Fibrothorax

Diseases of the pleural cavity are a common complication that develops after various bronchopulmonary pathologies. One of these complications may be fibrothorax - we are talking about an obliterating fibrous process in the pleural cavity. Obliteration is formed due to the accumulation of fibrous mass released from liquid pleural secretions, as well as fibrinous lumps in hemothorax.

Fibrothorax can be the result of exudative pleurisy (in particular, tuberculous), pleural empyema, various injuries or operations on the chest.

To put it simply, fibrothorax is the filling (overgrowth) of the pleural cavity with a high probability of further ossification. Normal sliding of the pleural sheets is hampered, and the lungs lose the ability to fully open during inhalation. The process is accompanied by severe pain, and can spread to the lung tissue: in this case, the organs inside the chest are able to shift to the affected side. [ 1 ]

Epidemiology

Respiratory pathologies are among the most common groups of diseases. Over the past few years, their share has grown by about 10%. Experts attribute this increase in morbidity to the deterioration of the socio-economic situation and ecology, a decrease in the standard of living, and increased migration.

In the vast majority of cases, fibrothorax occurs as a complication of other diseases. The frequency in the structure of morbidity as a whole is estimated at approximately 2-4%. Among patients of surgical clinics, this figure may be somewhat higher, however, to date, such statistical data are not available. [ 2 ]

Causes fibrothorax

Fibrothorax in the vast majority of cases develops as a complication of other pathologies of the respiratory system and, in particular, the pleura. Thus, the initial causes of fibrothorax are often:

• inflammation of the pleura (dry or exudative);
• empyema of the pleura;
• aseptic pleurisy with inflammation of the pancreas;
• tumor process (mesothelioma);
• oncological metastases;
• penetrating chest injuries;
• pulmonectomy (total or partial);
• pleural tuberculosis;
• pleurisy resulting from autoimmune processes.

Initial signs of fibrotic disorder usually appear several months after the underlying disease. For example, fibrothorax after pulmonectomy can develop approximately 6-18 months after surgery.

As we have already noted, fibrothorax is almost never encountered as an independent pathology, but rather as a complication of other diseases. Such provoking diseases can be both pulmonary disorders and other diseases that do not cause direct damage to lung tissue.

Risk factors

Fibrothorax is a complex and multifactorial disease. The most significant predisposing factors for the development of pathology are:

• long-term or frequent inflammatory pathologies of the respiratory system;
• metabolic disorders (primarily diabetes mellitus, obesity);
• old age;
• cardiac insufficiency, dysfunction of the left ventricle;
• acute renal failure, acute stages of chronic renal failure; [ 3 ]
• patients who are on hemodialysis;
• pathologies of the peripheral vascular network;
• lung surgery (in particular, pulmonectomy).

The above points refer to the so-called general risk factors.

In addition, the risk of developing fibrothorax increases with respiratory failure, prolonged artificial ventilation and a long stay in the intensive care unit.

Pathogenesis

Fibrothorax is the obliteration of the pleural cavity by adhesive adhesions and fibrous connective tissue, as a result of which the respiratory system loses the ability to perform its functions. Most often, the problem occurs as a late consequence of pulmonectomy.

Another common cause of pathology development is considered to be inflammatory pleural processes - in particular, empyema, hemothorax. As a result of such reactions, massive pleural adhesions are formed, and the size of the lung is significantly reduced due to pleurogenic fibrosis.

The pleural cavity of a healthy person is an anatomical slit-like space limited by the parietal and visceral pleural sheets (serous membrane). Normally, this space should not contain any inclusions. However, under the influence of certain etiological factors, pathological changes occur in the pleura, which entails increased formation of fibrosis and filling (fusion) of the cavity.

Symptoms fibrothorax

The symptoms of fibrothorax are non-specific, which makes it difficult to diagnose the disease. In general, the clinical picture may be reflected in the following symptoms:

• Signs of pleural damage:
  • chest pain;
  • decrease in the volume of the affected side of the chest, respiratory delay on one half;
  • recession of the spaces between the ribs.
• Signs of respiratory failure:
  • difficulty breathing even with minimal physical activity;
  • cyanosis of the fingertips and nails, as well as the nasolabial triangle area;
  • increased fatigue, feeling of weakness.

The severity of symptoms may vary, depending on the extent of pleural damage and the presence of complications in the form of respiratory failure. Most often, we are talking not about one, but about several obvious signs at once, which form the corresponding syndrome.

