Cardiospasm (achalasia of the cardia)

ACHALASIA

(CARDI~SPASM)

Cardiospasm ARTHUR M. OLSEN,

M.D.,

(Achalasia F. HENRY ELLIS, JR., M.D. Rochester, Minnesota

From tbe Sections of Medicine and Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, The Mayo Foundation is a part of the Graduate School of tbe Unioersit,y oJ Minnesxa.

HE experience at the Mayo Clinic with the condition known as cardiospasm has been reviewed in a number of communications [r-3]. The most recent statistica reports from the Clinic were published in 1951 [4] and 1933 [5,6], when a series of 601 cases seen in the twelveyear period ending in 1946 were studied. Further statistical anaIyses wiII not be attempted in this paper. Rather, our present concepts of diagnosis &II be reviewed in the light of recent physiologic observations and methods of treatment wiI1 be outlined, with due consideration for our increasing surgical experience in the management of the condition. Cardiospasm is a term that has been used for many years [7,8] for the condition of benign stenosis of the termina1 portion of the esophagus associated with deficient or absent peristaIsis of the esophagea1 musculature. OriginaIIy the condition was thought to represent spasm at the cardia and hence was caIIed cardioLater, Hurst and Rake [9] notec1 spasm. degeneration of Auerbach’s plexus in the esophagea1 waI1 and stated that the muscuIar contractions of the esophagus were inadequate, often failing to reach the cardia. Hurst offered the name achaIasia, which means faiIure to relax, and this term is the best yet proposed to describe the nature of the disturbance in question. Mega-esophagus mereIy indicates the diIatation of the organ noted in the Iater stages of the disease and is synonymous

T

of the Cardia) AND

BRIAN CREAMER,

M.D.,

with such designations as esophagectasin and dolicho-esophagus. AmpIe evidence exists that a physiologic sphincter is present in the terminal part of the esophagus. Like a11 sphincters, the one just above the esophagogastric junction opens in response to orderIy peristaItic movements of the viscus proxima1 to it. In turn, norma peristalsis apparently depends on the integrity of the tenth crania1 nerve and its various ramifications. The exact role of the parasympathetic and sympathetic nerves is not cIearIy understood, yet disturbances in neurogenic mechanisms of the esophagus do Iead to abnormaIities in the propagation of peristaItic waves and thus to various types of dysphagia. AchaIasia (or cardiospasm) denotes a state of hypomotiIity of the esophagus and must be carefuIIy distinguished from the functional disturbance known as diffuse spasm of the esophagus and from IocaIized spasm of the esophagus or cardia. In these Iatter disorders there is not onIy hypermotility but aIso a disturbance in orderIy propagation of peristaItic waves. PhvsioIogic methods of recording esophageal motility are now avaiIabIe [ro--121 and are extremely vaIuabIe aids in the differentia1 diagnosis of both functional and organic disorders. ETIOLOGY The cause of achaIasia is not known. That the condition is a neuromuscuIar disturbance has been confirmed by both pathoIogic studies [9,r3] and physioIogic technics. However, no one has expIained satisfactoriIy why degeneration of the gangIionic ceIIs in Auerbach’s pIexus takes pIace. In the days when spasm at the

OIsen, EIIis and Creamer cardia was the prevalent theory, the degenerative changes were thought to be secondary to dilatation and stretching of the waIIs of the esophagus. However, degenerative changes have been noted in extremely early stages of that the disease [14]. Etzel [IS] considered dietary deficient?of thiamine hydrochloride was responsible for this condition as well as for megacoIon (Hirschsprung’s disease) and megaureter. Etzel emphasized the poor economic status of Brazilian patients who had cardiospasm, yet in North America authorities agree that a11 cIasses of people may be afflicted and that achaIasia is not related to either eating habits or financial circumstances. The contention that achalasia is a psychosomatic disorder has been most controversia1 [r6]. It is diff< to refute the argument that the initial malfunction might be related to emotional stress or nervous tension. When the disorder can be recognized clinically by radiopressure studies or histologic Iogic means, demonstration, the patient is no longer susceptibIe to psvchiatric treatment. Furthermore, in our experience, patients who have cardiospasm are no more neurotic than are any other group of patients. CLINICAL

