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Treatment of Achalasia of the Esophagus
Symposium on Esophageal Surgery
Treatment of Achalasia of the Esophagus W. Spencer Payne, M.D.,* and R. Michael King, M.D. t
Achalasia is a specific motility disturbance of the esophagus characterized by an absence of peristalsis in the body of the esophagus and nonrelaxing hypertension of the lower esophageal sphincter. It affects males and females of all ages. The pathophysiology of this disorder is related to a loss of vagal innervation to the body of the esophagus and its lower sphincter. Neural degeneration from the dorsal motor nucleus of the vagus nerve in the brainstem to the myenteric plexus in the esophageal wall has been documented. Although many possibilities exist, as yet no specific cause of the neural degeneration has been defined. The clinical features of achalasia are distal esophageal obstruction with esophageal retention and spontaneous, effortless regurgitation of bland, undigested fresh food and saliva. The major complications of the condition are nutritional depletion and suppurative lung disease (Table 1). The suppurative lung disease is secondary to accidental respiratory aspiration of regurgitated esophageal contents. Initially, dysphagia is episodic and unrelated to the mechanical properties of material ingested. At times, entire meals may be ingested with little or no distress; then, inexplicably, symptoms worsen to the extent that all or portions of a meal fail to pass. The frequency and duration of dysphagia imperceptibly increase and are interspersed with irregular and unpredictable episodes of apparent remission. Patients with achalasia not only experience respiratory and nutritional problems but also have a high incidence of esophagitis and cancer. Approximately one third of patients with achalasia have objective esophagitis with subjective heartburn, but this is not due to gastroesophageal reflux. Such changes in the untreated patient are caused by chronic esophageal retention and stasis secondary to achalasia. The cause for the increased incidence of cancer of the esophagus and cardia in patients who have achalasia is not 'known, although cancer tends to occur in patients who have long-standing (more than 20 years in duration) untreated achalasia. Such cancers are *James C. Masson Professor of Surgery, Mayo Medical School; Consultant, Department of Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota tSenior Resident in Surgery, Mayo Graduate School of Medicine, Rochester, Minnesota
Surgical Clinics of North America-Val. 63, No. 4, August 1983
963
964
W. Table 1.
SPENCER PAYNE, AND
R.
MICHAEL KING
Complications of Achalasia
Obstruction to swallowing Malnutrition Dehydration Delayed growth and development Regurgitation Respiratory aspiration Esophageal retention Subjective heartburn Stasis esophagitis Neurosis Solitary dining Valsalva maneuver Drinking of copious liquids Coughing and choking Regurgitation Sleep deprivation Late development of carcinoma of esophagus From Payne, W. S.: Disorders of oesophageal motility. In Dyde, J. A., and Smith, R. E., (eds.): The Present State of Thoracic Surgery: The Fifth Coventry Conference. London, Pitman Medical Publishing Co., 1981, p. 137, with permission.
Figure l. Radiographic findings in patients undergoing treatment for achalasia of esophagus. Note that 20 per cent of patients had minimal or no increase in esophageal caliber, 45 per cent showed moderate dilatation and tortuosity, and 35 per cent showed severe or advanced dilatation and tortuosity. (From Payne, W. S.: Disorders of oesophageal motility. In Dyde, J. A., and Smith, R. E., (eds.): The Present State of Thoracic Surgery: The Fifth Coventry Conference. London, Pitman Medical Publishing Co., 1981, p. 137, with permission.)
