Surgical treatment of achalasia in the 21st century.

Background: Achalasia is a primary motility disorder of the esophagus characterized by poor mid-esophageal motility and failure of the lower esophageal sphincter to properly relax. The optimal treatment of the disease would improve esophageal peristalsis and promote lower esophageal sphincter relaxation. Currently, such therapy is not possible, so treatment of the disorder is aimed at relief of symptoms by disruption of the lower esophageal sphincter.

Methods: Data were collected prospectively on all patients undergoing laparoscopic myotomy and Toupet fundoplication during a 6-year period.

Results: Fifty-nine patients with a mean age of 44 years were treated during a 6-years period. Fifty-three patients underwent laparoscopic myotomy with Toupet fundoplication (91%), and four had laparoscopic myotomy without a fundoplication (6%). Fundoplication was not performed in two patients who had a megaesophagus. Two patients required conversion to an open operation. Sixty percent of patients were discharged the day after surgery; the average length of stay for all patients was 2.1 days. Ten percent of patients had minor complications; none required reoperation. Mortality was 0%, and 96% of patients rated their postoperative swallowing ability as excellent or good.

Conclusion: Surgical myotomy is becoming first-line therapy for all patients with achalasia. A strong working relationship between surgeon and gastroenterologist helps to optimize patient care.

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Achalasia is a primary motility disorder of the esophagus that is characterized by poor mid-esophageal motility and failure of the lower esophageal sphincter (LES) to properly relax. The term achalasia is of Greek origin and means "failure to relax." The disorder has been recognized since the late 1600s and was given the name achalasia by Lendrum in 1937. (1) Clinically, patients most often present with progressive dysphagia and frequently have the concomitant symptoms of regurgitation, chest pain, or weight loss. Men and women are equally affected. Symptoms typically appear between the third and fifth decades, but achalasia can be seen in children and the elderly.

Physiologic changes in achalasia include failure of LES relaxation, esophageal aperistalsis, and eventual esophageal dilation. Pathology reveals selective loss of the postinhibitory neurons of Auerbach's plexus. These neurons inhibit smooth muscle contraction of the LES, and their loss results in unopposed stimulation of contraction by acetylcholine. (2) A decrease in the amount of nitric oxide released from the post-ganglionic cells, which normally mediates the relaxation of the LES, also occurs. The actual cause of achalasia is unknown, but viral and autoimmune mechanisms have been proposed. The optimal treatment of the disease would improve esophageal peristalsis and promote LES relaxation. Currently, such therapy is not possible, so treatment of the disorder is aimed at relief of symptoms by disruption of the LES.

Diagnosis

The diagnosis of achalasia begins with a thorough history and physical. Particular attention should be paid to the symptoms of dysphagia and regurgitation. Patients with achalasia often complain of regurgitation of solid food during or at the end of a meal or at night while in the recumbent position. The dysphagia associated with achalasia must be differentiated from dysphagia caused by stricture, spasm, or obstruction from a benign or malignant mass. Barium swallow will often show the typical "bird beak" appearance of the esophagus (Fig. 1) and is helpful to identify dilation. Upper endoscopy is used to rule out stricture or malignancy. Esophageal manometry should demonstrate nonperistaltic esophageal contraction and failure of LES relaxation. Manometry is important to distinguish achalasia from other disorders that cause impaired esophageal motility, such as scleroderma. Occasionally, patients presumed to have gastroesophageal reflux disease (GERD) will be found to have achalasia; the presenting symptoms can be similar.

Treatment

Treatment of achalasia can be divided into pharmacologic, endoscopic, and surgical management. Medical therapy typically consists of calcium-channel blockers (nifedipine) or nitrates (isosorbide dinitrate). A small number of patients report relief of symptoms with pharmacologic treatment, but the majority have persistent dysphagia and many suffer side effects from the medications. (3)

Endoscopic treatment includes forceful dilation of the LES to disrupt the muscle fibers of the lower esophagus or injection of botulinum toxin at the gastroesophageal junction to paralyze the muscle. Dilation is performed with a 3-cm to 4-cm balloon and relieves dysphagia in 60 to 70% of patients. Repetitive dilations may be performed for persistent symptoms, increasing the success rate to nearly 90%. However, balloon dilation is usually ineffective for patients younger than 45 years of age. (4) In this age group, less than 50% of patients report significant improvement in swallowing after dilation.

