PATHOLOGY OF the DIAPHRAGM and  MEDIASTINum

 

Diaphragmatic hernias

Diaphragmatic hernia represents herniation of abdominal organs through natural openings of diaphragm, its weak places or ruptures.

 

Etiology and pathogenesis

The cause of occurrence of congenital hernia is the disturbance of embryogenesis with transformation in anomaly of diaphragm. The acquired diaphragmatic hernia more often arise owing to age-dependent involution of diaphragm, its ptosis in the people with a mainly sedentary mode of life, increase of intraperitoneal pressure, obesity, cough, overfeeding, constipation, meteorism and pregnancy. The cause of sliding hernias can be draw of esophagus upward in reflux esophagitis owing to intensive contraction of its longitudinal musculature.

 

1– norm, 2 –  sliding hiatal hernia, 3 – paraesophageal hernia

 

Pathology

As well as any hernia, diaphragmatic has hernial ring, hernial sac and hernial content. The tissues in the region of hilus, due to tension and pressure, result in atrophy and sclerosis. necrosis, chronic inflammation, adhesions of the hernial content can develop.

 

Classification

There are such types of hernia:

1)    congenital;

2)    acquired;

3)    posttraumatic;

4)    true;

5)    false.

А. Diaphragmatic hernia.

I. Sliding (axial) diaphragmatic hernia:

1)    esophageal;

2)    cardial;

3)    cardiofundal.

ІІ. Diaphragmatic hernia of paraesophageal type:

1)    fundal;

2)    antral;

3)    intestinal (small and large intestine);

4)    combined intestinal-gastric hernias;

5)    epiploic.

ІІІ. Huge diaphragmatic hernia:

1)    subtotal gastric;

2)    total gastric.

ІV. A short esophagus:

1)    acquired short esophagus;

2)    congenital short esophagus (thoracic stomach).

B. Parasternal hernias:

1)    retrosternal;

2)    retrocostosternal.

C. Lumbocostal diaphragmatic hernias.

D. Hernia of atypical localization.

 

Esophageal hernias:

 

Esophageal

 

Cardiofundal

 

Mixed paraesophageal

 

Paraesophageal fundal

 

Symptomatology and clinical course

The predominant manifestations resulting from sliding diaphragmatic hernia (about 90 % of diaphragmatic hernias) are the signs of gastroesophageal reflux. It is characterized by the pain behind breastbone or epigastric region. It more often appears in supine position after meal or after intensive physical exertion.

Heartburn is the second according to the frequency sign and caused by the injury of esophageal mucosa by gastric juice as a result in turn of gastroesophageal reflux.

Belching by air, as a rule, observed, which commonly results in pain relief and decrease of arching feeling in epigastric region.

Regurgitation arises owing to gastroesophageal reflux, which reaches pharynx and oral cavity. More often observed regurgitation by gastric acid or bitter liquid or food.

The sign of “lacing shoes“ is expressed when the patient bends down after liquid food, and the latter is partially poured out into the mouth. It is caused by incompetence of the lower esophageal sphincter (gastroesophageal junction).

Nausea and vomiting are rare. The latter some patients cause by themselves to achieve some relief.

Dysphagia is rarely observed. More often it is the outcome of complications of diaphragmatic hernia (esophageal stricture, malignancy).

Roentgenological signs: 1) the sign of “bell”; 2) blunt His angle; 3) lack of air bubble of the stomach.

Sliding diaphragmatic hernia

 

The clinical manifestations of paraesophageal, retrosternal or lumbocostal hernias basically depend on the character of organs, which the hernial sac contents, and their compressing by hernial ring. Sometimes the clinical course even of major hernias is asymptomatic, and they are occasionally found out during X-ray examinations. For the first time the disease can manifest under the influence of physical exertion, trauma, pregnancy, labors etc.

 

Paraesophageal hernia

 

Variants of clinical course and complications

The sliding hiatal hernia commonly has typical clinical course and rather rich symptomatology, which enable to establish the diagnosis with a great degree of probability. Nevertheless occasionally gastroesophageal reflux as the sequel of a sliding hiatal hernia can result in misdiagnostics (stenocardia, acute cholelithiasis etc.).

