Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of. Phlegmasia cerulea dolens pcd is a rare form of venous gangrene that arises secondary to occlusion of venous outflow from a limb. 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 refers to congestion and cyanosis of a limb due to massive venous thrombosis. Pacemakerassociated phlegmasia cerulea dolens treated. Phlegmasia alba and cerulea dolens are rare diseases along a spectrum of deep venous thromboses. In phlegmasia alba dolens, the thrombosis involves only major deep venous channels of the extremity, therefore sparing collateral veins. Endovascular treatment of phlegmasia cerulea dolens with impending venous gangrene. Phlegmasia cerulea dolens pcd represents the most severe acute consequence of dvt. Postoperatively he developed painful diffuse swelling of his left lower extremity with bluish discoloration figure 581. Intervention is usually required in order to prevent gangrene. We present a unique case of phlegmasia cerulea dolens and compartment syndrome secondary to external.
Catheterdirected thrombolysis is an effective treatment modality and has been shown to reduce the occurrence of postthrombotic limb. 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 from radiationinduced venous. Pdf download for trends in management of phlegmasia cerulea dolens. Clinically there is sudden severe pain in a part of an extremity which quickly spreads to involve the whole limb. No popliteal or distal pulse could be found, with anesthesia. The distal pulses were entirely absent, and only a faint left femoral pulse was palpable. The patients pain and edema quickly subside and the discoloration resolves. 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 alba dolens an overview sciencedirect topics. The swelling of the lower limb and coloring improved gradually, and he was discharged after an uneventful course. Acousticassisted catheterdirected thrombolytic therapy. Phlegmasia cerulea dolens phlegmasia cerulea dolens.
We conclude that although vascular complications are fortunately rare after hip arthroplasty. Phlegmasia cerulea dolens definition of phlegmasia. Guidelines for treatment are still not clearly documented. This is the first report of posttraumatic phlegmasia cerulea dolens induced by iliac vein ligation. Phlegmasia cerulea dolens pcd is an uncommon but potentially lifethreatening complication of acute dvt characterized by marked swelling of the extremities with pain. We report a case of upper extremity pcd developing abruptly in a 37yearold female with an indwelling cardiac pacemaker who presented to the emergency department. 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 painful cyanotic swollen leg is a rare and severe form of acute deep vein thrombosis dvt characterized by marked limb pain, swelling, and blue discoloration. Phlegmasia cerulea dolens pcd is a rare entity that is associated with significant morbidity and mortality, including limb ischemia and pulmonary embolism. On physical examination edema with fovea in the left lower extremity with cyanosis below the knee figs.
The development of a standard treatment for phlegmasia cerulea dolens is ongoing. 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. Trends in management of phlegmasia te d cerulea dolens. Phlegmasia coerulea dolensphlegmasia cerulea dolens. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. 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. Classically described in the lower extremity, it is rare in the upper extremity. We present a unique case of phlegmasia cerulea dolens and compartment syndrome secondary to external iliac vein disruption after revision total hip arthroplasty.
Phlegmasia cerula dolens is a grave complication of deep thrombophlebitis, characterized by a sudden massive venous occlusion which simulates arterial embolism in its effects and seriously endangers both the patients life and the affected limb. Early fasciotomy is an effective way of limb salvage for these patients. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens with compartment syndrome. A 65yearold gentleman was referred with left lower extremity phlegmasia cerulea dolens pcd 1 day following an exploratory laparotomy. Phlegmasia cerulea dolens postgraduate medical journal. Phlegmasia cerulea dolens is a severe form of venous thrombosis presenting as an edematous, painful, purplishblue lower extremity.
In the lower extremities, leftside involvement is three to four times more common. Left foot of the patient diagnosed with phlegmasia cerulea dolens. Phlegmasia cerulea dolens pcd, painful blue leg is a rare manifestation of deep vein thrombosis. A 50 year old man, with a prior history of stage iv rectal cancer in palliative treatment, came to the emergency department with sudden severe pain of 12 h duration in the left lower extremity. High index of suspicion must be kept for the development of acute compartment syndrome in such patients. Phlegmasia cerulean dolens is a severe form of deep venous thrombosis. Pdf phlegmasia cerulea dolens with compartment syndrome. Most cases described encompass the lower extremities. Lewis and charles short 1879 a latin dictionary, oxford. Phlegmasia cerulea dolens pcd is a rare and severe form of deep venous thrombosis that is classically associated with the lower extremities. Phlegmasia cerulea dolens from latin for inflamed, blue, and painful was the clinical impression 1 and confirmed. To our knowledge, this complication has not yet been described following revision total hip arthroplasty.