TREATMENT OF ACHALASIA OF THE ESOPH.\CUS
965
distributed throughout the length of the esophagus and have no specific anatomic predilection. Because of the long-standing preexisting esophageal symptoms of achalasia, most of these cancers are undiagnosed until quite advanced. The diagnosis of achalasia can usually be suspected by the clinical symptoms alone, but esophageal radiography, manometry, and endoscopy are required for complete evaluation. There appears to be some vague correlation between the degree of esophageal dilatation and tortuosity seen on the x-ray film (Fig. 1) and the duration or severity of obstructive esophageal symptoms; for example, esophageal retention and regurgitation are more bothersome in patients with more advanced radiographic stages. In our experience, only one third of patients have classic megaesophagus, and many of these have symptoms of relatively short duration and indistinguishable from those with less significant radiographic changes. Thus, we regard radiographic stages as interesting but noncritical, descriptive features of a patient's condition. Radiographic features, incidentally, are unlikely to improve significantly after successful treatment of achalasia. Manometric study of esophageal motility with the use of perfused catheters is the most reliable and sensitive method of diagnosing achalasia. Classically, esophageal contractions are small, repetitive, simultaneous, and nonperistaltic. Eighty-seven per cent of our patients have this finding, whereas 13 per cent have the manometric features of vigorous achalasia. 4 The resting pressure tone of the lower esophageal sphincter in all patients with achalasia is actually elevated above normal, and the sphincter fails to relax normally with swallowing. This latter feature is essential for establishing the diagnosis of achalasia. Endoscopy should always be performed at diagnosis to rule out other esophageal and gastroduodenal conditions; for instance, in rare cases, the x-ray and manometric findings of classic achalasia actually are manifestations of cancer of the esophagogastric junction.
TREATMENT The goal of treatment in patients with achalasia is to relieve symptoms of obstruction by weakening the lower esophageal sphincter and thereby improving the emptying of the esophagus. There is no medical or surgical cure for this disease, and all current efforts at treatment should be considered palliative. Regardless of the type of current therapy employed, peristalsis and lower esophageal sphincter function are not restored. Treatment consists of either forceful pneumatic dilatation or surgical esophagomyotomy. Medication has been of limited value. 1
Forceful Dilatation Pneumatic dilatation (Fig. 2) utilizes an inflatable bag that is passed over a previously swallowed thread to straddle the gastroesophageal junction and then is inflated. Proper positioning of the bag at the cardia and in the region of the lower esophageal sphincter is determined either by fluoroscopy or by inference after the preliminary passage of a bougie. Balloon inflation is carefully performed after proper positioning with pressures of 549 to 670 cmH 2 0 (hydrostatic dilatation) or 150 to 200 mmHg (pneumatic dilatation).
966
W.
\
SPENCER PAYNE, AND
R.
MICHAEL KING
-
\
Figure 2. Method of performing dilatations. a, Olive-tipped (41F) bougie being passed to stomach. b, Sound (50 or 60 F) guided by flexible wire spiral is passed into stomach. c, Hydrostatic dilator is passed to cardia and (d), distended within cardia. (From Olsen, A. M., Harrington, S. W., Moersch, H. J., et al.: The treatment of cardiospasm: Analysis of a twelveyear experience. J. Thorac. Cardiovasc. Surg., 22:164, 1951, with permission.)
With either method, the pressure is sustained for several seconds before the procedure is terminated. The patient is allowed to resume a normal diet after the procedure. Esophagomyotomy The current technique of esophagomyotomy is identical to that employed at the Mayo Clinic since 1949 (Fig. 3). 2• 3 The procedure consists of a left transthoracic longitudinal esophagomyotomy that begins at the esophagogastric junction and extends 7 to 10 em proximally to the level of the left inferior pulmonary vein. Care is taken to avoid undue traction on the esophagus and dissection around the esophageal hiatus, which might lead to the development of hiatal hernia. Approximately 50 per cent of the circumference of the esophageal mucosal tube is freed of overlying muscularis to allow the mucosa to pout through the site of the myotomy. Care is taken to avoid injury to vagal fibers and to avoid extending the myotomy onto the stomach. If an anatomic sliding esophageal hiatal hernia is present, it is repaired. The esophagus is returned to its mediastinal bed, and the mediastinal pleura is reapproximated. The lung is reexpanded, a nasogastric tube and a chest tube are inserted, and the chest is closed. On the morning after operation, the nasogastric tube and chest tube are removed, and diet is resumed.
