Phlegmasia Cerulea Dolens: A Complication of Filter Use in the Inferior Vena Cava, Successfully Treated With Ultrasound-Assisted, Catheter-Directed Thrombolysis

Abstract

Phlegmasia cerulea dolens is a severe form of deep vein thrombosis characterized by swelling, pain, and bluish discoloration. Treatment delay may cause venous gangrene, tissue ischemia, limb loss or death. Here, we present a case of inferior vena cava (IVC) filter thrombosis presenting with phlegmasia cerulea dolens and treated by ultrasound-assisted, catheter-directed thrombolysis.

Introduction 

For deep venous thromboembolism, the primary treatment and prophylaxis is anticoagulation. The only widely accepted and validated indications for vena cava filter placement in patients with thromboembolism are an absolute contraindication to therapeutic anticoagulation, complication to anticoagulation, and failure of anticoagulation when there is acute proximal venous thrombosis.¹ It is important to be judicious in the use of IVC filters considering the associated risks. Although IVC filters can significantly decrease morbidity associated with pulmonary embolism, they are associated with an increased risk of recurrent filter erosion/migration or embolization, and chronic thrombosis/recurrent thromboembolism and phlegmasia cerulea dolens (PCD).^2,3 PCD is a rare manifestation of deep venous thrombosis that results from total or near-total venous occlusion of a limb and as a result, threatening amputation, shock, or death.^4,5 We present a case of PCD in a patient who had IVC filter thrombosis, with the clot extending into the renal veins. The patient was successfully treated with ultrasound-assisted, catheter-directed thrombolysis. 

Case

A 78-year-old male presented to our facility with a previous history of right lower-extremity deep venous thrombosis and pulmonary embolism 10 years prior. At that time, the patient had a negative workup for a hypercoagulable condition and was treated with coumadin for 6 months. In 2010, the patient had another episode in his right lower extremity while travelling. At that time, to prevent pulmonary embolism, the patient had a VenaTech LP permanent vena cava filter placement (B. Braun Interventional Systems, Inc.) and was again started on anticoagulation (Figure 1). 

This patient presented to our facility with an acute onset of lower abdominal pain and severe pain, swelling, cyanosis, and edema of bilateral lower extremities consistent with phlegmasia cerulea dolens. The patient had returned from a 10-hour flight from Alaska 1 day earlier and had not been on anticoagulation for several months. The initial venous ultrasound showed extensive deep venous thrombosis in bilateral lower extremities.

The patient was taken to the cardiac catheterization laboratory. Two accesses were obtained in the bilateral posterior tibial veins using 5 French (Fr) Terumo Radial Glidesheath Slender Introducer Sheaths. The initial venogram showed extensive deep venous thrombosis in bilateral lower extremities (Figure 2;  Videos 1-2). The Glidewire crossed easily into the IVC from the left posterior tibial vein; however, due to extensive scarring of the right below-the-knee veins from the previous occurrences of deep venous thrombosis, it was difficult to cross from the right posterior tibial vein. A third access was obtained in the right common femoral vein. The venogram showed complete occlusion of the IVC filter, with the clot extending into the renal veins (Figure 3;  Videos 3-4).

Two EKOS catheters (EndoWave Infusion Catheter System, EKOS Corporation) were deployed through the left posterior tibial vein and the right common femoral vein, respectively (Figure 4; Video 5). Ultrasound-assisted, catheter-directed thrombolysis was performed, using tPA (tissue plasminogen activator) for 16 hours. The final venogram showed complete resolution of the IVC clot and complete resolution of the symptoms (Figure 5;  Videos 6-7). The patient was discharged home on lifelong anticoagulation.

Discussion 

Phlegmasia cerulea dolens is an uncommon manifestation of deep venous thrombosis that results from total or near-total occlusion of venous outflow from the limb.^4-11 The venous occlusion causes increased pressure in the capillaries and as a result, arterial blood flow to the limbs is compromised. Hence, it presents with acute swelling, severe pain, and cyanosis of the affected limb due to the resultant ischemia. In case of an inability to diagnose and treat this condition at the early phase, it can result in gangrene, shock, and eventual death.⁸ Risk factors that predispose to PCD include IVC filter use, malignancy, hypercoagulability (factor V Leiden mutation, antithrombin deficiency, protein C or S deficiency, or activated protein C resistance), contraceptive use, recent trauma, ulcerative colitis, mitral valve stenosis, and venous stasis.¹¹ The left leg is affected more frequently than the right. Therapeutic options include anticoagulation therapy with heparin, systemic thrombolysis, angioplasty, angioplasty with stenting, and surgical venous thrombectomy.^12,13 There has been an increasing use of catheter-directed thrombolysis for proximal iliocaval DVT and PCD treatment.^14-16 Some studies have shown a complete return of arterial pulses and limb rescue after catheter-directed thrombolysis in patients with phlegmasia cerulea dolens.^17,18 While comparing systemic anticoagulation with catheter-directed thrombolysis, catheter-directed thrombolysis was associated with significant reduction in the risk of post-thrombotic syndrome and venous obstruction, lower risk of bleeding, and limited use of thrombolytics.^19,20 

This case is unique in using an ultrasound-accelerated thrombolytic system that combines high frequency, low-intensity ultrasound with simultaneous use of a catheter-directed thrombolytic to accelerate clot dissolution. By using pedal access for catheter insertion, the entire segment of the venous thrombus was exposed to ultrasound energy. This technique ultimately reduced the infusion time and the treatment dosage of the thrombolytic agents, evident after 16 hours of treatment with complete resolution of IVC thrombus on angiogram and a prompt improvement in symptoms. Judicious use of IVC filters, as well as prompt retrieval of IVC filters that are intended to be temporary, substantially reduces the risk of IVC filter-related complications, including thrombosis and PCD.

References 

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¹Louisiana State University, Baton Rouge, Louisiana; ²St. Tammany Parish Hospital, Covington, Louisiana.

Disclosures: The authors report no conflicts of interest regarding the content herein.

The authors can be contacted via Dr. Sohail Khan at sohailkhan.md@gmail.com