Cardiospasm

Esophageal dilations are characterized by a gigantic enlargement throughout the entire length of the esophageal cavity with characteristic morphological changes in its walls with a sharp narrowing of its cardiac segment, called cardiospasm.

It is generally accepted that the first description of cardiospasm was given by the English surgeon T. Willis in 1674. After the introduction of the esophagoscope and X-ray examination, cases of diagnosis of this disease became much more frequent. Thus, in the leading surgical clinics of European countries from 1900 to 1950, about 2000 cases of megaesophagus were registered. Similar data were given in the works of B.V. Pstrovsky, E.A. Berezov, B.A. Korolev and others. The frequency of cardiospasm in relation to all diseases of the esophagus and cardia, according to different authors, is from 3.2 to 20%. According to epidemiological data, cardiospasm with megaesophagus is most often found in underdeveloped countries, which is associated with poor nutrition (avitaminosis strongi), as well as with the invasion of some "exotic" parasitic infections, such as Trepanosoma Crusii. Cardiospasm is observed at any age, but it is more common in people over 20-40 years old with equal incidence in both sexes.

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Cause of cardiospasm

The causes of megaesophagus can be numerous internal and external pathogenic factors, as well as embryogenesis disorders and neurogenic dysfunctions leading to its total expansion.

Internal factors include prolonged spasms of the cardia, supported by an ulcer of the esophagus, its traumatic injury associated with swallowing disorders, the presence of a tumor, as well as exposure to toxic factors (tobacco, alcohol, vapors of harmful substances, etc.). These factors should also include stenosis of the esophagus associated with its damage in scarlet fever, typhoid, tuberculosis and syphilis.

External factors include various types of diaphragmatic diseases (sclerosis of the esophageal opening of the diaphragm, accompanied by adhesions, subdiaphragmatic pathological processes of the abdominal organs (hepatomegaly, splenomegaly, peritonitis, gastroptosis, gastritis, aerophagia) and supradiaphragmatic pathological processes (mediastinitis, pleurisy, aortitis, aortic aneurysm).

Neurogenic factors include damage to the peripheral nervous system of the esophagus, which occurs with some neurotropic infectious diseases (measles, scarlet fever, diphtheria, typhus, poliomyelitis, influenza, meningoencephalitis) and poisoning with toxic substances (lead, arsenic, nicotine, alcohol).

Congenital changes in the esophagus leading to its gigantism apparently occur at the stage of its embryonic development, which subsequently manifests itself in various modifications of its walls (sclerosis, thinning), however, genetic factors, according to S. Surtea (1964), do not explain all the reasons for the occurrence of megaesophagus.

Contributing factors leading to esophageal dilation may include neurotrophic disorders, which entail imbalance in the body's acid-base balance and changes in electrolyte metabolism; endocrine dysfunctions, in particular the pituitary-adrenal system, the sex hormone system, and dysfunctions of the thyroid and parathyroid glands. The contributing influence of allergies, which cause local and general changes in the function of the neuromuscular apparatus of the esophagus, is also possible.

The pathogenesis of cardiospasm has not been sufficiently studied due to the rarity of this disease.

There are several theories, but none of them individually explains this essentially mysterious disease. According to many authors, the basis of this disease is the phenomenon of cardiospasm, interpreted as a deterioration in the patency of the cardia, occurring without an organic stricture, accompanied by dilation of the overlying sections of the esophagus. The term "cardiospasm", introduced in 1882 by J. Mikulicz, became widespread in German and Russian literature, where this disease was sometimes called "idiopathic" or "cardiotonic" dilation of the esophagus. In Anglo-American literature, the term "achalasia" is more common, introduced in 1914 by A. Hurst and denoting the absence of a reflex to open the cardia. In French literature, this disease is often called "megaesophagus" and "dolichoesophagus". In addition to the above terms, the same changes are described as esophageal dystonia, cardiostenosis, cardiosclerosis, phrenospasm, and hiatospasm. As T.A. Suvorova (1959) notes, such a variety of terms indicates not only the ambiguity of the etiology of this disease, but also, to no lesser extent, the lack of clear ideas about its pathogenesis. Of the existing "theories" of the etiology and pathogenesis of megaesophagus, T.A. Suvorova (1959) cites the following.