TREATMENT OF ACHALASIA OF THE EsOPHAGUS
967
'
'vagus n
Aorta
Esophagus
Figure 3. Top, Transthoracic exposure of distal esophagus for esophagomyotomy. Note that left lung has been retracted cephalad, and the mediastinal pleura has been opened. This procedure exposes the distal esophagus, which has been encircled by a soft rubber drain. Bottom, Technique of esophagomyotomy. A, Beginning the incision. B, Dissection of the mucosa from the muscularis. C, Restoration of esophagogastric junction to intraabdominal position with sutures to narrow esophageal hiatus, if necessary. (From Ellis, F. H., Jr., Kiser, J. C., Schlegel, J. F., et al.: Esophagomyotomy for esophageal achalasia: Experimental, clinical, and manometric aspects. Ann. Surg., 166:640, 1967, with permission.)
If a defect is created in the esophageal mucosa at the time of myotomy, it is repaired with interrupted 5-0 silk suture in the mucosal layer only. In such circumstances, radiographic examination is performed the morning after operation before the nasogastric tube and chest tube are removed and before diet is resumed. If there is good esophageal emptying and no leakage is apparent, normal postoperative routine is followed. However, if there is delayed esophageal emptying or leakage, parenteral alimentation is begun,
968
W.
SPENCER PAYNE, AND
R. MICHAEL KING
100
"'~
80
:::1
"C Q) (.J
e
c.
60
0
'E
Q)
(.J .... Q) c.
40
20
YEAR 1948
58
78
Figure 4. Observed trends in management of achalasia of the esophagus in 899 patients seen at the Mayo Clinic from 1949 through 1975. Percentage distribution of the two forms of treatment by year is shown. Note shift in emphasis toward esophagomyotomy (solid curve) in lieu of forceful hydrostatic or pneumatic dilatation (dotted curve) since 1949. (From Okike, N., Payne, W. S., Neufeld, D. M., et a!.: Esophagomyotomy versus forceful dilation for achalasia of the esophagus: Results in 899 patients. Ann. Thorac. Surg., 28:119, 1979, with permission.)
and it is continued for at least ten days, or until complete sealing is apparent radiographically. By following this routine, we have avoided significant clinical morbidity in those patients in whom mucosal tear has occurred. RESULTS During the 27-year period of 1949 through 1975, 899 patients underwent treatment for achalasia of the esophagus at the Mayo Clinic: 468 by transthoracic esophagomyotomy and the remaining 431 by one or more forceful hydrostatic or pneumatic dilatations (Fig. 4). 4 Early Results The early results of treatment are compared in Table 2. Except for transient, rare episodes of cardiac arrhythmia or retention of tracheobronchial secretions, the early postoperative course of patients underg()ing esophagomyotomy was usually benign. In five patients, however, a definable esophageal leak developed; three of them required additional surgical intervention for management. The single death among the 468 patients brought to the operating room for myotomy was unrelated to the operation. In fact, malignant hyperthermia developed in this patient while anesthesia was being induced, and so the operation was not actually performed. Nevertheless, the outcome is counted as a surgical death. Among the 431 patients managed by forceful dilatation, most had minimal morbidity after treatment. Nineteen patients experienced esophageal perforation, and ten of these required surgical management. All 19
969
TREATMENT OF ACHALASIA OF THE ESOPHAGUS
Table 2.
Early Complications of Treatment of Achalasia (1949-1975) (899 Patients)
Deaths Esophageal leak
DILATATION (431 Pt.)
ESOPHAGOMYOTOMY (468 Pt.)
2* 19 (10)t
1 5 (3)t
*Deaths not related to procedure. tNumbers in parentheses are operations required to control leakage. Data from Okike, N., Payne, W. S., Neufeld, D. M., et a!.: Esophagomyotomy versus forceful dilation for achalasia of the esophagus: Results in 899 patients. Ann. Thorac. Surg., 28:119, 1979.