The most often complications of sliding diaphragmatic hernia are gastric bleeding, peptic stricture of esophagus and malignancy.

The causes of the bleeding can be erosion and ulcers of stomach, which result from compression of the organ in esophageal hiatus. More often observed small bleeding, but at long-stand recurrent course they result in chronic anemia. The profuse bleeding arise rarely. The strangulation of a sliding diaphragmatic hernia never occurs.

Nevertheless for diaphragmatic hernias of other locations the most dangerous complication is naturally strangulation. Such pathology manifests by the signs of s strangulation intestinal obstruction. However the correct diagnosis frequently possible to establish only during operation.

 

The diagnostic program

1. Anamnesis and physical findings.

2. X-radiography of chest and abdomen.

3. Esophagogastroscopy with biopsy and histological investigation.

4. Contrast X-radiography of esophagus and stomach in three positions: upward, supine and upside-down position.

5. General blood and urine analyses.

6. Coagulogram.

Sliding hernia

 

Paraesophageal hernia

 

Mixed hernia

 

 

Differential diagnostics

Stenocardia. Diaphragmatic hernias frequently cause the pain, which character not only the patient, but also doctor can identify as anginal. However in diaphragmatic hernia the pain more often is vague, spread to the stomach region and depends on body position. The pain, as a rule, arises in supine position and disappears, if the patient upward. More often it spreads to the right and anginal vice versa to the left. In diaphragmatic hernia the ECG can manifest the coronary failure, nevertheless standing up, owing to the stop of strangulation leads to disappearance of these pathological sings. The pain caused by diaphragmatic hernia does not relieve after nitroglycerin. In this case more effective and prompt is atropine.

Peptic ulcer. The pain in gastric and duodenal ulcer frequently localized in epigastric region with irradiation in the left or right hypochondrium. Nevertheless, it is characterized by periodicity, which caused by meal and disappears after the usage of soda.

Lung atelectasis, pleurisy, pneumonia are also should be differentiated with diaphragmatic hernia. Thus it is always necessary to remember, that the extrapulmonary shadow of supradiaphragmatic disposed hernia on a plain roentgenogram can resemble intrapulmonary. For correct diagnosis it is possible to recommend polypositional X-radiography, contrast roentgenography of esophagus and stomach.

 Hypochromic anemia frequently associated due to repeated or permanent small bleedings. They are caused by a regional destruction a gastric mucosa. In the females of senior age if it is fail to explain genesis of the revealed anemia, it is necessary to think about the opportunity of diaphragmatic hernia and carry out appropriate X-ray examination.

 

Tactics and choice of treatment

The medical tactics toward diaphragmatic hernias of different localization essentially differs.

In case of sliding hiatal hernia the method of a choice is the conservative therapy:

1)    the diet the same, as in peptic ulcer;

2)    position of the patient during sleeping – with elevated upside, during exacerbation – sedentary;

3)    suppression of gastric secretion by administering of н2-blockers;

4)    neutralization of gastric acid;

5)    intensifying of evacuation of the food from stomach;

6)    avoidance of constipation;

7)    anesthetics and sedative agents.

The indication for surgical treatment of sliding diaphragmatic hernia is the considerable expression of clinical signs, diminish of patient’s working capacity, fail of conservative treatment, bleeding, peptic stricture, malignancy.

Surgical treatment. Upper median laparotomy is mainly used. Nevertheless some surgeons prefer transthoracic accesses.

Stages of the operation:

1.     Drawing of the stomach into abdominal vacuity by disjunction of adhesions in the region of its cardial part, esophagus, excision of hernial sac.

2.     The plastics of esophageal hiatus of diaphragm (cruroplasty). The most widespread cruroplasty by Hill and narrowing of esophageal ring according to Garrington.

3.     Elimination of valvular failure of esophagocardial junction. The purpose of operation is to prevent gastroesophageal reflux by means of formation of His angle and esophagocardial valve. Also Nissen fundoplication is applied.

4.     Gastropexia – fixation of gastric wall to parietal peritoneum.

Another tactics is applied in the patients with paraesophageal, parasternal and lumbocostal hernias. The method of choice is the surgery. Such tactics is explained by the hazard of strangulation. The essence of the operation consists of drawing down of hernial content (stomach, intestine, omentum) into abdominal cavity, removing of hernial sac and liquidation (suturing) of hernial ring.

