Treatment Of Phlegmasia Cerulea Dolens Research Articles

Man with leg pain.

A 66-year-old-male with a history of type 2 diabetes and hypertension presented as a transfer for rapid progression of lower extremity pain, swelling, and blue-purple discoloration of the entire limb (Figure 1) with concern for a possible necrotizing infection. His symptoms began earlier in the day and progressed over just a few hours. He had no known thromboembolic risk factors. Initial vital signs were notable only for a heart rate of 115. Point-of-care ultrasound revealed extensive deep venous thrombosis burden confirmed by formal duplex sonography. The patient was admitted on a heparin infusion and underwent EKOSTM catheter directed tissue plasminogen therapy with stent placement. He was admitted for 5 days and successfully discharged on oral anticoagulation back to his baseline level of function. Phlegmasia cerulea dolens Phlegmasia cerulea dolens (PCD) is a rare ischemic complication of massive venous thromboembolism with high rates of morbidity and mortality. Patients present with limb edema, pain, and cyanosis that can quickly develop into compartment syndrome, limb ischemia, and venous gangrene with amputation and death rates as high as 50% and 40%, respectively.1 PCD tends to affect the iliofemoral segment of the lower extremities.2 Non-traumatic cases of PCD are most commonly associated with malignancy.3 The preferred imaging modality is Doppler ultrasound and use of point-of-care ultrasound by physicians can expedite treatment of PCD.4 Management includes limb elevation, intravenous fluids, and either systemic anticoagulation, catheter-directed thrombolysis, thrombectomy, or a combination of these definitive therapies.5

Single-Stage Endovascular Thrombectomy and Stenting as the First-Line Strategy in the Treatment of Phlegmasia Cerulea Dolens Caused by Iliac Vein Lesions

Phlegmasia cerulea dolens (PCD) is a rare and serious complication of deep venous thrombosis and iliac vein lesions (IVLs) are the most common cause of PCD. The purpose of this study was to explore the safety and efficacy of single-stage endovascular thrombus removal and stenting to treat PCD caused by IVLs. Clinical data of 13 patients with PCD secondary to IVL were retrospectively analyzed. They underwent endovascular thrombus removal, including rheolytic thrombectomy, manual aspiration thrombectomy, and simultaneous iliac vein stenting after thrombus removal. The safety and efficacy of single-stage endovascular thrombectomy and stenting in the treatment of PCD were evaluated. The technical success rate was 100% (13/13). Postoperative symptoms were significantly relieved in all patients. There were no perioperative major bleeding complications or other critical adverse events. Two (15.4%) patients had slightly elevated serum creatinine concentration after surgery, which returned to normal before discharge. At the 12-month follow-up, the stent primary patency rate was 81.8% and there were no cases of severe post-thrombotic syndrome. Single-stage endovascular thrombectomy and stenting in PCD due to IVLs was minimally invasive, safe, and effective; it is recommended as a first-line treatment for PCD caused by IVLs.

