia Cerulea Dolens?

The prognosis worsens with the progression of symptoms. The mortality rate is between 20 percent to 40 percent.

Conclusion

PCD is a rare complication of deep vein thrombosis characterized by cyanosis, pain, swelling, and ischemia (reduced blood flow) of extremities. The condition has a high risk of amputation and mortality, so immediate medical attention is required. Management of PCD involves supportive and definitive management measures.