Fibrothorax syndrome as a whole is a set of symptoms that are united by one pathogenetic mechanism. Combinations of such symptoms may vary within the above-mentioned manifestations.

Initial manifestations of fibrothorax may be as follows:

• the patient complains of intrathoracic pain, which intensifies when coughing, as well as shortness of breath of varying intensity;
• In severe fibrothorax, the patient experiences tachycardia, dizziness, chest pain, pale skin, and dark circles under the eyes;
• If fibrothorax develops against the background of an infectious and inflammatory process, then there is an increase in temperature, chills, general weakness, and signs of intoxication.

Regardless of the extent of the disease progression, all patients experience breathing problems and chest pain to varying degrees. However, these symptoms cannot be called specific, so an X-ray examination or CT scan is required to establish a diagnosis.

Chest changes characteristic of fibrothorax

Chest examination	Sinking and respiratory delay of the affected side, drawing in of the spaces between the ribs at the moment of inhalation.
Palpation examination	Vocal tremor is weak or (–).
Percussion	A dull or blunt sound.
Auscultation	Breathing is weak or not audible, pleural noise is present, bronchophony is weak or (–).
X-ray	The fibrothorax lesion appears as a homogeneous darkening.

• Right fibrothorax

Fibrothorax can equally often occur on the left and right. In the case of a right-sided pathological process, the leading symptom is often shortness of breath - both during physical activity and at rest. As the disease progresses, other symptoms appear, such as tachycardia, swelling of the lower extremities, swelling and pulsation of the venous vessels of the neck, pain behind the breastbone on the right side. Patients complain of severe weakness, fatigue, and inability to exercise.

• Left-sided fibrothorax

Left-sided localization of fibrothorax often requires differential diagnostics with diseases of not only the respiratory system, but also the cardiovascular system. Patients may complain of pain in the heart, radiating to the left shoulder or left side of the abdomen.

It is not uncommon for left-sided fibrous lesions to have rapid heartbeat and shortness of breath. The pain usually worsens during coughing, sneezing, and sudden body movements.

Forms

Fibrothorax can be classified according to a number of features that depend on a certain criterion.

Depending on the causative factor, the disease can be:

• postoperative (as a result of pulmonectomy);
• pathological (due to another underlying disease).

Depending on the development of the inflammatory process, fibrothorax can be:

• increasing;
• stable.

If we take into account the localization of the pathology, then the following types of fibrothorax are distinguished:

• apical;
• interlobar;
• paracostal;
• supradiaphragmatic;
• paramediastinal.

Idiopathic, or primary fibrothorax, is a type of disease that occurs without an apparent cause.

Complications and consequences

The main adverse effects of fibrothorax are:

• chronic respiratory failure (oxygen deficiency in the body);
• pulmonary hypertension;
• chronic pulmonary heart disease;
• secondary infection and development of the inflammatory process in the lungs.

During the development of the preceding inflammatory process and fibrothorax, the vascular network and heart may shift to the side. This condition threatens to increase chest pressure and disrupt cardiac blood flow. In this situation, it is important to prevent the development of cardiopulmonary insufficiency: if organ displacement is detected, the patient is recommended to undergo emergency surgery.

The probability of adverse effects and complications depends on the degree and prevalence of fibrothorax. When pathology is detected at late stages, they often speak of unfavorable further developments. Timely referral to doctors and adequate treatment increases the patient's chances of relative recovery, namely, inhibition of fibrosis processes. However, even a recovered patient needs regular medical supervision.

Diagnostics fibrothorax

The main research methods for suspected fibrothorax are the following:

• Basic methods:
  • general examination of the patient;
  • examination of the chest area;
  • palpation method;
  • percussion examination;
  • listening (auscultatory method).
• Additional methods:
  • chest x-ray;
  • blood and sputum tests.

Fibrothorax is usually characterized by patient complaints of shortness of breath, chest pain. During a general examination, attention is drawn to the paleness of the skin, cyanosis. [ 4 ]

During a medical examination, a recession of the affected side of the chest, rapid shallow breathing, and a lag of the affected side during deep inhalation are detected.

What percussion sound appears with fibrothorax? As a rule, dullness of percussion sound is noted.

On auscultation, breathing is weak and vesicular, or cannot be heard at all. Pleural friction noise is noticeable. Bronchophony is weak or absent.

Palpation reveals a sharp weakening of vocal fremitus, or its absence. [ 5 ]

Blood and sputum tests do not show any specific changes.

