Cardiospasm

Expansions of the esophagus are characterized by a giant increase throughout the cavity of the esophagus with characteristic morphological changes in its walls during a sharp narrowing of its cardiac segment, called cardiospasm.

It is considered that the first description of cardiospasm was given by the English surgeon T. Willis (T. Willis) in 1674. After the introduction of the esophagoscope and X-ray examination, the diagnosis of this disease has become much more frequent. Thus, in the leading surgical clinics of European countries from 1900 to 1950, about 2000 cases of mega esophagus were reported. Similar data were cited in the works of BV Pstrovsky, Ye.A.Berezov, B.A. Korolev, and others. According to different authors, the frequency of cardiospasm in relation to all diseases of the esophagus and cardia is from 3.2 to 20%. According to epidemiological data, cardiospasm with mega esophagus is most common in underdeveloped countries, which is associated with malnutrition (avitaminosis strongi), as well as with the invasion of some “exotic” parasitic infections, such as Cruz trephine. Cardiospasm is observed at any age, but more often it occurs in persons older than 20-40 years with an equal incidence in both sexes.

[1], [2], [3], [4], [5], [6], [7], [8], [9],

Cause of cardiospasm

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

Internal factors include long spasms of the cardia, supported by the ulcer of the esophagus, its traumatic damage associated with impaired swallowing, the presence of a tumor, as well as exposure to toxic factors (tobacco, alcohol, a pair of harmful substances, etc.). These factors should also include esophageal stenosis associated with its lesion in scarlet fever, typhus, tuberculosis and syphilis.

The external factors include various kinds diaphragm diseases (multiple sclerosis hiatal accompanied adhesions, subdiaphragmatic pathological processes of the abdominal cavity (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 that occurs in some neurotropic infectious diseases (measles, scarlet fever, diphtheria, typhus, polio, influenza, meningoencephalitis) and toxic substances (lead, arsenic, nicotine, alcohol).

Congenital changes of the esophagus, leading to its gigantism, appear to occur at the stage of its embryonic bookmark, which subsequently manifests itself by various modifications of its walls (sclerosis, thinning), but genetic factors, according to S.Surtea (1964), do not explain all the causes the occurrence of megaesophagus.

Contributing factors leading to the expansion of the esophagus can be disorders of a neurotrophic nature, leading to an imbalance in the body's COS and changes in electrolyte metabolism; endocrine dysfunctions, in particular, the pituitary-adrenal system, the system of sex hormones, dysfunction of the thyroid and parathyroid glands. The contributory influence of allergy is also not excluded, in which local and general changes occur regarding the function of the neuromuscular apparatus of the esophagus.

The pathogenesis of cardiospasm is not well understood due to the rarity of this disease.

There are several theories, but each individually does not explain this, in essence, a mysterious disease. According to many authors, the basis of this disease is the phenomenon of cardiospasm, interpreted as a deterioration of the cardia's patency, occurring without an organic stricture, accompanied by an expansion of the overlying esophagus. The term "cardiospasm", introduced in 1882 by J. Mikulic (J. Mikulicz), was extended in German and Russian literature, where the disease was sometimes called "idiopathic" or "cardiotonic" expansion of the esophagus. In the Anglo-American literature, the term “achalasia” is more common, introduced in 1914 by A. Hurst (A. Hurst) and denoting the absence of a reflex in the opening of the cardia. In the French literature, this disease is often referred to as “mega esophagus” and “dolichoesophagus”. In addition to these terms, the same changes are described as dystonia of the esophagus, cardiostenosis, cardiosclerosis, phrenospasm and chiatospasm. According to TA Suvorov (1959), such a variety of terms indicates not only the ambiguity of the etiology of this disease, but no less than the absence of clear ideas about its pathogenesis. From the existing "theories" of etiology and pathogenesis of mega esophagus, T.A. Suvorov (1959) gives the following.

