ACHALASIA – online surgical procedure

ACHALASIA – online surgical procedure

It was Hurst who first gave the more accurately descriptive name of achalasia to a curious abnormality of the esophagus frequently and incorrectly called cardiospasm. I say incorrectly because the site of obstruction is never at the cardia but at a point in the lower esophagus, 1 to 2 cm proximal to the cardia. Furthermore, spasm of the cardia is never a feature of the disease. Another term frequently applied to this condition is “megaesophagus.” This is more descriptive of the large size of Ihe esophagus proximal to the obstruction than of the underlying physiological mechanism producing the disease.

ACHALASIA – surgical procedure textbook online

While no exact cause of achalasia has ever been demonstrated, there is sufficient evidence to indicate that, in many cases, if not all, there is a disturbance of ihe intramural plexuses of Meissner and Auerbach ranging from inflammation to complete destruction of the ganglion cells. Confirmatory evidence of this theory is supplied by a study of Chagas’ disease, prevalent in the northern reaches of Brazil. It is a disease caused by a specific trypanosome which destroys the plexuses of Meissner and Auerbach resulting in disturbed physiological activity of the esophagus and also the colon, producing, on the one hand, achalasia and, on the other, a condition of the colon similar to that seen in Hirschsprung’s disease.

ACHALASIA

Fig. 12. Roentgenogram of a patient with achalasia showing the markedly con­stricted area just above the cardia with esophageal dilatation above but without elonga­tion of the organ.

Physiologically, there is little doubt that achalasia is a disorder of the emptying mechanism of the lower esophagus which is dependent on an underlying neuromuscular disturbance. Hurst’s theory emphasizes the fact that there is a lack of normal coordination between the upper portion of the esophagus and the pinch – cock lower segment, which leads to a failure of the latter to open at the appropriate moment. Studies since Hurst’s time would seem to confirm his findings. For instance, intraluminal pressure recordings show little, if any, coordinated motor activity during the act of swallowing. Cineroentgenography has graphically demonstrated the irregular, uncoordinated contractions characteristic of the upper dilated segment. Finally, cholinergic drugs (Mecholyl) produce violent contractions of the esophagus, while anticholinergic drugs (Banthine) cause a prompt reduction in the increased tonicity initiated by Mecholyl. These studies confirm Cannon’s law of denervation with respect to the marked response of the achalasic esophagus to agents which stimulate the parasympathetic nervous system. Conversely, drugs which stimulate the sympathetics do not produce any mobility of the esophagus in the patient with achalasia.

Clinically and radiologically, I have noted two varieties of achalasia. It is extremely difficult to state definitely that these are separate entities or that they represent different stages of the same disease. I am inclined to feel that they are not separate entities; Sweet’s description would seem to indicate that they are. In any event, one variety of achalasia presents an esophagus which is dilated and hypertrophied above the obstructed segment, but there is not the elongation or S-shaped deformity usually associated with the second variety (Figs. 12, 13, 14, 15).

ACHALASIA

Fig. 13. Further stage in the development of achalasia of the esophagus with well-defined S-shaped reduplication and deformity of the organ.

Furthermore, in the latter type, the muscle layer at the site of obstruction is atrophic and thinned-out, quite different from the markedly thickened musculature noted proximal to the obstruction. In the first variety, the muscle layer at the point of obstruction is frequently much thicker but not nearly as hypertrophied as the musculature proximally. Physiologically, the Mecholyl test is positive in both varieties. From clinical observation of the life history of over 100 patients with this disease and analysis of the findings at operation and subsequent course in the follow-up period, I would venture the opinion that these individuals vary in the speed with which they dilate and elongate their esophagi. Some dilate and elongate in a relatively short time, while others, constituting the minority, maintain a fairly straight organ, dilated and tapering down to a point at the site of obstruction.

ACHALASIA

Fig. 14. Severe esophageal deformity in a long-standing case of achalasia.

