Chylothorax formed at break thoracic duct and entered lenii chyle in the pleural cavity.
Pathophysiological characteristics
Consumption of oil in a large part remains nerasscheplen nym in the form of chylomicrons into the bloodstream for about breast current. First, the fat enters the chylous vessels of the intestine, and then into the cisterna chyli , which is a lymphatic mass on the body of the second lumbar vertebra. Usually from cyster na chyli departs one large lymphatic vessel, the thoracic duct, which through the esophageal aperture enters into the chest cavity. Thoracic duct rises ekstraplev eral at the back of the mediastinum along the right side of the ne Independent user spine surface located between Nepara hydrochloric cava and descending aorta near the esophagus and perikar house. At the level of IV-VI of the thoracic vertebrae thoracic duct ne intersects spine, turning left and several extension ryayas, enters the upper part of the mediastinum between the aortic arch and the left subclavian artery from the esophagus.
At the exit of the upper opening of the chest it bending etsya at a distance of 3-5 cm above the clavicle and rotates KPE mong towards the subclavian and vertebral arteries and the thyroid gland, resulting in the left jugular and subclavian veins. All sections of the thoracic duct can have a large number of anatomical variants. More than one thoracic duct can move away from cisterna chyli . The duct can be raised along the right side of the spine and with the unifying vein right subclavian area. Between cereals GOVERNMENTAL lymphatic vessels usually there are many anastomoses, it is possible to shunt lymphovenous unpaired vein
On the thoracic duct is the movement of hilus. Mack macroscopic chyle similar to opalescent milky liquid which, if given to settle, typically separates camping into three layers: the upper, cream containing chylomicrons , medium, milky, and the lower containing cellular elements, mostly small lymphocytes . One to if the patient does not eat, chyle can only be insignificant but cloudy as the lipid content will be reduced. Hilus has bacteriostatic properties, it does not proish dit development of infection, even if it is left for several weeks at room temperature . Lampson reported that no growth of Escherichia coli and Staphylococcus aureus was observed in 100% hilus . Chyle, penetrated into the pleural of smallness, not irritate you and do not usually binds education is moored or the fibrous sheath.
During the day, 1500-2500 ml of hylus enters the venous system . Over the next few hours, fat intake may increase the flow of lymph into the thoracic duct by 2 to 10 times compared with the resting state . Fluid intake also increases the formation of chyle, while protein and carbohydrates only slightly affect the quantitative in lymph . The protein content of chyle is typically greater than 3 g / 100 ml, and its electrolyte composition is the same as that syvo ROTKO blood .
Chylothorax is developing at break lymph proto ka. In experimental animals the ligation of the thoracic about the current in any part of it is not ra ot education chylothorax . This is probably due to the presence of a large number of collaterals and lymphovenous anastomoses. Ligation of the superior vena cava in experimental animals in 50% of cases ve children to the formation of chylothorax .
Etiological factors
In tab. 11 presents the causes of chylothorax formation in 143 patients in 5 different groups of observations . For convenience, the main reasons can be divided into four categories. More than 50% of cases the formation of chylothorax associated with tumor related approximately 75% of SLE teas to lymphomas . In fact, chylothorax can be considered one of the symptoms of lymphoma . Proceeding from this, the presence of a non-traumatic origin of the patient with chylothorax It is an indication for a close examination of the patient in order to detect lymphoma . Roy et al . in 4 cases revealed
The second of the major causes of chylothorax are etsya injury ; This is usually surgery on the heart and blood vessels. Very often chylothorax occurs in patients who le was mobilized during the operation Vai subclavian artery . Overview Maloney and Spencer 2660 cardiovascular operations showed that 0.5% SLE teas developed chylothorax . In the same series of observations Bower incidence of chylothorax after cardiac surgery amounted Vila 0.24%. There are reports of the occurrence of chylothorax as a complication of high translumbus aortography and dissection of the cervical node . Education chylothorax so well may be associated with the subclavian vein thrombosis [IIJ- frequency chylothorax therefore likely to be vozras thief because subclavian Vienna increasingly used in cardio-pulmonary monitoring.
