Tietze's syndrome (Costochondral syndrome, Costochondritis)

Tietze syndrome was first described by the German surgeon Alexander Tietze in 1921. Tietze syndrome usually affects the third, fourth, and fifth costochondral joints. Less commonly, the manubriosternal joint and the xiphoid joint are affected. The disease is characterized by chest pain (with coughing and deep breathing) and morning stiffness, as well as local swelling and hyperemia of the costal cartilages.

Joint swelling distinguishes Tietze's syndrome from costochondritis, or chronic inflammation of the cartilage of one or more ribs at the junction of the sternum and the ribs.

Characteristics/Clinical picture

The cartilage connects the sternum to the ribs and collarbone and allows the chest to move during breathing. Inflammation of the cartilage of one or more ribs causes swelling and redness of the skin. This is visible and tangible. The pain can vary in intensity, but is usually worse with trunk movements, coughing, deep breathing and straining.

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These problems appear gradually and may disappear spontaneously after a few days, but sometimes they can take years to disappear. Even after spontaneous recovery, the disease may return, and pain may occur in the same or a different location. Pain syndrome can lead to disruption of normal movement in other joints, for example, shoulder movements can be accompanied by crepitus and pain. Patients with Tietze syndrome may experience a variety of functional limitations. Daily activities such as ironing, combing hair or lifting objects may be difficult.

Causes

Costochondritis is an inflammatory process in cartilage tissue, usually without any specific cause. Repeated minor injuries to the chest and acute respiratory infections can cause pain in the area where the ribs are attached (the impact of both the viruses themselves and frequent coughing on the area where the ribs are attached). Sometimes costochondritis occurs in people taking parenteral drugs or after surgery on the chest. After surgery, damaged cartilage tissue is more susceptible to infection due to impaired blood supply.

Differential diagnosis

Possible differential diagnoses are:

• Seronegative spondyloarthropathy.
• Rheumatoid arthritis.
• Xyphoidalgia.
• Slipping rib syndrome.
• Myelomalacia.
• Neoplasms of bones and soft tissues.
• Chondrosarcoma of the costochondral joints.
• Costochondritis.
• Tumors of the breast and/or lungs with extension to the costal cartilage.
• Metastases of neoplasms of the breast, kidneys and prostate gland.
• Rib injury and painful swelling of the ribs.
• Arthritis of the sternoclavicular and manubriosternal joints.

Symptoms

The main manifestation of Tietze's symptom is acute chest pain. It gradually increases and becomes more intense with deep breaths, sneezing, laughing, sudden movements, as well as with increased emotional and physical stress.

It is unilateral and often radiates to the arm on the corresponding side. Patients spare the affected side and try to close the sore spot. With this disease, there is no clear relationship between the occurrence of an attack of pain and the time of day. In some cases, the pain becomes so severe that patients cannot lie on their side; any movement brings them agony and suffering. Crepitation appears at the lesion site. The costal cartilage is hypertrophied and unusually curved. No histochemical changes are found in it. The surrounding soft tissues are swollen and inflamed.

Outwardly, the disease manifests itself sluggishly. Irregular acute attacks of pain can last several days, months, years. Pain is often combined with hypersensitivity of the xiphoid process. Palpation reveals a dense and clear spindle-shaped swelling. If you press on the junction of the ribs and sternum, noticeable pain appears. Discomfort and chest pain go away on their own and do not require specific treatment. The syndrome does not pose a threat to the life and health of the patient.

Secondary signs of pathology include:

• Violation of the depth, frequency and rhythm of breathing,
• Lack of appetite,
• Cardiopalmus,
• Insomnia,
• Local hyperthermia, hyperemia and swelling,
• Unmotivated fear, irritability and anxiety.