 

Steps of cruroplastic

 

 

 

Diaphragmatic relaxation (Diaphragmatic Eventration)

The term “diaphragmatic relaxation ” was used for the first time in 1906 by Witting. It means a relaxation of diaphragm, its high standing and displacement upward of abdominal organs.

The term ‘diaphragmatic eventration’ is used in common practice to describe a condition of relaxation of the diaphragmatic dome. It may present at birth as a congenital condition due to a defect of diaphragmatic development or in a later stage of life as an acquired condition (‘acquired diaphragmatic paralysis’ or ‘acquired diaphragmatic elevation’).

 

Etiology and pathogenesis

The cause of the disease is the congenital or acquired decrease of diaphragmatic resistance, which during elevation of intraperitoneal pressure results in its outpouching. The great importance in the development of acquired relaxation belongs to the damage of diaphragmatic nerve. The cause of the latter could be inflammatory processes in chest and abdominal cavity, intoxication, poisoning, operations on chest organs and  birth injury.

 

Pathology

In congenital form of a diaphragmatic relaxation revealed muscular aplasia, in acquired – atrophy of muscular fibers.

 

Classification

1)    Complete: left-side, right-side;

2)    Incomplete: anterior, posterior, restricted (partial).

 

Symptomatology and clinical course

Minor manifestation or asymptomatic course characterizes diaphragmatic relaxation. Therefrom, it is always necessary to thoroughly analyze the occurrence of multiple signs from the organs of digestive, respiratory and cardiovascular system. The clinical symptomatology basically depends on dysfunction of the diaphragm by itself and organs, which adjoin to it both in chest, and in abdominal cavity. In left-side diaphragmatic relaxation the asymptomatic course rarely occurs.

General symptomatology. The patients with diaphragmatic relaxation can feel a pain of different character, localization and intensity. The pain syndrome frequently results from gastric inflection or compression of vessels and nerves by filled stomach.  Inflection of vascular bundles of pancreas, lien, kidneys, mesentery of small and large intestines as a result of shift of abdominal organs also contribute to the development of pain syndrome. Frequently patients complain of general weakness and loss of weight.

Gastrointestinal symptomatology. Dysphagia almost always arises as a result of inflection of abdominal part of esophagus. The heaviness after meal should be caused by atony of stomach and its evacuation dysfunction. Ulceration and erosive gastritis, which occurs in some patients, are the outcome of a regional ischemia from gastric inflection or torsion. Chronic constipation is basically caused by disturbance of massage influence of the diaphragm on intestine. Meanwhile heartburn, belching, nausea, vomiting and meteorism also observed.

A phrenocardiac Uden-Ramcheld’s syndrome represents cardiopulmonary signs. It is characterized by dyspnea, discomfort in the region of heart, anginal pain, extrasystole and ECG changes (elongation of Р wave, РQ interval and complex QRS).

Respiratory disturbances result from dynamic dysfunction of the diaphragm. The high standing of the diaphragm leads to compression of lung on the side of lesion and disturbed ventilation of the lower part. It causes the diminishing of vital capacity of the lungs and development of dyspnea.

Roentgenologically revealed the high standing of diaphragmatic dome (to ІІ-ІІІ intercostal space), restriction of its excursion and reduce of the inferior pulmonary field. Frequently observed the mediastinal shift to the opposite side. The contrast X-radiography of esophagogastric junction can find out the inflection of abdominal part of esophagus. The X-ray examination enables to establish the diagnosis with a high degree of reliability.

 

Diaphragmatic relaxation

 

 

Variants of clinical course and complications

Asymptomatic course of diaphragmatic relaxation in the majority of patients has caused interpretation of this pathology as “innocent disease”. Nevertheless the shift and rotation of heart can cause the heart failures, and the restriction of pulmonary excursion sometimes leads to chronic pneumonia. The gastric inflection frequently may result in disturbance of the valvular mechanism of esophagogastric junction and occurrence of reflux esophagitis.