A CASE OF PHLEGMASIA CERULEA DOLENS AND MAY-THURNER SYNDROME IN A YOUNG MALE WITH TRIPLE-POSITIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Phlegmasia cerulea dolens (PCD) is an uncommon form of deep vein thrombosis (DVT) typically presenting with acute limb swelling, pain, and cyanosis. Diagnosis of PCD should be made promptly as these patients may develop limb-threatening complications such as gangrene and compartment syndrome. We describe a young male presenting with his first episode of provoked venous thromboembolism (VTE) as PCD complicated with May-Thurner syndrome (MTS) and Antiphospholipid antibody syndrome (APS). CASE PRESENTATION: A 37-year-old obese male with type 2 diabetes, hypertension, and hyperlipidemia was directly admitted to the medicine floor with extensive left lower extremity DVT (including ileofemoral vein thrombosis) found on outpatient duplex ultrasonography. At initial hospitalization, he was tachycardic but without respiratory distress or hemodynamic instability. His left lower extremity was tender and edematous with cyanosis. Diagnosis of PCD was made and patient was treated with subcutaneous enoxaparin. He received catheter-directed thrombolysis for PCD with alteplase and heparin infusion. During left leg venogram, he was discovered to have MTS, and subsequently underwent stent placement of left common iliac vein, external iliac vein, and common femoral vein. After stent placement, patient received bridging to warfarin with enoxaparin and was discharged home two days later. Patient’s family history was significant for Systemic Lupus Erythematosus. During hospitalization, his antibody testing revealed positivity for anti-cardiolipin, anti-beta-2 glycoprotein antibodies in addition to positive functional lupus anticoagulant assay. Positivity confirmed with repeat testing of anti-cardiolipin antibodies and lupus anticoagulant assay at 15 weeks after hospitalization - confirming the diagnosis of triple-positive APS. DISCUSSION: Treatment of PCD is anticoagulation therapy with adjunctive catheter-directed thrombolysis. Outcomes comparing additional catheter-directed thrombolysis versus anticoagulation alone have shown that combination treatment reduces the incidence of post-thrombotic syndrome in patients with acute iliofemoral DVT. Recognition of DVT associated with MTS is important as treatment includes stent placement to maintain venous outflow. Recognition of triple-positive APS is an indication for extended anticoagulation as this portends a high risk of recurrent VTE. Patient was discharged with warfarin therapy because the use of direct oral anticoagulation in patients with APS remains controversial. Recent studies showed increased risk of recurrent thrombosis, particularly in patients with APS who are triple-positive. CONCLUSIONS: We reported a case, 37 year old male with phlegmasia cerulea dolens and May-Thurner syndrome resulting from triple-positive antiphospholipid antibody syndrome. Reference #1: Casey ET, Murad MH, Zumaeta-garcia M, et al. Treatment of acute iliofemoral deep vein thrombosis. J Vasc Surg. 2012;55(5):1463-73 Reference #2: Haig Y, Enden T, Grøtta O, et al. Post-thrombotic syndrome after catheter-directed thrombolysis for deep vein thrombosis (CaVenT): 5-year follow-up results of an open-label, randomised controlled trial. Lancet Haematol. 2016;3(2):e64-71 Reference #3: Dufrost V, Risse J, Reshetnyak T, et al. Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis. Autoimmun Rev. 2018;17(10):1011-1021 DISCLOSURES: No relevant relationships by Waqas Adeel, source=Web Response No relevant relationships by Robert Decker, source=Admin input No relevant relationships by Shuisen Li, source=Web Response No relevant relationships by Preysi Patel, source=Web Response No relevant relationships by Chun Siu, source=Web Response

Surgical Thrombectomy and Simultaneous Stenting for Phlegmasia Cerulea Dolens Caused by Iliac Vein Occlusion

Phlegmasia cerulea dolens (PCD) is a severe complication of deep veions thrombosis, and there are several treatment methods. This study aimed to investigate the clinical efficacy of surgical thrombectomy and simultaneous iliac vein stent implantation for the treatment of PCD caused by iliac vein occlusion. This study aimed to investigate the clinical efficacy of surgical thrombectomy and simultaneous iliac vein stent implantation for the treatment of phlegmasia cerulea dolens (PCD) caused by iliac vein occlusion. From February 2014 to December 2016, 5 patients with secondary PCD complicated with iliac vein occlusion were treated in our center. Thrombectomy by venous incision was performed with simultaneous iliac vein balloon dilatation and stents implantation. Efficacy and stents patency were assessed. Iliac vein occlusions were confirmed in all 5 patients by angiography after the thrombectomy. Stents implantation were performed after balloon dilatation. Three stents were implanted in 1 case of iliac vein and inferior vena cava (IVC) occlusion, whereas 1 stent was implanted in each of the other 4 cases. Symptoms were significantly relieved after surgery, with no complications. Patients were followed up for 6-24months, and minor swelling of the affected limb was found in 1 case, with no thrombosis recurrence in all cases and vascular stent patency. Thrombectomy by venous incision and simultaneous iliac vein stent implantation for the treatment of PCD caused by iliac vein occlusion can quickly relieve symptoms, is easily implemented, is associated with fewer complications, and has good midterm efficacy and a high patency rate, making this technique a good treatment method.