Undoubtedly, a penetrating wound of the chest or neck, such as a knife or a gunshot, can cause rupture of the thoracic duct and the formation of chylothorax . In case of injury, with accompanied by hyperextension or spine fracture, the likelihood of developing chylothorax is great, especially if it happened soon after a fatty meal . Chylothorax , formed as a result of closed trauma, usually right -sided, and the area of the gap is most often at the level of TIÕ –TX . Such an injury can be caused by a fall from a height, car accident, trunk compression, severe with a blow to the back or stomach and childbirth. Damage mo Jette be less significant cases reported hee lotoraksa caused by coughing, vomiting, and weight lifting. It has been reported that bilateral chylothorax , accom pany bulging in the left supraclavicular fossa, was caused by the fact that the person with the power yawned and stretched .
The third main reason for the formation of chylothorax is idiopathic. This group includes most cases of congenital chylothorax . Lymphoma in this group should be excluded as non-idiopathic zabole ments. In adults, in most cases chylothorax of idiopathic nature is the result of small herbs we, for example, when coughing or hiccups that occur after at EMA fatty foods. Chylothorax – the most common type of pleural effusion in infants in developing trans stems days of life , but still rarely meet ongoing: in 1967 , only 37 cases were registered . Chylothorax develops in term infants born to uncomplicated labor. The causes of congenital chylothorax are not known. In those few children who underwent test thoracotomy, no anomalies of the thoracic duct were detected . It is possible that the rupture of the weak thoracic duct was caused by an increase in the venous pressure of the fetus during labor.
In the literature, there is evidence of many other causes of chylothorax , but even if all summarize them, they make up only a small percentage of the total number of cases hilotorak sa . The most interesting of these rare causes of chylothorax is pulmonary lymphangiomyomatosis , accompanied by interstitial infiltrates of the parenchyma (discussed later in this chapter). Chylothorax may be a complication of filariasis, enlarged lymph nodes, cirrhosis, serdech insufficiency, limfangiita thoracic duct, anevriz we thoracic aorta, lymph vessels anomalies, for example measures in cases lymphangiectasia AI or reticular hyperplasia .
Clinical picture
The first symptoms of chylothorax are usually caused by the presence of fluid in the chest cavity, which leads to the development of shortness of breath. Pleural pain and fever are rarely observed, since chyle does not irritate the pleura. In traumatic chylothorax usually observed latency period (2-10 days) from the time of injury to form plevu eral effusion . After the rupture of the thoracic duct prois goes Extrapleural accumulation of lymph in the mediastinum, thereby forming a heel , which manifests itself as the volume Noe formation in the rear part of the mediastinum . Can proi zoyti gap mediastinal pleura, resulting in the chyle enters the pleural cavity, causing compression easy to go and a sudden shortness of breath. In some cases, the breakthrough of the chyloma into the pleural cavity is accompanied by hypotension, cyanosis and pronounced shortness of breath . After breaking through the chyloma radiologically, it is no longer visible.
When nontraumatic chylothorax symptoms usually nachi nayut be gradual. With congenital chylothorax in a newborn in the first days of life, respiratory distress (respiratory distress ) develops . In 50% of cases, the symptoms appear in the first 24 hours, by the end of the 1st week of the symptoms observed are given in 75% of patients .
The main threat to the patient in cases of chylothorax for consists in the development of depletion due to power failure. By Since on the thoracic duct daily fed 2500 ml of liquid containing large amounts of protein, fats, electrolytes, and lymphocytes, as a result of daily withdrawn out such an amount of chyle when draining or repeated thoracentesis patient may ensue rapidly exhausted. In addition, lymphopenia develops and the immunological status is disturbed. Until 1948 , when Lampson [3] successfully wasp exists ligation thoracic duct, lethality sickly thorax was 50%. When managing patients with chylothorax should go from conservative treatment to surgeons cal before violation develop immunological status and exhaustion.
Diagnostics
Establishing a diagnosis of chylothorax is not difficult, since chyle is a white, milk-like, odorless liquid. When such a fluid is detected, it is mainly required to differentiate the chylothorax from empyema and pseudochilothorax . In cases of empyema milky pleural fluid caused in suspension lei Cocytus. After centrifuging such a liquid, the supernatant will be clear. Turbidity hilezopodobnogo pleural Nogo effusion caused by high lipid – hall sterol or letsitinglobulinovyh complexes. When chylous and chyle-like pleural effusions; the pleural fluid remains turbid after centrifugation.
