In the latest Clinical Problem-Solving article, a 55-year-old man with a history of heart failure presented to the emergency department with pain and swelling of his right foot and leg, which had begun 3 days earlier. On the day of presentation, he noted new dusky discoloration of his right toes.
The triad of asymmetric leg swelling, cyanosis, and pain is highly suggestive of phlegmasia cerulea dolens, a rare syndrome seen in cases of massive proximal venous thrombosis that is severe enough to cause arterial insufficiency.
- What are the important management tasks in the acute care of phlegmasia cerulea dolens?
Phlegmasia cerulea dolens, like other causes of acute limb ischemia, is a medical emergency, since prolonged tissue ischemia can jeopardize the viability of the limb. The leg should be elevated immediately. Prompt evaluation and initiation of treatment are essential. Hypovolemic shock can occur in phlegmasia cerulea dolens as a consequence of fluid loss due to “third spacing.” The release of inflammatory mediators can also result in a vasodilatory state. Volume resuscitation is essential.
- What is the difference between phlegmasia alba dolens and phlegmasia cerulea dolens?
Phlegmasia alba dolens (“alba” is from the Latin word for “white”) is characterized by extensive swelling of the entire leg with no tissue ischemia, because some venous drainage through superficial collateral vessels is preserved. By contrast, phlegmasia cerulea dolens (“cerulea” is from the Latin word for “blue”) occurs in the context of complete obstruction of both superficial and deep venous return. The resultant severe venous congestion leads to increased pressure on small arterioles and to tissue ischemia, characterized by a cyanotic or dusky appearance, that may progress to frank gangrene.
Morning Report Questions
Q: What feature associated with arterial insufficiency is not present in phlegmasia cerulea dolens?
A: A clinical diagnosis of phlegmasia cerulea dolens requires recognition of extensive deep venous thrombosis plus signs of arterial insufficiency, including pulselessness, pain, poikilothermy (inability to regulate temperature), and paralysis. In contrast to the pallor characteristic of the acute cessation of blood flow seen with an arterial embolism, purplish-blue cyanosis results from the concomitant venous congestion in phlegmasia cerulea dolens. This discoloration typically begins distally but may extend more proximally, depending on the duration and degree of ischemia. Paresthesias, muscle weakness, or both generally precede paralysis, which is usually a late finding.
Q: What is the most common risk factor for phlegmasia cerulea dolens?
A: Although there are many recognized causes of thrombophilia, cancer is the most common cause of phlegmasia cerulea dolens documented in published case series; obesity, advanced age, and immobilization are other risk factors. Registry data suggest that certain cancers are particularly thrombogenic; these include gastric, esophageal, lung, pancreatic, renal, and ovarian cancers, as well as acute myelogenous leukemia and non-Hodgkin’s lymphoma. The risk of venous thromboembolism in patients with cancer is further potentiated by numerous associated risk factors. For instance, patients with cancer are frequently bedbound, commonly have indwelling venous catheters, often require surgical procedures, and may be treated with drugs that have procoagulant properties. Patients with cancer in whom thrombosis develops have a poorer prognosis than those without thrombosis, which is perhaps related in part to overlapping mechanisms for thrombosis and the propensity for local or metastatic spread.