 

The diagnostic program

1.     Anamnesis and physical findings.

2.     Plain chest X-radiography.

3.     Esophagogastroduodenoscopy.

4.     Roentgenoscopy of esophagus and gastrointestinal tract.

5.     General blood and urine analyses.

 

Differential diagnostics

Diaphragmatic elevation is the secondary high standing, which can arise as a result of ascites, pregnancy, expressed meteorism, peritonitis, tumours of abdomen, splenomegaly or megacolon.

Pneumothorax, pyopneumothorax, pleurisy. Such misdiagnostics in the patients with diaphragmatic relaxation frequently caused by chest pain, cough, dullness and tympanic sound revealed at percussion, and weak breathing at auscultation. Chest X-radiography rather contributes to exact diagnostics.

Diaphragmatic hernia. The differential diagnosis of diaphragmatic relaxation with this pathology is the most difficult. Nevertheless it has the important practical value, because the threat of strangulation of diaphragmatic hernia requires an active surgical tactics. During the establishment of the diagnosis it is always necessary to remember, that clinical manifestation of diaphragmatic hernia more expressed. However, the sharp inflection of abdominal organs in the patients with diaphragmatic relaxation also can associate with severe pain, which resembles strangulation. Thereafter, a reliably differentiation of these diseases is possible only after a goal-oriented X-ray examination.

Cancer of esophagus and cardial part of stomach. A sharp gastric shift upward with inflection of abdominal part of esophagus can lead to dysphagia, substernal pain, disturbance of digestion, considerable loss of weight etc. For differential diagnostics applied a contrast X-ray examination of esophagus and stomach.

In difficult for differential diagnostics cases a pneumoperitoneum with further X-ray examination is performed. This method allows with a major degree of reliability to establish the diagnosis of diaphragmatic relaxation.

 

Tactics and choice of treatment

In most cases the asymptomatic course of diaphragmatic relaxation requires no special treatment.

Conservative therapy applied at presence of symptomatology:

1)    avoidance of physical exertions, which increases intraperitoneal pressure;

2)    diet – eating by small portions and exception of food, which form waste and gases;

3)    therapeutic gymnastics for improving of intestinal function and decrease of the patient’s weight;

4)    symptomatic therapy for regulation of cardiovascular and respiratory systems.

The indication for operation: gastric torsion or severe cardiorespiratory dysfunction. If clinical manifestations are absent, the surgical treatment can be recommended only for women with further pregnancy and labors, because these conditions cause a sharp increase of intraperitoneal pressure with further shift of the diaphragm and abdominal organs.

Surgical treatment. By means of a lateral access in VІІ intercostal space a phrenoplasty is performed, which consist of incision of diaphragm from costal edge to esophageal ring with following diaphragmatic duplication.

 

Acute mediastinitis

Acute mediastinitis is a purulent inflammation of mediastinum.

 

Etiology and pathogenesis

The penetration of pathogenic agents into mediastinum can result from perforation and chemical burns of esophagus; injuries of trachea, bronchi, operations on mediastinal organs and lungs. Also is possible the contamination from neck fat tissue and tracheobronchial lymph nodes.

 

Classification of mediastinitis

According to localization:

1)    anterior;

2)    posterior;

3)    superior;

4)    medial;

5)    inferior.

According to pathogenesis:

1)    primary;

2)    secondary.

According to the clinical course:

1)    acute: purulent, aseptic;

2)    chronic.

According to the character of infection:

1)    nonspecific;

2)    specific.

 

Symptomatology and clinical course

The clinical manifestation of acute mediastinitis is characterized by prompt progressing course, dependence on extent of the process, gravity of infection and peculiarities of underlying disease.

Body temperature raises up to 39-40°С and of hectic character, the patients complain of dyspnea, cyanosis, fever and profuse sweating.

The local symptomatology of the disease depends on location of the process and involvement of esophagus, trachea, heart, n. vagus, n. рhrеnісus, n. recurrens, tr. sуmраtісus.

Also is possible dysphagia, dyspnea, constant cough, hoarseness, change of cardiac rhythm.

On percussion revealed a mediastinal widening, on auscultation – weak cardiac tones.