1. Congenital origin of megaesophagus, as an expression of gigantism of internal organs as a result of malformation of elastic connective tissue (K. Strongard). Indeed, although megaesophagus in the overwhelming majority of cases is observed after 30 years, it is often found in infants. R. Hacker and some other authors consider megaesophagus a disease similar to Hirschsprung's disease - hereditary megacolon, manifested by constipation from early childhood, enlarged abdomen (flatulence), intermittent ileus, poor appetite, developmental delay, infantilism, anemia, the ampulla of the rectum is usually empty; radiologically - expansion of the descending colon, usually in the sigmoid region; occasionally - diarrhea due to the fact that fecal matter irritates the intestinal mucosa for a long time. An objection to this theory is provided by those observations where it was possible to radiologically trace an initial small expansion of the esophagus with subsequent significant progression.
2. Mikulicz's theory of essential spasm of the cardia: active spasm of the cardia caused by loss of influence of the vagus nerve and the reflex of opening during the passage of the food bolus.
3. Phrenospasm theory. A number of authors (J.Dyllon, F.Sauerbruch, etc.) believed that the obstruction in the esophagus is created as a result of primary spastic contracture of the diaphragm crura. Numerous experimental and clinical observations have not confirmed this assumption.
4. Organic theory (H. Mosher). Deterioration of the patency of the cardia and expansion of the esophagus occur as a result of epicardial fibrosis - sclerotic processes in the so-called liver tunnel and adjacent areas of the lesser omentum. These factors create a mechanical obstacle to the penetration of the food bolus into the stomach and, in addition, cause irritation of the sensitive nerve endings in the area of the cardia and contribute to its spasm. However, sclerotic changes are not always detected and, apparently, are a consequence of a long-term and advanced disease of the esophagus, and not its cause.
5. Neuromyogenic theory, which describes three possible variants of the pathogenesis of megaesophagus:
   1. the theory of primary atony of the esophageal muscles (F. Zenker, H. Ziemssen) leads to its expansion; an objection to this theory is the fact that during cardiospasm, muscle contractions are often more energetic than normal; subsequent muscle atony is obviously secondary in nature;
   2. theory of vagus nerve damage; in connection with this theory it should be recalled that the 10th pair of cranial nerves ensures peristaltic activity of the esophagus and relaxation of the cardia and juxtacardiac region, while n.sympathycus has the opposite effect; therefore, when the vagus nerve is damaged, there is a preponderance of sympathetic nerves with the resulting spasm of the cardia and relaxation of the muscles of the esophagus; with cardiospasm, inflammatory and degenerative changes in the fibers of the vagus nerve are often detected; according to K.N. Sievert (1948), chronic neuritis of the vagus nerve, arising on the basis of tuberculous mediastinitis, causes cardiospasm and subsequent stenosis of the cardia; this statement cannot be considered sufficiently substantiated, since, as clinical studies have shown, even with advanced pulmonary tuberculosis and involvement of the mediastinal tissue in the process, cases of cardiospasm are very rare;
   3. achalasia theory - absence of reflex to open the cardia (A. Hurst); currently this theory is shared by many authors; it is known that opening of the cardia is caused by the passage of the food bolus through the esophagus due to the generation of its peristaltic movements, i.e. irritation of the pharyngeal-esophageal nerve endings. Probably, due to some reasons, this reflex is blocked, and the cardia remains closed, which leads to mechanical stretching of the esophagus by the efforts of the peristaltic wave.

According to most authors, of all the theories listed above, the most substantiated is the theory of neuromuscular disorders, in particular achalasia of the cardia. However, this theory does not answer the question: damage to which part of the nervous system (vagus nerve, sympathetic nerve or corresponding structures of the central nervous system involved in the regulation of esophageal tone) leads to the development of megaesophagus.

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Pathological anatomy

The dilation of the esophagus begins 2 cm above the cardia and covers its lower part. It differs from the changes in the esophagus in diverticula and from its limited dilations in strictures, which occupy only a certain segment above the stenosis of the esophagus. Pathological changes in the esophagus and cardia vary significantly depending on the severity and duration of the disease. Macro- and microscopic changes occur mainly in the juxtacardial region of the esophagus and are manifested in two types.

Type I is characterized by an extremely small diameter of the esophagus in its lower segment, reminiscent of a child's esophagus. The muscular membrane in this area is atrophic, and microscopically, a sharp thinning of the muscle bundles is detected. Between the muscle bundles, there are layers of coarse fibrous connective tissue. The overlying sections of the esophagus are significantly dilated, reaching 16-18 cm in width, and have a saccular shape. Dilation of the esophagus is sometimes combined with its elongation, due to which it acquires an S-shape. Such an esophagus can hold over 2 liters of liquid (a normal esophagus holds 50-150 ml of liquid). The walls of the dilated esophagus are usually thickened (up to 5-8 mm), mainly due to the circular muscle layer. In rarer cases, the walls of the esophagus atrophy, become flabby and easily stretchable. Stagnation and decomposition of food masses lead to the development of chronic non-specific esophagitis, the degree of which can vary from catarrhal to ulcerative-phlegmonous inflammation with secondary phenomena of periesophagitis. These inflammatory phenomena are most pronounced in the lower sections of the dilated esophagus.