If the turbidity of the pleural fluid is caused by the presence of cholesterol crystals, it is easy to see them when analyzing the sediment of the centrifuged pleural fluid (Fig. 43). If the cause of turbidity pleural fluid – high cholesterol, then adding it disappears in about tag 1-2 ml of ethyl ether; if the liquid remains turbid, it means that it contains chylomicrons or lecithin complexes .
Determination of triglycerides. The best way to diagnose chylothorax is to determine the content of triglycerides (see Fig. 22). If the content of triglycerides in the pleural fluid exceeds 110 mg / 100 ml, the patient is likely chylothorax , if it is below 50 mg / 100 ml, then pain Nogo have chylothorax . If the triglyceride content is between 50 and 110 mg / 100 ml, it is necessary
Large polyhedral crystals are typical of cholesterol containing effusions. The patient has pleural effusions of rheumatoid etiology.
research of pleural fluid lipoproteins. If the identified chylomicrons , then confirmed the diagnosis hilot Rax [4]. Some patients with psevdohilotoraksom contains zhanie triglycerides in the pleural fluid exceeds 250 mg / 100 ml [17]. Differentiation between chylothorax and pseudochilothorax is usually not difficult. When chylothorax slaughtering Levan begins acutely, and the pleura is not changed, when psevdohilotorakse due to the long existence of pleural effusion, pleural thickening is observed [17]. If they remain There are doubts about the diagnosis, it is necessary to study the pleural fluid for the presence of chylomicrons .
When chylothorax pleural fluid is not always ha acteristic milky color. In cases of congenital chylothorax, at first, pleural fluid will be serous and become chyle-free only after milk starts feeding the baby [14]. By Since chylothorax is the most likely kind of Pleven eral effusion in newborns , definition contains Nia triglycerides in the pleural fluid and study whether poproteidov needed in all newborns with pleural effusion.
In adult patients with chylothorax, pleural fluid does not always look like typical hyle. Staats et al . [4] reported that in 20 (53%) of 38 cases of chylothorax, pleural fluid was not chyle. However, in all these 20 cases, the pleural fluid was bloody or turbid. For SET Lenia diagnosis such pleural fluid should be centrifuged . If the supernatant remains turbid, it is necessary to determine the content of triglycerides in the pleural fluid.
Use lipophilic dye. Another test for the diagnosis of chylothorax is a fatty meal with a lipophilic dye and subsequent (after 30-60 min) vypol nenie thoracentesis to determine whether the color has changed plevu tral fluid [181. The most commonly used dye is Drug and Cosmetic Green N6, made on the basis of a coal mole. One gram of this dye is thoroughly mixed with ^ 4 pounds (113.39 g) of butter, and then spread the mixture onto bread. After 30-60 minutes after the patient has eaten this sandwich, produce thoracocentesis . When chylothorax received naya liquid will be green in color. We applied this test in 6 patients. At the same time it was most difficult before for us to lay downthe patient to eat that disgusting green mass. Since the diagnosis of chylothorax can usually be easily established based on the definition of lipoproteins and triglycerides, we no longer use this test if you suspect chylothorax .
Treatment
Chylothorax represents a danger to the patient, because the repeated thoracentesis and drainage of pleural Lost excreted displayed a large number of proteins, Ms moat, electrolytes and lymphocytes, which can lead to ISTO scheniyu patient and immune deficiency. In the last scrap of approximately 50% of cases of chylothorax led to le tal outcome. When managing patients with chylothorax , use effective measures, such as ligation of the thoracic duct, and apply them before Noah will become too exhausted to undergo surgery. Since the approach to the management of patients with different types of chylothorax is the same, we consider separately the treatment of pain GOVERNMENTAL traumatic, non-traumatic and congenital sickly thorax .