X-ray examination. The method of X-ray examination should be chosen according to the cause of occurrence of acute mediastinitis. If the disease is caused by cervical phlegmon the X-radiography examination should be restricted only by chest X-ray film in three plains. In such situations observed widening of mediastinum, shadowing of its anterior part and shift of trachea. Compression of esophagus revealed by barium swallow.

The contrast X-ray examination of esophagus after its iatrogenic perforation it is possible to see penetration of barium into mediastinum, shadowing and widening of its consequent parts. Fiberesophagoscopy as the method of diagnostics of esophageal perforation is not recommended due to pneumatic pressure during this manipulation.

 

Variants of clinical course and complications

According to the features of clinical symptomatology, acute mediastinitis is divided into anterior and posterior mediastinites.

 

 

The most often complications of acute mediastinitis are: pyopneumothorax, which has arisen after the abscess discharge into pleural space, pleural empyema, purulent pericarditis, erosive bleeding and lung abscesses.

 

The diagnostic program

1.     Complaint and history of the disease.

2.     Physical findings.

3.     Chest X-ray examination.

4.     Contrast esophagography.

5.     ECG.

6.     Fibrobronchoscopy.

Acute mediastinitis

 

Differential diagnostics

Acute mediastinitis requires express differential diagnostics.

Acute pneumonia, as a rule, is the outcome of catarrhal factor, which evidence is showed by the patients. Besides, more long duration, high temperature, and cough with expectoration of mucopurulent sputum characterize the pneumonia; on auscultation –fine bubbling wet rales on the side of lesion and infiltration of pulmonary tissue at chest X-ray examination. All these findings enable to confirm or to rule out pneumonia.

Exsudative pleurisy mostly arises as the result of complication of pleuropneumonia. The process lasts, as a rule, 5-7 days. The most typical manifestations are cough and chest pain on the side of lesion, which intensifies at deep breathing. Percussion reveals a shortening of percussion sound. On auscultation – weak breathing sounds and pleural friction rub. The presence of intensive homogeneous shadow with oblique upper contour on chest X-radiography and also thoracentesis confirm the diagnosis of pleurisy.

Exsudative pericarditis. It most often results from rheumatic lesion of heart, acute myocardial infarction or polyserositis. Dyspnea, pain, heavy feeling behind breastbone, general malaise, forced sedentary patient’s position are the chief signs of pericarditis. On X-ray films observed a trapezoid shape of heart, and on ECG – diminished waves. Puncture of the pericardium with obtaining of exudate finally confirms the diagnosis.

 

Tactics and choice of treatment

The treatment of mediastinitis is only surgical. Its character and volume significantly depend on the cause, location and extent of purulent process. Established perforation of esophagus, trachea or bronchus requires an urgent operation. The foreign bodies thus removed, and operation ends by drainage. If the process located in anterosuperior mediastinum used a cervical mediastinotomy.

Cervical mediastinotomy

 

 

Nevertheless cervical mediastinotomy is insufficient at low localization of the process. In such cases performed anterior mediastinotomy. Meanwhile, isolated posterior mediastinitis is the indication for drainage by means of posterior extrapleural mediastinotomy.

In postoperative period is necessary application of intensive antibacterial, antiinflammatory and detoxycation therapy, and also treatment direct on the increase of immunological resistance of the organism. The mortality after such operative approaches has been 26-36 %, and after conservative treatment – about 70 %.

Mediastinal tumors

The mediastinal tumors include true tumours, cyst and masses.

 

Classification of tumours

1. Neurogenic tumours:

a)     ganglioneuroma;

b)    neurinoma;

c)     neurogenic sarcoma;

d)    neuroblastoma;

e)     sympathoblastoma.

2. Mesenchymal tumours:

a)     fibroma;

b)    lipoma;

c)     hemangioma;

d)    fibrosarcoma;

e)     liposarcoma;

f)      angiosarcoma.

3. Tumours originated from reticular tissue of lymph nodes:

a)     lymphosarcoma;

b)    reticulosarcoma;

c)     lymphogranulomatosis.

4. Tumour originated from thymus (thymoma), and thyroid gland (substernal, intrathoracic goiter).

5. Germ cell tumours:

a)     dermoid cyst;

b)    teratoma;

c)     mediastinal seminoma;

d)    choriocarcinoma.

