Superior vena cava syndrome consists of a group of symptoms that develop due to disruption of blood flow in the superior vena cava caused by external compression or thrombosis of the vessel itself. SVPV is a manifestation of other diseases, in particular lung cancer. Therefore, when signs of superior vena cava syndrome occur, it is necessary to immediately consult a doctor and conduct a comprehensive examination. What is the cause of cava syndrome, symptoms and methods of treatment - in detail in the article.
Features of anatomy and provoking factors
The superior vena cava is located in the mediastinum. It is adjacent to the bronchi, chest wall, trachea, lymph nodes, and aorta. Damage to these organs or an increase in their size can cause compression of the said vessel and disruption of the outflow of blood from the upper part of the body, i.e., from the head, heart, lungs, and upper chest. Thus, when cava syndrome occurs, life-threatening conditions may occur. Manifestations of this disease most often occur in men between 30 and 60 years of age. Such patients are usually encountered by cardiologists, pulmonologists, phlebologists, and oncologists. The mechanisms of occurrence of cava syndrome are tumor invasion into the vessel wall, external compression, and thrombosis.
The most common cause of superior vena cava syndrome is lung cancer. However, tumor invasion into the wall of the superior vena cava can also be provoked by the following neoplasms:
- Sarcoma.
- Lymphoma.
- Melanoma.
- Tumors of the digestive organs located near the vein.
- Mammary cancer.
Non-tumor causes of cava syndrome are the following: retrosternal goiter, cardiovascular failure, infectious diseases, purulent mediastenitis, post-radiation fibrosis, constrictive pericarditis, idiopathic mediastinal fibrosis, sarcoidosis, mediastinal teratoma, prolonged placement of a catheter in the superior vena cava, aortic aneurysm. Compression of the terminal section of the superior vena cava can cause expansion of the inferior vena cava, the causes of obstruction of blood flow in which are also quite varied and no less dangerous to health.
Most often, the syndrome occurs due to lung cancer.
Main manifestations
The syndrome of impaired blood flow in the superior vena cava is characterized by three main syndromes: cyanosis, swelling, and an increase in the diameter of the superficial veins of the upper half of the body. Patients are concerned about shortness of breath at rest, difficulty swallowing, hoarseness, cough, swelling of the face and neck. All these symptoms are significantly worse at rest, so a person suffering from this disease tends to take a semi-recumbent position.
Symptoms of suffocation that develop due to swelling of the larynx occur somewhat less frequently. This may lead to respiratory failure. Due to impaired outflow of venous blood, cerebral edema may develop. In this case, symptoms such as headaches, convulsions, confusion, tinnitus, drowsiness, and loss of consciousness occur.
Symptoms of dysfunction of the oculomotor and auditory nerves, expressed in lacrimation, double vision, exophthalmos, auditory hallucinations, hearing loss, and tinnitus, develop somewhat less frequently.
Increased pressure in the venous system leads to nasal, esophageal and pulmonary bleeding. In addition, swelling of the collar area and upper extremities and cyanosis of the skin occurs. These symptoms are clearly visible in the photo.
Methods for identifying the disease
In addition to standard examination methods, including interviewing the patient, visual examination and laboratory tests, diagnostic methods such as:
- Radiography in two projections.
- Computer and magnetic tomography.
Additional research methods aimed at detecting the cause of the syndrome include: examination of the fundus, measurement of intraocular pressure, bronchoscopy, sputum analysis, ultrasound of the carotid and supraclavicular veins, sternal puncture. In case of emergency, diagnostic thoracoscopy and parasternal thoracotomy can be performed.
To identify the disease, you can use the phlebography method.
Therapy methods
Treatment of superior vena cava syndrome includes complex therapy aimed at eliminating the cause of this condition and alleviating the patient’s condition. This can be achieved using conservative and surgical methods.
Conservative treatment includes:
- Oxygen inhalations used for airway obstruction, as well as tracheostomy and tracheal intubation.
- For cerebral edema, diuretics and glucocorticosteroids are prescribed. If necessary, anticonvulsants are added to treatment.
- If a malignant neoplasm is present, treatment should begin with radiation therapy. When intercellular lung cancer is combined with lymphoma, chemotherapy is added to radiation therapy.
- Percutaneous placement of a stent allows you to cope with the lack of air.
If the cause of disruption of blood flow in the superior vena cava is a thrombus, then treatment is carried out with fibrinolytic drugs. If there is no effect from conservative treatment or if there is a significant deterioration in the patient’s health, surgical intervention is performed.
If the superior vena cava is compressed from the outside, then radical removal of the tumor is performed. If such surgical treatment is not possible, then surgery is performed for palliative purposes. Among the methods aimed at improving the patient's condition, the following are distinguished: bypass surgery, stenting, percutaneous endovascular balloon angioplasty, removal of part of the tumor for the purpose of decompression.
Thus, the syndrome resulting from impaired blood flow in the superior vena cava can manifest itself with several ambiguous signs at once. Therefore, it is important to know its main symptoms. Depending on the severity of the condition and the cause of its occurrence, the method of treatment is selected; accordingly, the earlier the disease causing this syndrome is detected, the more complications can be avoided.
Medicine is not considered an exact science and is based on many assumptions and probabilities, but not facts. Inferior vena cava syndrome is a fairly rare occurrence in medical practice. It can occur in men and women at any age, most often in the elderly. Pregnant women are the first at risk. In this case, the pregnant woman’s condition is characterized by polyhydramnios, venous and arterial hypotension. Most often the fruit is large. When a vein is compressed, blood flow to the liver, kidneys, and uterus is impaired, which negatively affects the development of the child. The condition can result in stratification of the placental tissue, and this is a huge risk of developing varicose veins and thrombophlebitis in the lower extremities. If childbirth is carried out by caesarean section, then there is a high probability of collapse.
The concept of the inferior vena cava
The inferior vena cava is a wide vessel. It is formed by the fusion of the left and right iliac veins located in the abdominal cavity. The vein is located at the level of the lumbar region, between the 5th and 4th vertebrae. Passes through the diaphragm and enters the right atrium. The vein collects blood that passes through neighboring veins and delivers it to the heart muscle.
