ACHALASIA – Malignant tumors of the esophagus

ACHALASIA – Malignant tumors of the esophagus

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 the esophagus proximal to the obstruction than of the underlying physiological mechanism producing the disease.

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 the 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.

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). 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.

Malignant tumors of the esophagus

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.

Malignant tumors of the esophagus

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

Malignant tumors of the esophagus

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

Malignant tumors of the esophagus

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

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.

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.

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 esophagoscopist 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.

Malignant tumors of the esophagus

Malignant tumors of the esophagus

Malignant tumors of the esophagus

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. Esophagoscopy and biopsy should be performed in situations of this sort.

Malignant tumors of the esophagus

Fig. 17. Carcinoma of the cardiac end of the stomach in a patient with longstanding achalasia.

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. 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.

Treatment. At the outset, may I say that I have not been impressed with the results of conservative therapy of well-advanced achalasia. As a matter of fact, the results have been uniformly disappointing. In the earlier stages, the patients learn to change their eating habits and seem to get along in reasonable comfort. Various parasympatholytic and sympatholytic drugs have been tried extensively with practically negative results. The one remaining nonoperative measure which has been used widely is instrumental dilatation. The regular passage of an olive – tipped bougie through the cardia or a mercury-weighted rubber tube may permit the patient to carry on a relatively normal existence. I have known patients who learned to pass the mercury-weighted tube by themselves just before each meal and continued with this treatment for many years. It seems to me that there is some danger in this form of treatment in patients with the S-shaped deformity. Because of the tortuous elongation of the organ, direction of the dilating agent toward the cardia is difficult, and perforation might ensue should too much force be exerted.

There is one remaining type of conservative therapy, if it can be called such. I refer to the use of forceful dilatation by the employment of mechanical devices such as the Mosher apparatus, the Starck dilator, and the hydrostatic dilator of Plummer. While a few good results have been reported, most writers on the subject believe this method of forceful dilatation is dangerous and ill-advised. I have seen three examples of tearing of the lower esophagus following such therapy with subsequent mediastinitis requiring extensive surgery.

The surgical treatment of achalasia has and is still going through many vicissitudes. It sems pertinent to stress the fact that, up to the present writing, there is no operative procedure which can assureevery patient with achalasia that lasting relief will be obtained. With so little known concerning the essential mechanisms involved in this disease, one is forced to the conclusion that the operations utilized today are makeshift procedures with unpredictable results. With a demonstrable constriction in the lower esophagus, the obvious mechanical approach to the problem would be the fashioning of an anastomosis between the dilated esophagus above the constriction and the upper stomach. In fact, this was first proposed by Heyrovsky who used a U-shaped anastomosis, after the manner of a Finney pyloroplasty, between the esophagus and the proximal greater curvature of the stomach and, also, by Grondahl who made a simple lateral anastomosis. About 20 years ago, I considered the Heyrovsky procedure the operation of choice for achalasia and carried it out on 22 patients. The immediate relief of symptoms was dramatic but, as time went on, over 40 percent developed severe esophagitis due to reflux from the stomach, with bleeding, stenosis, and recurrence of dysphagia, more severe than before. The resulting disability required subsequent extensive surgical procedures before relief was attained. As a result of this experience, I would advise emphatically against the employment of any operation which permits reflux of gastric contents into the esophagus.

I believe it was Evarts Graham who popularized a revival of the myotomy operation reported by Heller in 1914, a procedure known everywhere by his name. At best, it must be considered a simple makeshift procedure which does not attack the underlying mechanism that causes the disease. It aims to remove the obstructing factor in the same way as one carries out the Ramstedt procedure for hypertrophic pyloric stenosis, but it does not restore the normal peristaltic activity of the esophagus. Although there was much satisfaction among surgeons when the operation was first revived, extensive experience with it since then has dampened somewhat the original enthusiasm. From my own experience with this procedure, I am able to draw four significant conclusions. In the first place, the immediate and late results with respcct to alleviation of symptoms are unpredictable. Some patients respond dramatically, while others do not. Secondly, the results are far better and more lasting with the first variety of achalasia than with the second S-shaped type. Thirdly, I have seen 11 examples of reflux esophagitis following a properly performed Heller operation. Lastly, I have been disappointed with the appearance of the x-ray films of the esophagus in the follow-up period. Orderly peristaltic activity is not restored. There may be marked delay in the passage of the barium one day and rapid passage the next. In the S-shaped or sigmoid deformity, decrease in size of the esophagus rarely takes place (Figs. 13, 14, 15). Some diminution in the width of the esophagus may occur in the first variety of the disease. I have noted a 50 percent decrease in size one week after operation. In spite of these reservations, I still am of the opinion that the Heller operation is, at the present moment, the procedure of choice. The surgeon should, however, expect disappointments.