Type II changes in the juxtacardial segment of the esophagus are characterized by less pronounced atrophic changes and, although the esophagus in this segment is narrowed compared to the lumen of the normal esophagus, it is not as thin as with type I changes. With this type of megaesophagus, the same histological changes are observed in the dilated part of the esophagus, but they are also less pronounced than with type I. The overlying sections of the esophagus are not dilated to the same extent as with type I, the esophagus has a fusiform or cylindrical shape, however, due to less pronounced congestion, inflammatory changes do not reach the same extent as with a giant S-shaped esophagus. Existing long-term observations (more than 20 years) of patients suffering from type II esophageal dilation refute the opinion of some authors that this type is the initial stage leading to the formation of type I megaesophagus.

In both types of macroanatomical changes in the esophageal wall, certain morphological changes are observed in the intramural nerve plexus of the esophagus, characterized by regressive-dystrophic phenomena in the ganglion cells and nerve bundles. All types of dystrophy are observed in the ganglion cells - dissolution or wrinkling of the protoplasm, pycnosis of the nuclei. Significant morphological changes are observed in the thick and medium-caliber pulpal nerve fibers of both the afferent pathway and the efferent fibers of the preganglionic arch. These changes in the intramural plexus occur not only in the narrowed segment of the esophagus, but throughout its entire length.

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Symptoms and clinical picture of cardiospasm

The initial period of the disease proceeds unnoticed, possibly from childhood or adolescence, but during the period of formed cardiospasm and megaesophagus, the clinical picture manifests itself with very vivid symptoms, the leading one being dysphagia - difficulty in passing a food bolus through the esophagus. The disease may develop acutely or manifest itself with gradually increasing symptoms. As A.M. Ruderman (1950) notes, in the first case, during a meal (often after a nervous and mental shock), a sensation of a delay in the esophagus of a dense food bolus, and sometimes liquid, suddenly occurs, accompanied by a feeling of bursting pain. After a few minutes, the food slips into the stomach and the unpleasant sensation passes. Later, such attacks are renewed and lengthened, the time of food retention is lengthened. With a gradual development of the disease, at first there are slight, barely noticeable difficulties in the passage of dense food products, while liquid and semi-liquid food passes freely. After some time (months and years), the symptoms of dysphagia increase, and difficulties in passing semi-liquid and even liquid food arise. Swallowed food masses stagnate in the esophagus, and fermentation and putrefaction processes begin to develop in them with the release of corresponding "gases of decay of organic substances". The food blockage itself and the released gases cause a feeling of distension of the esophagus and pain in it. In order to move the contents of the esophagus into the stomach, patients resort to various techniques that increase intrathoracic and intraesophageal pressure: they make a series of repeated swallowing movements, swallow air, compress the chest and neck, walk and jump while eating. Regurgitated food has an unpleasant rotten smell and an unchanged character, so patients avoid eating in society and even with their families; they become withdrawn, depressed and irritable, their family and work life is disrupted, which generally affects their quality of life.

Thus, the most pronounced syndrome in cardiospasm and megaesophagus is the triad - dysphagia, a feeling of pressure or chest pain and regurgitation. Cardiospasm is a long-term disease that lasts for years. The general condition of patients gradually worsens, progressive weight loss, general weakness appear, and work capacity is impaired. In the dynamics of the disease, stages of compensation, decompensation and complications are distinguished.

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Complications

Complications are observed in advanced stages of the disease. They are subdivided into local, regional and generalized. Local complications, in essence, are part of the clinical manifestations of the developed stage of megaesophagus and manifest themselves from catarrhal inflammation of the mucous membrane to its ulcerative-necrotic changes. Ulcers can bleed, perforate, and degenerate into cancer. Regional complications in cardiospasm and megaesophagus are caused by the pressure of the huge esophagus on the organs of the mediastinum - the trachea, recurrent nerve, and superior vena cava. Reflex cardiovascular disorders are observed. Pneumonia, abscesses and pulmonary atelectasis can develop due to aspiration of food masses. General complications arise due to exhaustion and the general severe condition of patients.

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Diagnostics

Diagnosis of cardiospasm in typical cases does not cause difficulties and is based on the anamnesis, patient complaints, clinical symptoms and instrumentally obtained signs of the disease. The anamnesis and characteristic clinical picture, especially clearly manifested in the progradient stage of the disease, give grounds to suspect cardiospasm. The final diagnosis is established using objective research methods. The main ones are esophagoscopy and radiography; probing is of lesser importance.