Residual signs of pathology occur extremely rarely.
Usually the general condition of patients is not disturbed and remains satisfactory. The muscles of the shoulder girdle and neck contract tonically. The skin over the affected area does not change, regional lymph nodes do not enlarge. As Tietze syndrome progresses, excessive calcification of cartilage develops, which is eventually replaced by bone tissue. As a result of fibrous metaplasia, the costal cartilages become deformed and lose their functions. The pain becomes intense and constant. A dense swelling on the chest interferes with the patient’s normal life. A rigid chest disrupts the normal breathing process, resulting in respiratory failure.

Diagnostic procedures

Appropriate studies such as electrocardiography (ECG) and computed tomography (CT) are necessary. They are used to rule out other diseases. Research suggests that this diagnosis is a diagnosis of exclusion. Plain radiographs are often normal/non-diagnostic. CT may reveal sclerosis of the sternum, partial calcification of the costal cartilage, and soft tissue edema. A costal cartilage biopsy may show chronic inflammation with fibrosis and ossification. Gallium scans have been reported to show increased uptake, similar to costochondritis.

Rating scales

• Visual analogue scale (VAS).
• Digital pain rating scale.
• McGill Pain Questionnaire (short form).

Classification

Depending on the distribution of chondritis, it happens:

• damage most often to only one costal cartilage is usually II and accounts for up to 60% of all known cases;
• several costal cartilages are involved in the pathological process, for example II and III, and occurs in 15-30% of patients;
• in rare cases - 1.5-10%, inflammation affects the entire sternoclavicular joint.

Relapsing polychondritis

Costochondritis may be part of relapsing polychondritis , an episodic inflammatory and destructive disease affecting the chondrocostal joints and knee joints. Less commonly, the disease may cause the development of conjunctivitis, scleritis, keratitis, iritis, choreoretinitis , destruction of the cartilage of the larynx, trachea, bronchi, and inner ear. It can affect the excretory and integumentary systems, but it is most dangerous when it affects the cardiovascular system, causing aortic regurgitation, pericarditis, myocarditis, aortic aneurysm or aortitis .

Polychondritis has a remitting nature, which manifests itself in the form of attacks of acute inflammation that disappear after a few weeks or months, and then return as repeated attacks several years later.

Inspection

On physical examination, swelling of the affected area and sometimes hyperemia is noted. Pain may occur during palpation of this area; The pectoralis major and minor muscles and the sternum are also painful.

The pain can be reproduced with retraction, protraction, or elevation of the shoulder or with deep inspiration. Tietze syndrome results in limited muscle strength and range of motion in the upper extremities. In such patients, the level of functional activity may decrease. Typically, difficulties arise when ironing, lifting objects, or combing hair, but the disruption in functionality is minor.

As said above, if there is inflammation of the cartilage of one or more ribs, then you can talk about costochondritis, if there is also swelling/redness that is very painful, then you can talk about Tietze's syndrome.

Etiology

Currently, the etiopathogenetic factors of Tietze syndrome remain unknown. Several theories of the occurrence and development of the disease have been developed. The main ones:

The traumatic or mechanical theory explains the occurrence of the disease in athletes, people engaged in heavy physical labor, or those who have suffered traumatic damage to the ribs in the past. Direct trauma to the shoulder results in damage to the costal cartilage. This irritates the perichondrium and disrupts further differentiation of cartilage cells. As a result of such changes, pathological cartilage tissue is formed, which compresses the nerve fibers, which is manifested by pain. The traumatic theory is considered the most popular.

According to the infectious theory, Tietze syndrome develops after suffering an acute respiratory infection, which provoked a decrease in the general resistance of the body.

Dystrophic theory - the development of pathology occurs as a result of calcium metabolism disorders and deficiency of vitamins C and B. This is one of the earliest theories developed by Tietze himself. It is not confirmed by objective data and is considered doubtful.

Factors contributing to the development of pathology:

• Pronounced and regular loads on the shoulders and chest,
• Chronic chest injuries in athletes,
• Acute infections
• Arthrosis-arthritis,
• Bronchopulmonary pathology,
• Occupational scoliosis,
• Metabolic disorders
• Endocrinopathies,
• Allergic reactions,
• Postmenopausal osteoporosis,
• Collagenoses.