If a person is healthy, then the vein works synchronously with the respiratory process, that is, it expands when exhaling, and contracts when inhaling. This is its main difference from the aorta.
The main purpose of the inferior vena cava is to collect venous blood from the lower extremities.
Why problems occur
According to statistics, approximately 80% of all pregnant women after 25 weeks experience compression of the vein, to a greater or lesser extent.
If there is no inferior vena cava syndrome, then the pressure in the vein is at a fairly low level - this is a normal physiological state. However, problems in the tissues that surround the vein can compromise its integrity and dramatically alter blood flow. For some time, the body is able to cope by finding alternative paths for blood flow. But if the pressure in the vein rises above 200 mm, then a crisis invariably occurs. At such moments, without urgent medical attention, everything can end in death. Therefore, you should know the inferior vena cava in order to call an ambulance in time if a crisis begins for the patient himself or someone close to him.
Clinical picture
The first thing you need to pay attention to is swelling, which can be on the face, neck, and larynx. This symptom is observed in 2/3 of patients. You may be bothered by shortness of breath, cough, hoarseness, even at rest and in a lying position, and this is a huge risk of airway obstruction.
Also, inferior vena cava syndrome may be accompanied by:
Pain in the groin and abdomen;
Swelling of the lower extremities;
Swelling on the buttocks and genitals;
Varicose veins of small vessels in the thigh area;
Impotence;
High body temperature;
Brittle nails and hair loss;
Constant pallor;
Problems with legs - it is difficult for the patient to move even short distances;
Bone fragility;
High blood pressure;
Silicosis;
Purulent mediastinitis;
Fibrosis.
Diagnostics
Naturally, compression of the inferior vena cava alone is not determined. A thorough diagnosis is required.
First of all, the doctor collects a complete medical history and conducts an examination. The condition of the veins in the neck and upper extremities can “tell” a lot; as a rule, they are dilated. A physical examination also gives an idea: the patient has cyanosis or plethora, whether the venous networks in the chest area are dilated, whether there is swelling, especially in the upper parts of the body.
X-ray examination and venography are also prescribed. X-ray examination can be carried out using a contrast agent. Magnetic resonance and computer topography, possibly spiral, is required.
In some cases, the diagnosis of inferior vena cava syndrome is accompanied by an in-depth examination by an ophthalmologist. The purpose of diagnosis is to identify, if present, dilation of the retinal veins, possible swelling of the peripapillary region, to determine whether intraocular pressure has increased, or whether there is congestion in the optic nerve.
For a complete picture you may need:
Bronchoscopy;
Biopsy of sputum and lymph nodes;
Sternal puncture;
Mediastinoscopy.
Therapeutic measures
Treatment of inferior vena cava syndrome is symptomatic. This pathology is still a concomitant disease, and first of all it is necessary to cure the underlying disease that caused the syndrome.
The main goal of treatment is to activate the body’s internal reserve forces in order to maximize the patient’s quality of life. The first thing that is recommended is a virtually salt-free diet and oxygen inhalation. It is possible that drugs from the group of glucocorticosteroids or diuretics will be prescribed.
If the syndrome appears against the background of tumor development, the approach to treatment is completely different.
Surgical intervention is indicated in the following cases:
The syndrome progresses rapidly;
There is no collateral circulation;
Blockage of the inferior vena cava.
Surgery does not eliminate the problems, but only improves venous outflow.
Syndrome and pregnancy
During the period of gestation, all the woman’s organs experience a heavy load, the volume of circulating blood increases and, as a result, stagnation appears. The uterus enlarges and compresses not only surrounding organs, but also blood vessels. In case of inferior vena cava syndrome in pregnant women, treatment should be carried out with extreme caution.
Problems begin with the fact that it is very difficult for a woman to lie on her back; usually this condition begins from the 25th week of gestation. There is slight dizziness, weakness, periodically lack of air. Blood pressure usually decreases. Very rarely, a pregnant woman may lose consciousness.
Naturally, we are not talking about drastic therapeutic measures during pregnancy, but some rules will still help to tolerate the syndrome easier:
You will have to give up all exercises that are performed in a supine position, on your back;
You should also not sleep on your back;
Nutrition should be adjusted to reduce salt intake;
It is necessary to reduce the amount of fluid consumed;
To improve the condition, it is better to walk more, in this case the muscles on the lower leg contract, and this process stimulates the upward movement of venous blood;
Prognosis and prevention
Doctors are optimistic about patients with the syndrome if it is detected at an early stage. The only condition is constant monitoring of the patient’s health and compliance with all recommendations of the attending physician.
Preventive measures include the prevention of cardiovascular diseases. If there are problems with blood clotting, then the pathology should also be under constant medical supervision, since such patients are at risk. You should give up even the thought of self-medication.
During pregnancy, a woman's body experiences significant stress. The volume of circulating blood increases, conditions for venous stagnation appear.
The growing uterus puts pressure on blood vessels and surrounding organs, causing disruption of the blood supply. One result of these changes is inferior vena cava syndrome. Its hidden manifestations are present in more than half of women, and clinically it manifests itself in every tenth pregnant woman. Severe cases of this disease occur in one in a hundred pregnant women.
Synonyms for this condition:
- hypotensive syndrome on the back;
- aortocaval compression syndrome;
- postural hypotensive syndrome;
- hypotensive syndrome of pregnant women in the supine position.
Why does this condition occur?
Inferior vena cava compression syndrome usually occurs when the pregnant woman is lying on her back. The inferior vena cava is a large diameter vessel through which venous blood is drained from the legs and internal organs. It is located along the spine. Its walls are soft, the pressure in the venous system is low, so the vein is easily compressed by the enlarged uterus.
Signs of such compression begin to occur periodically in the third trimester of pregnancy if the woman is in a supine position.
When this large vein is compressed, the outflow of blood through it to the heart is hampered, that is, venous return is reduced. As a result, the volume of blood passing through the lungs through the pulmonary circulation decreases. Blood oxygen saturation decreases, hypoxemia occurs.
Cardiac output decreases - the amount of blood ejected by the heart into the aorta. As a result of a small amount of blood and a reduced oxygen content in it, a lack of this gas occurs in all tissues - hypoxia. All organs of the woman and fetus suffer.