Phlegmasia Cerulea Dolens in the Pediatric Population: A Life-threatening Condition

BackgroundPhlegmasia cerulea dolens is a rare complication of deep venous thromboembolism that can have life-threatening consequences. It is more commonly reported in the adult population, and rarely in the pediatric population, especially in a healthy child. Case ReportWe report a case of a healthy 7-year-old girl who presented with right leg pain and fever, found to have a thrombus involving her inferior vena cava and right lower extremity. Why Should an Emergency Physician Be Aware of This?This case illustrates the importance of early recognition and a multimodal approach to treatment of phlegmasia cerulea dolens.

Pharmacomechanical Thrombolysis for Phlegmasia Cerulea Dolens

Phlegmasia cerulea dolens (PCD) is limb-threatening. Traditional treatments are very morbid. We examine the efficacy of percutaneous treatment of PCD. Between May 2005 and September 2008, we treated 21 limbs in 20 patients with lower extremity PCD who were candidates for thrombolysis. Diagnosis was by clinical examination and duplex ultrasound. Catheter access to the deep venous system was obtained through a popliteal vein. Therapy used pulse spray thrombolysis with tissue plasminogen activator (tPA). Infusion catheters and adjunctive percutaneous techniques were used as indicated. Postoperatively, patients were treated with systemic anticoagulation, compression hose, and interval follow-up. Limbs were graded according to the CEAP classification. Twenty patients (13 male) were treated with a mean age of 55.8 years. Nine patients had hypercoagulable states, four May Thurner syndrome, three a history of cancer, one postcolon resection, one acute myocardial infarction, and one postfemoral vein puncture. All patients had resolution of PCD without the need for open surgery. The initial tPA dose was 19.5 mg with pulse spray thrombolysis. Infusion catheters were required in 18 patients and used for 16.1 hours (range, 8 to 36 hours) until complete thrombolysis. Venous angioplasty was necessary in 14 patients with nine of these requiring venous stents. One patient required above-knee amputation despite successful treatment of her PCD. Mean follow-up was 10.7 months (range, 1 to 39 months). All patients demonstrated no or minimal residual thrombus and intact valvular function and a mean clinical CEAP score of 2.4. Percutaneous treatment of PCD produced excellent results with minimal morbidity.

80-Year-Old Man With Massive Leg Swelling

80-Year-Old Man With Massive Leg Swelling

Endovascular Treatment of Phlegmasia Cerulea Dolens with Impending Venous Gangrene: Manual Aspiration Thrombectomy as the First-Line Thrombus Removal Method

Our purpose was to report the outcome of endovascular treatment with manual aspiration thrombectomy as the first-line thromboablative method for phlegmasia cerulea dolens. Between October 2006 and May 2010, seven consecutive patients (5 women, 2 men; age range, 31-80 years) with the diagnosis of phlegmasia cerulea dolens secondary to acute iliofemoral deep venous thrombosis had endovascular treatment with manual aspiration thrombectomy. Catheter-directed thrombolysis and stent placement were used as adjunctive procedures. Phlegmasia was left-sided in five and right-sided in two patients. All patients had associated great saphenous vein thrombosis in addition to iliofemoral deep vein thrombosis (DVT). Aspiration thrombectomy completely removed the thrombus from the popliteal vein to the inferior vena cava (IVC) in all cases. Three patients with May-Thurner syndrome had stent placement in the left common iliac vein. Two patients had early recurrences. Repeated aspiration thrombectomy was unsuccessful in one patient. There were no complications related to the procedure. One patient who had been successfully treated died of sepsis and another patient who had unsuccessful repeated interventions had below-the-knee amputation. Overall, the clinical success and survival rates of patients in this study were 86%. On follow-up, three patients with successful treatment were asymptomatic with no deep venous insufficiency. One of these patients died during the 4-month follow-up period. Two patients had mild ankle swelling with deep venous insufficiency. Manual aspiration thrombectomy with adjunctive use of catheter-directed thrombolysis and stent placement is an effective endovascular treatment method with high clinical success and survival rates for phlegmasia cerulean dolens.