survived. The two early postdilatation deaths were due to acute myocardial infarction and were not directly attributable to the dilatation. Late Results Table 3 compares the late follow-up results on 767 patients 1 to 18 years after treatment. The criteria for assessing results of treatment were identical in the two treatment groups (Table 4). The late results of esophagomyotomy were significantly better than those for forceful dilatation (p<0.001) (Table 3). Indeed, 85 per cent of those treated surgically had "excellent" or "good" results on late follow-up compared with only 65 per cent treated by dilatation. Among those patients treated by forceful dilatation, most underwent the treatment once, 16 per cent required two treatments, and 2 per cent three or more. When the late results of surgical treatment were analyzed by patient age and sex, duration of symptoms, degree of esophageal dilatation, presence or absence of hiatal hernia, or the presence of classic or vigorous acalasia on manometric study, none of these factors appeared to be important in determining the outcome. Even previous forceful dilatation did not affect results, provided it had not been performed more than one or two times before operation. Multiple forceful dilatations occasionally resulted in fusion and fibrous obliteration of the tissue plane between the mucosa and the muscularis and made effective myotomy technically difficult Table 3.
Late Results of Treatment of Achalasia (1949-1975) (899 Patients) DILATATIOK 1431 Pt.)
Result(%) Excellent Good Fair Poor
213} 65* 37 lG
19
ESOPHAGOMYOTO~IY
(468 Pt.)
.5o } 8 _* :35 ;) 9 6
Follow-up (yr) No. of patients Per cent
1 to 18 311 72
1 to 17 456 98
Age (yr)
1 to 85
4 to 81
*Significantly different (p<0.001). From Okike, N., Payne, W. S., Neufeld, D. M., eta!.: Esophagomyotomy versus forceful
dilation for achalasia of the esophagus: Results in 899 patients. Ann. Thorac. Surg., 28:119, 1979, with permission.
970
W.
Table 4. Excellent: Good: Fair: Poor:
SPENCER PAYNE. AND
R.
MICIIAEL KING
Criteria for Assessing Results of Treatment of Achalasia
Asymptomatic, gained weight, returned to normal activities Significant improvement, occasional dysphagia, no regurgitation Definite improvement, occasional dysphagia with regurgitation Worsening of achalasia or no improvement
or, at times, impossible to perform. Results were uniformly poor under such circumstances.
CONCLUSIONS Myotomy is more effective and safer than forceful dilatation. "Fair" or "poor" results occurred twice as often among patients undergoing dilatation as among those treated by myotomy. Further chronic gastroesophageal reflux was a complication of either form of therapy but was rare: only 2.5 per cent of patients had this complication on late follow-up. Thus, with either form of treatment, the most common cause for an imperfect late result was failure to relieve completely the obstruction caused by achalasia rather than the development of gastroesophageal reflux and its complications. It seems imprudent to extend the myotomy onto the stomach to destroy the sphincter totally and then to attempt restoration of competence with one of the antireflux procedures, because the antireflux procedures historically have had a higher reflux rate than that which occurs with the operation we describe. It does appear, however, that hiatal hernia repair is prudent if an esophageal hiatal hernia is present at myotomy. In our experience, partial fundoplication of the Belsey type is more desirable than the 360° wrap of the Nissen type. The Nissen procedure is unpredictably obstructive to the amotile esophagus of achalasia and often results in worsening of preoperative symptoms. Whether a single trial of forceful dilatation should be made before operation has not been specifically addressed by our studies. However, a single episode of forceful dilatation that fails to provide lasting relief of achalasia does not appear to preclude a subsequent successful result from myotomy. Indeed, the success rate of myotomy after a previous single unsuccessful dilatation was identical to that observed in patients treated by myotomy alone.
REFERENCES l. Bortolotti, M., and Labo, G.: Clinical and manometric effects of nifedipine in patients with esophageal achalasia. Gastroenterology, 80:39, 1981.