Traumatic chylothorax . Treatment of patients with traumatic skim chylothorax is aimed at the elimination of shortness of breath in the midst of stvom removal chyle, preventing dehydration, secu chenie supply and reduction of the rate of chyle. Vyve denie chyle is best done by draining IU Todd torakostomii . Since chyle is a bacteriostatic fluid , the likelihood of developing intrapleural infection during drainage is small. Thanks to the drainage, the lung is always in a straightened state, which is Includes the probability of mooring. Drainage OJEC ensures, contact between the visceral and parietal pleura and may contribute pleurodesis . In addition, drain vanie accurately determine the arrival rate hee Lusa.
In traumatic chylothorax often occurs spontaneously Noah defect closure of the thoracic duct. The likelihood of closure is higher with a minimum amount of hilus. In the latter case the tea also reduces the loss of nutrients and electro litas. The intake of chylus is reduced when cessation of oral ingestion and constant aspiration of the contents of the stomach, so that the gastrointestinal tract is as clean as possible . In addition, the patient must comply with bed nymode, because any movement of the lower limbs Uwe lichivaet flow of lymph .
Fatal in case of chylothorax is usually caused by depletion eat, dehydration or infection development caused them munologicheskoy failure. It is therefore necessary adequacy Noe powered patient best by parenteral hyperalimentation . As a source of fat is recommended to use medium chain triglycerides , as it was observed that the medium chain triglycerides (ten or less carbon atoms) are absorbed into the portal vein and the tread in the blood stream directly, and not via the thoracic duct. If a patient with chylothorax receives oral nutrition, then fats should certainly be in the form of triglycerides from media. her chain. Since the chyle flow is minimal, if the gastrointestinal tract is idle, patients with hilotorak catfish are advised to keep on parenteral nutrition, singularity but given the fact that currently in most medical centers have considerable experience in parenteral Foot Power by hyperalimentation .
In most cases the traumatic chylothorax at blizitelno through 7 days after the administration of the amount of generated chyle drainage is considerably reduced or completely stopped. If, after 7 days of drainage selection hee Lusa does not stop and does not decrease, it is a display Niemi to more intensive treatment of the patient. Adier and Levinsb reported 2 patients with pleural obliteration of Lost and the elimination of chylothorax have been achieved as a result of intrapleural injection of talc .
If after 7 days chyle selection is not terminated, in order to ensure pleurodesis recommended drainage through the Introduction STI into the pleural cavity of tetracycline in the dose of 20 mg / kg, YaV -governing effective sclerosing agent . Me todika administration of tetracycline is the same as in cancerous n th pleural effusion . If for some then where our doctor refrained from the use of tetracycline, and if after the introduction of tetracycline selection chyle continues, consideration should be given a trial thoracotomy with ligation of thoracic duct.
For the first time the effectiveness of ligirovatsiya thoracic duct in le chenii chylothorax was shown Lampson . Probably, due to a plurality of anastomoses between different lymph ical vessels and shunts lymphovenous ligation thoracic duct has no adverse effect on the patient .
When unilateral chylothorax thoracotomy should be about the plague on the side of the effusion , with bilateral-on the right side, as on this side of the thoracic duct to more stages .
During the operation, you need to find the location of the thoracic duct perforation and produce ligation on both sides de Fecteau 1191. However, in many cases, the defect can not be detected, so it is recommended ligation of the thoracic duct. To facilitate intraoperative identification of the defect piles special technique was developed by Foot but the best method is the introduction of Evans blue at a dose of 0.7-0.8 mg / kg in subcutaneous tissue legs, the total dye dose should not exceed 25 mg. After a 5 min chyle will turn first luboy color . Behind 3-4 hours before the operation, the patient can be given to eat butter or sour cream. However, in this case, before Anesti Zia stomach may be filled, then it contains zhimoe removed through a nasogastric tube by Aspira tion .
If thoracotomy for any reason fails to ligate the thoracic duct, in order to pleural obliteration of the body cavity should be made pleurectomy .
If the flow of hilus along the drainage does not stop, and the patient becomes more and more exhausted, thoracotomy cannot be postponed. In one series of observations of all 3 patients with herbs cally chylothorax , who underwent thoracotomy, died in the postoperative period . The lethal outcome was caused by the exhaustion of patients at the time of the operation.