1. Congenital origin of megaesophagus, as an expression of the gigantism of the internal organs as a result of the malformation of the elastic connective tissue (K.strongard). Indeed, although megaesophagus is overwhelmingly observed after 30 years, it is often found in infants. R.Hacker and some other authors consider megaesophagus to be a disease similar to Hirshsprung's disease - a hereditary megacolon, manifested by constipation from early childhood, an increased stomach (flatulence), intermittent ileus, poor appetite, developmental delay, infantilism, anemia, ampoule the rectum is usually empty; radiographically - expansion of the descending part of the colon, usually in the sigmoid region; occasionally - diarrhea due to the fact that fecal masses irritate intestinal mucosa for a long time. Objections against this theory are those observations, where it was possible to radiographically trace the initial small expansion of the esophagus, followed by its significant progression.
2. Theory of the essential spasm of the cardia of Mikulich: the active spasm of the cardia caused by the loss of the influence of the vagus nerve and the opening reflex to the passage of the food lump.
3. Theory of phrenospasm. A number of authors (J. Dyllon, F. Sauerbruch, and others) believed that the obstruction in the esophagus is created due to the primary spastic contracture of the diaphragm legs. Numerous experimental and clinical observations have not confirmed this assumption.
4. Organic Theory (H.Mosher). The deterioration of the cardia's patency and the expansion of the esophagus occur as a result of epicardial fibrosis - sclerotic processes in the so-called hepatic tunnel and adjacent areas of the omental gland. These factors create a mechanical obstacle to the penetration of the food bolus into the stomach and, moreover, cause the phenomenon of irritation of sensitive nerve endings in the region of the cardia and contribute to its spasm. However, sclerotic changes are not always detected and, apparently, are the result of a long and advanced esophageal disease, and not its cause.
5. Neuromyogenic theory describing three possible pathogenesis of mega esophagus:
   1. the theory of primary atony of the muscles of the esophagus (F.Zenker, H.Ziemssen) leads to its expansion; an objection to this theory is the fact that with cardiospasm muscle contraction is often more energetic than normal; subsequent muscle atony, obviously, is secondary;
   2. theory of lesion of the vagus nerves; in connection with this theory, it should be recalled that X a pair of cranial nerves provides peristaltic activity of the esophagus and relaxation of the cardia and juxtacardiac region, while n.Sympathycus has the opposite effect; therefore, with the defeat of the vagus nerve, a preponderance of the sympathetic nerves occurs with the cardia spasm resulting from this and relaxation of the esophagus muscles; with cardiospasm, inflammatory and degenerative changes in the fibers of the vagus nerve are often detected; in the opinion of K.N. Sievert (1948), chronic neuritis of the vagus nerve, arising from the soil of tuberculous mediastinitis, causes cardiospasm and subsequent cardiac stenosis; this statement cannot be considered sufficiently substantiated, since, as shown by clinical studies, even with advanced pulmonary tuberculosis and involvement of mediastinum in the process of cellulose, cases of cardiospasm are very rare;
   3. achalasia theory - lack of reflex to the opening of the cardia (A.Hurst); currently this theory is shared by many authors; It is known that the 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 cardia remains closed, which leads to mechanical stretching of the esophagus by the efforts of a peristaltic wave.

According to most authors, of all the above theories, the theory of neuromuscular disorders, in particular achalasia of the cardia, is the most reasonable. However, this theory does not allow to answer the question: the defeat of which part of the nervous system (the vagus nerve, the sympathetic nerve or the corresponding CNS structures involved in the regulation of the tone of the esophagus) leads to the development of mega esophagus.