Suddenly, blood pressure drops quickly, in some cases to 50/0 mmHg. Art.
On the other hand, a compressed inferior vena cava cannot pass the entire volume of venous blood from the legs and lower torso to the right atrium. Therefore, venous congestion develops in the veins of the lower extremities.
In the development of inferior vena cava syndrome, an increase in intra-abdominal pressure due to the growing uterus, elevation of the diaphragm and compression of all major vessels of the abdominal cavity and retroperitoneal space are important. Many pregnant women develop a network of collaterals - bypass routes of venous outflow, as a result of which the syndrome in question does not occur in them.
How does the condition manifest?
The inferior vena cava is compressed by the enlarged uterus when the woman is lying on her back. At long gestation periods or with polyhydramnios, this can also occur in an upright position of the body.
The first symptoms appear at about 25 weeks. It becomes difficult for a woman to lie on her back, and she may experience dizziness, shortness of breath, and weakness. Blood pressure decreases. In some cases, even collapse with fainting occurs.
In severe cases, a woman quickly turns pale 2 to 3 minutes after turning on her back, complains of dizziness and darkening of the eyes, nausea and cold sweat. More rare signs are ringing in the ears, heaviness behind the sternum, a feeling of strong fetal movement.
Suddenly developing pallor and hypotension are very similar to signs of internal bleeding, so the doctor may mistakenly suspect such a pregnant woman has placental abruption, uterine rupture, etc.
The appearance of a vascular pattern is also associated with the described syndrome. One of the common manifestations of this condition is.
The described pathological condition leads to fetal hypoxia and disturbance of its heartbeat. The development of organs and systems of the unborn child suffers. If it occurs during childbirth, it can cause fetal asphyxia. The connection of this disease with premature detachment of a normally located placenta has been proven.
What to do in this condition
The optimal position for a pregnant woman during sleep is lying on her left side. What not to do in the third trimester of pregnancy:
- A pregnant woman over 25 weeks should not sleep on her back;
- It is forbidden to engage in physical exercises performed while lying on your back, including tensing the abdominal muscles.
- It is recommended to rest lying on your left side or in a semi-sitting position;
- It is useful to use special pillows for pregnant women, which are placed under the back or between the legs when lying on your side. Changing body position helps prevent compression of abdominal vessels by the uterus;
- To normalize venous outflow and improve hemodynamics, rational physical activity, especially walking, is recommended. While walking, the muscles of the legs actively contract, which helps move venous blood upward;
- Exercises in water are useful. Water has a compression effect, squeezing blood out of the veins of the lower extremities;
- During childbirth, the preferred position is lying on the left side or with the head end of the bed raised high.
Angiosarcoma is a malignant tumor. Vascular cancer is quite rare compared to other oncological diseases - approximately 3% of all diseases of this type. The disease is characterized by rapid development, rapid transition of metastases and their development. Vascular cancer is difficult to treat, and the prognosis is disappointing, especially if the disease is not at an early stage.
What it is
Vascular cancer is angiosarcoma, malignant neoplasms that very quickly begin to metastasize. They are located on the inner side of the vascular wall. Develops with equal frequency in men and women. Cancerous tumors most often occur on the skin, liver, breasts, brain and spleen.
The causes of its occurrence have not been fully studied; it is this factor that largely explains the impossibility of treatment with traditional types of therapy. The causes of development are considered to be exposure to arsenic, thorium dioxide, and ionizing radiation. Chronic lymphedema and mutational processes are also called as the cause of the tumor.
The main distinguishing feature of vascular cancer is that the disease develops rapidly. Metastasis occurs instantly, in most cases, when the patient begins to notice symptoms, the disease is at an advanced stage, therefore, cannot be treated.
Vein tumors are vascular cancer characterized by obstruction of blood flow in the vessels of the veins. In addition to the main factors, this is caused by blood clots in the vessels, as well as varicose veins.
Vein cancer is diagnosed independently only if the formation is located on the subcutaneous layer of the epithelium. In this case, you may notice a purple spot on the skin. But in most cases, vessels that are not on the surface are affected.
The impossibility of self-diagnosis and the late onset of symptoms (only when nerve fibers are compressed) make vein cancer a serious oncological disease that is practically untreatable.
Arterial cancer is a tumor on the carotid artery. It does not develop so quickly, it is often benign, but it also often and suddenly begins to metastasize. It appears only in an advanced state, then the symptoms are expressed in the sensation of a pulsating mass on the neck.
Classification
The malignant formation has a dense texture with an uneven contour; inside it is filled with voids with blood. The formations are permeated with small vessels and capillaries, and can affect any part of the human body.
The disease is classified not only by how quickly it spreads in the body, but also by its location and the areas of the body that are primarily affected.
Highlight:
- vascular cancer general;
- vascular cancer heads;
- breast cancer;
- cancer caused by radial irradiation;
- hemangioendothelioma.
Common vascular cancer is small nodules, bluish-violet in color, that can spread to any area of the skin. It is usually found on the legs, thighs, chest, and arms.
Over time, small nodules merge into a node. The usual cause of this type of cancer is disruption of the proper functioning of the lymphatic flow.
Cancer of the blood vessels of the head, idiopathic angiosarcoma, is characterized by the appearance of small compactions and nodules, which also eventually merge into a nodule. This cancer begins to metastasize very quickly, and the prognosis is negative in most cases.
It can affect both the area where there is hair, as well as the larynx, neck, pharynx, and tonsils. Mostly men (twice as often as women) over the age of 65 suffer from vascular cancer of the head.
Breast cancer appears in the form of small nodules, which can be pink, red, burgundy or blue in color. At the same time, the nodes are painful.
Over the course of several months, they transform into a tumor, which is surgically removed. Breast cancer, which is essentially vascular cancer, affects women between 35 and 45 years of age.
Cancer caused by radiation is called radiation cancer. Angiosarcoma rapidly spreads throughout the body and metastasizes instantly.
In terms of their manifestations, they are like ordinary vascular cancer, that is, the seals are small and do not cause discomfort. Localized on the chest, hips and abdomen.
Hemangioendothelioma is a tumor consisting of endothelial cells. The origin of the disease is practically unknown, and it is extremely difficult to treat.
