Pdf venous thromboembolism vte is a major healthcare problem that results in significant mortality. We report a case of a 56yearold male medically free with a history of long travel. Phlegmasia definition of phlegmasia by medical dictionary. Endovascular treatment of phlegmasia cerulea dolens with impending venous gangrene. 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. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. The leg becomes more swollen and increasingly more painful. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. Early fasciotomy is an effective way of limb salvage for these patients. He has a previous stroke but left them with partial paralysis of. Pdf phlegmasia cerulea dolens in a long distance driver. Jan 24, 2016 diagnosis and management of phlegmasia cerulea dolens in the emergency department. Phlegmasia cerulean dolens is a severe form of deep venous thrombosis characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish. Phlegmasia alba dolens pad describes the patient with swollen and white leg because of early compromise of arterial flow secondary to.
He received heparin and inferior vena cava ivc filter placement followed by anticoagulation therapy with warfarin. Phlegmasia definition of phlegmasia by the free dictionary. Treatment options for phlegmasia cerulea dolens journal. Phlegmasia cerulea dolens pcd is more advanced and considered a precursor of frank venous gangrene. Phlegmasia alba dolens and the relation of the lymphatics to. Phlegmasia cerulea dolens a rare life threatening condition. Aug 27, 2018 phlegmasia cerulea dolens and phlegmasia alba dolens can present as compartment syndrome. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens with compartment syndrome. Surgical thrombectomy and simultaneous stenting for. Phlegmasia cerulea dolens color atlas and synopsis of.
Phlegmasia cerulea dolens pcd is an uncommon but potentially lifethreatening complication of acute. Severe sequelae threatening life and limb are rare and include phlegmasia cerulea dolens and renal vein thrombosis. Phlegmasia cerulea dolens a rare life threatening condition 4 p44 he defined inflammation or phlegmasia as the most frequent and where the seat of the irritation becomes painful, warm, swollen, and red from the increased afflux of blood into its capillary system. Access to society journal content varies across our titles. Phlegmasia article about phlegmasia by the free dictionary. It is a medical emergency and can rapidly lead to venous gangrene of the extremity.
Pronunciation of phlegmasia cerulea dolens with 1 audio pronunciation, 1 meaning, 2 translations and more for phlegmasia cerulea dolens. Phlegmasia cerulea dolens with compartment syndrome nejm. The patient is started on anticoagulation, and the involved extremity is elevated. Phlegmasia cerulea dolens pcd is a complication of acute deep venous thrombosis dvt characterized by pain, swelling, and skin discolouration. Also known as milk leg explanation of phlegmasia alba dolens.
What is the pathophysiology of phlegmasia alba dolens pad. Abouzamzam jr, md, loma linda, calif phlegmasia cerulea dolens is a rare but serious complication of deep venous thrombosis, which can lead to arterial. Phlegmasia cerulea dolens is a rare syndrome caused by acute, extensive venous thrombosis. High index of suspicion must be kept for the development of acute compartment syndrome in such patients. Phlegmasia cerulea dolens request pdf researchgate. Phlegmasia cerulea dolens definition of phlegmasia cerulea. No clear guidelines exist for the management of phlegmasia cerulea dolens. Phlegmasia cerulea dolens with compartment syndrome.
Phlegmasia cerulea dolens is a rare syndrome defined as a clinical triad of acute limb swelling, ischemic pain and 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 phlegmasia erulea dolens pd erulea means lue referred to as painful lue leg rare condition that occurs in phlegmasia cerulea dolens with compartment syndrome. At this stage it is called phlegmasia cerulea dolens. 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 phlebitis blue of gregoire. Phlegmasia alba dolens and the relation of the lymphatics. Phlegmasia cerulea dolens information mount sinai new york. A painful swelling of the leg usually seen postpartum, due to femoral vein thrombophlebitis or lymphatic obstruction. 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 with compartment syndrome ncbi.
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. In phlegmasia alba dolens, the thrombosis involves only major deep venous channels of the extremity, therefore sparing collateral veins. Phlegmasia cerulea dolens is an acute limbthreatening venous thrombosis which requires prompt and aggressive management. No popliteal or distal pulse could be found, with anesthesia and. Phlegmasia cerulea dolens describes clot formation in the venous system over a large area. Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant. Prompt fasciotomies were performed, but the underlying massive venous thrombosis was not addressed surgically. Tell a friend about us, add a link to this page, or visit the webmasters page for free fun content. Lewis and charles short 1879 a latin dictionary, oxford. Postphlebitic edema after phlegmasia alba dolens in a typical situationat ar. Apr 25, 2019 phlegmasia cerulea dolens refers to congestion and cyanosis of a limb due to massive venous thrombosis. 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 an uncommon but potentially life threatening.
It is liable to occur in individuals of young adult life and middle age, who are for any reason long confined to bed, particularly by debilitating diseases, whether or not infectious, by operations and by injuries. Guidelines for treatment are still not clearly documented. It is characterized by the sudden onset of severe leg pain, massive edema, cyanosis. Catheterdirected thrombolysis is an effective treatment modality and has been shown to. 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. A diagnosis of phlegmasia cerulea dolens was made, and to prevent potential irreversible venous gangrene and subsequent limb loss, the patient underwent catheterdirected thrombolysis combined with thrombectomy and showed subsequent symptomatic improvement. It was first described by hildanus in the 16 th century. Phlegmasia alba dolens article about phlegmasia alba. Postphlebitic edema and induration after phlegmasia alba dolens a very advanced stage of ulceration. A massive backlog of venous blood results in a substantial increase in tissue pressure.