Technique of the Heller Operation. A study of the preoperative roentgenograms of the esophagus will give the surgeon a fairly accurate impression of the height of the left diaphragm and the location of the cardia with respect to the ensiform process and rib cage. This will aid him to make the choice between an abdominal and a thoracic approach. I have always preferred the abdominal approach and have used it almost routinely. In most individuals, adequate exposure is easily attained, and the details of the myotomy can be carried out without difficulty. It is certainly less traumatic to the patient.

The abdominal incision, usually through the midline, should be of adequate length to permit fairly wide retraction. Two broad flat retractors pulled upward and outward bring into view the left lobe of the liver. By grasping the extreme edge of the lobe with a sponge forceps and exerting careful downward traction, the surgeon brings the suspensory ligament into clear focus and permits him to divide it with a long scissors. He should avoid injury to the transverse phrenic vein which crosses anterior to the ligament. The left lobe is folded on itself, covered with a thick abdominal pad and retracted toward the right by the second assistant with the aid of a deep Deaver retractor. This maneuver brings into view the eosphageal hiatus. A transverse incision is now made in the peritoneum and phrenoesophageal ligament overlying the abdominal esophagus which is now bluntly freed from its areolar attachments (Fig. 18). A piece of Penrose tubing is placed around the esophagus for traction purposes. This helps considerably to bring the lower thoracic esophagus downward into the field of operation. Every bleeding point should be accurately ligated with fine silk. A constant dry field will simplify the myotomy incision. The vagus nerves should be pushed to one side if they appear in the field. With the aid of a No. 15 sharp blade, a longitudinal incision is made in the musculature of the esophagus starting about 2 inches above the site of constriction and continuing downward past the cardia onto the anterior wall of stomach for a distance of about 1 inch. As the circular fibers are divided the mucosa projects through the incision. Great care is needed in completing the muscle incision in the region of the constriction, the cardia, and stomach wall because of the thinness of the muscle layer. It is easy to make an opening in the mucosa at these sites. Should this occur, a few sutures of fine silk will suffice to repair the opening. To guard against reunion of the muscle fibers, which might be a possible reason for subsequent recurrence, it has been suggested by Ellis of England that a section of muscle on each side of the incision be excised after fashioning muscle flaps. At the completion of the muscle incision, its length should measure between 3 and 4 inches, and the mucosa should be prolapsed between the cut edges. The field should be completely dry (Fig. 18).

The final step should consist of an accurate repair of the phrenoesophageal ligament using interrupted sutures of 00 silk. I believe this is an important step in the prevention of postoperative herniation through the hiatus. The abdominal incision is now closed without drainage. If the mucosa has been inadvertently opened, it is good practice to drain the subphrenic area with one cigarette drain.

The postoperative care of the patient should offer no difficulties. Sips of fluid are given the next day. The amount is increased rapidly thereafter. Soft food is permitted on the fifth day, with gradual increase until the eleventh or twelfth day when a full diet is ordered. Thorough mastication and the swallowing of small boluses are necessary safeguards during the first three or four weeks (Fig. 19).

In recent years, attention has been called to the frequent association of pyloro – spasm in many of the patients with achalasia. For this reason, pyloroplasty has been advised as an adjuvant procedure at the time of myotomy. Whether it should be carried out as a routine procedure is debatable. It should not be forgotten as a possible additional step by the surgeon, because it has much to commend it.