Causes
The reasons for the appearance of malignant formations on the walls of blood vessels are not fully understood. However, the most common are:
- radiation, including those that were used as therapy in the treatment of other oncological diseases;
- mechanical injuries;
- mutation benign tumor.
It should be noted that angiosarcoma may appear as a result of training after several years. It can also form if a person regularly interacts with substances that can release polyvinyl chloride and arsenic.
Among other factors, vascular cancer can be caused by dysfunction of immune functions, vascular diseases, including varicose veins, and hemochromatosis.
Symptoms
The appearance of vascular cancer initially appears as small lumps, usually purple or bluish in color. However, they can be of a different color, especially if they are located on the mammary glands - red, pink or blue. After a certain period (each patient has a different term), these small compactions merge and form a single round-shaped ball of pronounced purple color.
Other signs of vascular cancer development:
- dysfunction liver;
- general weakness body;
- manifestation swelling;
- anemia;
- sharp jumps temperature;
- unconditional decline weight;
- nausea and vomiting;
- manifestation ulcerative diseases.
The symptoms on the list are not associated only with vascular cancer. It also occurs with the development of other diseases that are not necessarily related to cancer. Only a specialist after examination can make an accurate diagnosis and prescribe a treatment plan.
Diagnostics
Determination and diagnosis occurs in several stages. After the medical history, an examination is scheduled, during which the oncologist determines the size of the tumor and its consistency. Palpation required.
If there is a suspicion of cancer, then further diagnostic measures are carried out as follows:
- x-ray examination (degree of spread of metastases);
- MRI and CT scan (allows you to find out about the condition of tissues);
- biopsy(taking a small piece of tumor for analysis);
- Ultrasound;
- angiography(radiography is performed in this way);
- taking tests blood;
- detection of cancer markers.
The main method for this is biopsy. It is through clinical examination of a tissue sample that the diagnosis is confirmed or refuted. Other methods help determine the state of the blood and the spread of metastases.
Treatment
The initial stage, that is, the location of the tumor on the surface, in most cases is successfully treated. In this case, the tumor, if no metastases have appeared, is drained and sutures are applied. If the tumor is not located on the surface, then surgical removal of the entire limb is possible.
Surgical intervention is performed using modern techniques. Using computer technology, the optimal angle of amputation and the force of impact are calculated.
After surgery, the patient is prescribed radiation and chemotherapy. They help reduce the risk of relapse after surgery.
Detection of a neoplasm at the initial stage has a positive prognosis. At the same time, the picture worsens if the tumor has invaded the lymph node, metastases have appeared, and are located on the mammary glands and head.
After the tumor is removed, the patient is registered with an oncologist. In the first two years, visits to the doctor are scheduled once a quarter, after this period - once every six months.
During the examination, the specialist examines the skin of the lymph nodes and veins for the presence of an incipient disease (detects whether there are nodules or tumors). If necessary, lymph nodes are palpated. The patient also undergoes x-rays and tests. A biopsy is not required at this stage.
A detailed examination after surgery is necessary in order to promptly detect the spread of metastases and relapse of vascular cancer and prevent it.
Complications
Complications relate primarily to relapse and spread of metastases. A cancerous tumor, even if a limb is completely removed, may appear again. There is also a risk that the remaining metastases will begin to spread even faster.
Forecast
The prognosis for patients with vascular cancer is disappointing. Even a timely operation does not provide guarantees. Interventions are successful in 70% of cases, but as a result of relapse from vascular cancer, patients die within two years. Less than 10% of those who undergo surgery manage to live five years or more after it.
Prevention
There are no measures to prevent the development of the disease. But since the cause of cancer is contact with chemicals, radiation exposure, it can be understood that the prevention of pathology will be their exclusion.
Vascular cancer is difficult to treat, and the prognosis is disappointing. Even with a successful operation, the disease requires careful attention. At the slightest suspicion of relapse, you should immediately contact an oncologist.
1915 0
Development of ideas about superior vena cava syndrome (SVVC) can be conditionally divided into 4 periods.
The first period covers 1740-1930. Bartolinus in 1740 mentioned a case described by Riolanus, who observed the death of a patient due to occlusion superior vena cava (SVC)“a small piece of meat with shapeless fat at the mouth of the SVC.”
W. Hunter in 1757 described a syndrome that arose in a patient as a result of compression of the SVC by a syphilitic aortic aneurysm.
Sketches made during the autopsy suggest that this case is the first reliable report of SVPV. In the 19th century, interest in VTOL increased significantly, as evidenced by the increased number of works published on this topic.
Ducart C. (1828), Stannius and Duchan (1854), Franc F. (1869) in their works make the first attempts to systematize previously accumulated material, classify this disease according to etiology, pathogenesis and clinical picture, for the first time describe the veins of the anterior abdominal wall as paths possible collateral outflow of blood.
In 1914, the Russian scientist Wagner K.E. in the monograph “On the issue of narrowing of the superior vena cava” he gave the most complete description of the clinical picture of SVC occlusion, analyzing issues of anatomy, pathophysiology, topical and differential diagnosis based on 6 of his own observations and literature data. In the 20th century, the first attempts at surgical treatment of this disease were made. Sauerbruch (1912) performed a decompressive sternotomy, Waterfield (1928) successfully removed a thrombus from the SVC.
However, the general level of knowledge, diagnostic methods and surgical techniques did not allow us to begin extensive surgical treatment of SVPV. This period is characterized by the systematization of scattered data, the study of the etiology, pathogenesis and clinic of the superior vena cava syndrome, and the first experiments in its treatment.
The second period covers 1930-1950. and is associated with the emergence of phlebography in the arsenal of diagnostic techniques (Conte, Costa, 1933; Castellanos, Pereiras, 1947) and the experimental study of the pathogenesis and methods of treatment of SVPV (Carlson, 1934; Leo, Rundle, 1948; Romankevich V.M., 1949). In 1947 Katz, Hussey, Veal described in detail the technique of mediastinal venography, which makes it possible to determine the localization of the narrowing, its extent and identify the collateral pathways that have arisen.