The esophagoscopic picture depends on the stage of the disease and the nature of the changes in the esophagus. In megaesophagus, the esophagoscope tube inserted into the esophagus moves freely without encountering any obstacles, and a large gaping cavity is visible in which it is impossible to examine all the walls of the esophagus at the same time, for which it is necessary to move the end of the tube in different directions and examine the inner surface of the esophagus in parts. The mucous membrane of the dilated part of the esophagus, in contrast to the normal picture, is collected in transverse folds, inflamed, edematous, and gynecologic; it may have erosions, ulcers, and areas of leukoplakia (flat, smooth spots of a whitish-gray color that look like plaque that does not come off when scraped off; leukoplakia, especially the warty form, is considered a precancerous condition). Inflammatory changes are more pronounced in the lower part of the esophagus. The cardia is closed and looks like a tightly closed rosette or slit, located frontally or sagittally with swelling edges, like two closed lips. Esophagoscopy can exclude cancer, peptic ulcer of the esophagus, its diverticulum, as well as organic stricture caused by a chemical burn or cicatrizing peptic ulcer of the esophagus.

The chest pains observed with cardiospasm and megaesophagus can sometimes simulate heart disease. The latter can be differentiated with an in-depth cardiological examination of the patient.

X-ray examination of cardiospasm and megaesophagus provides very valuable data in relation to both direct and differential diagnosis. The visualized picture during radiography of the esophagus with contrast depends on the stage of the disease and the phase of the functional state of the esophagus during radiography. As A. Ruderman (1950) notes, in the initial, rarely detected stage, intermittent spasm of the cardia or distal part of the esophagus is detected without persistent retention of the contrast agent.

The swallowed suspension of the contrast agent slowly sinks into the contents of the esophagus and outlines the gradual transition of the dilated esophagus into a narrow symmetrical funnel with smooth contours, ending in the area of the cardiac or diaphragmatic sphincter. The normal relief of the mucous membrane of the esophagus completely disappears. Often it is possible to detect rough unevenly dilated folds of the mucous membrane, reflecting esophagitis accompanying cardiospasm.

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Differential diagnosis of cardiospasm

Each case of cardiospasm, especially in the initial stages of its development, should be differentiated from a relatively slowly developing malignant tumor of the cardiac segment of the esophagus, accompanied by narrowing of the juxtacardiac section and secondary expansion of the esophagus above the narrowing. The presence of uneven jagged outlines and the absence of peristaltic contractions should raise suspicion of a cancerous lesion. For differential diagnosis, all sections of the esophagus and its walls along its entire length are subject to examination. This is achieved by the so-called multi-projection examination of the patient. The lower section of the esophagus and especially its abdominal part are clearly visible in the second oblique position at the height of inspiration. In difficult cases, A. Ruderman recommends examining the esophagus and stomach with "effervescent" powder. During artificial inflation of the esophagus, the opening of the cardia and penetration of the contents of the esophagus into the stomach with the appearance of air in the cardiac section of the latter are clearly observed on the X-ray screen. Usually, with cardiospasm, there is no air in the cardiac region of the stomach.

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Treatment of cardiospasm

There is no etiotropic or pathogenetic treatment for cardiospasm. Numerous therapeutic measures are limited to symptomatic treatment aimed at improving the patency of the cardia and establishing normal nutrition for the patient. However, these methods are effective only at the onset of the disease, until organic changes in the esophagus and cardia have developed, and when dysphagia is transient and not so pronounced.

Non-surgical treatment is divided into general and local. General treatment involves normalizing the general and dietary regimen (high-energy nutrition, soft and semi-liquid foods, exclusion of spicy and sour foods). Medicinal preparations used include antispasmodics (papaverine, amyl nitrite), bromides, sedatives, mild tranquilizers (phenazepam), B vitamins, ganglionic blocking agents. Some clinics use suggestion and hypnosis methods developed in the mid-20th century.

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Method of mechanical expansion of the esophagus

T.A. Suvorova classifies these methods as "bloodless methods of surgical treatment". For mechanical expansion of the esophagus in case of cardiospasm, cicatricial stenosis after infectious diseases and chemical burns of the esophagus, various types of bougies (instruments for expansion, examination and treatment of some tubular organs; the technique of esophageal bougienage is described in more detail in the description of chemical burns of the esophagus) and dilators with various methods of their introduction into the esophagus have been used since ancient times. Bougienage as a method of bloodless expansion of the cardia has proven to be ineffective. The dilators used for this purpose are hydrostatic, pneumatic and mechanical, which have found application abroad. In the USA and England, the Plummer hydrostatic dilator is widely used. The principle of operation of these instruments is that the expanding part (balloon or spring expansion mechanism) is inserted into the narrowed part of the esophagus in a collapsed or closed state and there it is expanded by introducing air or liquid into the balloon to certain dimensions, regulated by a manometer or by a manual mechanical drive.

