Phlegmasia cerulea dolens a limb threatening problem. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. Rapid deterioration and death caused by bilateral phlegmasia. Rapid deterioration and death caused by bilateral phlegmasia cerulea dolens a 58yearold woman with a history of deep vein thrombosis and pulmonary embolism presented for evaluation of significant back and leg pain and difficulty ambulating. Diagnosis and management of phlegmasia cerulea dolens in the emergency department. There is no reason, at an early stage, for doing anything but giving such opiates as are needed and elevating the leg. Internal jugular and subclavian vein thrombosis in a case of ovarian cancer donati, cancer and thrombosis.
When venous outflow is seriously impeded, arterial inflow may be reduced. Phlegmasia alba and cerulea dolens clinical presentation. Deep venous thrombosis uncomplicated phlegmasia alba dolens. An 85yearold woman with newly diagnosed metastatic nonsmallcell lung cancer was admitted for pain control. Phlegmasia alba dolens is part of a spectrum of diseases related to deep vein thrombosis. Jan 24, 2016 diagnosis and management of phlegmasia cerulea dolens in the emergency department. A diagnosis of phlegmasia cerulea dolens pcd and pulmonary embolism pe was established.
A 75yearold man, known for nephrotic syndrome currently under investigation, presented to the emergency department with a. Phlegmasia cerulea dolens with compartment syndrome. Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high. Phlegmasia cerulea dolens is a rare form of massive proximal e. 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. Advances in the treatment of phlegmasia cerulea dolens treatment options for phlegmasia cerulea dolens. Phlegmasia cerulea dolens definition of phlegmasia cerulea. Phlegmasia cerulea dolens pcd is a rare form of venous gangrene. A 50yearold woman, a known diabetic and hypertensive with poor compliance to treatment, presented with fever, dyspnoea and leftsided pleuritic chest pain for 15 days and left lower limb swelling worsening over the past 1 week. Phlegmasia definition of phlegmasia by the free dictionary.
The imported definitions may be significantly out of date, and any more recent senses may be completely missing. More than 600000 cases of venous thromboembolism are estimated to occur each year in the united. Phlegmasia cerulea dolens pcd is a rare entity that refers to a painful, edematous, and cyanotic limb due to a massive deep vein thrombosis dvt. Rare case of unilateral phlegmasia cerulea dolens with. Phlegmasia cerulean dolens is a severe form of deep venous thrombosis. Plegmasia alba dolens phlegmasia alba dolens alba means white i. The swelling of the lower limb and coloring improved gradually, and he was discharged after an uneventful course. Phlegmasia cerulea dolens pcd, painful blue leg is a rare manifestation of deep vein thrombosis. 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 cerulea dolens is a rare condition involving total or neartotal intravenous thrombotic obstruction that can rapidly progress to limb ischemia and venous gangrene. Albino referred to as milk leg or white leg historically seen in pregnant women third trimester or mothers who had just given birth compression of lt iliac vein against the pelvic rim from an enlarged uterus. Aug, 2018 phlegmasia stems from a greek term phlegma meaning inflammation. The treatment of phlegmasia alba dolens ought to be planned to get rid of the edema at the earliest moment, to restore the.
Pdf phlegmasia cerulea dolens a rare, lifethreatening. The development of a standard treatment for phlegmasia cerulea dolens is ongoing. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene. Phlegmasia alba dolens pdf phlegmasia alba and cerulea dolens. 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 rim by the. Prompt fasciotomies were performed, but the underlying massive venous thrombosis was not addressed surgically. Herein we report a case of phlegmasia cerulea dolens, a form of venous thrombosis complicated by arterial ischaemia. A 58yearold woman with metastatic breast cancer presented with acute onset of pain in her right lower leg for 1. A fatal case of upperextremity deep vein thrombosis porto. Phlegmasia stems from a greek term phlegma meaning inflammation. Scvir, 1995 phlegmasia cerulea dolens pcd is a fulminant form of peripheral venous thrombosis characterized by massive edema, severe pain, and cyanosis.
It is a medical emergency and can rapidly lead to venous gangrene of the extremity. The results of treating germ cell cancer continue to improve. Phlegmasia cerulea dolens radiology reference article. In patients delaying initial presentation by less than three months survival. Endovascular treatment of phlegmasia cerulea dolens with impending venous gangrene.
A 58yearold woman with metastatic breast cancer presented with acute onset of pain in her right lower leg for 1 day. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. 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 postgraduate medical journal. Differential diagnosis clinical spectrum of venous thromboembolism. Phelgmasia cerulea dolens diagnosed by pointofcare. It is the consequence of complete obstruction of the venous drainage of a limb and may result in shock, distal gangrene 50%, pulmonary embolus, amputation. Schwarze, md, mpp perspectives in vascular surgery and endovascular therapy 2011 22. To our knowledge, this complication has not yet been described following revision total hip arthroplasty. Phlegmasia cerulea dolens has been described in every age group but occurs more frequently in the fifth and sixth decades. Phlegmasia alba dolens an overview sciencedirect topics. The complications of acute venous thromboembolism vte, including deep vein thrombosis dvt and pulmonary embolism pe, are the most common preventable causes of hospital death and a source of substantial longterm morbidity.