2. Ellis, F. H., Jr., and Olsen, A.M.: Achalasia of the esophagus. Major Probl. Clin. Surg., 9:1, 1969. 3. Payne, W. S., and Olsen, A. M.: The Esophagus. Philadelphia, Lea & Febiger, 1974. 4. Sanderson, D. R., Ellis, F. H., Jr., Schlegel, J. F., eta!.: Syndrome of vigorous achalasia: Clinical and physiologic observations. Dis. Chest, 52:508, 1967. Department of Surgery Mayo Clinic Rochester MN 55905
Treatment of Achalasia of the Esophagus W. Spencer Payne, M.D.,* and R. Michael King, M.D. t
Achalasia is a specific motility disturbance of the esophagus characterized by an absence of peristalsis in the body of the esophagus and nonrelaxing hypertension of the lower esophageal sphincter. It affects males and females of all ages. The pathophysiology of this disorder is related to a loss of vagal innervation to the body of the esophagus and its lower sphincter. Neural degeneration from the dorsal motor nucleus of the vagus nerve in the brainstem to the myenteric plexus in the esophageal wall has been documented. Although many possibilities exist, as yet no specific cause of the neural degeneration has been defined. The clinical features of achalasia are distal esophageal obstruction with esophageal retention and spontaneous, effortless regurgitation of bland, undigested fresh food and saliva. The major complications of the condition are nutritional depletion and suppurative lung disease (Table 1). The suppurative lung disease is secondary to accidental respiratory aspiration of regurgitated esophageal contents. Initially, dysphagia is episodic and unrelated to the mechanical properties of material ingested. At times, entire meals may be ingested with little or no distress; then, inexplicably, symptoms worsen to the extent that all or portions of a meal fail to pass. The frequency and duration of dysphagia imperceptibly increase and are interspersed with irregular and unpredictable episodes of apparent remission. Patients with achalasia not only experience respiratory and nutritional problems but also have a high incidence of esophagitis and cancer. Approximately one third of patients with achalasia have objective esophagitis with subjective heartburn, but this is not due to gastroesophageal reflux. Such changes in the untreated patient are caused by chronic esophageal retention and stasis secondary to achalasia. The cause for the increased incidence of cancer of the esophagus and cardia in patients who have achalasia is not 'known, although cancer tends to occur in patients who have long-standing (more than 20 years in duration) untreated achalasia. Such cancers are *James C. Masson Professor of Surgery, Mayo Medical School; Consultant, Department of Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota tSenior Resident in Surgery, Mayo Graduate School of Medicine, Rochester, Minnesota
Surgical Clinics of North America-Val. 63, No. 4, August 1983
963
964
W. Table 1.
SPENCER PAYNE, AND
R.
MICHAEL KING
Complications of Achalasia
Obstruction to swallowing Malnutrition Dehydration Delayed growth and development Regurgitation Respiratory aspiration Esophageal retention Subjective heartburn Stasis esophagitis Neurosis Solitary dining Valsalva maneuver Drinking of copious liquids Coughing and choking Regurgitation Sleep deprivation Late development of carcinoma of esophagus From Payne, W. S.: Disorders of oesophageal motility. In Dyde, J. A., and Smith, R. E., (eds.): The Present State of Thoracic Surgery: The Fifth Coventry Conference. London, Pitman Medical Publishing Co., 1981, p. 137, with permission.
Figure l. Radiographic findings in patients undergoing treatment for achalasia of esophagus. Note that 20 per cent of patients had minimal or no increase in esophageal caliber, 45 per cent showed moderate dilatation and tortuosity, and 35 per cent showed severe or advanced dilatation and tortuosity. (From Payne, W. S.: Disorders of oesophageal motility. In Dyde, J. A., and Smith, R. E., (eds.): The Present State of Thoracic Surgery: The Fifth Coventry Conference. London, Pitman Medical Publishing Co., 1981, p. 137, with permission.)