What can the surgeon do if the constriction recurs and dysphagia becomes more pronounced than before operation? Or if reflux esophagitis develops with resultant bleeding and stenosis? In my view, these complications constitute some of the most difficult problems that one can encounter in the surgery of the gastrointestinal tract. Before resorting to surgical intervention, recourse may be had to repeated bouginage under esophagoscopic control. I have encountered a few instances of complete relief by this therapy. But it must be admitted that most of these recurrences do not respond to this measure. Under such circumstances, I have on four occasions operated again through a left transthoracic approach, utilizing the procedure of Wendel. This consists of making a longitudinal incision through all layers of the stricted area and repairing the opening in a transverse direction,

Malignant tumors of the esophagus

Malignant tumors of the esophagus

Fig. 18. Details of the Heller operation showing the upper midline incision in A, division of the phrenoesophageal ligament in B, length of the incision in the esophagus and stomach in C, and protrusion of the mucosa after excision of a wedge of muscularis above the esophagus in D.

care being taken to avoid the cardia and to accurately repair the hiatus. In two instances, the result was satisfactory, but in two others, recurrence was prompt. This operation should never be utilized for stricture secondary to esophagitis because recurrence is the rule rather than the exception.

When reflux esophagitis occurs and produces organic stenosis and/or severe bleeding episodes, the surgeon is faced with a difficult problem. A uniformly suc-

Malignant tumors of the esophagus

Fig. 19. Postoperative roentgenogram of the effect of the Heller operation, showing a wide open cardia but with little diminution in the size of the esophagus.

Malignant tumors of the esophagus

Fig. 20. Roentgenogram of a patient with achalasia of 14 years duration. The almost complete closure at the cardia is noteworthy.

cessful operative procedure has not as yet been devised for this complication. In the past, I have utilized an operative procedure consisting of removal of the proximal half of the stomach and of the lower third of esophagus, bilateral vagotomy, and complementary pyloroplasty. It is a most difficult operation from the technical standpoint because of the extensive inflammatory reaction in the posterior mediastinum. Half of the patients did well. One patient is now 14 years postoperative and is free of symptoms. The remaining 50 percent eventually developed varying degrees of esophagitis which required rigid medical management.

The technical details of this operation are essentially the same as described in the chapter on carcinoma of the cardia.

To minimize the possibility of reflux which occurs in about half of the patients subjected to esophagogastrectomy, many surgeons, in recent years, have interposed a loop of bowel (jejunum or colon) between esophagus and stomach. There is no doubt that this procedure lessens considerably the incidence of reflux esophagitis. But even this operation is not completely free of complications. I know of two instances of erosive ulceration in transplanted loops of colon, and in one there was a perforation into the posterior mediastinum.

The technique of these procedures will be found in the chapter on hiatus hernia. Because of the generally estimated 20 percent incidence of reflux esophagitis after the Heller operation, surgeons have searched for alternative or adjuvant procedures which would tend to obviate this undesirable complication. Since the most important complication following the Heller operation is reflux of acid gastric juice, it occurred to me that if gastric acidity were greatly reduced or even eliminated, the likelihood of esophagitis would be markedly lessened. This theoretical consideration is in accord with the physiological evidence obtained so far by extensive laboratory and clinical experiments. Accordingly, in three patients presenting an achalasic esophagus with S-shaped deformity, the following operation was carried out. Through an upper midline incision, the Heller myotomy was performed as already described with the additional excision of hypertrophied muscle on each side of the incision as recommended by Ellis of England. The supplemental procedures of bilateral vagotomy and antrectomy with retrocolic gastrojejunostomy completed the operation. Aside from mild vagal effects (delayed gastric emptying and abdominal distention) which were of short duration and occurred only in the second patient, the immediate convalescence was smooth. The first patient was operated upon 2 Vi years ago, the second and third 20 months and 3 months respectively. Dysphagia has been relieved completely, there is neither symptomatic nor roentgenographic evidence of esophagitis, and all are anacid (free acid after histamine) (Figs. 20 and 21). Of course, it is too soon to draw definite conclusions and the group is too small, but the basic physiological reasons for this combined operation seem sound. I believe the problem of the operative approach to achalasia should proceed along these lines. In the absence of definite knowledge of the essential etiology of the disease, it is possible that surgeons will adopt the more radical approach of removal of the diseased esophagus and the interposition of a loop of colon as a substitute organ. However, it should be remembered that these colon loops act only as conduits and are incapable of duplicating the physiological propulsive function of the normal esophagus.

Malignant tumors of the esophagus

Fig. 21. Postoperative roentgenograms demonstrating the wide open cardia. Vagotomy and antrectomy were complementary procedures.

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