glottis cIosed and finaIIy force food into the stomach. Pain is not a common symptom of cardiospasm, occurring in Iess than 30 per cent of our patients [r]. It is more prominent in early stages and it is usually localized to the lolver substerna region and related to the effort ot getting food into the stomach. The patient who has cardiospasm usually loses weight, but extremeIy obese patients are seen occasionaIIy. Obvious deficiencies of vitamins are uncommon. Cough is not uncommon, and about IO per cent of our patients have had demonstrabIe evidence of aspiration pneumonitis or other compIications [ri]. Bleeding from the esophagus is rare. ROENTGENOLOGIC

FINDINGS

A detaiIed description of the roentgenologic findings in achalasia has been reported elsewhere [.$I; a brief summary will be given herein. In the early stages of cardiospasm, ineffectual hyperactivity may be noted. The primaryperistaItic wave is arrested in the upper part of the esophagus and does not trave1 to the cardia [r7]. In fact, barium may be “milked” upward instead of passing through the cardia. After some churning about, some of the barium passes into the stomach. In more advanced lesions, the coIumn of barium presents a smooth conica appearance with a beak-like extension into the region of stenosis at the cardia. (Fig. I .) As the esophagus becomes diffusely dilated the organ becomes passive and motility is absent. In advanced stages, the esophagus may become hugely diIated, tortuous, elongated and saccular. From a roentgenologic point of view, early cardiospasm may be considered as a “compensated stage ” in which frequent, ineffIcient, poorly co-ordinated esophageal contractions finally force food into the stomach. On the other hand, the esophagus in advanced lesions may be “siIent” or in the “decompensated phase” in which onIy gravity and changes in intrathoracic pressure can overcome the resistance at the cardia. PuImonary changes secondary to achalasia aIready have been mentioned. Most of these are caused by aspiration of retained material and present the picture of aspiration pneumonitis or fibrosis. The shadows noted on the thoracic roentgenogram usuaIly involve the

PICTURE

The principal symptoms of cardiospasm are reIated to esophageal obstruction. Food sticks in the loner part of the esophagus before it enters the stomach. At times the sensation of obstruction may be referred to the upper portion of the esophagus. Dysphagia may be intermittent in early stages and is often related to the type of food taken. As a rule, warm foods pass more readily than do coId foods and many times the patient wiI1 prefer solids to Iiquids. Regurgitation is common and may be first noted as soiling of the piIIow slip. Later, willful regurgitation may be employed to relieve distress. Inasmuch as esophageal contractions no Ionger open the cardia, the patient may reI3 on gravity and hydrostatic pressure to force food from the esophagus to the stomach. At times he wiII engage in certain exercises and maneuvers to alter the intrathoracic pressure. Thus it is not uncommon in advanced stages of the disease to see the patient with cardiospasm stand up while eating, lean forward with his hands on the manteIpiece or a simiIar support, grunt and strain with the breath held and 300

Cardiospasm

FIG. I. Roentgenographic appearance of achalasia. (a) Characteristic appearance of moderateIy advanced achalasia. (6) Same case; appearance after diIation. (c) Same case; appearance after subcutaneous injection of 10 mg. of mecholy1.

Measurements of esophageal pressure at rest and during the act of swaIIowing are made b? passing open-tip polyethylene tubes attached to small electromagnetic pressure transducers that are externa1 to the patient. SimuItaneous recording of pressures at various levels [12,r3] permits graphic demonstration of the orderIy progression of peristaItic waves in the norma person, whereas in patients who have cardiospasm, peristaltic waves are absent throughout most of the esophagus. (Fig. 2A and B.) In the early stages of cardiospasm, spontaneous changes in pressure often occur, apparentIy unreIated to the act of swaIIowing and probabIy corresponding to the poorIy co-ordinated non-peristaItic contractions of the esophagus

mid-portion of the lung fieIds, especially on the right side. The dilated esophagus of cardiospasm may produce mediastina1 widening on the routine roentgenogram. As a rule, the patient’s symptoms wiI1 provide an indication for roentgenoIogic studies after a swallow of barium and thus wiI1 Iead to the correct diagnosis. If dysphagia is not a prominent symptom and the esophagus is not suspected, mediastina1 widening occasionaIIy may pose a diagnostic probIem. PHYSIOLOGIC

