Phlegmasia cerulea dolens pcd is a rare form of venous gangrene. Phlegmasia alba dolens also colloquially known as milk leg or white leg is part of a spectrum of diseases related to deep vein thrombosis. Phlegmasia coerulea dolensphlegmasia cerulea dolens. May 25, 2014 phlegmasia cerulea dolens pcd represents the most severe acute consequence of dvt. Phlegmasia cerulea dolens is a medical emergency requiring escalation of therapy beyond simple anticoagulation to prevent sequelae such as postthrombotic limb. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene. There are two treatment options endovascular or surgical. Pronunciation of phlegmasia cerulea dolens with 1 audio pronunciation, 1 meaning, 2 translations and more for phlegmasia cerulea dolens. 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%. Phlegmasia cerulea dolens circulation aha journals. Common risk factors for pcd include malignancy and hypercoagulable states, and prompt. Phlegmasia cerulea dolens pcd is an uncommon but potentially life threatening complication of acute dvt. More than 600000 cases of venous thromboembolism are estimated to occur each year in the united. A 65yearold gentleman was referred with left lower extremity phlegmasia cerulea dolens pcd 1 day following an exploratory laparotomy.
Phlegmasia alba dolens pad and phlegmasia cerulea dolens pcd predominantly affect the lower extremities, with fewer than 5% of cases believed to involve the upper extremities. Aug, 2018 treatment of phlegmasia alba dolens pad or phlegmasia cerulea dolens pcd should be initiated as soon as the diagnosis is suspected. Phlegmasia cerulea dolens could be a result of improper management of acute proximal dvt in the. 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 information mount sinai new york. Dec 31, 2016 phlegmasia cerulea dolens pcd is a medical emergency that can lead to venous gangrene of the lower extremity. Jan 11, 2020 phlegmasia cerulea dolens is a medical emergency requiring escalation of therapy beyond simple anticoagulation to prevent sequelae such as postthrombotic limb. Phlegmasia cerulea dolens with compartment syndrome. Venous obstruction can lead to compartment syndrome, venous gangrene, and death in 25%40% of cases 1. If you have access to a journal via a society or association membership, please browse to your society journal, select an article to view, and follow the instructions in this box. An 85yearold woman with newly diagnosed metastatic nonsmallcell lung cancer was admitted for pain control.
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. In patients delaying initial presentation by less than. Prompt diagnosis along with pharmacological and endovascular therapy is the key to avoiding complications such as gangrene 4060 %, amputation 2050 %, and death 2540 %. 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 is an uncommon, severe form of deep venous thrombosis blood clots in the vein. Department of radiology, university of rochester school of medicine, 601 elmwood avenue, rochester, ny 14642. Postphlebitic edema after phlegmasia alba dolens in a typical situationat ar.
It is characterized by the sudden onset of severe leg pain, massive edema, cyanosis. Phlegmasia cerulea dolens blue, painful leg is an uncommon manifestation of deepvein thrombosis and results from massive thrombosis compromising venous outflow, which causes ischemia. 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. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. Postphlebitic edema and induration after phlegmasia alba dolens a very advanced stage of ulceration. Phlegmasia cerulea dolens with compartment syndrome nejm. Treatment options for phlegmasia cerulea dolens journal. This case report shows how a hybrid approach might be successful. Access to society journal content varies across our titles. Pdf phlegmasia cerulea dolens a rare, lifethreatening. Rapid deterioration and death caused by bilateral phlegmasia. Inicio cirugia espanola english edition phlegmasia cerulea dolens journal information.
The patient had painful swelling and bluishred discoloration of the right leg at the time of presentation. Phlegmasia alba and cerulea dolens clinical presentation. Catheterdirected thrombolysis is an effective treatment modality and has been shown to reduce the occurrence of postthrombotic limb. Phlegmasia cerulea dolens is a severe form of venous thrombosis presenting as an edematous, painful, purplishblue lower extremity. Phlegmasia cerulean dolens is a rare condition, whose prevalence is unknown, and it represents the intermediate state between venous thrombosis and phlegmasia alba dolens. Abstract phlegmasia dolens is a rare form of massive venous thrombosis of the lower extremities that is associated with a high degree of morbidity. Phlegmasia alba dolens and the relation of the lymphatics to. Phlegmasia cerulea dolens is preceded by a condition called phlegmasia alba dolens.