[10], [11], [12], [13],

Pathological anatomy

The expansion of the esophagus begins 2 cm above the cardia and covers the lower part. It differs from changes in the esophagus during a diverticulum and from its limited extensions in strictures that occupy only a certain segment above the stenosis of the esophagus. Pathological changes in the esophagus and cardia vary considerably depending on the severity and duration of the disease. Macro and microscopic changes occur mainly in the juxtacardialis segment 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, resembling the child's esophagus. The muscular layer in this area is atrophic, and a sharp thinning of the muscle bundles is microscopically detected. Between the muscle bundles there are layers of coarse fibrous connective tissue. The overlying sections of the esophagus are significantly enlarged, reaching a width of 16-18 cm, and have a bag-shaped shape. Expansion of the esophagus is sometimes combined with its lengthening, because of which it becomes S-shaped. Such an esophagus can hold more than 2 liters of fluid (a normal esophagus can hold 50-150 ml of fluid). The walls of the enlarged esophagus are usually thickened (up to 5-8 mm), mainly due to the circular muscle layer. In more rare cases, the walls of the esophagus atrophy, become flabby and easily stretchable. Stagnation and decomposition of food masses lead to the development of chronic nonspecific esophagitis, the extent of which can vary from catarrhal to ulcerative-phlegmonous inflammation with secondary effects of periesophagitis. These inflammatory phenomena are most pronounced in the lower parts of the enlarged esophagus.

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

In both types of macroanatomical changes in the wall of the esophagus, certain morphological changes are observed in the intramural neural plexus of the esophagus, characterized by regressive-dystrophic phenomena in the ganglion cells and nerve bundles. All types of dystrophy are noted in ganglion cells - dissolution or shrinkage of protoplasm, pycnosis of the nuclei. Thick and medium-caliber pulpy nerve fibers of both the afferent pathway and efferent fibers of the preganglion arc are subjected to significant morphological changes. These changes in the intramural plexus occur not only in the narrowed segment of the esophagus, but throughout its length.

[14]

Symptoms and clinical picture of cardiospasm

The initial period of the disease proceeds unnoticed, perhaps from childhood or adolescence, but during the period of cardiospasm and megaesophagus that developed, the clinical picture manifests very vivid symptoms, of which dysphagia is the main difficulty in passing the food bolus through the esophagus. The disease may be acute or manifest gradually increasing intensity of symptoms. As A.M. Ruderman (1950) notes, in the first case, during a meal (often after a neuropsychic shock), a sensation of a tight lump in the esophagus and sometimes fluid accompanied by a feeling of arching pain suddenly comes. After a few minutes, the food slips into the stomach and the unpleasant feeling disappears. In the future, such attacks are renewed and prolonged, the delay time of food is extended. With the gradual development of the disease in the beginning, there are lightly noticeable difficulties in the passage of dense foods, while liquid and semi-liquid food passes freely. After some time (months and years), the phenomena of dysphagia increase, there are difficulties in passing through a semi-fluid and even liquid poor. Swallowed food masses stagnate in the esophagus, fermentation and putrefaction processes begin to develop in them with the release of the corresponding "gases of decomposition of organic substances." The food blockage itself and the gases emitted cause a sensation of pain in the esophagus and pain in it. In order to promote the contents of the esophagus into the stomach, patients resort to various techniques that increase intrathoracic and internal pressure: they make a number of repeated swallowing movements, swallow air, compress the chest and neck, walk and jump during meals. The regurgitated food has an unpleasant, rotten smell and unchanged character, so patients avoid eating in society and even with their family; they become withdrawn, depressed and irritable, their family and working life is disturbed, which generally affects their quality of life.

Thus, the triad is the most pronounced syndrome in cardiospasm and mega esophagus - dysphagia, feeling of pressure or chest pain and regurgitation. Cardiospasm is a long-lasting disease that lasts for years. The general condition of the patients gradually worsens, progressive weight loss, general weakness, and disability are appearing. In the dynamics of the disease there are stages of compensation, decompensation and complications.

[15], [16], [17]

Complications

Complications are observed when the disease is neglected. They are divided into local, regional and generalized. Local complications, in essence, are part of the clinical manifestations of the developed stage of mega esophagus and manifest from catarrhal inflammation of the mucous membrane to its ulcerative-necrotic changes. Ulcers can bleed, perforate, degenerate into cancer. Regional complications of cardiospasm and mega esophagus are caused by the pressure of the huge esophagus on the mediastinal organs - the trachea, the recurrent nerve, the superior vena cava. Reflex cardiovascular disorders are observed. Due to the aspiration of food masses, pneumonia, abscesses and atelectasis of the lung can develop. Common complications arise due to exhaustion and the general serious condition of patients.