The widespread introduction of this diagnostic method has significantly accelerated the development of surgical treatment of SVVC, facilitating the choice of surgical approach and type of operation. The experimental study of SVPV carried out during these years made it possible to expand information about the pathophysiological mechanisms of compensation and to develop surgical techniques that were later transferred to the clinic.
The third period (1950-1970) is characterized by extensive experimental development of prosthetic issues and plastic replacement operations for superior vena cava syndrome, and the first attempts at radiation treatment of SVVC. Numerous developments to replace the superior vena cava with synthetic prostheses have not lived up to expectations.
Tubes from the pericardium and peritoneum were subject to thrombosis in all cases (Collins, De Bakey, 1960); the use of aortic autografts required too cumbersome interventions (Moore, 1958); the use of autovenous grafts limited their use due to the discrepancy in the diameter of the vessels being sutured (Sampson, Scannel, 1951, 1954).
Evaluation of the quality of synthetic prostheses in the clinic and in experiments turned out to be very controversial. Most experimental studies have shown that prostheses made of Dacron, Ivalon, nylon, and Orlon are unsuitable (Ohara, Sakai, 1957; Lasenby, Howard, 1958, etc.).
However, there are isolated reports of the successful use of these materials in the clinic; Currently, about 25 types of operations have been proposed to unload the SVC basin; there are no clear indications or contraindications for surgical treatment for this syndrome. Operations for SVPV are characterized by high mortality and frequent complications, which does not allow the widespread use of the surgical method for the treatment of SVPV.
From the 70s to the present, priority in treatmentsuperior vena cava syndromebelongs to radiation therapy, which can be explained by the following factors:
1. In most cases, the cause of SVPV is malignant neoplasms. According to Lockich et al. (1975) a malignant tumor was detected in 90% of patients with SVPV. D. Schraufnagel et al. (1981) states that in general hospitals the proportion of “benign” causes of SVPV ranges from 10 to 25%, and in oncology clinics it is much less (0-3%).
2. radiation treatment is a “universal” technique - most tumors are sensitive to the effects long-term hormone therapy (DHT), while there is a minimal risk for the patient and a quickly onset effect.
Concluding a short essay on the development of the doctrine of superior vena cava syndrome, it should be noted that at present the problem of treating SVVC cannot be considered completely resolved.
Etiology and pathogenesis
The development of SVVC is caused by three main processes: compression of the vein from the outside, invasion of the vein by a malignant tumor, and thrombosis of the SVC. Zhmur V.A. (1960) divided all processes leading to occlusion of the SVC into the following groups: compression, stricture, obstruction and mixed.According to Savelyev V.S. et al. (1977) the most favorable prognostically is the development of venous obstruction as a result of compression, because Moreover, in all patients, the patency of the vessel was restored after removal of the tumor. Obstruction and stricture of the vein leads to gross morphological changes in the vessels, and therefore clinical manifestations are usually more pronounced.
Zhmur V.A. (1960) proposed to classify the etiological factors leading to the development of SVPV as follows:
A. Tumor processes
1. malignant
Bronchogenic cancer of the right lung
thymus tumors
thyroid tumors
lymphomas
pericardial tumors
2. benign
Thymomas
substernal goiter
mediastinal cysts, etc.
B. Tumor-like processes
1. aortic aneurysm
2. damage to the lymph nodes
B. Inflammatory processes
1. primary thrombosis of the SVC
2. fibrous mediastinitis
3. lymphadenitis
4. pericarditis
An idea of the frequency of various etiological factors in superior vena cava syndrome is given in table. 11.1.5.1, based on data from 111 observations (Mazhorov V.A.)
Table 11.1.5.1. Causes of SVPV
According to our own data, the causes of SVPV were: lung cancer (86%), lymphoproliferative tumors (12%), other tumors (2%). The anatomy of the SVC adequately explains the clinical picture caused by obstruction or compression of this vessel.
It drains blood flow from the head, neck, chest wall and upper extremities, with the lower half of the vessel surrounded by a fibrous layer of the pericardium, limiting its mobility.
The SVC is particularly vulnerable to obstruction because it is a thin-walled, low-pressure vessel that is confined to a tight space surrounded by lymph nodes, trachea, bronchi, and aorta.
Manifestations of SVVC are caused by venous hypertension in the area of the drained SVC. Moreover, they are directly dependent on the degree of localization and speed of obstruction, as well as on the degree of development of collaterals.
Components of superior vena cava syndrome, in addition to increased venous pressure, are: slowing of blood flow, development of venous collaterals, symptoms associated with the disease that caused obstruction
ERW.
Clinic
All clinical signs of SVPV are separated by A.N. Bakulev. (1967) into 2 groups:1. Symptoms resulting from venous congestion in the superficial and deep veins of the face and neck (classic triad of SVPV)
A. Swelling of the face, upper half of the torso and upper extremities (noted most often; in severe cases, swelling can spread to the vocal cords and lead to asphyxia.
B. Cyanosis caused by dilation of venous and narrowing of arterial capillaries. Sometimes, against the background of cyanosis of the mucous membranes, an earthy-pale coloration of the facial skin is noted, caused by concomitant lymphostasis
B. Dilatation of the saphenous veins of the neck, upper half of the body. The degree of this expansion and its nature are an important sign in the topical diagnosis of the level of occlusion of the SVC and its relationship to the mouth of the azygos vein.
One of the striking clinical symptoms of a disorder of venous outflow is nasal, esophageal and tracheobronchial bleeding, resulting from rupture of thinned vein walls (Friedberg, 1948). In contrast to portal hypertension, with SVC occlusion, varicose veins are localized in the proximal esophagus (Sheiner, 1969).
During physical activity, fatigue occurs quickly, and your hands get tired especially quickly; performing even light physical work becomes impossible due to a rush of blood to the head. The feeling of palpitations, pain in the heart area, and a feeling of compression behind the sternum are caused by impaired blood supply to the myocardium and swelling of the mediastinal tissue.
2. Symptoms that are a manifestation of venous stagnation in the brain:
A. General cerebral symptoms - headache, which is sometimes paroxysmal in nature; shortness of breath with attacks of suffocation of central origin, resulting from “tissue stasis of the brain” (Altshuler, 1945) with increased intracranial pressure. Long-term impairment may be aggravated by swelling of the vocal cords and larynx (Allansmith, 1958).