Article information, pdf download for phlegmasia cerulea dolens in. Patients usually present with edema, pain, blanching and it may progress to gangrene and systemic shock in severe cases. Phlegmasia cerulea dolens flegmasia cerulea dolens. Phlegmasia cerulea dolens request pdf researchgate. The swelling of the lower limb and coloring improved gradually, and he was discharged after an uneventful course. Rarely, open surgical venous thrombectomy may be indicated in postoperative patients to relieve the severe swelling in phlegmasia alba dolens or phlegmasia cerulea dolens. A diagnosis of phlegmasia cerulea dolens pcd and pulmonary embolism pe was established. Advances in the treatment of phlegmasia cerulea dolens. Diagnosis of pulmonary embolism by multidetector ct alone or combined with venous ultrasonography of the leg. It is a medical emergency and can rapidly lead to venous gangrene of the extremity. Phlegmasia cerulea dolens pcd, painful blue leg is a rare manifestation of deep vein thrombosis. Advanced testicular cancer presenting with phlegmasia cerulea. On arrival, he underwent a venous duplex that demonstrated thrombosis of his tibial, popliteal, femoral, common femoral, and. Aug, 2018 phlegmasia alba dolens pad and phlegmasia cerulea dolens pcd predominantly affect the lower extremities, with fewer than 5% of cases believed to involve the upper extremities.
Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of. The patient is started on anticoagulation, and the involved extremity is elevated. 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 syndrome. Early fasciotomy is an effective way of limb salvage for these patients.
Low molecular weight heparins have not been effectively studied in the setting of phlegmasia alba or cerulea dolens. Phlegmasia cerulea dolens presenting with acute compartment. Clinically there is sudden severe pain in a part of an extremity which quickly spreads to involve the whole limb. We conclude that although vascular complications are fortunately rare after hip arthroplasty. Phlegmasia alba dolens pdf phlegmasia alba and cerulea dolens. Phlegmasia cerulea dolens pcd is a severe complication of deep veions thrombosis, and there are several treatment methods. It is the more severe form of phlegmasia alba dolens pad, and can lead to venous gangrene. Phlegmasia cerulea dolens and external iliac vein disruption. 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. Phlegmasia cerulea dolens pcd is an uncommon, severe form of lower extremity deep venous thrombosis characterized by extremity swelling, cyanosis, and. Case report surgical thrombectomy for phlegmasia cerulea. Phlegmasia cerulea dolens refers to congestion and cyanosis of a limb due to massive venous thrombosis. The management of phlegmasia alba dolens and mild cases of phlegmasia cerulea dolens includes initiation of heparin infusion, warfarin bridging, fluid resuscitation, and steep limb elevation.
Postoperatively he developed painful diffuse swelling of his left lower extremity with bluish discoloration figure 581. Phlegmasia cerulea dolens cirugia espanola english edition. Trends in management of phlegmasia te d cerulea dolens. Early diagnosis and prompt treatment is crucial for limb salvage. No clear guidelines exist for the management of phlegmasia cerulea dolens. Phlegmasia cerulean dolens is a severe form of deep venous thrombosis. Phlegmasia alba dolens definition of phlegmasia alba dolens. Phlegmasia cerulea dolens in a patient with breast cancer. Phlegmasia alba dolens an overview sciencedirect topics. Deep venous thrombosis dvt affects mainly the veins in the lower leg and the thigh. Despite all of the therapeutic modalities described above, phlegmasia cerulea dolens and venous gangrene still remain lifethreatening and. 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. Pdf download for trends in management of phlegmasia cerulea dolens.