[FIGURE 1 OMITTED]

Endoscopic dilation results in an uncontrolled disruption of the LES and has a 2 to 13% risk of esophageal rupture. (3-6) In addition, the disruption of the LES leaves an incompetent barrier to gastroesophageal reflux, and up to 30% of patients demonstrate GERD after dilation therapy. (7) For patients failing dilation, subsequent surgical myotomy can be performed with outcomes similar to those of patients without previous dilation.

Endoscopic injection of botulinum toxin (Botox) into the LES relaxes the muscle by inhibiting the release of acetylcholine. Approximately 85% of patients experience short-term relief, but 50% report return of their symptoms within 6 months. (8), (9) Patients can undergo multiple injections, but they have increasing resistance to each treatment. Vaezi and Richter (3) reported that only one-third of patients treated with Botox were in clinical remission after 1 year, and others have noted that Botox treatment is more expensive than other forms of treatment. Hunter et al (10) noted that morbidity of surgical myotomy was increased in patients with previous Botox treatment secondary to intense submucosal scarring. This results in a higher risk of esophageal mucosal injury during myotomy and is also related to a less effective clinical outcome. (10) Endoscopic Botox injection should be reserved for patients who are unfit for surgery or unwilling to undergo dilation.

Surgical myotomy has been used to successfully treat achalasia since the early 1900s. It was originally performed through a left chest incision and later through an upper abdominal incision. The morbidity of these techniques had made nonoperative management of achalasia appealing. However, the excellent success rate of the surgical division of the LES cannot be reproduced by endoscopic techniques. Relief of dysphagia is achieved in 90 to 95% of patients, and symptoms are relieved in the long term. (11) The advent of laparoscopic techniques has again brought surgical myotomy to the forefront of achalasia treatment. The minimally invasive approach allows for a shorter hospital stay, reduced postoperative pain, and faster patient recovery. The laparoscopic approach has produced successful results in 90 to 100% of patients, (10), (12) and our experience at Carolinas Medical Center demonstrates 96% of patients have an excellent or good outcome.

Methods

Under general anesthesia, the patient is positioned supine with legs abducted. Five laparoscopic trocars are placed in the upper abdomen to give the surgeon two working ports, the assistant two ports, and one camera port. A 30- or 45-degree laparoscope is used to provide good visualization of the esophageal hiatus. Dissection proceeds with division of the gastrohepatic ligament to expose the right diaphragmatic crus. The crus is dissected anteriorly over the esophagus by dividing the phrenoesophageal ligament. Posterior to the esophagus, blunt dissection is used to identify the posterior vagus nerve, which is lifted with the esophagus to prevent its injury. To allow for the creation of a floppy fundoplication to prevent postoperative reflux, the short gastric vessels along the greater curvature are divided. The stomach is rolled medially, which allows visualization of the left diaphragmatic crus. The esophagus is then encircled with a Penrose drain, and blunt dissection is used to mobilize it superiorly in the mediastinum for at least 8 cm. An anterior longitudinal myotomy is performed, and care is taken to preserve the vagus nerve throughout its course. The myotomy extends 7 cm above the gastroesophageal junction and 2 cm onto the stomach to ensure complete division of the LES (Fig. 2). Intraoperative endoscopy is used to verify that all muscle fibers have been divided. At this point the diaphragmatic hiatus is loosely closed, and a Toupet fundoplication is performed in standard fashion (Fig. 3).

[FIGURE 2 OMITTED]

Postoperatively, patients undergo a Gastrografin (Schering AG, Berlin, Germany) swallow the morning after surgery to rule out a leak. After a normal study, patients are started on a soft diet, which prohibits ingestion of bread, meat, and carbonated beverages for 3 weeks. At that time the diet is liberalized. The majority of patients are discharged to home the day after surgery.