OBSERVATIONS

Studies of esophagea1 motiIity in heaIthy peopIe and in patients who have cardiospasm have been reported in detai1 [ro-12,181. 30’

Olsen,

Ellis

and Creamer

ACHALASlA

HEALTH

PNEUMOGRAPH

MECHOLYL

FIG. 2. EsophageaI pressures ilIustrating Iower half of esophagus of patient with peristaItic wave recorded from heaIthy mecholyl in patient with achalasia. D, heaIthy person.

the essentia1 differences between achalasia and health. A, recording from achaIasia, showing absence of peristaItic wave on swaIIowing. B, norma person. C, increase in pressure after subcutaneous injection of IO mg. of absence of increase of pressure after injection of IO mg. of mechoIy1 in

noted roentgenoIogicaIIy. In the more advanced stages, however, motility is practicaIIy absent. Of particuIar diagnostic significance is the response of achaIasia to the injection of methacholine (mechoIyl@) chIoride [rg,zo]. The patient usuaIIy compIains of severe substernal pain. The radioIogist may see pronounced contraction of the entire esophagus (Fig. I b and c), and pressure detectors in pIace at the time of the injection wiI1 show a distinct and prolonged increase in pressure. (Fig. 2C.) In norma persons and in patients who have other esophagea1 disorders, this response to mecholyl is virtuaIIy absent. (Fig. 2D.) ENDOSCOPIC

TEST

DIAGNOSIS

Esophagoscopy is not of much vaIue in the diagnosis of cardiospasm but may be important in the differentiation of cardiospasm from other diseases. We do not routineIy use esophagoscopy in cases of typica cardiospasm. This examination discIoses reddening and thickening of the esophagea1 mucosa, pIus varying amounts

of mucus and retained secretions. The stenotic zone at the cardia may give way to the esophagoscope or, in some cases, it may be impossibIe to pass the instrument into the stomach. RareIy, it becomes necessary to perform esophagoscopy for the purpose of initiating diIation therapy. The passage of an oIive-tipped bougie over a previousIy swaIlowed thread is of considerabIe vaIue in the diagnosis of cardiospasm. In experienced hands the type of resistance noted when the sound passes through the stenotic cardia is unmistakable. AIthough the obstruction may be firm, it suddenly “gives” and the sensation is different from that experienced with an organic Iesion. DIFFERENTIAL

DIAGNOSIS

For obvious reasons it is most important that cardiospasm be differentiated from organic lesions affecting the Iower portion of the esophagus, particuIarIy maIignant tumors. When an oIder person with a short history of dysphagia 302

Cardiospasm spasm of the esophagus. It must be distinguished from the asymptomatic corkscrew (curIing) esophagus and the “tertiary” contractions sometimes seen roentgenographically in oIder people, and aIso from the bizarre irregularity of the esophagus in treated cardio-

presents himself, the problem may be diffIcuIt. The roentgenoIogist has much more troubIe with the diagnosis of carcinoma of the cardiac end of the stomach than he does with esophageal carcinoma. Gastric carcinomas often encircIe the terminal part of the esoph%gus without invading the mucosa. The roentgenoIogist may be unable to distinguish the obstruction from that seen in cardiospasm. Likewise, the esophagoscopist may be unabIe to see a carcinoma of the cardia and biopsy may be unsatisfactory. In such cases, smears and secretions studied by cytoIogic technics may revea1 the true nature of the condition [21]. The passage bougie over a preof a No. 41 olive-tipped viousIy swallowed thread may give considerabIe information concerning the nature of the obstruction. In case of serious doubt, transthoracic expIoration of the cardia is indicated. Benign tumors of the esophagus are rare but large peduncuIated fibroIipomas occasionaIIy may give a roentgenoIogic picture strongIy suggesting cardiospasm [22]. Even the endoscopist may not recognize the nature of such a lesion, as the mucosa covering such tumors is identical to that of the esophageal waI1. Huge sausage-Iike tumors are particuIarIy 1ikeIy to give troubIe in diagnosis. The most common benign organic stricture of the Iower part of the esophagus is that often associated with hiata1 hernias and esophagitis. The patient usuaIIy gives a Iong history of heartburn and “acid indigestion,” suggesting an incompetent cardia. By the time the stricture has formed, the esophagus is shortened. In such cases the examiner must be on the Iookout for carcinoma in the intrathoracic portion of the stomach. ScIeroderma of the esophagus may present a cIinica1 picture resembIing that of cardiospasm, and the roentgenographic changes aIso may be somewhat simiIar. The esophagea1 disorder occasionaIIy wiI1 precede the dermatoIogic manifestations. Raynaud’s phenomena are aImost invariably present when the esophagus is invoIved. In many cases of advanced scIeroderma one finds a short esophagus with an intrathoracic stomach and a stricture at the esophagogastric junction. Of the non-organic disorders, diffuse spasm of the esophagus most frequentIy is confused with achaIasia [23]. (TabIe I.) Diffuse spasm has been described variousIy as non-sphincteric pseudodiverticuIosis and segmenta spasm,

