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. Phlegmasia cerulea dolens, a rare complication of deep. The patient is started on anticoagulation, and the involved extremity is elevated. Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of. It also shows how rare pathologies can combine to create a life and limbthreatening condition. 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. Guidelines for treatment are still not clearly documented. This often causes compartment syndrome with a subsequent decrease in arterial blood flow in the affected extremity or even venous gangrene. Treatment of phlegmasia alba dolens pad or phlegmasia cerulea dolens pcd should be initiated as soon as the diagnosis is suspected.
Phlegmasia cerulea dolens and phlegmasia alba dolens can present as compartment syndrome. No clear guidelines exist for the management of phlegmasia cerulea dolens. Phlegmasia cerulea dolens pcd is a complication of acute deep venous thrombosis dvt characterized by pain, swelling, and skin discolouration. Catheterdirected thrombolysis is an effective treatment modality and has been shown to reduce the occurrence of postthrombotic limb. Treatment options for phlegmasia cerulea dolens journal. In phlegmasia alba dolens, the thrombosis involves only major deep venous channels of the extremity, therefore sparing collateral veins. Phlegmasia cerulea dolens pcd is an uncommon but potentially life threatening. Phlegmasia cerulea dolens with compartment syndrome. 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%. This case report shows how a hybrid approach might be successful. To make the correct diagnosis, 4 cardinal signs are necessary. We presenta rare case of a phlegmasia cerulea due to an aortocaval fistula secondary acf to an aaa. It was first described by hildanus in the 16 th century. Common risk factors for pcd include malignancy and hypercoagulable states, and prompt.
Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant. An 81yearold man presented to the emergency department with acute pain and swelling of the. 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. The development of a standard treatment for phlegmasia cerulea dolens is ongoing. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. Phlegmasia cerulea dolens pcd is more advanced and considered a precursor of frank venous gangrene.
A multimodal therapeutic approach to phlegmasia cerulea dolens in a pediatric patient isabella kuo, md, ajason smith, md,b and ahmed m. A rare case of upper extremity phlegmasia cerulea dolens. Prompt fasciotomies were performed, but the underlying massive venous thrombosis was not addressed surgically. An 85yearold woman with newly diagnosed metastatic nonsmallcell lung cancer was admitted for pain control. A massive backlog of venous blood results in a substantial increase in tissue pressure.
Phlegmasia cerulean dolens is a severe form of deep venous thrombosis characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish discoloration, and even venous gangrene if the condition is untreated. 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. Anticoagulation with heparin was initiated, but there was progressive swelling and cyanosis of the leg panels a and b. 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. He declared no past medical history exceptactive smoking. Full text is available as a scanned copy of the original print version. 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. A 75yearold man, known for nephrotic syndrome currently under investigation, presented to the emergency department with a. Phlegmasia cerulea dolens with compartment syndrome ncbi.
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 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 a fulminant condition of acute massive venous thrombosis that may result in major amputation or death unless treated in an early phase. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.
Intervention is usually required in order to prevent gangrene. A total occlusion of the venous outflow is called phlegmasia cerulea dolens pcd. Phlegmasia cerulea dolens american journal of medicine. Phlegmasia alba dolens an overview sciencedirect topics. A multimodal therapeutic approach to phlegmasia cerulea. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens with compartment syndrome.
It can cause arterial insufficiency, venous gangrene, systemic shock. A 72 yearold woman with recently diagnosed squamous cell cancer of the neck. Case report phlegmasia cerulea dolens in a patient with breast cancer and inferior vena cava hypoplasia a. Phlegmasia cerulea dolens in a patient with breast cancer. Phlegmasia cerulea dolens pcd represents the most severe acute consequence of dvt. Shackford md, facs, in abernathys surgical secrets seventh edition, 2018. Phlegmasia cerulea dolens a limb threatening problem. 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. Phlegmasia cerulea dolens with compartment syndrome nejm. Acousticassisted catheterdirected thrombolytic therapy. Phlegmasia alba dolens pad describes the patient with swollen and white leg because of early compromise of arterial flow secondary to.
Phlegmasia cerulea dolens pcd is a rare condition of massive proximal venous thrombosis obstructing superficial and deep venous drainage in the lower extremities. Pdf venous thromboembolism vte is a major healthcare problem that results in significant mortality, morbidity, and expenditure of. Its rapid recognition as venous thrombosis rather than arterial insufficiency is important in avoiding gangrene and other life threatening complications. Pdf phlegmasia cerulea dolens with compartment syndrome. All three manifestations result from acute massive venous thrombosis and. It compounds with pulmonary embolism pe and deep vein thrombosis dvt. Venous obstruction can lead to compartment syndrome, venous gangrene, and death in 25%40% of cases 1. Trends in management of phlegmasia cerulea dolens khamin.
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 is characterized by the sudden onset of severe leg pain, massive edema, cyanosis. 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. Phlegmasia cerulea dolens phlegmasia erulea dolens pd erulea means lue referred to as painful lue leg rare condition that occurs in phlegmasia alba dolens. Pdf phlegmasia cerulea dolens in a long distance driver. Phlegmasia cerulea dolens in a teenage boy found to have. Phlegmasia cerulea dolens pcd is a rare venous disorder that continues to be a major therapeutic challenge. 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. It is the more severe form of phlegmasia alba dolens pad, and can lead to venous gangrene. Phlegmasia cerulea dolens is a severe form of venous thrombosis presenting as an edematous, painful, purplishblue lower extremity.
Phlegmasia cerulea dolens pcd is near or total occlusion of the venous system causing severe congestion and a characteristic painful, blue and edematous limb. 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 refers to congestion and cyanosis of a limb due to massive venous thrombosis. The patient was referred for prophylactic placement of an inferior vena cava filter. 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.
Links to pubmed are also available for selected references. Doppler ultrasonography revealed a left femoropopliteal deepvein thrombosis. Pcd carries a published mortality rate of 25% with a 12% to 25% risk of major amputation in survivors. Malignant disease was the most common underlying condition seven patients. Pdf phlegmasia cerulea dolens a rare, lifethreatening. 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 is a medical emergency requiring escalation of therapy beyond simple anticoagulation to prevent sequelae such as postthrombotic limb. Get a printable copy pdf file of the complete article 264k, or click on a page image below to browse page by page.
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