Intervention is usually required in order to prevent gangrene. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene. All three manifestations result from acute massive venous thrombosis and. Phlegmasia cerulea dolens is a severe form of venous thrombosis presenting as an edematous, painful, purplishblue lower extremity. Treatment options for phlegmasia cerulea dolens journal. Phlegmasia cerulea dolens pcd is a complication of acute deep venous thrombosis dvt characterized by pain, swelling, and skin discolouration. Abouzamzam jr, md, loma linda, calif phlegmasia cerulea dolens is a rare but serious complication of deep venous thrombosis, which can lead to arterial compromise, tissue ischemia, and gangrene. Phlegmasia cerulea dolens in a teenage boy found to have. Phlegmasia cerulea dolens pcd represents the most severe acute consequence of dvt. Full text is available as a scanned copy of the original print version.
A 72 yearold woman with recently diagnosed squamous cell cancer of the neck. Phlegmasia cerulea dolens is a medical emergency requiring escalation of therapy beyond simple anticoagulation to prevent sequelae such as postthrombotic limb. Phlegmasia cerulea dolens pcd is a rare venous disorder that continues to be a major therapeutic challenge. Phlegmasia cerulea dolens with compartment syndrome nejm. We present a case of upper extremity pcd progressing to venous gangrene in a patient with acute respiratory distress syndrome ards and intraventricular hemorrhage ivh. We presenta rare case of a phlegmasia cerulea due to an aortocaval fistula secondary acf to an aaa. Phlegmasia cerulea dolens pcd is an uncommon but potentially lifethreatening complication of acute dvt characterized by marked swelling of the extremities with pain and cyanosis, which in turn may lead to arterial ischemia and ultimately cause gangrene with high amputation and mortality rates. It compounds with pulmonary embolism pe and deep vein thrombosis dvt. A multimodal therapeutic approach to phlegmasia cerulea. Doppler ultrasonography revealed a left femoropopliteal deepvein thrombosis. Guidelines for treatment are still not clearly documented.
Pdf venous thromboembolism vte is a major healthcare problem that results in significant mortality, morbidity, and expenditure of. Phlegmasia cerulea dolens is a rare syndrome caused by diffuse venous thrombosis that is characterized by sudden an 83yearold man with a recent history of thyroid carcinoma presented with sudden pain and swelling of the left leg with bluish discoloration and livedo reticularis throughout. Phlegmasia alba dolens pad describes the patient with swollen and white leg because of early compromise of arterial flow secondary to. P hlegmasia cerulea dolens pcd, a lifethreatening complication of deep venous thrombosis dvt, is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. Phlegmasia cerulea dolens pcds is a rare and serious complication of massive deep venous thrombosis of the lower extremities, which has a high mortality rate. Acousticassisted catheterdirected thrombolytic therapy. Malignant disease was the most common underlying condition seven patients. Prompt diagnosis along with pharmacological and endovascular therapy is the key to avoiding complications such as gangrene 4060 %, amputation 2050 %, and death 2540 %. Links to pubmed are also available for selected references. It also shows how rare pathologies can combine to create a life and limbthreatening condition.
A total occlusion of the venous outflow is called phlegmasia cerulea dolens pcd. Pdf images in cardiovascular medicine phlegmasia cerulea. Phlegmasia cerulea dolens a limb threatening problem. Phlegmasia cerulea dolens and phlegmasia alba dolens can present as compartment syndrome. Catheterdirected thrombolysis is an effective treatment modality and has been shown to reduce the occurrence of postthrombotic limb. Prompt fasciotomies were performed, but the underlying massive venous thrombosis was not addressed surgically. Pdf phlegmasia cerulea dolens with compartment syndrome.
It can cause arterial insufficiency, venous gangrene, systemic shock. Pdf phlegmasia cerulea dolens in a long distance driver. This often causes compartment syndrome with a subsequent decrease in arterial blood flow in the affected extremity or even venous gangrene. Phlegmasia cerulea dolens phlegmasia erulea dolens pd erulea means lue referred to as painful lue leg rare condition that occurs in phlegmasia alba dolens. A massive backlog of venous blood results in a substantial increase in tissue pressure. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens with compartment syndrome. Phlegmasia cerulea dolens in a patient with breast cancer. The patient is started on anticoagulation, and the involved extremity is elevated. Phlegmasia cerulea dolens with compartment syndrome.