B. Symptoms associated with a violation of cortical neuroregulation: drowsiness, emotional fatigue, attacks of dizziness with loss of consciousness - are signs of chronic brain hypoxia, developing as a result of circulatory disorders. One of the severe manifestations of cortical neuroregulation disorder is confusion and auditory hallucinations.
B. Symptoms associated with dysfunction of the cranial nerves. Tinnitus, hearing loss and diplopia are caused by a disorder of the auditory and oculomotor nerves. Tearfulness, decreased visual acuity, increased intraocular and intracranial pressure. In our opinion, for a more complete description of the patient’s condition and the reliability of the clinical picture, it is necessary to identify a third group of symptoms caused by the underlying disease (weight loss, cough, hoarseness, hemoptysis, etc.).
In the Chelyabinsk Regional Oncology Center during 1976-2000. There were 253 patients with SVPV. Clinical manifestations are presented in Table 11.1.5.2.
Table 11.1.5.2. Clinical manifestationssuperior vena cava syndrome
Classification
Currently, there is no single, generally accepted classification of SVPV according to the severity of clinical manifestations. The classifications used in various clinics are based, as a rule, on three indicators or a combination of them (the level of venous pressure, the degree of narrowing of the SVC, detected angiographically, and patient examination data).Meanwhile, Lebedev V.A. (1971) at the Moscow Oncology Institute named after. P.A. Herzen convincingly proved the absence of strict parallelism between the degree of occlusion of the main veins of the mediastinum, indicators of venous pressure and other clinical symptoms of SVPV. SVC occlusion may be manifested by only one increase in venous pressure in the absence of other symptoms of SVVC. At the same time, with complete occlusion of the SVC, venous pressure may not exceed 170 mm water column.
The author explains this by the fact that the height of venous pressure, as well as the severity of other clinical symptoms, has a direct connection with the time factor, i.e. the duration and degree of development of collaterals, the ability of the latter to function. The level of occlusion in relation to the mouth of the azygos vein is also of known importance. The most severe circulatory disorders occur when the SVC is blocked above or at the level of the azygos vein entering it.
Consequently, venous pressure indicators cannot serve as reliable criteria for the degree of occlusion of the main veins of the mediastinum.
The most widely used classifications are those of B.V. Petrovsky. (1962) and Bakulev A.N. (1967).
According to the classification of Petrovsky B.V., according to the severity of venous pressure in the upper extremities, all patients can be divided into 3 groups:
1 (weak) degree - venous pressure up to 150-200 mm water column.
2 (medium) degree - from 250 to 300 mm water column.
3 (severe) degree - over 300 mm water column.
Classificationsuperior vena cava syndrome(Bakulev A.N.):
1. Impairment of the patency of the superior vena cava of the 1st degree.
Patients are periodically bothered by headaches and shortness of breath, sometimes in the morning a puffy face appears, which completely disappears during the day. Performance is fully preserved. The color of the skin is normal, but when bending forward, swelling of the face appears.
Moderate expansion of the superficial veins only in the anterosuperior part of the chest wall. Venous pressure is within 200-250 mm water column. Angiography reveals a narrowing of the lumen of the innominate or superior vena cava by 1/2-2/3 of the diameter.
2. Impaired patency of the superior vena cava of the 2nd degree.
Patients are bothered by headaches, puffiness of the face (persists throughout the day), hands swell during physical activity, and when bending forward - a sharp rush of blood to the head. Efficiency drops sharply.
On examination, the face is puffy, the neck is swollen, and there is mild cyanosis of the mucous membranes and skin of the face. The veins of the neck and anterior chest wall are expanded to the level of the 3-5 ribs. Venous pressure is within 250-350 mm water column. Angiographic examination reveals narrowing of the SVC with preservation of patency.
3. Impairment of the patency of the superior vena cava of the 3rd degree. Complaints of paroxysmal headaches, sometimes drowsiness, dizziness. There is a feeling of compression in the chest area and palpitations, shortness of breath with attacks of suffocation, rapid physical and mental fatigue.
Hemoptysis, nasal and esophageal bleeding occur periodically. The veins of the neck are dilated and tense, and the venous network extends to the lateral surfaces of the chest wall and the back, telangiectasias are visible. Cyanosis of the face and upper half of the body is pronounced. Venous pressure ranges from 300 to 400 mmH2O. Angiography reveals complete occlusion of the SVC.
4. Impairment of the patency of the superior vena cava of the 4th degree.
Cyanosis and shortness of breath are more pronounced. The expanded venous network extends to the anterior abdominal wall. Attacks of suffocation are repeated several times a day, nosebleeds and hemoptysis also become regular.
Patients report tinnitus, dizziness, periodic fainting, drowsiness, and seizures. Patients exhibit hoarseness due to swelling of the vocal cords, dysphagic disorders, as well as asphyxial conditions requiring emergency care. Venous pressure - 400 mm water column. and higher.
Diagnostics
In the diagnosis of SVPV, two stages can be distinguished:1. Primary diagnosis in non-specialized institutions.
An initial examination and anamnesis allow a presumptive diagnosis to be made when the patient first consults a doctor. The classic clinical picture of SVPV (swelling of the face, neck in combination with dilated saphenous veins of the chest, increased symptoms in a horizontal position) and X-ray data in frontal and lateral projections, often showing an additional shadow in the chest, allow you to correctly diagnose and refer the patient to a specialized department.
2. Clarifying diagnostics in specialized institutions, aimed at morphological confirmation of the diagnosis. The need for morphological verification is determined by the choice of the optimal treatment regimen - radiation, chemotherapy, their combination, or (if the SVPV is benign) - surgical treatment.
Due to their serious condition, patients cannot always undergo the necessary diagnostic tests before starting treatment. During radiation therapy, it can also be difficult to verify the diagnosis morphologically due to the rapid regression of the tumor and the development of radiation tissue pathomorphosis.
Indications for further diagnostic procedures are determined taking into account radiological data. Indication for fibrobronchoscopy (FBS) The topographic-anatomical localization of the process is considered (central cancer of the right lung, the presence of enlarged lymph nodes in the tracheobronchial, paratracheal groups).