Instrumental diagnostics are represented mainly by plain radiography or tomography of the chest cavity. In order to differentiate fibrinous accumulations and fluid secretions in the pleura, some patients undergo pleural puncture. This diagnostic procedure involves performing a puncture with pumping out the intracavitary contents. The material is then sent for laboratory testing for the presence of infectious signs, atypical cells or fibrin threads. [ 6 ]

The scale of further measures depends on the results of the initial diagnosis.

• Fibrothorax on x-ray

The X-ray picture of fibrothorax is displayed as a homogeneous shadow. There is a reduced and to varying degrees intense darkening of the affected lung field, a high position of the diaphragmatic dome, obliterating closure of the costophrenic sinuses, and a shift of the mediastinal organs to the affected side. The appearance of pleural calcification zones is possible.

Thickening of the pleural surfaces and areas with formed adhesions are noticeable.

Radiological signs of fibrothorax may be supplemented by tuberculous foci in the lungs, pleura, and bone tissue. Sometimes it is possible to record the presence of calcifications – dense deposits of calcium salts. [ 7 ]

Differential diagnosis

Fibrothorax should be distinguished from the following pathologies:

• focal or lobar compaction of lung tissue;
• cavities in the lung tissue;
• obstructive or compression atelectasis;
• hydrothorax;
• pneumothorax;
• exudative bronchial constriction;
• bronchial obstruction;
• respiratory distress syndrome.

Treatment fibrothorax

It is impossible to cure fibrothorax with medications, regardless of the stage of the disease. There are no medications that can dissolve fibrous growths and cleanse the pleural cavity, restoring the functionality of the pleura.

However, medications for fibrothorax are prescribed primarily with the aim of influencing the underlying cause of the problem.

For example, if the disease was preceded by a chronic microbial disease, then it is appropriate to prescribe antibiotics in accordance with the resistance of the identified bacteria.

In case of intense inflammation, non-steroidal anti-inflammatory drugs can be used.

At the initial development of the tumor, chemotherapy and other appropriate treatment are prescribed, depending on individual indications.

In the case of an initial inflammatory process of rheumatic or other autoimmune etiology, the use of corticosteroids is appropriate.

If the underlying cause of fibrothorax is no longer relevant or cannot be determined, then with a stable deterioration in the dynamics of the pathology, they resort to the help of a surgeon. You cannot do without surgery if there is obvious compression of the lung - in such a situation, decortication, or pleurectomy, is performed. The intervention is the removal of pleural areas to release the lung, which makes it possible to restore its function.

For patients who have developed fibrothorax after pulmonectomy, additional intervention is inappropriate. Therefore, in such situations, emphasis is placed on symptomatic therapy. The main objectives of such treatment are as follows:

• minimize the manifestations of shortness of breath;
• optimize the functionality of a healthy lung;
• relieve the cardiovascular system.

Medicines

Medicines will not be able to eliminate the developing fibrotic process - for this it is necessary to perform an operation, which is not always safe and appropriate. But this fact does not mean that drug treatment is not carried out: drugs are prescribed to improve the patient's quality of life, to relieve symptoms.

• To combat the infectious and inflammatory process in fibrothorax, antibiotics with a broad spectrum of antibacterial activity are prescribed. Such drugs include Ceftriaxone, Doxycycline, Azithromycin. Often, drugs with anti-tuberculosis action are also used - in particular, Isoniazid, Rifampicin, etc.

Ceftriaxone	A 3rd generation cephalosporin antibiotic with prolonged action and a broad spectrum of activity. The drug is used on average 1-2 g every 24 hours, for several days (usually no more than a week). The most common side effects of Ceftriaxone: diarrhea, skin rashes, elevated liver enzymes, eosinophilia, leukopenia.
Azithromycin	A representative of macrolide antibiotics, characterized by a wide range of antibacterial activity. The drug is taken once a day, daily, between meals. The duration of treatment is determined by the doctor (usually 3-5 days). The likelihood of side effects is low. In rare cases, dyspepsia, skin rash, vaginitis, neutropenia are possible.

• To eliminate the symptoms of intoxication, oral and intravenous drip rehydration, taking anti-inflammatory and antipyretic drugs (acetylsalicylic acid, paracetamol, ibuprofen, etc.) are appropriate.

Paracetamol	An analgesic and antipyretic agent that can be taken 1-2 tablets 3 times a day. It is advisable to maintain an interval of at least 4 hours between doses. Side effects during treatment with Paracetamol occur very rarely: this may include allergies, nausea, increased activity of liver enzymes.
Regidron	Oral rehydration solution is used to restore fluid-electrolyte balance and correct acidosis. Regidron is taken as prescribed by a doctor. Side effects are extremely unlikely.