The risk group includes:

• Athletes,
• Persons engaged in heavy physical labor
• Drug addicts,
• Persons after thoracotomy.

At the junction of the cartilages of the ribs and sternum, metabolic processes are disrupted. Prolonged aseptic inflammation leads to dystrophy, the appearance of areas of sequestration in the cartilage, metaplasia of cartilage tissue, its calcification and sclerosis. Benign reversible swelling of the costal cartilages causes pain. Degenerative changes lead to deformation of the cartilage, a decrease in its size, and weak intractability. As a result of ossification of the cartilage, the configuration of the chest changes, its mobility and elasticity decrease, and it becomes rigid.

Treatment

Drug treatment includes relative rest for 4-6 weeks, injections of a corticosteroid anesthetic, topical or oral analgesics, and other drugs, including Sulfasalazine or Caspofungin in combination with Fluconazole.

Physical therapy

Currently, there are no reliable clinical studies on the treatment of this disease. Treatment of Tietze syndrome is mainly symptomatic.

What can be done?

• Reassure the patient by explaining what is happening to him.
• It is important for the physical therapist to educate the person about proper body positioning while sitting or performing daily activities. It is also important that the patient avoids repetitive movements/activities. In general, these patients need a good balance between exercise and rest.
• The treatment program may include exercises to increase range of motion. Typically, patients tolerate such exercises well, but if they aggravate symptoms, then they should be abandoned or postponed.
• Stretching the pectoralis major muscle is helpful. You can stretch the pectoralis major muscle by standing in a doorway with emphasis on your forearms. You need to repeat this exercise several times a day for 1 or 2 minutes.
• Use hot/cold compresses and massage to reduce pain and symptoms of muscle overuse. For this purpose, you can also use a variety of sprays.
• Mobilizations of the spine and ribs are also helpful. This will help to avoid chest stiffness and reduce complaints.
• It is important to teach the patient breathing exercises and methods of working with pain.
• Exercises for the shoulder girdle and back can be provocative, so they should only be prescribed if symptoms disappear.
• Transcutaneous electrical stimulation and electroacupuncture can be used. In the latter case, an acupuncture needle is placed into the affected segment of the spine.
• Injection therapy with local anesthetics or corticosteroids has previously been described as a treatment for chest pain associated with costochondral joint lesions. Results from previous studies have shown that dry needling may be as effective as injection therapy in treating similar conditions.

TIETZE SYNDROME

Tietze syndrome

(A. Tietze, German surgeon, 1864-1927; synonym:
Tietze's disease, rib dystrophy, costochondral syndrome, painful swelling of the costochondral junction, raised costal cartilages, protruding costal cartilages, fatigue fractures of the costal cartilage, pseudotumor of the costal cartilages,
etc. .) - damage to the costal cartilage, characterized by the appearance in it of a painful tumor-like formation of a non-tumor nature.

The syndrome was described by Tietz in 1921. In Russian literature, the first mention of Tietz syndrome belongs to E. N. Kalinovskaya (1959). To date, this syndrome is relatively little known and insufficiently studied.

Tietze syndrome occurs equally often in men and women, more often in people aged 20-40 years, engaged in manual labor, and in athletes. In most cases, the lesion is unilateral and localized in the left half of the chest. According to E. N. Kalinovskaya, in 60% of patients the pathological process developed in the cartilage of the 2nd rib, in 30% - in the cartilage of the 3rd - 4th ribs, and in 10% - in the cartilage of the 1st, 5th, 6th ribs.