In the presence of affected peripheral lymph nodes, a biopsy was performed followed by histological examination. In the absence of verification based on FBS data and biopsy of peripheral lymph nodes, a diagnostic mediastinotomy was performed.
Angiography, as a method for diagnosing superior vena cava syndrome, is currently used extremely rarely. This study allows us to identify the location and degree of obstruction of the SVC and is performed in large vascular centers that use various types of plastic surgery to treat SVVC. In oncology clinics, angiography has not found practical use in diagnosing SVPV.
The effectiveness of the diagnostic procedures performed at the second stage of the examination can be assessed by the proportion of morphologically verified diagnoses.
Patients with SVPV admitted to the Chelyabinsk Regional Oncology Center were divided into 3 groups according to the severity of their general condition (general clinical classification was used). Tables 11.1.5.3 and 11.1.5.4 illustrate the effectiveness of diagnostic measures depending on the severity of the patient’s condition.
Table 11.1.5.3. The effectiveness of diagnostic measures depending on the severity of the patient’s condition 
Table 11.1.5.4. The effectiveness of various diagnostic methods depending on the severity of the patient’s condition 
As you can see, the ability to perform diagnostic procedures and their effectiveness are inversely related to the severity of the patient’s condition.
Treatment
Currently, the treatment of superior vena cava syndrome remains one of the most complex and controversial problems in oncology. Considering that SVPV in the vast majority of cases is caused by a malignant process (90-97% - Trakhtenberg A.H., 1988), all types of special methods are used in its treatment - surgical, radiation, chemotherapy. Let's look at the advantages and disadvantages of each of them.Priority in choosing a treatment method is most often given to radiation therapy. Since 1963, the main discussions have been around two schemes. Some authors consider it advisable to carry out radiation treatment in small doses - 1-1.5 Gy per day (Slow lowdose) throughout the entire course radiation therapy (RT)(Simpson J.R., Presant C.A., 1985).
Others hold the opposite point of view, believing that it is more effective to start RT with a dose of 4 Gy, followed by a dose reduction to 1.5-2 Gy - (Rapid High-dose) (Davenprot D., Ferree C., 1976). Proponents of the first regimen consider it inappropriate to use high doses due to the risk of “radiation edema,” which can lead to increased obstruction.
Proponents of the second scheme provide data on the possibility of progression of the tumor process (including SVPV) at low doses of radiation, and the atypicality of “radiation edema”. We were unable to find any randomized trial data in the literature to support the superiority of one of these regimens.
Thus, the universality of application is considered to be the positive qualities of radiation therapy in the treatment of SVPV; DHT is effective for various histological types of tumors, minimal risk for the patient, rapid onset of effect.
Negatives include a wide range of contraindications to DHT (general severe condition, tumor disintegration, hemoptysis, leukopenia, etc.). If complications arise during treatment (an increase in the superior vena cava syndrome against the background of “radiation edema”, intoxication syndrome, etc.), DHT is interrupted for a long time or is stopped altogether.
Chemotherapy (CT) has found widespread use in the treatment of SVPV caused by small cell lung cancer and malignant lymphoproliferative diseases. According to Motorina L.I. (1989), SVPV occurs in 6-11% of patients with small cell lung cancer.
During chemotherapy, it was possible to achieve complete disappearance of clinical symptoms of SVC compression in 62% of patients, partial disappearance in 38%. Considering that SVPV occurs, as a rule, in stages 3-4 of the disease, it can be assumed that the lesion is systemic and that the process extends beyond the thoracic cavity.
In this case, systemic therapy is fundamentally more justified than radiation therapy. Maddox A. et al. (1983) observed distant metastases in 70% of patients. Dombernavsky P. et al. received a response to chemotherapy in 75% of patients, and in addition - a significant improvement in survival compared to RT.
Many authors note that radiation therapy and chemotherapy often successfully complement each other, successfully combining the positive aspects of each method. Motorina L.I. et al (1989) note that the percentage of complete disappearance of clinical symptoms of the superior vena cava syndrome after the appointment of RT to patients who underwent chemotherapy increased from 62 to 93.
The issue of using a surgical method in the treatment of SVPV causes the most controversy. Since 1912, about 25 types of operations have been proposed to unload the SVC basin, but there are no clear indications and contraindications for surgical treatment for this syndrome.
Proponents of surgical treatment consider the rapid progression of the process and the absence of compensated collateral circulation to be absolute indications, and severe concomitant pathology and old age to be contraindications, calling shunt surgery the only effective method of reducing pressure in the SVC system.
Opponents argue their point of view by the initial unresectable nature of the process and the possibility of using conservative treatment methods (RT and chemotherapy). It is an indisputable fact that the positive effect appears already in the first hours after the shunt is applied. Choosing the type of surgery for SVPV is a difficult task.
According to Mazhorov V.A. (1989) only in 17.3% of patients it is possible to perform intrathoracic bypass bypass, in 30.6% of patients - extrathoracic bypass with large-diameter prostheses. It should be noted that, according to the literature (Klioner et al., 1970), with tumors of the lungs and mediastinum, the intrathoracic anastomosis is quickly involved in the process and ceases its function.
A negative point when performing surgical intervention is the need for a long horizontal position of the patient and intubation anesthesia, which leads to increased symptoms of SVPV and increases the risk of intraoperative complications.
In some large foreign clinics, X-ray endovascular prosthetics of the superior vena cava are increasingly used. The endovascular prosthesis provides expansion from the inside of the narrowed section of the vessel, creates an internal supporting frame of the wall, preventing the development of restenosis, keeping the lumen of the superior vena cava from compression from the outside and allowing long-term preservation of normal blood flow. This technique is of particular value in the absence of effect from radiation therapy, chemotherapy, or in case of recurrence of the superior vena cava syndrome after treatment.
The method of installing a shunt is quite simple in technical execution, but requires a lengthy and expensive examination of the patient before surgery. The technique is characterized by a fairly high cost, which is unlikely to allow it to be widely used in the treatment of SVPV.
We present data from our own analysis of 253 cases of SVPV (1976-2000), and the treatment tactics developed on its basis. A fruitful development of this problem turned out to be possible after the creation of the ChOOD on the initiative of prof. Vazhenina A.V. Center for Oncoangiosurgery (headed by Prof. Fokin A.A.).