• To support liver function, hepatoprotectors are prescribed (Essentiale, Karsil, Gepabene), and in more complex cases of fibrothorax, corticosteroid drugs.

Essentiale forte N	A hepatoprotective drug that is taken 2 capsules three times a day with food. Duration of administration is 2-3 months, at the discretion of the doctor. Possible side effects: digestive disorders, rarely - allergic reaction.
Prednisolone	Hormonal glucocorticoid drug with anti-inflammatory, anti-allergic, desensitizing and immunosuppressive effects. The dosage is set individually. Treatment is completed by gradually reducing the dose. With prolonged use of the drug, vision may be impaired, dyspepsia, sleep disorders, thrombosis, changes in blood pressure, and nervous system disorders may occur.

Physiotherapy treatment

The use of oxygen for therapeutic and prophylactic purposes is called oxygen therapy. This method is especially important in fibrothorax, as it helps to compensate for the lack of oxygen in the tissues.

Against the background of oxygen therapy, patients experience an increase in the oxygen content in the plasma, improved breathing, an increase in the level of oxyhemoglobin in the blood, and a decrease in metabolic acidosis by minimizing the amount of under-oxidized products in the tissues.

Oxygen therapy is not able to stop the progression of fibrothorax, but it significantly improves the quality of life of patients. As a result of treatment, well-being improves significantly, sleep is normalized. Both stationary and mobile devices can be used to perform the procedure, which greatly simplifies the situation.

In addition to oxygen treatment, the patient is recommended to master the technique of correct breathing and is prescribed breathing exercises that help improve pulmonary ventilation.

Surgical treatment

All patients whose fibrothorax is causing compression of the lung are treated with a surgical procedure called pleurectomy with decortication. The procedure involves removing the necessary portion of the pleura to free up the lung and restore its function.

During a pleurectomy, a portion of the parietal and visceral pleura is removed. Using a lateral approach, the doctor performs a tissue dissection with resection of the fifth to sixth rib. A portion of the pleura is peeled off using a blunt method, separated from the lung, and then removed. [ 8 ]

In order to fully straighten the lung, the surgeon performs decortication – removal of fibrous accumulations. The chest cavity is drained using a pair of drains.

As a result of the intervention, the affected lung straightens out and the respiratory system begins to function normally.

If fibrothorax develops after pulmonectomy, then surgical intervention is not performed due to inappropriateness. In such a situation, symptomatic supportive therapy is prescribed - to reduce shortness of breath and reduce the load on the cardiovascular system.

Prevention

The formation of fibrothorax can be prevented in many cases. To minimize the likelihood of the development of the pathological process, it is necessary to listen to the following recommendations:

• promptly consult a doctor regarding the diagnosis and treatment of respiratory disorders;
• strengthen the immune system, avoid hypothermia;
• give up bad habits, in particular smoking;
• avoid passive inhalation of cigarette smoke;
• to avoid chemical damage to the respiratory system, if necessary, use personal protective equipment;
• promptly eliminate any infectious and inflammatory processes in the body;
• eat well, lead an active lifestyle, avoid physical inactivity;
• systematically undergo preventive comprehensive diagnostics of the body, and have a fluorographic image of the lungs taken annually.

Forecast

If fibrothorax development is stopped in time, the prognosis can be called more or less favorable. The probability of complications of the pathology is assessed in accordance with the severity of the initial disease, the age and general health of the patient, as well as the degree of spread of the fibrous process.

Most often, fibrosis phenomena progress without a linear dependence on time. The more stages fibrothorax goes through, the more severe its course and the more unfavorable the prognosis.

If a patient is diagnosed with fibrothorax, treatment should always be prescribed by a qualified physician. However, there are no therapeutic standards in this situation. Since in many cases the development and progression of the disease is preceded by inflammatory processes, anti-inflammatory drugs can be prescribed. Corticosteroids are indicated only for the treatment of patients with autoimmune processes and acute intoxication lesions of the respiratory organs.

A favorable outcome of the disease can be discussed when performing decortication of the lung. After such an intervention, the patient can actually "forget" about the problem and continue to live a completely normal life. If we consider non-surgical management of patients diagnosed with fibrothorax, then even with minimal respiratory failure there is a risk of further progression of the pathology. The most unfavorable outcome is observed in patients who fell ill after pulmonectomy, or with obvious intense respiratory failure.