Nontraumatic chylothorax . Treatment of patients with netravma cally chylothorax pursues the same objectives as the treatment of patients with traumatic chylothorax . However, it is necessary to establish the cause of non-traumatic chylothorax . As can be seen from the table. II, the most common cause of nontraumatic of chylothorax is a lymphoma . Because the current BPE on me lymphoma can be treated with radiation and chemotherapy, should make every effort to identify her. Very often in patients with lymphoma and chylothorax no other Symp toms lymphoma . For the detection of lymphadenopathy of the mediastinum all patients with non-traumatic chylothorax need computed tomography of the mediastinum. Women should be the races to watch the probability of pulmonary lymphangiomyomatosis if there are I also infiltrates in the parenchyma.
Initial activities at nontraumatic chylothorax should be the same as in traumatic hilotorak se . The patient is administered drainage zholudochno -kishechny tract proportion women to be free, and power is provided by parenteral hyperalimentation . If chylothorax caused insignificant tion injury, a week later, these measures usually ending are the recovery of patients. In the event that the computer naya imaging mediastinum revealed no lymphadenopathy or other bulk process, the treatment consists in Dren tion. In group of 35 patients with chylothorax induced tumors, in any case, treatment with the core nirovaniya pleural and repeated punctures was not successful nym . If after 7 days of drainage, the intake of hilus does not stop or a tumor is detected during computed tomography, a trial thoracotomy is recommended to the patient. In vre mja operation mediastinum carefully examined in order to identify Lenia tumors, particularly lymphomas , and ligated piles hydrochloric duct.
If it is known that a patient has lymphoma or metastatic cancer, drainage and trial thoracotomy are not indicated, since in such patients, chylothorax responds well to treatment by irradiating the mediastinum. Roy and colleagues reported that in 68% of cases of lymphoma and 50% of cases of metastatic cancer of the beam -hand mediastinal therapy will eliminate chylothorax for the remainder of the period of life of the patient. If radiotherapy and chemotherapy do not ensure the elimination of chylothorax in these patients, then probably the test thoracotomy is also not indicated due to a clearly poor prognosis . However if chylothorax gives clinical symptoms should try produ STI thoracostomy with the administration of sclerosing agents, for example tetracycline.
Congenital chylothorax . Chylothorax in young children, WHO Rasta can be fatal. Mortality rate was 30% in the group of 10 patients with congenital hilot Rax . Death was the result of exhaustion and secondary Noah infection in all patients, thoracentesis they performed bo Lee 14 times. In 5 children (50%) after 1-3 thoracentesis relapse Islands chylothorax were observed. All 7 surviving patients no abnormalities have been identified. In connection with the data presented in this work, we can recommend the following scheme for the management of children with congenital chylothorax . First, the child should be treated conservatively, applying for vtornye thoracentesis . If after the third thoracentesis observation given recurrence, enter the drainage at the base of a light back and start parenteral nutrition. If, over the next three days, the drainage of the hilus does not stop, or there is a relapse when switching to oral nutrition, then a thoracotomy trial with ligation of the thoracic duct should be considered .
Pulmonary lymphangiomyomatosis
Lymphangio myomatosis of the lungs is a rare disease characterized by widespread proliferation of smooth muscle fibers in the peribronchial, perivascular and perilymphatic areas of the lung . For 1977 , there were only 67 cases of this disease . In most cases, perilymphatic proliferation when lead to lymphatic obstruction and chylothorax . In this disease may also be observed infiltration of mediastinal lymph nodes and retroperitoneal pro immature smooth muscle cells, which can cause even greater impairment of lymphatic drainage. Piles hydrochloric duct in such cases can be obliterated or races panded . The germination of smooth muscle fibers in the perivascular space can cause obstruction of the venules of the lungs and lead to pulmonary hemorrhage, hemoptysis and hemosiderosis of the lungs. Proliferation peribronchial gladkomy antiplaque fibers can cause partial or complete obstructive tion of the respiratory tract, the formation of cysts and bullae, as well as a significant number of cases of pneumothorax .