[18], [19], [20], [21], [22], [23], [24], [25]

Diagnostics

Diagnosis of cardiospasm in typical cases of difficulties does not cause and is based on a pas history, patient complaints, clinical symptoms and instrumentally obtained signs of the disease. Anamnesis and a characteristic clinical picture, especially pronounced 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 less important.

Esophagoscopic picture depends on the stage of the disease and the nature of changes in the esophagus. When megaesophagus, the esophagoscope tube inserted into the esophagus, without encountering obstacles, moves freely in it, while there is a large gaping cavity in which it will not be possible to inspect all the walls of the esophagus at the same time, for which it is necessary to displace the end of the esophagus in different directions and examine the inner surface of the esophagus in parts. The mucous membrane of the enlarged part of the esophagus, in contrast to the normal picture, is assembled in transverse folds, inflamed, swollen, hyperemic; there may be erosion, ulcers and leukoplakia areas (flat, smooth, whitish-gray spots that look like a plaque that cannot be removed during scraping; leukoplakia, especially the warty form, is considered as a precancerous condition). Inflammatory changes are more pronounced in the lower part of the esophagus. The cardia is closed and has the form of a tightly closed rosette or gap, located frontally or sagitally with swelling edges, like two closed lips. With esophagoscopy, you can exclude a cancerous lesion, peptic ulcer of the esophagus, its diverticulum, as well as an organic stricture arising from the soil of a chemical burn or scarring peptic ulcer of the esophagus.

Observed with cardiospasm and megaesophagus, chest pain may sometimes simulate heart disease. Differentiate the latter is possible with in-depth cardiac examination of the patient.

X-ray examination of cardiospasm and mega esophagus provides very valuable data in relation to the formulation of both direct and differential diagnosis. The visualized picture during X-ray diffraction of the esophagus with contrasting depends on the stage of the disease and the phase of the functional state of the esophagus during X-ray. As A. Ruderman (1950) notes, in the initial, rarely detectable stage, an intermittent spasm of the cardia or the distal part of the esophagus is found without a persistent delay in contrasting

The swallowed suspension of the contrasting substance slowly sinks in the contents of the esophagus and describes the gradual transition of the expanded esophagus into a narrow, symmetrical funnel with smooth contours, ending in the region of the cardiac or diaphragmatic pulp. The normal relief of the mucous membrane of the esophagus disappears completely. Often it is possible to detect coarse unevenly expanded folds of the mucous membrane, reflecting the accompanying cardiospasm of esophagitis.

[26], [27], [28], [29], [30],

Differential diagnosis of cardiospasm

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

[31], [32], [33], [34], [35], [36]

Cardiospasm Treatment

Etiotropic and pathogenetic treatment for cardiospasm is absent. Numerous therapeutic measures are limited only to symptomatic treatment aimed at improving the patency of the cardia and the establishment of a normal diet for the patient. However, these methods are effective only at the onset of the disease, until organic changes of 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 provides for the normalization of the general and nutritional regime (high-energy nutrition, food products of a soft and semi-liquid consistency, the exclusion of spicy and acidic foods). Of the drugs used antispastic drugs (papaverine, amyl nitrite), bromides, sedatives, light tranquilizers (phenazepam), B vitamins, ganglioblokiruyuschie substances. In some clinics, methods of suggestion and hypnosis, developed in the middle of the 20th century, are used.

[37], [38], [39], [40], [41], [42]

Method of mechanical expansion of the esophagus

These methods TA Suvorova refers to "bloodless methods of surgical treatment." For a mechanical expansion of the esophagus with cardiospasm, cicatricial stenosis after infectious diseases and chemical burns of the esophagus, for a long time various bougies (tools for expanding, researching and treating certain tubular organs) have been used; the esophagus bougienage technique is described in more detail when describing chemical burns of the esophagus and dilators with various ways of introducing them into the esophagus. Bougienage as a method for non-blood expansion of the cardia was ineffective. The dilators used for this purpose are hydrostatic, pneumatic and mechanical, which have found application abroad. Plummer hydrostatic dilators are widely used in the USA and England. The principle of operation of these tools lies in the fact that the expanding part (balloon or spring expansion mechanism) is introduced into the constricted part of the esophagus in a collapsed or closed state and is expanded there by introducing air or liquid into the balloon to a certain size, adjustable with a manometer or manual mechanical drive.