TREATMENT OF ACHALASIA OF THE ESOPH.\CUS
965
distributed throughout the length of the esophagus and have no specific anatomic predilection. Because of the long-standing preexisting esophageal symptoms of achalasia, most of these cancers are undiagnosed until quite advanced. The diagnosis of achalasia can usually be suspected by the clinical symptoms alone, but esophageal radiography, manometry, and endoscopy are required for complete evaluation. There appears to be some vague correlation between the degree of esophageal dilatation and tortuosity seen on the x-ray film (Fig. 1) and the duration or severity of obstructive esophageal symptoms; for example, esophageal retention and regurgitation are more bothersome in patients with more advanced radiographic stages. In our experience, only one third of patients have classic megaesophagus, and many of these have symptoms of relatively short duration and indistinguishable from those with less significant radiographic changes. Thus, we regard radiographic stages as interesting but noncritical, descriptive features of a patient's condition. Radiographic features, incidentally, are unlikely to improve significantly after successful treatment of achalasia. Manometric study of esophageal motility with the use of perfused catheters is the most reliable and sensitive method of diagnosing achalasia. Classically, esophageal contractions are small, repetitive, simultaneous, and nonperistaltic. Eighty-seven per cent of our patients have this finding, whereas 13 per cent have the manometric features of vigorous achalasia. 4 The resting pressure tone of the lower esophageal sphincter in all patients with achalasia is actually elevated above normal, and the sphincter fails to relax normally with swallowing. This latter feature is essential for establishing the diagnosis of achalasia. Endoscopy should always be performed at diagnosis to rule out other esophageal and gastroduodenal conditions; for instance, in rare cases, the x-ray and manometric findings of classic achalasia actually are manifestations of cancer of the esophagogastric junction.
TREATMENT The goal of treatment in patients with achalasia is to relieve symptoms of obstruction by weakening the lower esophageal sphincter and thereby improving the emptying of the esophagus. There is no medical or surgical cure for this disease, and all current efforts at treatment should be considered palliative. Regardless of the type of current therapy employed, peristalsis and lower esophageal sphincter function are not restored. Treatment consists of either forceful pneumatic dilatation or surgical esophagomyotomy. Medication has been of limited value. 1
Forceful Dilatation Pneumatic dilatation (Fig. 2) utilizes an inflatable bag that is passed over a previously swallowed thread to straddle the gastroesophageal junction and then is inflated. Proper positioning of the bag at the cardia and in the region of the lower esophageal sphincter is determined either by fluoroscopy or by inference after the preliminary passage of a bougie. Balloon inflation is carefully performed after proper positioning with pressures of 549 to 670 cmH 2 0 (hydrostatic dilatation) or 150 to 200 mmHg (pneumatic dilatation).
966
W.
\
SPENCER PAYNE, AND
R.
MICHAEL KING
-
\
Figure 2. Method of performing dilatations. a, Olive-tipped (41F) bougie being passed to stomach. b, Sound (50 or 60 F) guided by flexible wire spiral is passed into stomach. c, Hydrostatic dilator is passed to cardia and (d), distended within cardia. (From Olsen, A. M., Harrington, S. W., Moersch, H. J., et al.: The treatment of cardiospasm: Analysis of a twelveyear experience. J. Thorac. Cardiovasc. Surg., 22:164, 1951, with permission.)
With either method, the pressure is sustained for several seconds before the procedure is terminated. The patient is allowed to resume a normal diet after the procedure. Esophagomyotomy The current technique of esophagomyotomy is identical to that employed at the Mayo Clinic since 1949 (Fig. 3). 2• 3 The procedure consists of a left transthoracic longitudinal esophagomyotomy that begins at the esophagogastric junction and extends 7 to 10 em proximally to the level of the left inferior pulmonary vein. Care is taken to avoid undue traction on the esophagus and dissection around the esophageal hiatus, which might lead to the development of hiatal hernia. Approximately 50 per cent of the circumference of the esophageal mucosal tube is freed of overlying muscularis to allow the mucosa to pout through the site of the myotomy. Care is taken to avoid injury to vagal fibers and to avoid extending the myotomy onto the stomach. If an anatomic sliding esophageal hiatal hernia is present, it is repaired. The esophagus is returned to its mediastinal bed, and the mediastinal pleura is reapproximated. The lung is reexpanded, a nasogastric tube and a chest tube are inserted, and the chest is closed. On the morning after operation, the nasogastric tube and chest tube are removed, and diet is resumed.