TABLE DlFFERENTlAL

DIAGNOSIS

I

OF CAKDIOSPASSf

AND

DIFFUSE

SPASM

Symptoms Signs

and

Pain. Obstruction. Regurgitation. Retention .. Psyche......... Roentgenologic findings.

.

Cardiospasm

Diffuse Spasm

Uncommon AIways Common Frequent StabIe

Abnost constant Variabk Rare Never Nervous

Diffuse dilatation

Segment:11 spasm

spasm. (Fig. ~b.) This condition tends to occur in nervous, tense people of both sexes but is somewhat more common in women. The most striking symptom is pain, usuaIIy substernal and often referred.to the back, neck, jaws, ears or even the arms. The pain may or may not be reIated to eating. Most of these patients have some diffIcuIty in swaIIowing. The dysphagia is extremeIy variabIe and is subject to nervous and emotiona inff uences. RoentgenographicaIIy, the condition invoIves the Iower haIf to two-thirds of the esophagus. It may appear as an irreguIar spasm (Fig. 3a), as diffuse narrowing (Fig. 3b) or as muItipIe pseudodiverticula. (Fig. 3c.) PhysioIogicaIly, as measured by studies of esophageal motiIity, diffuse is the opposite of cardiospasm or spasm achaIasia [24]. It is a condition of hypermotiIity with high pressures, repetitive waves and simuItaneous contractions at various IeveIs in response to the stimuIus of swallowing. Diffuse spasm frequentIy is confused with earIy cardiospasm, both cIinicaIIy and roentgenoscopicaIIy. Measurements of pressure and the mechoIy1 test are extremeIy heIpfu1 in making the differentiation. The findings are different when bougies are passed and, of course, diffuse spasm responds Iess we11 to treatment than does cardiospasm. Diffuse spasm frequently is associated with some other Iesion of the digestive tract such as duodena1 uIcer, ChoIeIithiasis, pancreatitis or hiatal hernia [23]. The correction 303

Olsen, EIIis and Creamer

Frc;. 3. Diffuse spasm

of esophagus.

(c) PseudodiverticuIosis. twelve-year experience.

of the associated disease often inffuence on diffuse spasm. NON-SURGICAL

(a) Diffuse irregular spasm.

(b) Diffuse constant narrowing.

(From: OLSEN, A. M. et a1. The treatment J. Thoracic Surg., z.2: 164~187, 1951.)

of cardiospasm:

analysis of a

peristalsis. It is unIikeIy, however, that a means wiI1 be found to replace the degenerated gangIionic ceIIs in the esophagea1 waI1. Up to the present time the most effective treatment has consisted of measures that decrease resistance at the cardia. Dilation of the cardia has been employed to great advantage for many years. Passage of bougies (Figs. 4a and b) usuaIIy gives 0nIy temporary resuIts. If IongIasting or permanent reIief is to be obtained, rather drastic and forcefu1 stretching of the cardia must be done. This usuaIIy is accompIished by inflating a properly pIaced cyIindric bag with either air or water. We have used the PIummer hydrostatic bag (Figs. 4c and cl), which is passed over a previousIy swaIIowed thread. Permanent reIief has been obtained in

has a favorable

TREATMENT

Satisfactory medical treatment for cardiospasm does not exist. Antispasmodic drugs and sedatives have Iittle effect, and nitrites open the cardia briefly, if at aI1. Psychiatric therapy is worthIess in cardiospasm, aIthough it may be beneficia1 for patients who have diffuse spasm. Thiamine hydrochloride has not proved heIpfu1 in our experience, and oraIIy administered IocaI anesthetic agents apparentIy do not benefit patients who have achaIasia. IdeaIIy, the treatment of cardiospasm should be directed toward restoration of norma 304