Quality Improvement Guidelines for the Treatment of Lower Extremity Deep Vein Thrombosis with Use of Endovascular Thrombus Removal

Suresh Vedantham, MD, Akhilesh K. Sista, MD, Seth J. Klein, MD, Lina Nayak, MD, Mahmood K. Razavi, MD, Sanjeeva P. Kalva, MD, Wael E. Saad, MD, Sean R. Dariushnia, MD, Drew M. Caplin, MD, Christine P. Chao, MD, Suvranu Ganguli, MD, T. Gregory Walker, MD, and Boris Nikolic, MD, MBA, for the Society of Interventional Radiology and Cardiovascular and Interventional Radiological Society of Europe Standards of Practice Committees

Successful Treatment of Phlegmasia Cerulea Dolens with Combination Surgical and Catheter-Assisted Thrombectomy

Phlegmasia cerulea dolens is a rare condition caused by complete venous occlusion that leads to impaired arterial flow. Prompt diagnosis and treatment initiation are paramount in preventing progression to gangrene, the need for amputation, and possibly the death of the patient. Thrombolysis or surgical thrombectomy, for advanced disease and cases in which anticoagulation is contraindicated, represent the current standard treatment. Here we report the case of a 47-year-old male who sustained severe trauma during a car accident. After having a prophylactic retrievable inferior vena cava filter placed, he developed caval thrombosis with progression to phlegmasia cerulea dolens. He was successfully treated with a combination of surgical thrombectomy of the lower extremity veins and catheter-assisted thrombectomy of the iliac veins and inferior vena cava, in the setting in which an initial attempt at balloon thrombectomy of the iliac veins and inferior vena cava failed. He was discharged from the hospital in stable condition and on follow-up visits had full resolution of symptoms. Ultrasound evaluation showed no residual venous clot. The presented case illustrates how the use of catheter-assisted thrombectomy alleviated the need for a laparotomy and direct caval evacuation of the thrombus and provided a timely and effective treatment of phlegmasia cerulea dolens with impending gangrene.

Phlegmasia Cerulea Dolens in a Patient with Heparin-Induced Thrombocytopenia

Objective: To report a case of the venous obstructive condition known as phlegmasia cerulea dolens (PCD) in the presence of heparin-induced thrombocytopenia (HIT). Case Summary: A 50-year-old white female presented to the emergency department with a 2-day history of a bluish discoloration of her toes and hands accompanied by chest pain and shortness of breath. The evident edema, tenderness on palpation, and cyanosis of the extremities were suggestive of PCD. She had been hospitalized approximately one month previously due to a fibular fracture and again within the past 2 weeks for intractable abdominal pain and nausea. During her current hospital stay, she was diagnosed with multiple venous thromboembolisms (VTEs); at the time of admission, an unfractionated heparin (UFH) drip was initiated to treat her VTEs. Due to a decreased platelet count on admission, a platelet factor 4 (PF4) antibody assay was performed and found to be positive. After discontinuation of UFH, her platelet count slowly returned to normal range. Discussion: The pathogenesis of HIT is due to formation of antibodies against the complex of heparin and PF4. HIT is characterized by a reduction in the platelet count approximately 4–14 days after the initiation of heparin therapy plus a paradoxical prothrombotic state. The typical diagnostic clues are a drop in platelet count of 50% from baseline with the initiation of heparin and a positive assay for heparin-PF4-immunoglobulin G. This condition may result in PCD, which presents as the triad of pain, edema, and cyanosis. This condition often results in venous or arterial thrombus formation. The treatment for PCD includes immediate discontinuation of heparin products and anticoagulation with a direct thrombin inhibitor. Conclusions: Thromboembolic complications such as PCD are often observed as a presenting feature of HIT. To avoid these potentially limb- and life-threatening complications, clinicians must be vigilant in their monitoring of platelets and clinical signs and symptoms of HIT while patients are on heparin therapy.