Phlegmasia alba dolens and the relation of the lymphatics. We present a unique case of phlegmasia cerulea dolens and compartment syndrome secondary to external iliac vein disruption after revision total hip arthroplasty. Phlegmasia cerulea dolens in a patient with breast cancer and. Phlegmasia cerulea dolens phlegmasia dolens following influenza. In phlegmasia alba dolens, the thrombosis involves only major deep venous channels of the extremity, therefore sparing collateral veins. He received heparin and inferior vena cava ivc filter placement followed by anticoagulation therapy with warfarin. Phlegmasia cerulea dolens pcd is an uncommon but potentially lifethreatening. A 75yearold man, known for nephrotic syndrome currently under investigation, presented to the. Advanced testicular cancer presenting with phlegmasia. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and. Treatment options for phlegmasia cerulea dolens journal of.
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. This case gives us the chance to focus on three rare pathologies. Phlegmasia cerulea dolens from radiationinduced venous stenosis. Phlegmasia cerulea dolens pcd is a rare venous disorder that continues to be a major therapeutic challenge. Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant. Trends in management of phlegmasia cerulea dolens khamin.
Phlegmasia cerulea dolens pcd is a fulminant condition of acute. 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. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. 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. Phlegmasia cerulea dolens pcd, a lifethreatening complication of deep venous thrombosis dvt, is characterized by massive iliofemo ral thrombus that. Phlegmasia cerulea dolens pcd is an uncommon but potentially lifethreatening complication of acute dvt. We conclude that although vascular complications are fortunately rare after hip arthroplasty, they can have significant. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens with compartment syndrome. Maythurner syndrome as the cause of phlegmasia cerulea dolens. In the endovascular era, catheterdirected thrombolysis is the treatment of choice to achieve venous outflow.
The results of treating germ cell cancer continue to improve. Phlegmasia dolens is a rare form of massive venous thrombosis of the lower extremities that is. Surgical thrombectomy and simultaneous stenting for. Phlegmasia cerulea dolens color atlas and synopsis of. Phlegmasia cerulea dolens, a rare complication of deep. It also shows how rare pathologies can combine to create a life and limbthreatening condition.
Pad is known by the triad of edema, pain, and blanching alba without cyanosis. 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. 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. Phlegmasia cerulea dolens is a rare form of massive proximal e. Phlegmasia cerulea dolens postgraduate medical journal. In the lower extremities, leftside involvement is three to four times more common. Phlegmasia cerulea dolens is a rare syndrome caused by diffuse venous. 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. Phlegmasia cerulea dolens phlebitis blue of gregoire. Phlegmasia cerulea dolens is an acute limbthreatening venous thrombosis which requires prompt and aggressive management. Phlegmasia cerulea dolens pcd is a severe presentation of massive dvt, and it is often associated with extremity. Oct 21, 2017 we present a unique case of phlegmasia cerulea dolens and compartment syndrome secondary to external iliac vein disruption after revision total hip arthroplasty. Phlegmasia cerulea dolens describes clot formation in the venous system over a large area. Its rapid recognition as venous thrombosis rather than arterial insufficiency is important in avoiding gangrene and other life threatening complications.
Pdf phlegmasia cerulea dolens in a long distance driver. Phlegmasia cerulea dolens pcd is a medical emergency that can lead to venous gangrene of the lower extremity. A diagnosis of phlegmasia cerulea dolens was made, and to prevent potential. Case report phlegmasia cerulea dolens in a patient with breast cancer and inferior vena cava hypoplasia a. Manual aspiration thrombectomy for treatment of pcd has been reported by l. Phlegmasia cerulea dolens pcd is a complication of acute deep venous thrombosis dvt characterized by pain, swelling, and skin discolouration. High index of suspicion must be kept for the development of acute compartment syndrome in such patients. To our knowledge, this complication has not yet been described following revision total hip arthroplasty. Two days after admission, bluish discoloration of the left great toe was noted. In cases of pregnancy, it is most often seen during the third trimester, resulting from a compression of the left common iliac vein against the pelvic. Pdf phlegmasia cerulea dolens with compartment syndrome.
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