Sep 25, 2018 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. Pdf phlegmasia cerulea dolens with compartment syndrome. Severe sequelae threatening life and limb are rare and include phlegmasia cerulea dolens and renal 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 arterial compromise. 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. Manual aspiration thrombectomy with stent placement. A multimodal therapeutic approach to phlegmasia cerulea. Prompt diagnosis is crucial for limb salvage and to minimize morbidity. Trends in management of phlegmasia te d cerulea dolens. The swelling of the lower limb and coloring improved gradually, and he was discharged after an uneventful course.
Pdf phlegmasia cerulea dolens a rare, lifethreatening. A 75yearold man, known for nephrotic syndrome currently under investigation, presented to the emergency department with a. A diagnosis of phlegmasia cerulea dolens pcd and pulmonary embolism pe was established. 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. Deep venous thrombosis dvt affects mainly the veins in the lower leg and the thigh. A 65yearold gentleman was referred with left lower extremity phlegmasia cerulea dolens pcd 1 day following an exploratory laparotomy. There is a high risk of massive pulmonary embolism. This patient presented to the emergency department with the swollen and painful right lower extremity. Patients usually present with edema, pain, blanching and it may progress to gangrene and systemic shock in severe cases. The pathogenesis begins with massive thrombosis causing total or arterial flow, and ultimately, varying degrees of ischemic damage.
After 25 days of hospitalization, he was discharged while receiving rivaroxaban. Nov 16, 2019 phlegmasia coerulea dolensphlegmasia cerulea dolens. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Manual aspiration thrombectomy for treatment of pcd has been reported by l. Images in clinical medicine from the new england journal of medicine phlegmasia cerulea dolens. Venous obstruction can lead to compartment syndrome, venous gangrene, and death in 25%40% of cases 1. This study aimed to investigate the clinical efficacy of surgical thrombectomy and simultaneous iliac vein stent implantation for the treatment of. Trends in management of phlegmasia cerulea dolens khamin. Phlegmasia cerulea dolens presenting with acute compartment. Serial ultrasounds were performed in patients before the insertion of femoral venous catheters and sequentially every 48 hrs while the catheters were in place.
Postoperatively he developed painful diffuse swelling of his left lower extremity with bluish discoloration figure 581. Health, general venous thrombosis care and treatment diagnosis. Phlegmasia cerulea dolens refers to congestion and cyanosis of a limb due to massive venous thrombosis. Phlegmasia alba dolens an overview sciencedirect topics. It is characterized by severe swelling and cyanosis and blue discoloration of the extremity. This case report shows how a hybrid approach might be successful. At the moment, the edema is receding, and desquamation of the skin is going on. Phlegmasia cerulea dolens is an uncommon, severe form of deep venous thrombosis blood clots in the vein. Phlegmasia cerulea dolens in a patient with breast cancer. 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. Additionally, the edema and loss of venous outflow impedes the arterial inflow. Aug, 2018 oguzkurt l, ozkan u, demirturk os, gur s. On rare occasions, severe leg swelling due to iliofemoral dvt can lead to compartment syndrome, and an emergency fasciotomy is required for limb salvage.
Aug, 2018 treatment of phlegmasia alba dolens pad or phlegmasia cerulea dolens pcd should be initiated as soon as the diagnosis is suspected. It compounds with pulmonary embolism pe and deep vein thrombosis dvt. Treatment options for phlegmasia cerulea dolens journal of. Phlegmasia alba dolens definition of phlegmasia alba. Low molecular weight heparins have not been effectively studied in the setting of phlegmasia alba or cerulea dolens. Phlegmasia cerulea dolens and phlegmasia alba dolens can present as compartment syndrome. Phlegmasia alba dolens definition of phlegmasia alba dolens. Lewis 1891 an elementary latin dictionary, new york. Phlegmasia cerulea dolens postgraduate medical journal. A multimodal therapeutic approach to phlegmasia cerulea dolens in a pediatric patient isabella kuo, md, ajason smith, md,b and ahmed m. Phlegmasia cerulea dolens pcd is a severe complication of deep veions thrombosis, and there are several treatment methods. Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of. Phlegmasia coerulea dolensphlegmasia cerulea dolens.
Oct 28, 2016 a total occlusion of the venous outflow is called phlegmasia cerulea dolens pcd. Phlegmasia cerulea dolens a limb threatening problem. Case report, clinical report by journal of evolution of medical and dental sciences. On arrival, he underwent a venous duplex that demonstrated thrombosis of his tibial, popliteal, femoral, common femoral, and external. Treatment of phlegmasia alba dolens pad or phlegmasia cerulea dolens pcd should be initiated as soon as the diagnosis is suspected. 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. Seven days later, during oral anticoagulation therapy. This often causes compartment syndrome with a subsequent decrease in arterial blood flow in the affected extremity or even venous gangrene. The patients condition improved panel b, and he recovered renal function. It also shows how rare pathologies can combine to create a life and limbthreatening condition. On physical examination edema with fovea in the left lower extremity with cyanosis below the knee figs. 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. Rarely, open surgical venous thrombectomy may be indicated in postoperative patients to relieve the severe swelling in phlegmasia alba dolens or phlegmasia cerulea. Phlegmasia cerulea dolens is a medical emergency requiring escalation of therapy beyond simple anticoagulation to prevent sequelae such as postthrombotic limb.