6. True mediastinal cysts:

a)     mesothelial cyst;

b)    pericardial diverticula;

c)     bronchogenic cyst;

a)     enteric cyst.

7. Parasitogenic (echinococcal) cysts.

 

Symptomatology and clinical course

On early stages of the development the tumours are almost asymptomatic, and 40 % of mediastinal neoplasms are revealed at preventive chest X-ray examination. The patients most often complain of chest pain. The intensity of pain depends on degree of tumour compression or growth into nervous structures. In malignant growth the pain has more intensive character, than in benign. Frequently the pain precedes by feeling of heaviness, discomfort and foreign body in chest. Sometimes observed dyspnea caused by compression of airways, and major vessels both of anterior, and posterior mediastinum.

Owing to compression of the lumen of superior vena cava the syndrome of superior vena cava develops, which manifest by cyanosis of face, neck and upper half of chest, distend cervical veins, edema and dyspnea. Resulting from the rise of blood pressure and disrupture of venous walls, the nasal, esophageal and pulmonary bleedings develop. As the characteristic features considered headache, loss of consciousness and hallucinations. In overwhelming cases superior vena cava syndrome results from malignant tumours of lungs and mediastinum. Only in 5-7 % of patients they are benign.

The basic method of diagnostics is a complex X-ray examination: roentgenoscopy, polypositional X-radiography, tomography, computer tomography. The examination should be start from roentgenoscopy in different plains (multiaxial roentgenoscopy). It gives the possibility to find out a pathological shadow, its location, shape, size, mobility, intensity, contours and to reveal the presence or lack of pulsation of walls. Computer tomography is also a high-grade method of diagnostics. It helps to receive the image of transversal plain of chest at any level, to confirm the location of mediastinal tumour and its communication with adjacent organs. In suspicion on  a vascular nature of the process, angiography is used. It enables to rule out aneurysm of heart, aorta and its branches, reveal compression of superior vena cava and growth of the tumour into major arterial trunks.

If it is necessary to differentiate the tumour from cyst and reveal its different deposits, it is expedient to apply ultrasonic examination (sonography).

With the purpose of improvement of localization, size of mass, its communication with mediastinal organs performed pneumomediastinography (X-radiography of mediastinum with introducing of oxygen or air). For pneumomediastinography, depending on tumour locating the gas is introduced through a puncture above jugular incissure of breastbone, under xyphoid process or parasternally. Thus gas at first is spread in anterior mediastinum, and in 45-60 min. penetrates in posterior. The introduced gas achieves a good visualization of tumour contours and its growth into adjacent organs. Sometimes an artificial pneumothorax is performed on the affected side. In such patients collapse of lung gives the opportunity to differentiate pulmonary tumour from mediastinal tumours and cysts.

For morphological verification of the tumour applied such additional methods:

–         thoracoscopy, which allows to examine a pleural space, to take biopsy from mediastinal lymph nodes or tumour;

–         mediastinoscopy (through a small incision above the breastbone exposed trachea, and along its position performed the canal in anterior mediastinum with following insertion of a special endoscope) enables to examine anterior mediastinum, and take a biopsy from lymph nodes and tumour;

–         transthoracic aspiration biopsy is performed if tumour is located near chest wall;

–         transbronchial puncture of lymph nodes is carried out during bronchoscopy.

During diagnostics of mediastinal neoplasms applied according to indications bronchography, esophagography and pneumoperitoneum.

 

Variants of clinical course and complications

Neurogenic tumours are the most commoeoplasms of mediastinum, which occur in 20 % among the tumours of this location. There arise in any age and more often benign. Their predominant localization is the posterior mediastinum. The origin of such tumours could be nervous trunks, ganglions and other nervous structures of mediastinum. From the cells of sympathetic nervous trunk arise ganglioneuromas, neuroblastomas and sympathoblastomas. Slide

The tumours, which arise from peripheral nerves are represent by neuromas and neurofibromas. The paragangliomas and mediastinal pheochromocytoma develop from chemoceptor cells of and according to the structure resemble the tumours of sinocarotid zone. They frequently produce hormones, and manifests by hypertension with often crises. In half of patients these tumours are malignant.

