Superior vena cava syndrome (Mediastinal syndrome) | |
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Other names | SVC obstruction [1] |
Superior vena cava syndrome in a person with bronchogenic carcinoma. Note the swelling of his face first thing in the morning (left) and its resolution after being upright all day (right). | |
Specialty | Pulmonology |
Superior vena cava syndrome (SVCS), is a group of symptoms caused by obstruction of the superior vena cava ("SVC"), a short, wide vessel carrying circulating blood into the heart. The majority of cases are caused by malignant tumors within the mediastinum, most commonly lung cancer and non-Hodgkin's lymphoma, directly compressing or invading the SVC wall. Non-malignant causes are increasing in prevalence due to expanding use of intravascular devices (such as permanent central venous catheters and leads for pacemakers and defibrillators), which can result in thrombosis. [2] Other non-malignant causes include benign mediastinal tumors, aortic aneurysm, infections, and fibrosing mediastinitis. [3]
Characteristic features are edema (swelling due to excess fluid) of the face and arms and development of swollen collateral veins on the front of the chest wall. Shortness of breath and coughing are quite common symptoms; difficulty swallowing is reported in 11% of cases, headache in 6% and stridor (a high-pitched wheeze) in 4%. The symptoms are rarely life-threatening, though edema of the epiglottis can make breathing difficult, edema of the brain can cause reduced alertness, and in less than 5% of cases of SVCO, severe neurological symptoms or airway compromise are reported. Resolution of superior vena cava syndrome is directly related to the treatment of the underlying compression. [4]
Shortness of breath is the most common symptom, followed by face or arm swelling. [5]
Following are frequent symptoms:
Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness. [5]
Over 80% of cases are caused by malignant tumors compressing the superior vena cava. Lung cancer, usually small cell carcinoma, comprises 75–80% of these cases and non-Hodgkin lymphoma, most commonly diffuse large B-cell lymphoma, comprises 10–15%. Rare malignant causes include Hodgkin's lymphoma, metastatic cancers, leukemia, leiomyosarcoma of the mediastinal vessels, and plasmocytoma. [3] Syphilis and tuberculosis have also been known to cause superior vena cava syndrome. [5] SVCS can be caused by invasion or compression by a pathological process or by a deep vein thrombosis in the vein itself, although this latter is less common (approximately 35% due to the use of intravascular devices). [5]
The main techniques of diagnosing SVCS are with chest X-rays (CXR), CT scans, transbronchial needle aspiration at bronchoscopy and mediastinoscopy. [6] CXRs often provide the ability to show mediastinal widening and may show the presenting primary cause of SVCS. [6] However, 16% of people with SVC syndrome have a normal chest X-ray. CT scans should be contrast enhanced and be taken on the neck, chest, lower abdomen, and pelvis. [6] They may also show the underlying cause and the extent to which the disease has progressed. [6]
Several methods of treatment are available, mainly consisting of careful drug therapy and surgery. [5] Glucocorticoids (such as prednisone or methylprednisolone) decrease the inflammatory response to tumor invasion and edema surrounding the tumor. [5] Glucocorticoids are most helpful if the tumor is steroid-responsive, such as lymphomas. In addition, diuretics (such as furosemide) are used to reduce venous return to the heart which relieves the increased pressure. [5]
In an acute setting, endovascular stenting by an interventional radiologist may provide relief of symptoms in as little as 12–24 hours with minimal risks.[ citation needed ]
Should a patient require assistance with respiration whether it be by bag/valve/mask, bilevel positive airway pressure (BiPAP), continuous positive airway pressure (CPAP) or mechanical ventilation, extreme care should be taken. Increased airway pressure will tend to further compress an already compromised SVC and reduce venous return and in turn cardiac output and cerebral and coronary blood flow. Spontaneous respiration should be allowed during endotracheal intubation until sedation allows placement of an ET tube and reduced airway pressures should be employed when possible.[ citation needed ]
Symptoms are usually relieved with radiation therapy within one month of treatment. [5] However, even with treatment, 99% of patients die within two and a half years. [5] This relates to the cancerous causes of SVC found in 90% of cases. The average age of disease onset is 54 years. [5]
Veins are blood vessels in the circulatory system of humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are those of the pulmonary and fetal circulations which carry oxygenated blood to the heart. In the systemic circulation, arteries carry oxygenated blood away from the heart, and veins return deoxygenated blood to the heart, in the deep veins.
Edema, also spelled oedema, and also known as fluid retention, dropsy, hydropsy and swelling, is the build-up of fluid in the body's tissue. Most commonly, the legs or arms are affected. Symptoms may include skin which feels tight, the area may feel heavy, and joint stiffness. Other symptoms depend on the underlying cause.
Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare.