The balloon must be precisely positioned in the cardiac end of the esophagus, which is checked by fluoroscopy. The hydrostatic dilator can also be inserted under visual control using esophagoscopy, and some doctors, for greater safety, insert it along a guide thread swallowed 24 hours before the procedure. It should be borne in mind that during the expansion of the cardia, quite severe pain occurs, which can be reduced by a preliminary injection of an anesthetic. A positive therapeutic effect occurs only in some patients, and it manifests itself immediately after the procedure. However, in most cases, 3-5 procedures or more are required to achieve a longer-lasting effect. According to some foreign authors, satisfactory results from hydrostatic dilation of the esophagus reach 70%, but complications in the form of esophageal rupture, bloody vomiting and aspiration pneumonia exceed 4% of all procedures performed.

Of the metal dilators with a mechanical drive, the most widely used, especially in Germany in the mid-20th century, was the Stark dilator, which is also used by Russian specialists. The expanding part of the dilator consists of four diverging branches; the dilator is equipped with a set of removable guide attachments of different shapes and lengths, with the help of which it is possible to find the lumen of the narrowed cardiac canal. The Stark device is inserted into the cardia in a closed state, then quickly opened and closed 2-3 times in a row, which leads to forced expansion of the cardia. At the moment of expansion, severe pain occurs, which immediately disappears when the device is closed. According to published data, the author of the device (H. Starck) himself has the greatest number of observations on the use of this method: from 1924 to 1948, he treated 1118 patients, of which 1117 received a good result, only in one case there was a fatal outcome.

Esophageal dilation methods are indicated at the initial stage of cardiospasm, when gross cicatricial changes, severe esophagitis and ulceration of the mucous membrane have not yet developed. A single dilation fails to achieve a stable therapeutic effect, so the procedure is repeated several times, and repeated manipulations increase the likelihood of complications, which include strangulation and injury to the mucous membrane, ruptures of the esophageal wall. With an elongated and curved esophagus, the use of dilators is not recommended due to the difficulty of inserting them into the narrowed part of the cardia and the risk of esophageal rupture. According to domestic and foreign authors, when treating patients with cardiospasm using the cardiodilation method at the initial stage, recovery occurs in 70-80% of cases. The remaining patients require surgical treatment.

Complications during cardia dilation and the use of balloon probes are not uncommon. According to various authors, the frequency of ruptures when using pneumatic cardiodilators ranges from 1.5 to 5.5%. A similar mechanism of esophageal rupture at the diaphragm level is sometimes observed during rapid filling of a balloon probe used for closed hypothermia of the stomach, or a Sengstaken-Bleiker probe to stop gastric or esophageal bleeding. In addition, as B.D. Komarov et al. (1981) point out, esophageal rupture can occur when the patient tries to independently remove the probe with an inflated balloon.

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Surgical treatment of cardiospasm

Modern anesthesiology and thoracic surgery methods allow to significantly expand the indications for surgical treatment of cardiospasm and megaesophagus, without waiting for irreversible changes in the esophagus and cardia. Indications for surgical intervention are persistent functional changes in the esophagus that persist after repeated non-surgical treatment and, in particular, dilation of the esophagus by the described methods. According to many surgeons, if even after two dilations at the very beginning of the disease the patient's condition does not improve steadily, then he should be offered surgical treatment.

Various reconstructive surgical methods have been proposed both on the esophagus and diaphragm itself and on the nerves innervating it, many of which, however, as practice has shown, have proven ineffective. Such surgical interventions include operations on the diaphragm (diaphragmo- and crurotomy), on the expanded part of the esophagus (esophagoplication and excision of the esophageal wall), on the nerve trunks (vagolysis, vagotomy, symnatectomy). Most of the methods of surgical treatment of cardiospasm and megaesophagus were proposed at the beginning and first quarter of the 20th century. Improvement of surgical methods of treatment for this disease continued in the middle of the 20th century. The methods of these surgical operations are given in manuals on thoracic and abdominal surgery.

Esophageal injuries are divided into mechanical ones with a violation of the anatomical integrity of its wall and chemical burns, which cause no less, and in some cases more severe damage not only to the esophagus, but also to the stomach with symptoms of general intoxication.

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