Among the patients there were 222 (87.7%) men and 31 women (12.3%). Under the age of 40 years - 20 (7.9%), 40-49 years old - 36 (14.2%), 50-59 years old - 97 (38.4%), 60-69 years old - 85 (33.8 %), 70 years and older - 15 (5.9%) people. The most common cause of SVPV was lung cancer, diagnosed in 219 (86.6%) patients, with stage 3 diagnosed in 134 (52.7%), stage 4 in 83 (33.1%) patients. As we see, most patients are not subject to radical treatment due to the prevalence of the tumor process.
All patients admitted to the hospital with superior vena cava syndrome underwent emergency radiation therapy using the “rapid high-dose” regimen. In parallel, diagnostic measures were carried out aimed at clarifying the prevalence of the process and obtaining morphological verification of the process.
To objectively assess the effectiveness of diagnosis and treatment, depending on the severity of the patient upon admission, patients were divided into 3 groups: mild, moderate and severe (general clinical classification was used).
Group 1 (satisfactory condition) - 124 patients
Group 2 (moderate condition) - 114 patients
Group 3 (severe condition) - 14 patients.
The analysis allowed us to draw the following conclusions:
1. The success of diagnostic measures and the effectiveness of treatment depend on the severity of the patient’s condition (see tables 11.1.5.3 and 11.1.5.4).
2. There is a significant group of patients - 62 people (24.5%), whose treatment according to the traditional regimen is regarded as unsatisfactory due to the absence or insignificant positive effect against the background of the ongoing RT.
The results of this analysis forced us to look for a more effective treatment regimen for patients with severe SVPV and to supplement radiation treatment with surgical intervention. Patients in the first group (satisfactory condition) usually tolerate DHT well. SVPV is successfully terminated.
Patients whose radiation treatment was repeated mostly belong to the second and third groups. Thus, objective prerequisites have been created for the use of palliative surgery, which allows reducing pressure in the SVVC system and continuing radiation therapy in patients with severe superior vena cava syndrome.
In our attempts, we sought to find an operational aid: one that does not require a lengthy preoperative examination; minimally damaging the existing collateral connections; does not represent a high degree of risk associated with prolonged intubation of the patient; and even if it fails (shunt thrombosis) it does not aggravate the patient’s condition.
Initially, we settled on the operation of applying an external anastomosis connecting the basin of the superior and inferior vena cava, which was first proposed by Schramel et al (1961). The large saphenous vein of the thigh is used as a graft, to isolate which a longitudinal incision is made from the inguinal ligament to the ankle. Then a section of the internal jugular vein is exposed and a subcutaneous tunnel is formed on the anterolateral surface of the chest and abdomen.
Next, the large saphenous vein of the thigh is passed through the tunnel and an end-to-side anastomosis is performed with the jugular vein. Due to the small diameter of the veins, there is a need for bilateral bypass surgery. In 1999, we performed 2 such operations; SVPV was stopped within 2436 hours, radiation therapy was started on the sixth day after surgery. One of the patients died on the 7th day after surgery from acute myocardial infarction.
This operation has many negative qualities:
1. Major trauma when isolating the saphenous veins of the thigh and leg;
2. The saphenous veins are not always suitable for bypass surgery due to thrombosis and loose type of structure; often turn out to be short for anastomosis with the jugular vein, which requires the use of an additional vascular insert. In addition, the difference in the diameters of the vessels being stitched is quite large.
In 1999, 2 operations were performed using the bovine internal mammary artery as a graft, which significantly reduced the trauma of the operation, shortened the time the patient spent under endotracheal anesthesia, and increased the diameter of the shunt. The average duration of the operation is 80 minutes.
In the early postoperative period, 1 patient died (the cause of death was acute cerebrovascular accident). Improvement in condition was noted 2-3 hours after surgery; radiation therapy was carried out starting 3 days after surgery.
Despite the positive aspects, this operation has serious disadvantages: the difficulty of placing the graft under the skin of the lateral surface of the body, the need for intervascular anastomoses; The traumatic nature of the operation requires endotracheal anesthesia.
Initially, a palliative operation of temporary extracorporeal shunting from the SVC basin to the inferior vena cava system was performed according to a technique developed at the Oncoangiosurgery Center of the Chronological Oblast. Indications for surgery were: the patient's condition is moderate or severe, the presence of neurological symptoms, and rapid progression of SVPV.
The essence of the operation is to apply a shunt in the form of a plastic tube between catheters installed in the v. basilica and v. saphena magna. The operation is performed under local anesthesia, does not require a horizontal position of the patient, the average duration is 20 minutes.
Positive qualities of the proposed operation: minimal trauma, technical simplicity, does not require special instruments and equipment, easy replacement of the shunt in case of thrombosis, performed under local anesthesia. The positive effect manifested itself within 30-60 minutes after surgery.
1.0-1.5 hours after surgery, a course of DHT was started: the first three sessions with a single focal dose of 4 Gy, followed by a decrease to 1.5-2.0 Gy, reaching 30-60 Gy. Patients in satisfactory condition received DHT without previous surgery.
According to this scheme in 1999-2001. 33 patients received treatment. The condition was assessed as satisfactory in 14 (39.4%), moderate - in 17 (49.5%), severe - in 4 (12.1%), indications for surgical treatment (extracorporeal bypass) were given to 20 patients, two of which refused the proposed operation.
The operation was performed on 18 patients. A positive effect was observed in all patients within 2-5 hours after surgery. SVEP was completely stopped in all patients on days 4-7 from the start of treatment.
Conclusions:
1. The surgical and radiation components in the proposed technique are complementary; the method combines the positive aspects of each of them.
2. The proposed technique allows for quick and effective relief, including in patients in serious condition, and does not interfere with the emergency start of radiation therapy.
3. The technique is the least traumatic and accessible of all previously proposed; The operation is performed under local anesthesia.
In conclusion, it should be noted that there is currently an urgent need for a randomized study of the role and place of each method in the treatment of SVPV.
Novikov G.A., Chissov V.I., Modnikov O.P.
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