Phlegmasia cerulea dolens | |
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A person with a two hour history of phlegmasia cerulea dolens (left leg, right side of image) |
Phlegmasia cerulea dolens (PCD) (literally: 'painful blue inflammation'), not to be confused with preceding phlegmasia alba dolens, is an uncommon severe form of lower extremity deep venous thrombosis (DVT) that obstructs blood outflow from a vein. Upper extremity PCD is less common, occurring in under 10% of all cases. [1] PCD results from extensive thrombotic occlusion (blockage by a thrombus) of extremity veins, most commonly an iliofemoral DVT, of the iliac vein and/or common femoral vein. [2] [3] It is a medical emergency requiring immediate evaluation and treatment.
It is characterized by progressive lower extremity edema distal to the thigh, tight shiny skin, cyanosis (inadequate blood oxygenation), petechiae or purpura, and sudden severe pain of the affected limb in proportion to the level of venous blockage. Patients often have difficulty walking. Blisters, bullae, paresthesias, and motor weakness may develop in severe cases, along with gangrene in ~50% of cases. [4] [5] Distal pulses are palpable early on but may diminish over time, and doppler signal can be usually heard throughout disease progression. [6] The left limb is more commonly affected due to its vascular anatomy (the right internal iliac artery directly overlies the left iliac vein). [7] [8]
PCD is associated with an underlying malignancy in 20-40% of cases. [9] There is a high risk of massive pulmonary embolism, even under anticoagulation. [10]
Risk factors, present in around 50% of documented cases, include malignancy, hyper-coagulable states, cardiac disease, venous stasis, venous insufficiency, May-Thurner syndrome (right iliac artery compressing the left iliac vein that runs beneath it), surgery, trauma, pregnancy, inferior vena cava (IVC) filter, hormone therapy, oral contraceptives, prolonged immobilization, inflammatory bowel disease, heart failure, and central venous catheters. Etiology is unknown in ~10% of PCD cases. [11] [12] [13]
When a thrombus occludes an extremity vein, pressure backs up in the venous system leading plasma fluid to leak out into the interstitium of the affected limb. This increases the pressure of that limb compartment, which can collapse the arteries and lead to acute ischemia, gangrene, hypovolemia, and hemodynamic instability. [14] [15]
PCD is best diagnosed with contrast venography, but venous duplex ultrasonography is used more commonly in clinical practice. Magnetic resonance and computed tomography venography can also be used. [16]
DDx is as follows: [17]
Treatment for PCD includes immediate anticoagulation, fluid resuscitation, bed rest, limb elevation above 60º, limb wrap to reduce pain and edema, and either catheter-based thrombolysis, percutaneous transluminal angioplasty, or surgical venous thrombectomy +/- fasciotomy to remove the blood clot. [18] [19] [20] [21] Some people also suggest an IVC filter before thrombolysis. [22]
PCD is fully reversible if the causal venous thrombus is promptly removed. [23] In the 40-60% of people who go on to develop venous gangrene, there is a 20-50% risk of amputation and 20-40% mortality rate. [24] [25] Following PCD resolution patients are more likely to develop venous insufficiency and post-thrombotic syndrome [26]
A grading system has been established [ by whom? ]: [27] [28]
PCD is most likely to occur in people in their 50s and 60s, but can occur as early as 6 months old. There is slight male predominance of around 1.5:1. [29] [30]
This phenomenon was first discovered by Fabricus Hildanius in the 16th century, and was officially termed "phlegmasia cerulea dolens" by Gregoire in 1938. [31] [32] Phlegmasia originates from the Greek root phlegma (inflammation), cerulea originates from Latin root caeruleus (dark blue), and dolens originates from Latin word dolens (suffering). [33]
Angioplasty, is also known as balloon angioplasty and percutaneous transluminal angioplasty (PTA), is a minimally invasive endovascular procedure used to widen narrowed or obstructed arteries or veins, typically to treat arterial atherosclerosis. A deflated balloon attached to a catheter is passed over a guide-wire into the narrowed vessel and then inflated to a fixed size. The balloon forces expansion of the blood vessel and the surrounding muscular wall, allowing an improved blood flow. A stent may be inserted at the time of ballooning to ensure the vessel remains open, and the balloon is then deflated and withdrawn. Angioplasty has come to include all manner of vascular interventions that are typically performed percutaneously.
The brachiocephalic artery is an artery of the mediastinum that supplies blood to the right arm and the head and neck. It was previously known as the innominate artery, meaning unnamed artery.
The femoral artery is a large artery in the thigh and the main arterial supply to the thigh and leg. The femoral artery gives off the deep femoral artery and descends along the anteromedial part of the thigh in the femoral triangle. It enters and passes through the adductor canal, and becomes the popliteal artery as it passes through the adductor hiatus in the adductor magnus near the junction of the middle and distal thirds of the thigh.
Thrombolysis, also called fibrinolytic therapy, is the breakdown (lysis) of blood clots formed in blood vessels, using medication. It is used in ST elevation myocardial infarction, stroke, and in cases of severe venous thromboembolism.
Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and enlarged veins in the affected area, but some DVTs have no symptoms. The most common life-threatening concern with DVT is the potential for a clot to embolize, travel as an embolus through the right side of the heart, and become lodged in a pulmonary artery that supplies blood to the lungs. This is called a pulmonary embolism (PE). DVT and PE comprise the cardiovascular disease of venous thromboembolism (VTE). About two-thirds of VTE manifests as DVT only, with one-third manifesting as PE with or without DVT. The most frequent long-term DVT complication is post-thrombotic syndrome, which can cause pain, swelling, a sensation of heaviness, itching, and in severe cases, ulcers. Recurrent VTE occurs in about 30% of those in the ten years following an initial VTE.
Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.
A pseudoaneurysm, also known as a false aneurysm, is a locally contained hematoma outside an artery or heart due to damage to the vessel wall. The injury goes through all the three layers of the arterial wall causing a leak, which is contained by a new weak wall formed by the products of the clotting cascade. Hence, a pseudoaneurysm does not contain any layer of the vessel wall.
The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries without passing through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, and finally published in 1973.
Intraosseous infusion (IO) is the process of injecting medications, fluids, or blood products directly into the marrow of a bone; this provides a non-collapsible entry point into the systemic venous system. The intraosseous infusion technique is used to provide fluids and medication when intravenous access is not available or not feasible. Intraosseous infusions allow for the administered medications and fluids to go directly into the vascular system. The IO route of fluid and medication administration is an alternative to the preferred intravascular route when the latter cannot be established in a timely manner in emergent situations. Intraosseous infusions are used when people have compromised intravenous access and need immediate delivery of life-saving fluids and medications.
In human anatomy, the superior epigastric veins are two or more venae comitantes which accompany either superior epigastric artery before emptying into the internal thoracic vein. They participate in the drainage of the superior surface of the diaphragm.
An embolus, is described as a free-floating mass, located inside blood vessels that can travel from one site in the blood stream to another. An embolus can be made up of solid, liquid, or gas. Once these masses get "stuck" in a different blood vessel, it is then known as an "embolism." An embolism can cause ischemia - or damage to an organ from lack of oxygen. A paradoxical embolism is a specific type of embolism in which the emboli travels from the right side of the heart or "venous circulation," travels to the left side of the heart or "arterial circulation," and lodges itself in a blood vessel known as an artery. Thus, it is termed "paradoxical" because the emboli lands in an artery, rather than a vein.
In medicine, gonadal vein refers to the blood vessel that carries blood away from the gonad toward the heart. These are different arteries in women and men, but share the same embryological origin.
The popliteal artery entrapment syndrome (PAES) is an uncommon pathology that occurs when the popliteal artery is compressed by the surrounding popliteal fossa myofascial structures. This results in claudication and chronic leg ischemia. This condition mainly occurs more in young athletes than in the elderlies. Elderlies, who present with similar symptoms, are more likely to be diagnosed with peripheral artery disease with associated atherosclerosis. Patients with PAES mainly present with intermittent feet and calf pain associated with exercises and relieved with rest. PAES can be diagnosed with a combination of medical history, physical examination, and advanced imaging modalities such as duplex ultrasound, computer tomography, or magnetic resonance angiography. Management can range from non-intervention to open surgical decompression with a generally good prognosis. Complications of untreated PAES can include stenotic artery degeneration, complete popliteal artery occlusion, distal arterial thromboembolism, or even formation of an aneurysm.
In the anatomy of the heart, the smallest cardiac veins, also known as the Thebesian veins, are small valveless veins in the walls of all four heart chambers that drain venous blood into any of the heart chambers.
Chronic venous insufficiency (CVI) is a medical condition in which blood pools in the veins, straining the walls of the vein. The most common cause of CVI is superficial venous reflux which is a treatable condition. As functional venous valves are required to provide for efficient blood return from the lower extremities, this condition typically affects the legs. If the impaired vein function causes significant symptoms, such as swelling and ulcer formation, it is referred to as chronic venous disease. It is sometimes called chronic peripheral venous insufficiency and should not be confused with post-thrombotic syndrome in which the deep veins have been damaged by previous deep vein thrombosis.
Obstructive shock is one of the four types of shock, caused by a physical obstruction in the flow of blood. Obstruction can occur at the level of the great vessels or the heart itself. Causes include pulmonary embolism, cardiac tamponade, and tension pneumothorax. These are all life-threatening. Symptoms may include shortness of breath, weakness, or altered mental status. Low blood pressure and tachycardia are often seen in shock. Other symptoms depend on the underlying cause.
Paolo Zamboni is an Italian doctor and scientist. He is full Professor and Director of the School of Vascular Surgery at the University of Ferrara in Italy.
Acute limb ischaemia (ALI) occurs when there is a sudden lack of blood flow to a limb.
Venous access is any method used to access the bloodstream through the veins, either to administer intravenous therapy, parenteral nutrition, to obtain blood for analysis, or to provide an access point for blood-based treatments such as dialysis or apheresis. Access is most commonly achieved via the Seldinger technique, and guidance tools such as ultrasound and fluoroscopy can also be used to assist with visualizing access placement.
Arterial occlusion is a condition involving partial or complete blockage of blood flow through an artery. Arteries are blood vessels that carry oxygenated blood to body tissues. An occlusion of arteries disrupts oxygen and blood supply to tissues, leading to ischemia. Depending on the extent of ischemia, symptoms of arterial occlusion range from simple soreness and pain that can be relieved with rest, to a lack of sensation or paralysis that could require amputation.
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