Manual Aspiration Thrombectomy with Stent Placement: Rapid and Effective Treatment for Phlegmasia Cerulea Dolens with Impending Venous Gangrene

Phlegmasia cerulea dolens is an uncommon but potentially life-threatening complication of acute deep vein thrombosis. It is an emergency and delay in treatment may cause death or loss of the patient's limb. Surgical thrombectomy is the recommended treatment in venous gangrene. Catheter-directed intrathrombus thrombolysis has been reported as successful, but it may require a lengthy infusion. Manual aspiration thrombectomy may clear the entire thrombus with no need for thrombolytic administration and provide rapid and effective treatment for patients with phlegmasia cerulea dolens with impending venous gangrene.

Successful catheter-directed venous thrombolysis in phlegmasia cerulea dolens

Background Phlegmasia cerulea dolens is an extremely rare condition caused by complete venous occlusion and often results in tissue necrosis, limb amputation, or death. Treatment options include systemic anticoagulation, systemic thrombolytic therapy, fasciotomy, or surgical thrombectomy. Rare case reports have described the use of catheter-directed thrombolysis in the treatment of this condition. Prompt diagnosis and treatment initiation are important to prevent gangrene, amputation, and ultimately death. Case We report two unusual cases of phlegmasia cerulea dolens that presented in patients with aggressive gynecologic malignancies and who were successfully treated with catheter-directed thrombolytic therapy. Conclusion To maximize the opportunity for limb salvage, catheter-directed venous thrombolytic therapy should be considered in the treatment of phlegmasia cerulea dolens that presents in the gynecologic oncology patient.

Catheter-Directed Thrombolysis in the Treatment of Phlegmasia Cerulea Dolens

Phlegmasia cerulea dolens is a potentially devastating complication of extensive deep venous thrombosis for which there is currently no consensus for treatment. Heparin anticoagulation, surgical thrombectomy, thrombolytic therapy, fasciotomy, and amputation have each been advocated. We present two cases of phlegmasia cerulea dolens successfully treated with catheter-directed venous thrombolytic therapy.

Iliofemoral Venous Thrombosis Caused by Compression of an Internal Iliac Artery Aneurysm: A Minimally Invasive Treatment

To report the success of a minimally invasive treatment for phlegmasia cerulea dolens without gangrene caused by compression from an internal iliac artery aneurysm. An 81-year-old male with a 1-month history of paralysis owing to a hemorrhagic stroke presented with massive edema and skin mottling of the right lower extremity. Imaging confirmed right iliofemoral deep vein thrombosis caused by compression from a 4-cm internal iliac artery aneurysm. With thrombolysis ruled out, a minimally invasive treatment plan was undertaken, featuring percutaneous coil embolization of the aneurysm and surgical venous thrombectomy with proximal arteriovenous fistula creation and iliac vein stent placement. Failure of the coils to embolize the iliac aneurysm prompted the use of an endovascular graft to exclude the aneurysm. The patient's symptoms subsided, and he has a patent right iliofemoral venous system and internal iliac artery at his latest (16-month) follow-up. This case demonstrates that minimally invasive endovascular and open techniques can be combined to achieve an optimum outcome in patients at high risk for standard surgical approaches.

Phlegmasia Cerulea Dolens: The Role of Non-Operative Therapy

Thrombectomy and thrombolysis are often advocated in the treatment of phlegmasia cerulea dolens, but frequently result in incomplete clot removal, recurrence of thrombosis, local and systemic hemorrhagic complications and chronic venous stasis; this state is associated with a rate of major amputation and death of up to 50%. Non-operative therapy includes elevation, hydration and heparinization and excludes all methods aimed at surgical removal or chemical lysis of the thrombus. In 1982 it was decided to use non-operative therapy as the first line of treatment for phlegmasia cerulea dolens. In the last 9 years seven extremities in six patients with this condition have been treated. One patient had advanced gangrene on presentation and one underwent emergency thrombectomy. Five extremities (in five patients) were treated with non-operative therapy. Ischemia was rapidly corrected in all five patients. Edema resolved completely after 3-4 days in four patients. There were no complications attributable to the therapy. Two of six (33%) patients died from terminal disease. Non-operative therapy appears to be effective in preventing limb loss and avoiding the risks of thrombectomy and thrombolysis in critically ill patients.