Pregnancy increases the risk of venous thromboembolism by four to fivefold. Clinically there is sudden severe pain in a part of an extremity which quickly spreads to involve the whole limb. Successful venous thrombectomy significantly reduces early morbidity in patients with phlegmasia cerulea dolens and phlegmasia alba dolens. Article information, pdf download for phlegmasia cerulea dolens in. This case report shows how a hybrid approach might be successful. No clear guidelines exist for the management of phlegmasia cerulea dolens. Treatment options for phlegmasia cerulea dolens journal. Pad is known by the triad of edema, pain, and blanching alba without cyanosis. Patients usually present with edema, pain, blanching and it may progress to gangrene and systemic shock in severe cases. Treatment options for phlegmasia cerulea dolens journal of. Phlegmasia cerulea dolens in a patient with breast cancer.
Phlegmasia cerulea dolens, a rare complication of deep. Diagnosis of pulmonary embolism by multidetector ct alone or combined with venous ultrasonography of the leg. 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. This stage is called phlegmasia cerulea dolens literally, painful purple swelling. 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. It has been used in the medical literature in reference to extreme cases of lowerextremity deep venous thrombosis dvt that cause critical limb ischemia and possible limb loss. With alba you have just severe swelling of the limb without arterial compromise. Only 40% of ambulatory ed patients with pe have concomitant dvt. Phlegmasia cerulea dolens pcd is an uncommon complication of deep venous thrombosis, which results from extensive thrombotic occlusion of the major and collateral veins of an extremity usually the legs. Pdf phlegmasia cerulean dolens is a severe form of deep venous. In the lower extremities, leftside involvement is three to four times more common. Between phlegmasia alba dolens and phlegmasia cerulea.
On examination, her vitals were stable, and she had significant left lower limb oedema extending up to the upper thigh with livedo reticularis figure 1. 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 lifethreatening. Phlegmasia cerulea dolens describes clot formation in the venous system over a large area. Phlegmasia cerulea dolens of the upper extremity sciencedirect. He received heparin and inferior vena cava ivc filter placement followed by anticoagulation therapy with warfarin. Phlegmasia cerulea dolens is preceded by a condition called phlegmasia alba dolens. 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.
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. The patients pain and edema quickly subside and the discoloration resolves. Manifestations of phlegmasia alba dolens the clinical precursor to pcd include edema, pain and blanching. Intervention is usually required in order to prevent gangrene. Phlegmasia cerulea dolens, maythurner syndrome, and nutcracker syndrome pasithorn a. Pronunciation of phlegmasia cerulea dolens with 1 audio pronunciation, 1 meaning, 2 translations and more for phlegmasia cerulea dolens. As the disease progresses, cyanosis develops, and can lead to bleb and bullae formation and venous gangrene.
We conclude that although vascular complications are fortunately rare after hip arthroplasty, they can have significant. 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. Advanced testicular cancer presenting with phlegmasia. Symptoms severe pain, rapid swelling, and bluishskin coloring affect the area below the blocked vein. Phlegmasia cerulea dolens medical definition merriam. 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 cerulea dolens refers to congestion and cyanosis of a limb due to massive venous thrombosis.
There is a high risk of massive pulmonary embolism. In the united states, 600,000 cases of venous thromboembolism are thought to occur every year. It also shows how rare pathologies can combine to create a life and limbthreatening condition. Phlegmasia alba dolens pad describes the patient with swollen and white leg because of early. Pdf phlegmasia cerulea dolens with compartment syndrome.
Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Phlegmasia alba dolens and the relation of the lymphatics. Phlegmasia cerulea dolens, or blue phlebitis, is an acute fulminating form of deep venous thrombosis first described by tremolieres and veran 1 in 1929. Pdf phelgmasia cerulea dolens diagnosed by pointofcare. Phlegmasia alba dolens and phlegmasia cerulea dolens predominantly affect the lower extremities, involving the upper extremities in less than 5% of the cases. Phlegmasia alba dolens definition of phlegmasia alba dolens. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens with compartment syndrome. Phlegmasia cerulea dolens is the more severe form of the two. Phlegmasia cerulea dolens from latin for inflamed, blue, and painful was the clinical impression 1 and confirmed with doppler ultrasound, which demonstrated an occlusive thrombus in the left. Phlegmasia cerulea dolens a rare life threatening condition. Phlegmasia alba dolens and the relation of the lymphatics to.
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