Its rapid recognition as venous thrombosis rather than arterial insufficiency is important in avoiding gangrene and other life threatening complications. However, there are major differences in both the short and longterm outcomes for iliofemoral vein and inferior vena caval thrombosis, when compared with the much more serious variant of venous thrombosis, phlegmasia cerulea dolens pcd. Phlegmasia cerulea dolens, a rare complication of deep. Phlegmasia dolens is a rare form of massive venous thrombosis of the lower extremities that is associated with a high degree of morbidity, including venous gangrene, compartment type syndrome, and arterial compromise. Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of. Anticoagulation with heparin was initiated, but there was progressive swelling and cyanosis of the leg panels a and b. Phlegmasia cerulea dolens pcd is a rare disease entity involving complete thrombotic occlusion of venous outflow in an extremity, and is considered a vascular emergency. Trends in management of phlegmasia te d cerulea dolens. Phlegmasia cerulea dolens is a rare form of massive proximal e. Phlegmasia cerulea dolens pcd is more advanced and considered a precursor of frank venous gangrene. Phlegmasia cerulea dolens pcd is an uncommon but potentially life threatening. Phlegmasia cerulea dolens pcd is a fulminant condition of acute massive venous thrombosis that may result in major amputation or death unless treated in an. Get a printable copy pdf file of the complete article 264k, or click on a page image below to browse page by page. Phlegmasia cerulea dolens refers to congestion and cyanosis of a limb due to massive venous thrombosis.
Phlegmasia alba dolens an overview sciencedirect topics. Pdf phlegmasia cerulea dolens a rare, lifethreatening. No clear guidelines exist for the management of phlegmasia cerulea dolens. Common risk factors for pcd include malignancy and hypercoagulable states, and prompt. The development of a standard treatment for phlegmasia cerulea dolens is ongoing. Phlegmasia cerulea dolens pcd is a fulminant condition of acute massive venous thrombosis that may result in major amputation or death unless treated in an early phase. A multimodal therapeutic approach to phlegmasia cerulea dolens in a pediatric patient isabella kuo, md, ajason smith, md,b and ahmed m. Treatment of phlegmasia alba dolens pad or phlegmasia cerulea dolens pcd should be initiated as soon as the diagnosis is suspected. An 81yearold man presented to the emergency department with acute pain and swelling of the. The patient was referred for prophylactic placement of an inferior vena cava filter. He declared no past medical history exceptactive smoking. Phlegmasia cerulea dolens american journal of medicine. Shackford md, facs, in abernathys surgical secrets seventh edition, 2018.
Phlegmasia cerulea dolens pcd is a rare condition of massive proximal venous thrombosis obstructing superficial and deep venous drainage in the lower extremities. Venous obstruction can lead to compartment syndrome, venous gangrene, and death in 25%40% of cases 1. A rare case of upper extremity phlegmasia cerulea dolens. Trends in management of phlegmasia cerulea dolens khamin. An 85yearold woman with newly diagnosed metastatic nonsmallcell lung cancer was admitted for pain control. Pcd carries a published mortality rate of 25% with a 12% to 25% risk of major amputation in survivors. These two entities occur following iliofemoral venous thrombosis, 75% of which occur on the left side presumably because of compression of the left common iliac vein by the overlying right common iliac artery maythurner. Phlegmasia cerulea dolens, first recognised 400 years ago by fabricius hildanus, is a rare and often devastating complication of deep vein thrombosis with a reported mortality of 25%. Case presentation we report the case of a 58yearold man who consulted at the ed for a sudden on set of pain in the right leg. Phlegmasia cerulea dolens with compartment syndrome ncbi. Most cases described encompass the lower extremities. Case report phlegmasia cerulea dolens in a patient with breast cancer and inferior vena cava hypoplasia a. Phlegmasia cerulea dolens pcd is a rare form of massive venous thrombosis of the lower extremities associated with a high degree of morbidity including venous gangrene, compartment syndrome, and arterial compromise.
It is characterized by the sudden onset of severe leg pain, massive edema, cyanosis. To make the correct diagnosis, 4 cardinal signs are necessary. Phlegmasia cerulea dolens is a rare syndrome caused by diffuse venous thrombosis that is characterized by sudden pain, swelling, purple ecchymosis, and arterial ischemia with loss of distal pulses. Phlegmasia cerulea dolens pcd is near or total occlusion of the venous system causing severe congestion and a characteristic painful, blue and edematous limb. Pcd is the term used to describe lowerextremity dvt that causes limb ischemia, and is characterized by severe swelling and blue discoloration of the extremity. In phlegmasia alba dolens, the thrombosis involves only major deep venous channels of the extremity, therefore sparing collateral veins.
1298 1479 460 1269 1248 429 413 49 947 1472 43 878 843 1282 1058 748 87 641 903 1142 412 218 1493 442 87 342 1244 92 744 497 822 810 388 89 442 1267 1270 573 1287 1493 1211 1174 612 1213 915 331 482