Etiology and pathogenesis

are not clear. Opinion of Titze et al. about etiol. The role of nutritional deficiency leading to disruption of mineral (in particular calcium) metabolism has been refuted. Among other theories of the emergence of T. s. the most recognized are infectious-allergic and so-called. mechanical or traumatic. Adherents of the infectious-allergic theory proceed from the coincidence in time of development of T. with. and allergic process in some patients with diseases of the lungs and upper respiratory tract. According to the mechanical theory, damage to the costal cartilages is a consequence of mechanical effects on the perichondrium and cartilage, for example, with prolonged and persistent coughing in patients with respiratory diseases (the so-called cough fracture), occupational microtraumas, excessive tension of the pectoralis major and minor and adjacent muscles in athletes, with blunt chest trauma without damaging the integrity of the ribs, etc.

Patol. the process in the costal cartilages proceeds according to the type of aseptic inflammation of the perichondrium and cartilage. The results of microscopic studies are few and contradictory. Tietze noted a violation of the structure of cartilage in combination with the deposition of calcium salts and the formation of fibrous tissue; Bruin and Smook found no changes; Harttung, Satani, and others identified hyperplasia and fibrous metaplasia of cartilage with the formation of bone islands and areas of calcification. T. P. Vinogradova, E. N. Kalinovskaya and others in a number of cases noted the presence in the thickness of the cartilage of numerous areas of degeneration with tissue disintegration, disruption of the paired arrangement of cartilage cells and numerous vacuoles in them; at the junction of cartilage with bone there is a deep penetration of cartilage tissue into the bone tissue, and in the bone crossbars there are phenomena of increased restructuring. T.P. Vinogradova associated the noted changes with increased growth of cartilage tissue.

Clinical picture

characterized by acute or gradually increasing pain in the affected area. After a few hours (sometimes days), a uniformly dense painful swelling begins to form above the affected rib, gradually occupying the entire intercostal space and rising 0.5-3.0 cm above the chest wall. The pain intensifies when moving the arms, lifting heavy objects, sneezing, coughing, turning the body, or lying on the affected side. Often the pain radiates to the neck, shoulder girdle, arm, and shoulder blade. The skin over the swelling is of normal color and mobile. Regional lymph nodes are not changed, body temperature does not increase, there are no changes in the composition of blood and urine. The pain lasts from several days to several weeks and even months, the swelling disappears much later. In rare cases, the clinical manifestations of the disease disappear spontaneously in a short time.

Diagnosis

in the vast majority of cases, it is established on the basis of anamnesis and typical symptoms.
Differential diagnosis is carried out with osteomyelitis (see), specific forms of perichondritis (see) and osteochondritis (see), callus after a fracture (see Callus), benign and malignant tumors, etc. (see Chest). The results of radiographs are of decisive importance in differential diagnosis. research. On radiographs, sometimes already during the first study, and more often during dynamic observation, asymmetry of calcification and the structure of the corresponding sections of the ribs is revealed. X-ray of a part of the chest of a patient with Tietze syndrome (direct projection): the arrow indicates neoarthrosis that has developed in the area of ​​pathological restructuring of the tissue of the first rib.
On the affected side, the calcification is more pronounced, the rib looks thicker and more massive. After ossification of the entire costal cartilage, the sternocostal joints may become visible and a picture of deforming arthrosis may be observed in them (see Arthrosis). When the lesion is localized in the area of ​​osteochondral joints (usually in the first rib), a typical patol zone is visible on the x-ray. functional restructuring - a band of clearing with club-shaped thickening of the bone at this level due to periosteal bone formation, sometimes with the development of neoarthrosis (Fig.). A tomographic examination in a direct anterior projection can provide significant assistance in diagnosis (see Tomography).

Treatment

usually conservative: local subchondral administration of 0.5% novocaine solution with hydrocortisone (4-5 injections every 3-5 days), immobilization of the upper limb on the side of the affected costal cartilage, physiotherapy (potassium iodide electrophoresis, UHF, paraffin baths); microdoses of iodine with salicylates are prescribed internally; There is evidence of the effectiveness of radiotherapy. After the pain disappears, exercise therapy sessions are carried out. Surgical intervention (subperiosteal resection of the thickened part of the rib) is rarely performed, only if conservative treatment is unsuccessful.

Forecast

favorable, but relapses are possible, sometimes occurring after several years.