TREATMENT OF ACHALASIA OF THE EsOPHAGUS
967
'
'vagus n
Aorta
Esophagus
Figure 3. Top, Transthoracic exposure of distal esophagus for esophagomyotomy. Note that left lung has been retracted cephalad, and the mediastinal pleura has been opened. This procedure exposes the distal esophagus, which has been encircled by a soft rubber drain. Bottom, Technique of esophagomyotomy. A, Beginning the incision. B, Dissection of the mucosa from the muscularis. C, Restoration of esophagogastric junction to intraabdominal position with sutures to narrow esophageal hiatus, if necessary. (From Ellis, F. H., Jr., Kiser, J. C., Schlegel, J. F., et al.: Esophagomyotomy for esophageal achalasia: Experimental, clinical, and manometric aspects. Ann. Surg., 166:640, 1967, with permission.)
If a defect is created in the esophageal mucosa at the time of myotomy, it is repaired with interrupted 5-0 silk suture in the mucosal layer only. In such circumstances, radiographic examination is performed the morning after operation before the nasogastric tube and chest tube are removed and before diet is resumed. If there is good esophageal emptying and no leakage is apparent, normal postoperative routine is followed. However, if there is delayed esophageal emptying or leakage, parenteral alimentation is begun,
968
W.
SPENCER PAYNE, AND
R. MICHAEL KING
100
"'~
80
:::1
"C Q) (.J
e
c.
60
0
'E
Q)
(.J .... Q) c.
40
20
YEAR 1948
58
78
Figure 4. Observed trends in management of achalasia of the esophagus in 899 patients seen at the Mayo Clinic from 1949 through 1975. Percentage distribution of the two forms of treatment by year is shown. Note shift in emphasis toward esophagomyotomy (solid curve) in lieu of forceful hydrostatic or pneumatic dilatation (dotted curve) since 1949. (From Okike, N., Payne, W. S., Neufeld, D. M., et a!.: Esophagomyotomy versus forceful dilation for achalasia of the esophagus: Results in 899 patients. Ann. Thorac. Surg., 28:119, 1979, with permission.)
and it is continued for at least ten days, or until complete sealing is apparent radiographically. By following this routine, we have avoided significant clinical morbidity in those patients in whom mucosal tear has occurred. RESULTS During the 27-year period of 1949 through 1975, 899 patients underwent treatment for achalasia of the esophagus at the Mayo Clinic: 468 by transthoracic esophagomyotomy and the remaining 431 by one or more forceful hydrostatic or pneumatic dilatations (Fig. 4). 4 Early Results The early results of treatment are compared in Table 2. Except for transient, rare episodes of cardiac arrhythmia or retention of tracheobronchial secretions, the early postoperative course of patients underg()ing esophagomyotomy was usually benign. In five patients, however, a definable esophageal leak developed; three of them required additional surgical intervention for management. The single death among the 468 patients brought to the operating room for myotomy was unrelated to the operation. In fact, malignant hyperthermia developed in this patient while anesthesia was being induced, and so the operation was not actually performed. Nevertheless, the outcome is counted as a surgical death. Among the 431 patients managed by forceful dilatation, most had minimal morbidity after treatment. Nineteen patients experienced esophageal perforation, and ten of these required surgical management. All 19
969
TREATMENT OF ACHALASIA OF THE ESOPHAGUS
Table 2.
Early Complications of Treatment of Achalasia (1949-1975) (899 Patients)
Deaths Esophageal leak
DILATATION (431 Pt.)
ESOPHAGOMYOTOMY (468 Pt.)
2* 19 (10)t
1 5 (3)t
*Deaths not related to procedure. tNumbers in parentheses are operations required to control leakage. Data from Okike, N., Payne, W. S., Neufeld, D. M., et a!.: Esophagomyotomy versus forceful dilation for achalasia of the esophagus: Results in 899 patients. Ann. Thorac. Surg., 28:119, 1979.