The superior vena cava (SVC) is the superior of the two venae cavae, the great venous trunks that return deoxygenated blood from the systemic circulation to the right atrium of the heart. It is a large-diameter (24 mm) short length vein that receives venous return from the upper half of the body, above the diaphragm. Venous return from the lower half, below the diaphragm, flows through the inferior vena cava. The SVC is located in the anterior right superior mediastinum. It is the typical site of central venous access via a central venous catheter or a peripherally inserted central catheter. Mentions of "the cava" without further specification usually refer to the SVC.
Cerebral edema is excess accumulation of fluid (edema) in the intracellular or extracellular spaces of the brain. This typically causes impaired nerve function, increased pressure within the skull, and can eventually lead to direct compression of brain tissue and blood vessels. Symptoms vary based on the location and extent of edema and generally include headaches, nausea, vomiting, seizures, drowsiness, visual disturbances, dizziness, and in severe cases, death.
The azygos vein is a vein running up the right side of the thoracic vertebral column draining itself towards the superior vena cava. It connects the systems of superior vena cava and inferior vena cava and can provide an alternative path for blood to the right atrium when either of the venae cavae is blocked.
Pemberton's sign was named after Hugh Pemberton, who characterized it in 1946.
A Pancoast tumor is a tumor of the apex of the lung. It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae. Most Pancoast tumors are non-small-cell lung cancers.
The jugular veins are veins that take deoxygenated blood from the head back to the heart via the superior vena cava. The internal jugular vein descends next to the internal carotid artery and continues posteriorly to the sternocleidomastoid muscle.
Inferior vena cava syndrome (IVCS) is a very rare constellation of symptoms resulting from either an obstruction, or stenosis of the inferior vena cava. It can be caused by physical invasion or compression by a pathological process or by thrombosis within the vein itself. It can also occur during pregnancy. Pregnancy leads to high venous pressure in the lower limbs, decreased blood return to the heart, decreased cardiac output due to obstruction of the inferior vena cava, sudden rise in venous pressure which can lead to placental separation, and a decrease in kidney function. All of these issues can arise from lying in the supine position during late pregnancy which can cause compression of the inferior vena cava by the uterus. Symptoms of late pregnancy inferior vena cava syndrome consist of intense pain in the right hand side, muscle twitching, hypotension, and fluid retention.
Renal vein thrombosis (RVT) is the formation of a clot in the vein that drains blood from the kidneys, ultimately leading to a reduction in the drainage of one or both kidneys and the possible migration of the clot to other parts of the body. First described by German pathologist Friedrich Daniel von Recklinghausen in 1861, RVT most commonly affects two subpopulations: newly born infants with blood clotting abnormalities or dehydration and adults with nephrotic syndrome.
May–Thurner syndrome (MTS), also known as the iliac vein compression syndrome, is a condition in which compression of the common venous outflow tract of the left lower extremity may cause discomfort, swelling, pain or iliofemoral deep vein thrombosis.
Traumatic asphyxia, or Perte's syndrome, is a medical emergency caused by an intense compression of the thoracic cavity, causing venous back-flow from the right side of the heart into the veins of the neck and the brain.
A mediastinal tumor is a tumor in the mediastinum, the cavity that separates the lungs from the rest of the chest. It contains the heart, esophagus, trachea, thymus, and aorta. The most common mediastinal masses are neurogenic tumors, usually found in the posterior mediastinum, followed by thymoma (15–20%) located in the anterior mediastinum. Lung cancer typically spreads to the lymph nodes in the mediastinum.
Vascular disease is a class of diseases of the vessels of the circulatory system in the body, including blood vessels – the arteries and veins, and the lymphatic vessels. Vascular disease is a subgroup of cardiovascular disease. Disorders in this vast network of blood and lymph vessels can cause a range of health problems that can sometimes become severe, and fatal. Coronary heart disease for example, is the leading cause of death for men and women in the United States.
Compression stockings are a specialized hosiery designed to help prevent the occurrence of, and guard against further progression of, venous disorders such as edema, phlebitis and thrombosis. Compression stockings are elastic compression garments worn around the leg, compressing the limb. This reduces the diameter of distended veins and increases venous blood flow velocity and valve effectiveness. Compression therapy helps decrease venous pressure, prevents venous stasis and impairments of venous walls, and relieves heavy and aching legs.
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.
Congenital stenosis of vena cava is a congenital anomaly in which the superior vena cava or inferior vena cava has an aberrant interruption or coarctation.
Cancer pain can be caused by pressure on, or chemical stimulation of, specialised pain-signalling nerve endings called nociceptors, or by damage or illness affecting nerve fibers themselves.
Raghib syndrome is rare a congenital heart defect where the left superior vena cava (LSVC) is draining into the left atrium in addition to an absent coronary sinus and an atrial septal defect. This can be considered a dangerous heart condition because it puts the individual at a high risk of stroke. Other defects that are often associated with Raghib syndrome can include ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. While this is considered an extremely rare developmental complex, cases regarding a persistent left superior vena cava (PLSVC) are relatively common among congenital heart defects. It is also important to note that the PLSVC often drains into the right atrium, and only drains into the left atrium in approximately 10 to 20% of individuals with the defect.