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