Cardiospasm

FIG. 5. Esophagomyotomy cedure) .

FIG. 4. Method of performing dilation. (a) Passage of No. 41 French olive-tipped bougie into stomach. (b) A No. $0 or 60 French sound is passed into the stomach guided-by flexible wire spiral. (ci A hydrostatic diIator is oassed to the cardia. cd) Distention of the hvdrostatic dilator across cardia.
60 per cent of our patients with cardiospasm after a singIe course of such therapy. Of the remaining 40 per cent, about one-half experienced permanent relief after further courses. Thus, 20 per cent either did not respond to this treatment or required periodic dilation. Hydrostatic dilation of the esophagus is not without some risk. In our series of 601 patients, of whom ~$4 were treated by means of the hydrostatic diIator, ten had an esophageal “spht” [d]. This represents a complication rate of approximately 2 per cent. FortunateIy, such compIications can be handled satisfactoriIy by surgica1 measures and antibiotics in nearly a11 instances. SURGICAL

TREATMENT

Some form of surgica1 treatment must be considered for those patients who fai1 to respond to properly performed hydrostatic diIation. Enthusiasm for a surgica1 approach to the symptomatic reIief of cardiospasm has Auctuated over the years. The many operative procedures that have been and stiI1 are being empIoyed attest to the fact that the roIe of 305

(modified

HcIIer’s

pro-

surgery in the management of this disease has not yet been cIearIy defined. Among the surgical technics that have been empIoyed are transgastric diIation of the cardia, the WendeI cardiopIasty, the HeyrovskyGrondahI cardiopIasty and various types of esophagogastrectomy. CardiopIasty, in our experience [26]-and that of others [27], has Ied uniformIy to the deveIopment of severe ulcerative esophagitis. For this reason, operations of this type should be abandoned. A simiIar result may foIIow esophagogastrectomy unless extensive resection of the stomach down to the antrum is done. Such a radical approach to the probIem has not appealed to us. The operative procedure described by HeIIer [28] and Iater modified by Zaaijer [2g] has given the best resutts in the surgical management of cardiospasm. This procedure (esophagomyotomy) consists of a linear incision of the esophageal musculature down to the mucosa, carried out in such a fashion as to free compIeteIy a portion of the narrowed esophagea1 segment of its musculature. This operation ma?; be done through the abdomen or transthoraclcally; we prefer the Iatter approach. As originally described, the incision was carried we11 down over the stomach. Since the obstruction is in the esophagus, such a Iengthy incision is not necessary and may, in fact, be inadvisable. The incision shouId be carried onto the stomach onIy far enough to assure the surgeon that a11 the esophagea1 muscuIature has been divided. (Fig. 5.)

Olsen,

Ellis

Prior to September, 1936, a total of thirtyseven patients had undergone this rnodifred HeIIer procedure at the Clinic. There were no postoperative deaths. Ten of these patients either have been operated on too recentIy for evaIuation or have not been available for foIlow-up examination. Sixteen of the remaining twenty-seven patients have had exceIIent rest&s; they are asymptomatic, have gained weight and are working. The seven patients who have had good results are also asymptomatic except for occasiona dysphagia when nervous or when eating rapidly-. The three patients with fair resuIts are improved but have had either recurrence or persistence of some dysphagia. Severe postoperative esophagitis cleveloped in one patient who is cIassiIied as having a poor result. Thus, twenty-three of twenty-seven patients (85 per cent) have had good or excellent results. This cIinica1 impression of improvement is strengthened further by: roentgenographic studies showing regression m size of the esophagus toward normal. However, studies of motility continue to show an abnorma1 pattern characterized by lack of a co-ordinated peristaltic wave. These earIy results of esophagomyotomy done on a reIativeIy small number of patients are distinctIy encouraging. Such a procedure unquestionably shouId be done when hydrostatic diIation faiIs. It cannot yet be stated dehnitely whether surgical treatment shouId suppIant hydrostatic diIation in the initia1 management of this disease. The Iow degree of risk associated with it and the exceIIent resuIts it affords when properIy empIoyed certainIy justify its frequent use. SUMMARY