The balloon must be located exactly in the cardiac end of the esophagus, which is checked by fluoroscopy. A hydrostatic dilator can also be administered under visual control using esophagoscopy, and some doctors, for greater safety, guide it along a guideline swallowed a day before the procedure begins. It should be borne in mind that during the expansion of the cardia appears quite pronounced pain, which can be reduced by a pre-made injection of anesthetic. A positive therapeutic effect occurs only in some patients, and it manifests itself immediately after the procedure. However, in most cases, to achieve a longer-lasting effect requires 3-5 procedures and more. According to some foreign authors, satisfactory results from hydrostatic dilatation of the esophagus reach 70%, however, complications such as rupture of the esophagus, bloody vomiting and aspiration pneumonia exceed 4% of the total number of procedures performed.

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

Methods of dilatation of the esophagus are shown in the initial stage of cardiospasm, when no major cicatricial changes, pronounced esophagitis and ulceration of the mucous membrane have yet occurred. With a single dilatation resistant therapeutic effect is not achieved, so the procedure is repeated several times, and with repeated manipulations increases the likelihood of complications, which include pinching and injury of the mucous membrane, rupture of the esophagus wall. With an elongated and curved esophagus, the use of dilators is not recommended because of the difficulty of holding them in the narrowed part of the cardia and the danger of rupture of the esophagus. According to domestic and foreign authors, in the treatment of patients with cardiospasm by cardiodilation in the initial stage, recovery occurs in 70-80% of cases. The remaining patients need surgical treatment.

Complications with dilatation of the cardia and the use of probe balloons is not uncommon. When using pneumatic cardiodilators, the frequency of breaks, according to different authors, ranges from 1.5 to 5.5%. A similar mechanism of rupture of the esophagus at the level of the diaphragm is sometimes observed also with the rapid filling of the balloon probe used to conduct closed hypothermia of the stomach, or the Sengstaken-Baker probe to stop gastric or esophageal bleeding. In addition, as indicated by B. D. Komarov et al. (1981), a rupture of the esophagus can occur when a patient tries to self-extract a probe with a bloated balloon.

[43], [44], [45]

Surgical treatment of cardiospasm

Methods of modern anesthesiology and thoracic surgery can significantly expand the indications for surgical treatment of cardiospasm and megaesophagus, without waiting for irreversible changes in the esophagus and cardia. The indications for surgical intervention are persistent functional changes in the esophagus, which persist after repeated non-operational treatment and, in particular, dilatation of the esophagus by the described methods. According to many surgeons, if even after a double-dilatation performed at the very onset of the disease, the patient’s condition does not stably improve, then he should be offered surgical treatment.

Various reconstructive surgical methods were proposed both at the esophagus and diaphragm, and at the nerves innervating it, many of which, however, as practice has shown, turned out to be ineffective. Such surgical interventions include operations on the diaphragm (diaphragm and crucotomy), on the enlarged part of the esophagus (esophagoplication and excision of the esophagus wall), on the nerve trunks (vagolysis, vagotomy, simnatectomy). Most methods of surgical treatment of cardiospasm and megaesophagus were proposed at the beginning and the first quarter of the 20th century. Improvement of surgical methods of treatment for this disease continued in the middle of the XX century. Methods of these surgeries are given in the guidelines for thoracic and abdominal surgery.

Damage to the esophagus is divided into mechanical with violation of the anatomical integrity of its walls and chemical burns, causing not less, and in some cases, more severe damage not only to the esophagus, but also to the stomach with symptoms of general intoxication.

[46], [47], [48], [49], [50], [51],