Neurogenic sarcoma is a malignant tumour of nervous sheath. It is usually solitary, or in association with von Recklinhausen’s disease.

The neurogenic tumours commonly manifest by backache, hyperalgesia, pareses and paralyses at tumour growth trough the spinal canal. The pain reaction varies from slight to severe expressed neuralgias. Frequently ganglioneuroma is asymptomatic for many years. In the clinical pattern of malignant neurogenic tumours prevail general intoxication, loss of weight and pain syndrome.

Roentgenologically in neurogenic tumour of a vertebrocostal angle observed characteristic intensive rounded shadow, with vertebral and costal usuration, rib. Frequently revealed hemorrhagic pleural effusion on the side of lesion by malignant tumour.

Ganglioneuroma

 

 

Mesenchymal tumours. According to the histological origin mesenchymal benign tumours are represented by:

–      from a fibrous connecting tissue – fibroma;

–      from a cartilaginous and osseous tissue – chondroma, osteochondroma, osteoblastoclastoma;

–      from a spinal cord – chordoma;

–      from fat tissues – lipoma, hibernoma;

–      the tumours, which originate from vessels – hemangioma, lymphangioma;

–      from a muscular tissue – leiomyoma, rhabdomyoma.

Lipomas are the most frequent mesenchymal benign tumours with predominant location in cardiodiaphragmatic angle.

The clinical symptomatology of these tumours is atypical. At the small sizes they are usually asymptomatic. The malignant neoplasms manifest much earlier as the result of prompt infiltrative growth of the tumour and intoxication of the organism. Nevertheless, despite the malignant character, liposarcoma can grow rather slowly with late metastatic spread.

On roentgenogram such masses represented by homogeneous formations, that applies to heart shadow.

The lymphomas occur in 3-5 % of patients with mediastinal tumors and in 20-25 % with all malignant neoplasms of mediastinum. The lymphomas arise from mediastinal lymph nodes. Their common localization – anterior mediastinum, nevertheless lymph nodes of any part can be affected. There are three types of lymphomas: lymphosarcoma, reticulosarcoma and lymphogranulomatosis. All of them are characterized by malignant course. The initial signs of this pathology mainly caused by intoxication: malaise, subfebrile or febrile temperature with further remittent character, sweating and loss of weight. One of most typical manifestation of the disease should be considered itching of skin. Abnormally enlarged lymph nodes can compress mediastinal organs, which lead to dry cough, chest pain, and dyspnea. In lymphogranulomatosis, in contrast with other mediastinal tumours, the signs of compression are weakly expressed. It is characterized by bilateral lesion and blood changes (leukocytosis or leukopenia and elevation of erythrocyte sedimentation rate). Lymphosarcoma differs by more prompt course and considerable progression of mediastinal compression syndrome. X-ray examination, mediastinoscopy and biopsy of lymph nodes are the most valuable for diagnostics.

The dermoid cysts and teratomas arise owing to disturbance of embryogenesis and occur in 5-8 % of the patients with mediastinal tumors. The origin of dermoid cysts is the ectoderm, which transforms to a fibrous connecting tissue. The cystic cavity frequently contains similar to fat viscous mass of brown color with deposits of skin and hair. The teratomas arise from several germinal laminas and contain different structural tissues. They are divided on mature and immature. All mature teratomas, as a rule, are characterized by well-defined capsule, irregular rounded or oval shape, different size, and look like a cyst on slit. Immature ones look like solitary nodes, sometimes with small cavities. The structure of teratomas can include parts of glands, teeth, bones and sometimes even underdeveloped fetus. According to degree of cellular differentiation teratoma divided on benign (80-90 %) and malignant (10-20 %). Although this disease is always congenital, it diagnosed, mainly, in elderly age at occurrence of pain and “compression syndrome”. 95 % of such cysts are located in anterior mediastinum and frequently are accompanied with cardiovascular disturbances (tachycardia, pressing pain in the region of heart). The compression of major bronchi and trachea results in occurrence of dyspnea, paroxysmal cough and hemoptysis. The infection of the tumour frequently leads to fever and increase of intoxication. The presence in sputum of hair and other tissues is considered to be the sign of bronchial fistula.