Results

Our experience with laparoscopic myotomy and Toupet fundoplication for achalasia has been extensive. In the past 6 years, we have treated 59 patients with a mean age of 44 years. Most patients (69%) had undergone previous endoscopic treatments, including Botox injection (33%) or dilation (55%). Five patients had undergone at least three dilations and two Botox injections each, and two patients had undergone previous failed surgical myotomies. We also had one patient who presented 10 hours after an endoscopic dilation-induced esophageal perforation.

[FIGURE 3 OMITTED]

Fifty-three patients underwent laparoscopic myotomy with Toupet fundoplication (91%), and four had laparoscopic myotomy without a fundoplication (6%). Fundoplication was not performed in two patients who had a megaesophagus to avoid possible recurrent dysphagia (Fig. 4). Another patient had intense scarring from numerous Botox injections, which would increase the chance of dysphagia with the addition of a fundoplication, and a third had minimal esophageal muscle to which to anchor a fundoplication secondary to previous esophageal surgery. Only two patients required conversion to an open operation because of marked upper abdominal adhesions from previous operations. Sixty percent of patients were discharged the day after surgery, and the average length of stay for all patients was 2.1 days. Ten percent of patients had minor complications, none required reoperation, and the mortality rate was 0%.

[FIGURE 4 OMITTED]

Our patients rated their symptoms both preoperatively and postoperatively. One hundred percent complained of dysphagia and regurgitation before surgery and showed a statistically significant improvement in these symptoms after surgery. In addition, 96% of patients rated their postoperative swallowing ability as excellent or good. Three percent of patients rated their swallowing as fair, but no patients felt that their swallowing was poor. Patients who had undergone nonoperative treatment with dilation or Botox had outcomes similar to those of patients without previous treatment. Like clinicians at other institutions, we observed patients with previous Botox injections to have significant scarring in the plane used for myotomy. However, our patients did not experience esophageal mucosal injury or leak despite previous reports of increased incidence of these complications in patients with Botox-induced scarring. (10)

Discussion

Achalasia is a rare condition affecting 1 in 100,000 persons, but it is the most common primary motor disorder of the esophagus. Traditional treatment was surgical myotomy, which required a large incision and considerable morbidity. Endoscopic therapy provides a less invasive form of treatment by dilation or Botox injection. However, injection therapy does not provide good long-term relief of dysphagia and can make subsequent surgical myotomy more difficult. Botox should be reserved for patients unfit for surgery or dilation.

Endoscopic dilation provides relief of symptoms in the majority of patients, but its uncontrolled division of esophageal muscle runs the risk of esophageal perforation and destroys the barrier to reflux, leaving some patients with the new problem of GERD. In addition, dilation has very poor results in patients younger than 45 years of age, and it should not be used as first-line therapy in this patient population.

Surgical myotomy provides excellent relief of dysphagia and can be performed with minimal morbidity by laparoscopic techniques. Laparoscopic myotomy is appropriate for any patient who is fit for anesthesia, and can produce excellent outcomes in patients with previous treatment failures. The minimal morbidity of the operation makes it useful for patients with a dilated esophagus (>8 cm) who were previously thought to have esophagectomy as their only treatment option.

Some controversy exists over the addition of an antireflux procedure to esophageal myotomy because studies have shown little symptomatic reflux after myotomy. Patti et al, (7) however, demonstrated that 60% of asymptomatic patients actually had significant reflux when subjected to pH monitoring. In a series with 20-years follow-up, Malthaner et al (12) reported a low incidence of reflux and good outcome in patients at 1 year, but the same group had a 78% incidence of reflux by 20 years. Most authors believe that the most common reason for short-term failure is an incomplete myotomy, and long-term failure stems from GERD. Therefore, we advocate a long myotomy with full mobilization of the esophagus, coupled with an antireflux procedure.