Phlegmasia cerulea dolens pcd, a lifethreatening complication of deep venous thrombosis dvt, is characterized by massive iliofemo ral thrombus that. Flegmasia cerulea dolens phlegmasia cerulla dolens dra. Phlegmasia alba dolens, which translates to painful white inflammation, occurs when venous thrombosis progresses to a massive occlusion of the major deep venous system of the leg, but without ischemia as collateral veins are still patent. Phlegmasia alba dolens pdf phlegmasia alba and cerulea dolens. 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 cerulea dolens khamin. Phlegmasia cerulea dolens with compartment syndrome nejm. The imported definitions may be significantly out of date, and any more.
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 alba dolens also colloquially known as milk leg or white leg is part of a spectrum of diseases related to deep vein thrombosis. Surgical thrombectomy and simultaneous stenting for. Phlegmasia cerulea dolens is a medical emergency requiring escalation of therapy beyond simple anticoagulation to prevent sequelae such as postthrombotic limb. More than 600000 cases of venous thromboembolism are estimated to occur each year in the united. Phlegmasia cerulea dolens color atlas and synopsis of. Pointofcare ultrasound pocus can expedite the diagnosis, leading to earlier life and limbsaving treatment. Phlegmasia alba dolens and the relation of the lymphatics. Phlegmasia cerulea dolens pcd is a severe presentation of massive dvt, and it is often associated with extremity catheterdirected thrombolytic therapy in deep venous thrombosis of the lower extremity. Pronunciation of phlegmasia cerulea dolens with 1 audio pronunciation, 1 meaning, 2 translations and more for phlegmasia cerulea dolens. The patient was diagnosed as a case of phlegmasia cerulea dolens of left leg, compartment syndrome with pulmonary embolism pe.
Phlegmasia cerulean dolens is a severe form of deep venous thrombosis characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish. 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. Successful venous thrombectomy significantly reduces early morbidity in patients with phlegmasia cerulea dolens and phlegmasia alba dolens. It is a medical emergency and can rapidly lead to venous gangrene of the extremity. Rapid deterioration and death caused by bilateral phlegmasia. Phlegmasia cerulea dolens pcd is a fulminant condition of acute. A 58yearold woman with metastatic breast cancer presented with acute onset of. Successful treatment of posttraumatic phlegmasia cerulea.
Maythurner syndrome as the cause of phlegmasia cerulea dolens. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens. He received heparin and inferior vena cava ivc filter placement followed by anticoagulation therapy with warfarin. A diagnosis of phlegmasia cerulea dolens pcd and pulmonary embolism pe was established. Pdf phlegmasia cerulea dolens a rare, lifethreatening. There is a high risk of massive pulmonary embolism.
Its rapid recognition as venous thrombosis rather than arterial insufficiency is important in avoiding gangrene and other life threatening complications. Phlegmasia cerulea dolens is a rare form of massive proximal e. Here we report a rare case of posttraumatic phlegmasia cerulea dolens after ligation of the iliac vein to save the patients life, with successful treatment by reconstructing the external iliac vein. 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.
Phlegmasia cerulea dolens definition at, a free online dictionary with pronunciation, synonyms and translation. Pad is known by the triad of edema, pain, and blanching alba without cyanosis. On arrival, he underwent a venous duplex that demonstrated thrombosis of his tibial, popliteal, femoral, common femoral, and. The patient had painful swelling and bluishred discoloration of the right leg at the time of presentation. Phlegmasia cerulea dolens phlegmasia dolens following influenza. Phlegmasia cerulea dolens pcd is a severe complication of deep veions thrombosis, and there are several treatment methods. Phlegmasia cerulea dolens is an acute limbthreatening venous thrombosis which requires prompt and aggressive management. Phlegmasia cerulea dolens and external iliac vein disruption after. Phlegmasia alba dolens definition of phlegmasia alba.
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. Lewis 1891 an elementary latin dictionary, new york. Phelgmasia cerulea dolens diagnosed by pointof care. Phlegmasia alba and cerulea dolens clinical presentation.
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