Advances in the treatment of phlegmasia cerulea dolens

Phlegmasia cerulea dolens (PCD) is an uncommon, severe form of lower extremity deep venous thrombosis characterized by extremity swelling, cyanosis, and pain. Progression of the thrombotic process may result in extremity gangrene, amputation, and death. The relative value of specific therapeutic regimens in the treatment of this disease remains uncertain. Twelve patients, 9 females and 3 males, with PCD were treated during a 10-year period. Eighteen lower extremities were involved. Pre-existing conditions included malignancy (eight), postoperative state (four), diabetes (three), previous deep venous thrombosis (three), and hypercoagulation (two). Venous gangrene was present in four patients. All patients were treated initially with bedrest, fluid resuscitation, extremity elevation, and systemic high-dose heparin therapy. Five patients had complete resolution with this regimen alone. One patient required cessation of heparin therapy due to heparin-induced thrombocytopenia and developed gangrenous toes. Two patients whose condition failed to respond to heparin therapy underwent catheter-based delivery of urokinase with marked clinical improvement. Four patients, two with venous gangrene, died, three of whom had disseminated malignant disease. A significant percentage of patients with PCD will respond to extremity elevation, fluid resuscitation, and aggressive systemic anticoagulation therapy. Thrombolytic therapy selectively administered is beneficial in patients whose disease fails to respond promptly. Venous thrombectomy should be reserved for patients with contraindications to thrombolysis.

Thrombectomy and Isolated Limb Perfusion with Urokinase in the Treatment of Phlegmasia Cerulea Dolens

Phlegmasia cerulea dolens is a rare form of deep vein thrombosis. A patient with recurrent episodes of such thrombosis caused by protein C deficiency who developed phlegmasia cerulea dolens is reported. Limb perfusion with urokinase successfully restored venous outflow after unsuccessful attempts at thrombectomy.

Early and Late Results in the Surgical Treatment of Phlegmasia Cerulea Dolens

A series of 408 patients with acute thrombosis in the iliofemoral area was operated on over the last fourteen years. The operative indication and prognosis depend on localizing the origin and duration of the thrombus. We differentiate among three type of thrombosis: iliofemoral, sapheno-femoro-iliac, and femo roiliac. The duration of the thrombi varied from three to thirty days. In the first and second type, there is a great danger of pulmonary embolism. In such cases, operative treatment is indicated. In the third type of thrombosis, the confluent point is ascendingly obliterated. There is little danger of pulmonary embolism, for the peripheral pressure is not raised. Intraoperative prophylaxis for embo lism by means of a balloon catheter placed contralaterally is not necessary. Of this group, 284 patients were followed up over a period of one to fourteen years. Good results were seen following thrombectomy of type I and II throm bosis. The third type of thrombosis showed worse results. In type I and II thrombosis, fibrinolysis can easily cause pulmonary embo lism, for the peripheral venous pressure is high, but in thrombosis of the third type, the danger of pulmonary embolism is relatively small. The results of fi brinolysis in this ascending type are better than the results of surgery.

Intramuscular pressure in the lower leg in deep vein thrombosis and phlegmasia cerulae dolens.

The influence of deep vein thrombosis on intramuscular pressure was evaluated in 22 patients by means of the wick technique. Intramuscular pressure was measured in the anterior tibial and the deep posterior compartments in both legs before and during treatment. The intramuscular pressure was significantly (p less than 0.001) higher in the thrombosed leg than in the contralateral leg (0-16 mmHg). The increase in intramuscular pressure was related to the extension of the thrombus. Iliofemoral thrombosis caused a significantly (p less than 0.001) higher pressure (17-28 mmHg) than calf thrombosis (16-23 mmHg). A compartment syndrome was found to be a part of the entity phlegmasia cerulea dolens (rest pressure 47-56 mmHg). In the treatment of phlegmasia cerulea dolens, fasciotomy is suggested additional to other therapeutic procedures.