Most of these patients present a long-standing history by the time they seek help. It is usually difficult to establish when the disease began because of the length of the history and the initial mildness of symptoms. Eventually, two main symptoms, dysphagia and regurgitation, become prominent. A characteristic feature of these symptoms is the irregular periodicity in severity. At times, the patient seems relatively free of any swallowing difficulty, and then, without any demonstrable reason, the disability becomes severe. When symptoms are severe, the dilated esophagus becomes filled with food and fluid, producing chest and epigastric discomfort, relieved only by regurgitation. As time goes on, the esophageal dilatation becomes more pronounced, and spillover into the trachea may occur, especially during sleep. In fact, I have seen a number of examples of unexplained attacks of recurrent bronchopneumonia in patients with marked achalasia.

ACHALASIA

Fig. 15. Extensive esophageal dilatation and reduplication in a patient with achalasia of many years’ standing.

One curious feature about achalasia is the relative good nutrition of most of these patients in spite of the long history of severe symptoms. In my experience, it is unusual to find emaciation associated with achalasia, although it is certainly what one would expect. Another point is worthy of emphasis. Many writers on the subject have emphasized the many emotional difficulties exhibited by almost all these patients and some have labeled achalasia as a “psychosomatic” disease. I would like to express my disagreement in this regard. Careful survey of the history of these patients indicate that emotional troubles (worry, embarrassment, fear, exaggerated introspection, and withdrawal phenomena) began to appear after the symptoms of dysphagia and regurgitation became severe. Witness the disappearance of these so-called emotional aberrations after a successful surgical correction.

The diagnosis of achalasia can usually be made from the history alone, but the clinical investigation cannot be considered complete without roentgen examination and esophagoscopy. X-ray studies should, of course, include fluoroscopic observation of the esophagus while the patient swallows the barium mixture. In recent years, cinefluorography has become an important part of the work-up in many clinics. Esophagoscopy is especially important in the first variety of achalasia I have described because the picture of a straight dilated esophagus tapering to a point at the lower end can be duplicated exactly by an obstructing cardial carcinoma with submucosal infiltration of the lower esophageal segment. Here a esoph – agoscopist can establish the true diagnosis by noting rigidity of the organ at the lower end and by performing a punch biopsy from this area. I must emphasize this point because I have known of at least a half-dozen instances where the error of diagnosis was made in the direction of achalasia rather than carcinoma (Fig. 16). Unexpectedly finding a carcinoma of the cardia when operating for achalasia is something the surgeon should scrupulously avoid (Fig. 17). In the S-shaped variety, it may be difficult for the esophagoscopist to visualize the constricted segment because of the tortuosity, elongation, and deformity of the organ.

There is one condition of the esophagus which may be confused with true achalasia, namely, esophagospasm, usually of the acute variety. When this occurs in the lower segment, it may give rise to dysphagia of such severity as to prevent the passage of even water into the stomach. It usually occurs in hypersensitive people in otherwise good health. Acute dysphagia may occur in the middle of a meal. I recently saw an example of this disease in a robust man. The esophagoscopist was unable to pass the tight constriction at the site of spasm. Fluoroscopic examination discloses violent peristaltic and antiperistaltic waves with extreme tightness at the lower end of the esophagus. Continued attacks with retention of food may lead to various degrees of esophagitis.

ACHALASIA

ACHALASIA

ACHALASIA

Fig. 16. These x-rays indicate the great difficulty that frequently arises in making a differential diagnosis be­tween achalasia and carcinoma of the cardiac end of the stomach with sec­ondary esophageal invasion. A, Rep­resents a case of cardiospasm. B and C, Examples of carcinoma of the cardia with obstruction. Esophagos – copy and biopsy should be performed in situations of this sort.

Atropine or amyl nitrite may relieve the acute attack. Bouginage is one method of conservative treatment for patients with recurrent attacks. Recently Ellis has recommended and carried out a long myotomy of the esophagus extending from cardia to the aortic arch for this variety of diffuse esophageal spasm. I have had no experience with this procedure and can therefore offer no comment.

4 comments

  1. Joberi

    Hi, excellent article, like to check a little more regarding this process.

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