survived. The two early postdilatation deaths were due to acute myocardial infarction and were not directly attributable to the dilatation. Late Results Table 3 compares the late follow-up results on 767 patients 1 to 18 years after treatment. The criteria for assessing results of treatment were identical in the two treatment groups (Table 4). The late results of esophagomyotomy were significantly better than those for forceful dilatation (p<0.001) (Table 3). Indeed, 85 per cent of those treated surgically had "excellent" or "good" results on late follow-up compared with only 65 per cent treated by dilatation. Among those patients treated by forceful dilatation, most underwent the treatment once, 16 per cent required two treatments, and 2 per cent three or more. When the late results of surgical treatment were analyzed by patient age and sex, duration of symptoms, degree of esophageal dilatation, presence or absence of hiatal hernia, or the presence of classic or vigorous acalasia on manometric study, none of these factors appeared to be important in determining the outcome. Even previous forceful dilatation did not affect results, provided it had not been performed more than one or two times before operation. Multiple forceful dilatations occasionally resulted in fusion and fibrous obliteration of the tissue plane between the mucosa and the muscularis and made effective myotomy technically difficult Table 3.
Late Results of Treatment of Achalasia (1949-1975) (899 Patients) DILATATIOK 1431 Pt.)
Result(%) Excellent Good Fair Poor
213} 65* 37 lG
19
ESOPHAGOMYOTO~IY
(468 Pt.)
.5o } 8 _* :35 ;) 9 6
Follow-up (yr) No. of patients Per cent
1 to 18 311 72
1 to 17 456 98
Age (yr)
1 to 85
4 to 81
*Significantly different (p<0.001). From Okike, N., Payne, W. S., Neufeld, D. M., eta!.: Esophagomyotomy versus forceful
dilation for achalasia of the esophagus: Results in 899 patients. Ann. Thorac. Surg., 28:119, 1979, with permission.
970
W.
Table 4. Excellent: Good: Fair: Poor:
SPENCER PAYNE. AND
R.
MICIIAEL KING
Criteria for Assessing Results of Treatment of Achalasia
Asymptomatic, gained weight, returned to normal activities Significant improvement, occasional dysphagia, no regurgitation Definite improvement, occasional dysphagia with regurgitation Worsening of achalasia or no improvement
or, at times, impossible to perform. Results were uniformly poor under such circumstances.
CONCLUSIONS Myotomy is more effective and safer than forceful dilatation. "Fair" or "poor" results occurred twice as often among patients undergoing dilatation as among those treated by myotomy. Further chronic gastroesophageal reflux was a complication of either form of therapy but was rare: only 2.5 per cent of patients had this complication on late follow-up. Thus, with either form of treatment, the most common cause for an imperfect late result was failure to relieve completely the obstruction caused by achalasia rather than the development of gastroesophageal reflux and its complications. It seems imprudent to extend the myotomy onto the stomach to destroy the sphincter totally and then to attempt restoration of competence with one of the antireflux procedures, because the antireflux procedures historically have had a higher reflux rate than that which occurs with the operation we describe. It does appear, however, that hiatal hernia repair is prudent if an esophageal hiatal hernia is present at myotomy. In our experience, partial fundoplication of the Belsey type is more desirable than the 360° wrap of the Nissen type. The Nissen procedure is unpredictably obstructive to the amotile esophagus of achalasia and often results in worsening of preoperative symptoms. Whether a single trial of forceful dilatation should be made before operation has not been specifically addressed by our studies. However, a single episode of forceful dilatation that fails to provide lasting relief of achalasia does not appear to preclude a subsequent successful result from myotomy. Indeed, the success rate of myotomy after a previous single unsuccessful dilatation was identical to that observed in patients treated by myotomy alone.
REFERENCES l. Bortolotti, M., and Labo, G.: Clinical and manometric effects of nifedipine in patients with esophageal achalasia. Gastroenterology, 80:39, 1981.
2. Ellis, F. H., Jr., and Olsen, A.M.: Achalasia of the esophagus. Major Probl. Clin. Surg., 9:1, 1969. 3. Payne, W. S., and Olsen, A. M.: The Esophagus. Philadelphia, Lea & Febiger, 1974. 4. Sanderson, D. R., Ellis, F. H., Jr., Schlegel, J. F., eta!.: Syndrome of vigorous achalasia: Clinical and physiologic observations. Dis. Chest, 52:508, 1967. Department of Surgery Mayo Clinic Rochester MN 55905