Clinical picture. Lymphangiomyomatosis lung OS novnom common in women of reproductive age . Symptoms can occur between the ages of 18 to 70 years, more often – from 25 to 50 years. The majority of patients zabole vanie shown increasing shortness of breath, and in some cases s – hemoptysis, pneumothorax can occasionally be detected chylothorax . Almost all patients during Zabolev Nia formed infiltrates in the parenchyma, in 75% of patients – chylothorax and 40% – pneumothorax .
Lymphangiomyomatosis light sometimes is one of about phenomena tuberous sclerosis. It is a rare disease before Vai genetically characterized by three features: dropping ki, skin lesions (sebaceous adenoma) lagging and mental lethargy. Only in a small number of patients with tuberous sclerosis occurs with lung damage. Clinical manifestations of time to zheniya lungs at tuberous sclerosis lungs are the same as when lymphangiomyomatosis lungs. Pathological given nye and radiographic picture is identical in both slaughtering Levan. However, in tuberous sclerosis with involvement of the lungs lymph nodes are less frequently observed and chylothorax does not often develop .
Diagnosis lymphangiomyomatosis can be assumed to Ba Vania radiological data. On chest X-ray can detect reticulonodular changes that Kie same as in fibrosing alveolitis . However, unlike the fibrosing alveolitis , and nearly all other interstitial lung damage if lymphangiomyomatosis lay some increased in size, and is not reduced [27]. Interstitial infiltrates in the lung can be of different sizes, they are located both diffusely and basally. In patients with chylothorax, the pleural effusion is unilateral and bilateral. Nij usually extensive and recurrent, macroscopically – chylous. Studies of lung function in patients limfan giomiomatozom reveal normal or reduced vital capacity with increased total lung capacity. Typically, when there are signs of moderate or severe dysfunction obstructive ha raktera and reduce the diffusion of carbon monoxide . Analysis hectares Lost Girl shows hypoxia and hypocapnia.
Diagnosis . Lymphangiomyomatosis light should zapodoz rit every woman between the ages of 25 to 50 years hilotorak catfish , especially in the presence of interstitial infiltrates and an increase in lung volume. Diagnosis is at OPENING that thoracotomy based on histological identification of five pichnoy immature extensive proliferation of smooth muscle cells. When thoracotomy be take tissue samples for analysis of lease content progesteronestrogennyh receptors prichi not, which will be discussed later in this section. Macro scopic lung defined diffuse interstitial thickening and cystiform space different times measure how subpleural , and in the parenchyma . Microscope cally most typical extensive proliferation of smooth muscle fibers in areas with normal lymphatic network. This perilymphatic proliferation consider camping rather as gamartomatozny rather than a neoplastic process . The space between the bundles of smooth muscle fibers is lined by endothelium. Microscopically in the affected lymph cal nodes observed the same changes as in the lay FIR, t. e. proliferation of interwoven bundles gladkomyshech GOVERNMENTALfibers separated portions endothelium .
Treatment . In general, the prognosis for patients limfangiomiomato Zoom is not encouraging, as the majority of the pain GOVERNMENTAL live about 10 years after the onset of symptoms, but in some cases – 15 years or more . Although pleurodesis and ligation of the thoracic duct can ensure the elimination of chylothorax , patients nevertheless die from progressive respiratory failure . Some authors vyskaza whether the assumption that for a given disease can be proved hormonal therapy . Since the disease susceptible women of childbearing age only, smooth muscle proliferation, probably it has hormonal Ba Woo. McCarty and colleagues reported a case limfangiomioma toza at which the affected area harakterizova tissue were high affinity for the progestin , causes its strong binding . After treatment, medroxyprogesterone (400 mg per month intramuscularly) remission for bolevaniya. Kitzsteiner and Mallen examined the lung tissue of a single patient with lymphangiomyomatosis and found no estrogen receptors. The condition of this patient improved after the removal of the ovaries. Banner et al . Reported a similar case . Thus, some patients WHO action on the hormonal status may have a beneficial effect on the disease. If a patient is suspected of having lymphangiomyosis , samples of lung tissue should be taken to detect progesterone and estrogen receptors. When they are found, the patient should be treated with progesterone or estrogen in pharmacological doses. In the absence of such receptors in a patient, the issue of ovary removal should be considered.