AND

CONCLUSIONS

Achalasia of the cardia is a disturbance of esophagea1 motiIity characterized by feebIe or absent peristalsis and faiIure of the sphincter to open at the cardia. The cause is not known but when the disease is cIinicaIIy recognizabIe it is not a psychosomatic disorder. The diagnosis is suspected when characteristic symptoms are presented; it may be substantiated by roentgenographic studies and endoscopic means, especiaIIy by the passage of a bIunt oIive-tipped bougie over a previousIy swaIIowed thread. PhysioIogic methods that measure esophagea1 pressures are now avaiIabIe for exact diagnosis of achaIasia. Records of motiIity after injection

and Creamer of methacholine (mechoIy1) chloride ott’er further proof of the diagnosis. The object of treatment in cardiospasm is to obtain Iong-Iasting or permanent relief. Such resuIts can be obtained in 60 per cent of patients with a single course of hydrostatic diIation and may be ultimately achieved (after two or more courses) in 80 per cent. Hydrostatic diIation is a drastic method of treatment that is not without hazard, yet it is much preferable to haIf-hearted bouginage with bougies, soft rubber tubes or mercury-weighted balloons. During the course of such inadequate therapy, progressive diIatation of the viscus may take place and vaIuabIe time wiI1 be Iost. The use of a previousIy swalIowed thread, not onIy for safety but aIso for proper pIacement of the hydrostatic bag, deserves re-emphasis. The success of treatment cannot be entirely correIated with the size of the esophagus but, in generaI, resuIts of treatment are Iess satisfactory when the condition is advanced. This statement appIies to both endoscopic and surgica1 methods of treatment. It is obviously unfair to the patient and surgeon to continue with diIation when resuIts are poor and the condition progressive. Thus, if dysphagia is not promptIy and compIeteIy reIieved, surgica1 treatment shouId be undertaken. Also, if dysphagia recurs within weeks or months after satisfactory hydrostatic diIation, surgical treatment shouId be seriousIy considered. On the basis of extensive experience we can predict the IikeIihood of successful treatment with the hydrostatic diIator. We have neither a sufficient number of cases nor a Iong enough period of observation to provide a statistica anaIysis of surgica1 results after cardiomyotomy. However, a properIy performed HeIIer operation probabIy offers as much to the patient as does hydrostatic diIation, and the risk of compIication is probabIy less. Thus, there are two good methods of treating cardiospasm. The seIection of treatment for the individual patient depends on many factors, incIuding the persona1 preference of the patient on one hand and the physician on the other. Perhaps the more advanced condition is best managed by esophagomyotomy. It is usuaIIy wise to use diIation for earIy Iesions, especiaIIy when pain is prominent. The usua1 patient can be treated either by diIation or surgica1 intervention. SurgicaI treatment is empIoyed in many cases if diIation is not satisfactory.

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307

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26. RIPLEY, H. R., OLSEN, A. M. and KIRKLIN, J. W. Esophagitis after esophagogastric anastomosis. Surgery, 32: 1-9, 1952. 27. BARRETT, N. R. and FRANKLIN, R. H. Concerning the unfavourabIe late resuIts of certain operations performed in the treatment of cardiospasm. Brit. J. Surg., 37: Ig4-202, 1949. CardiapIastik beim 28. HELLER, E. Extramukiise chronischen Cardiospasmus mit DiIatation des Oesophagus. Mitt. a. d. Grenzgeb. d. Med. u. Cbir., 27: IqI-149, Ig*3--IgIq. 29. ZAAIJER, J. H. Cardiospasm in the aged. Ann. Surg., 77: 615-617, 1923.