In diagnostics of teratomas the major value has a complex X-ray examination. It is possible to find out teeth, calcification of cystic capsule and its contents. Prompt growth of the tumour, disappearance of its regular contours suspects the malignancy.

Cysts of pericardium are the cavity thin-walled formations, which according to the structure resemble pericardium. They occur in 7-8 % of cases of all mediastinal tumors. Their most often location is the right cardiodiaphragmatic angle, much less often – left one. A true cyst may be single-, double- or multichamber, connected or non-connected with pericardium. The cases, when the cystic cavity communicates with the cavity of pericardium, it should be considered as diverticulum. The disease, as a rule, asymptomatic and it is casually revealed only during prophylactic photoroentgenography. In cases of great size of cyst the patients complain of pain in the region of heart and cardiac arrhythmia. During X-ray examination mesothelial cyst or pericardial diverticulum observed as oval or semicircular homogeneous shadow with regular outline, which intimately applies to the shadow of heart.

 

Cysts of pericardium. Computer tomography

 

 

Bronchogenic and enteric cysts of mediastinum arise in the period of intrauterine development and originated from dystopic germs of bronchial or intestinal epithelium.

Bronchogenic cysts, as a rule, are single chamber, with location either in mediastinum, or in pulmonary tissue. The neighborhood of cyst with bifurcation of trachea can cause paroxysmal cough, dyspnea and respiratory disturbances. Paraesophageal location of the cyst manifests by dysphagia. The clinical manifestations of such pathology can be caused by inflammatory process in cyst or its sudden discharge into airways. If the cyst is communicated with airways, the roentgenogram reveals a fluid level, and during bronchography a contrast agent fills in the lumen of cyst. The diagnosis is possible to confirm by means of pneumomediastinography.

The enteric cyst (enterocystoma) arises from the dorsal parts of a primary intestinal tube, located more often in lower parts of posterior mediastinum and applies to esophagus. Depending on histology they are divided on esophageal, gastric and intestinal cyst. In cases, when a cystic wall is covered by gastric epithelium, which produces hydrochloric acid, an ulceration of wall, bleeding and its perforation can develop.

Frequently enteric cyst suppurates with the hazard of discharge into pleural space or pericardium, esophagus and bronchi. The most often signs of the disease is dyspnea and chest pain. Due to characteristic localization of the pathological focus in posterior mediastinum, to the right from median line, the roentgenological diagnostics is usually not difficult. In order to determine mutual relation of neoplasm to trachea, bronchi, and esophagus it is possible to apply pneumomediastinography. As there is always a danger of the development of complications, it is necessary to consider such pathology as absolute indication for operative removal.

Echinococcosis of mediastinum occurs rarely (1-2 % of all mediastinal tumors). If the parasite is of small size, the disease most often asymptomatic. The enlargement of echinococcal cyst causes pain, dyspnea, dysphagia and superior vena cava syndrome. Sometimes in such patients the cyst can discharge into bronchus or trachea. The suppuration of parasitogenic cysts is transformed into abscess and purulent mediastinitis. Roentgenological method should be considered to be predominant in the diagnostics of echinococcosis of mediastinum. The presence of homogeneous, round or oval shadow with regular outline (with further calcification) suggests echinococcosis. For confirming of the diagnosis reaction of latex-agglutination is performed. Echinococcosis of mediastinum frequently associated with anatomical lesion of lungs.

Principles of diagnostics in such situation are similar to primary mediastinal tumors.

 

Tactics and choice of treatment

The presence of mediastinal tumors requires surgical treatment. Expectant tactics and dynamic observation in such cases are unjustified.

In connection with a constant and substantial threat of infection, perforation and development of purulent intrapleural complications, the pericardial, bronchogenic and enteric cysts of mediastinum are the subjects to operative remove.

The operative treatment of malignant mediastinal tumors should be applied on early stages of the disease. In advanced cases it is expedient to apply antineoplastic and radiotherapy.

The benigeurogenic tumours are removed in surgical way through thoracotomy incision. If the tumour grow like a ” sandglasses” a fragment of the tumour removed with following resection of vertebral arches. The malignant tumours are removed “іn mass”, with maximal excision of the tumour, affected ribs, paraaortic, esophageal and paratracheal lymph nodes.