The treatment of achalasia has changed during the past 85 years and will likely continue to evolve. The development of laparoscopic techniques has allowed the surgical treatment of achalasia to be accomplished with minimal morbidity and excellent patient outcomes.

Laparoscopic esophageal myotomy and fundoplication should be considered first-line therapy for patients younger than 45 years of age, patients with a significantly dilated esophagus, those uncomfortable with the risk of perforation during dilation, and individuals who have failed medical or endoscopic treatment. Surgical myotomy is becoming first-line therapy for all patients. A strong working relationship between surgeon and gastroenterologist helps to optimize patient care.

From the Department of Surgery, Carolinas Medical Center, Charlotte, NC.

Reprint requests to B. Todd Heniford, MD, Department of General Surgery, Carolinas Medical Center, 1000 Blythe Boulevard, MEB #601, Charlotte, NC 28203. Email: [e-mail]

Accepted August 12, 2002.

Copyright [c] 2004 by The Southern Medical Association 0038-4348/04/9701-0007

References

(1.) Lendrum FC. Anatomic features of the cardiac orifice of the stomach with special reference to cardiospasm. Arch Intern Med 1937;59:474-511.

(2.) Umana JP, Whyte RI. Achalasia of the esophagus, in Cameron JL (ed): Current Surgical Therapy. St. Louis, Mosby, 2001, ed 7, pp 13-19.

(3.) Vaezi MF, Richter JE. Current therapies for achalasia: Comparison and efficacy. J Clin Gastroenterol 1998;27:21-35.

(4.) Katz PO, Gilbert J, Castell DO. Pneumatic dilatation is effective long-term treatment for achalasia. Dig Dis Sci 1998;43:1973-1977.

(5.) Parkman HP, Reynolds JC, Ouyang A, et al. Pneumatic dilatation or esophagomyotomy treatment for idiopathic achalasia: clinical outcomes and cost analysis. Dig Dis Sci 1993;38:75-85.

(6.) Spiess AE, Kahrilas PJ. Treating achalasia: From whalebone to laparoscope. JAMA 1998;280:638-642.

(7.) Patti MG, Arcerito M, Tong J, et al. Importance of preoperative and postoperative pH monitoring in patients with esophageal achalasia. J Gastrointest Surg 1997;1:505-510.

(8.) Gordon JM, Eaker EY. Prospective study of esophageal botulinum toxin injection in high-risk achalasia patients. Am J Gastroenterol 1997;92:1812-1817.

(9.) Pamphlett R. Early terminal and nodal sprouting of motor axons after botulinum toxin. J Neurol Sci 1989;92:181-192.

(10.) Hunter JG, Trus TL, Branum GD, et al. Laparoscopic Heller myotomy and fundoplication for achalasia. Ann Surg 1997;225:655-664.

(11.) Csendes A, Braghetto I, Henriquez A, et al. Late results of a prospective randomised study comparing forceful dilatation and oesophagomyotomy in patients with achalasia. Gut 1989;30:299-304.

(12.) Malthaner RA, Tood TR, Miller L, et al. Long-term results in surgically managed esophageal achalasia. Ann Thorac Surg 1994;58:1343-1346.

RELATED ARTICLE: Key Points

* Physiologic changes in achalasia include failure of lower esophageal sphincter relaxation, esophageal aperistalsis, and eventual esophageal dilation.

* The dysphagia associated with achalasia must be differentiated from dysphagia caused by stricture, spasm, or obstruction from a benign or malignant mass.

* Treatment of achalasia can be divided into pharmacologic, endoscopic, and surgical management.

* Endoscopic treatment includes forceful dilation of the lower esophageal sphincter to disrupt the muscle fibers of the lower esophagus or injection of botulinum toxin at the gastroesophageal junction to paralyze the muscle.

* Surgical myotomy has been used to successfully treat achalasia since the early 1900s. Relief of dysphagia is achieved in 90 to 95% of patients, and symptoms are relieved long term.

Kristi L. Harold, MD, Brent D. Matthews, MD, Kent W. Kercher, MD, Robert F. Sing, DO, and B. Todd Heniford, MD