Bibliography:

Brusinenko A.I. Tietze syndrome, Health. Belarus, No. 10, p. 75, 1978; Vinogradova T. P. On the physiology of human cartilage tissue, Arch. pathol., vol. 18, no. 2, p. 24, 1956; Greida B.P. Therapeutic tactics for Tietze syndrome, Vestn. chir., t. 126, no. 3, p. 29, 1981; aka, Tietze Syndrome, Surgery, No. 11, p. 89, 1982; 3aslavsky E. S. Tietze syndrome, Klin, med., t. 49, No. 11, p. 20, 1971; Kalinovskaya E. N. About “Tietz syndrome”, Surgery, No. 5, p. 31, 1959; Lyashchuk P. M. About Tietze syndrome, Klin, med., t. 53, No. 1, p. 117, 1975; Plakhov A. Ya. About tumor formation of costal cartilages (Tietze syndrome), Orthop. and traumat., No. 9, p. 24, 1966; Pratsko V. G. The role of microtrauma in the pathogenesis of Tietze’s disease, Vestn. chir., t. 114, no. 1, p. 74, 1975; Reinberg S. A. X-ray diagnosis of diseases of bones and joints, book. 2, p. 123, M., 1964; Giordano A. La roentgen-terapia nella sindrome di Tietze, Ann. Radiol. diagnosis (Bologna), v. 43, p. 452, 1971; Tietze A. t)ber eine eigenartige Haufung von Fallen mit Dystrophie der Rippenknorpel, Berl. klin. Wsehr., S. 829, 1921; Wiedemann Tietze-Syndrome (Chondroosteopathia costalis tuberosa) im friihen Kindesalter, Helv. paediat. Acta, Bd 21, S. 25, 1972.

I. N. Belov; P. L. Zharkov (rent.).

Origins of the disease

They started talking about the disorder on a large scale in 1983 thanks to the Swedish scientist Bengt Hagberg. At this time, he and his group studied 35 similar cases in 3 different countries: Portugal, France and Sweden.

However, Hagbert is not the discoverer of the syndrome. It was first discovered by pediatrician Andreas Rhett, whose name the disease bears. He observed two girls who had the same symptoms. He noticed them in the reception line. They sat on their mothers' laps, and they held their hands. The girls swung like pendulums, and then suddenly both began making stereotypical movements with their hands. The children were frozen in one position, detached from the world around them. The gaze was directed to one point. Their synchronicity in movements and behavior was amazing.

The doctor found similar case histories in his written archives, and then went to Europe to find the same patients there. In 1966, he made the first publications of his research, which, however, did not arouse much interest.

Rett called the disease he recorded brain atrophy syndrome. At first it was considered a manifestation of autism or schizophrenia, and only in 1983 was it brought into a separate nosological unit.

Currently, the syndrome is classified as a rather rare genetic disease. It occurs with a incidence of 1 in 15,000. The cause is said to be a mutation in the MECP2 gene. This gene is responsible for the synthesis of a certain protein that affects brain development. Normally this protein is

established some time after birth, must be suppressed by other genes to ensure normal brain development.

If the MECP2 gene is mutated, the protein is not completely inactivated, which causes abnormal brain maturation and provokes the development of Rett syndrome.

Usually the mutating gene is located on the X chromosome, so the disease mainly affects girls.

Symptoms

Damage to costal cartilages in Tietze syndrome

Tietze syndrome manifests itself with several characteristic symptoms. And although these symptoms can also occur in other pathologies, their combination allows the doctor to make an almost unmistakable diagnosis, although differential diagnosis with some diseases of the internal organs is required.

The main symptoms of the disease are as follows:

• swelling in the affected areas of the ribs;
• compaction of the affected parts of the cartilage;
• pain on one side - from mild to severe;
• irradiation of pain into the arm on the affected side.

Symptoms may subside or intensify and this lasts for years, which causes discomfort to the person and worsens the quality of his life.