Achalasia Treatment & Management

The goal of therapy for achalasia is to relieve symptoms by eliminating the outflow resistance caused by the hypertensive and nonrelaxing LES. Once the obstruction is relieved, the food bolus can travel through the aperistaltic body of the esophagus by gravity.

• Calcium channel blockers and nitrates are used to decrease LES pressure.

  • Approximately 10% of patients benefit from this treatment.
  • This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery.
• Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters.^[3]

  • This treatment has limited value. Only 30% of patients treated endoscopically still have relief of dysphagia 1 year after treatment.
  • This treatment can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult.
  • Use this treatment in elderly patients who are poor candidates for dilatation or surgery.
• Pneumatic dilatation performed by a qualified gastroenterologist is the recommended treatment in those sporadic cases in which surgery is not appropriate.^[4]

  • A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact.
  • The success rate is 70-80%, and the perforation rate is approximately 5%.
  • If a perforation occurs, emergency surgery is needed to close the perforation and to perform a myotomy.
  • As many as 50% of patients may require more than 1 dilatation.
  • The incidence of abnormal gastroesophageal reflux after the procedure is approximately 25%.
• A laparoscopic Heller myotomy is considered by many to be the appropriate primary treatment of patients with achalasia (see Surgical Care). A Heller myotomy and a partial fundoplication performed from the chest (thoracoscopic) have a high incidence of gastroesophageal reflux.^{[4, 5, 6]}

Because of excellent results, a short hospital stay, and a fast recovery time, the primary treatment is considered by many to be a laparoscopic Heller myotomy and partial fundoplication.^{[5, 4]} In the author's experience and in the experience of many authors, this treatment provides a fine balance in relieving symptoms of dysphagia by performing the myotomy and in preventing gastroesophageal reflux by adding a partial wrap. A prospective, randomized study from Vanderbilt University indicated that there is significantly less risk of postoperative reflux following a Heller myotomy plus a partial fundoplication than there is after a Heller myotomy alone.^[7] The authors of this study also showed that in patients with achalasia, adding a partial fundoplication not only is more effective in preventing postoperative reflux but also is more cost-effective at a time horizon of 10 years.^[8]

However, the use of preoperative endoscopic therapy remains common but has resulted in intraoperative complications (eg, esophageal perforation) and postoperative complications and in a high failure rate.

• Minimally invasive surgery for achalasia is performed under general anesthesia with the use of 5 trocars. A controlled division of the muscle fibers (myotomy) of the lower esophagus (5 cm) and the proximal stomach (1.5 cm) is carried out, followed by a partial fundoplication to prevent reflux. See the images below. Heller myotomy extending 1.5 cm onto the gastric wall. Dor fundoplication, left row of sutures (after division of short gastric vessels). Completed Dor fundoplication.
• Patients remain hospitalized for 24-48 hours and return to regular activities in about 2 weeks.
• The operation relieves symptoms in 85-95% of patients, and the incidence of postoperative reflux is 10-15%.
• For patients in whom surgery fails, they may be treated with an endoscopic dilatation first. If this fails, a second operation (extending the previous myotomy onto the anterior gastric wall) can be attempted once the cause of failure has been identified with imaging studies. The last resort is to surgically remove the esophagus (ie, esophagectomy).
• Treatment options vary for patients with different degrees of illness severity. A study by Reynoso et al suggests that among hospitalized patients with minor/moderate illness severity, laparoscopic myotomy for achalasia showed comparable or better outcomes than esophageal dilation.^[9] Laparoscopic myotomy is not associated with superior success rates for patients with newly diagnosed achalasia.^[10] For major/extreme illness severity, dilation showed comparable or better profile for hospitalized patients with achalasia.

Esophagectomy was the standard treatment in patients with achalasia and a markedly dilated or sigmoid-shaped esophagus, with Heller myotomy considered to be ineffective in such cases. However, in a study by Sweet and colleagues of 113 patients with achalasia, the investigators reported that (1) in most of the study's patients, even those with achalasia and a dilated esophagus, a laparoscopic Heller myotomy relieved dysphagia; (2) additional treatment was needed in about 20% of patients; and (3) in the end, 90% of patients had attained good swallowing ability. Esophagectomy was not required in any of the patients to maintain clinically adequate swallowing.^[11]

Cowgill et al reported on outcomes in 47 patients more than 10 years after they had undergone laparoscopic Heller myotomy for achalasia.^[6] The investigators found that notable complications were infrequent following the procedure and that no perioperative deaths had occurred. One patient underwent a second myotomy 5 years after the first, because of symptom recurrence.

There were 33 surviving patients at the time of the study; the authors reported that the other patients died from causes that were unrelated to myotomy. Using a Likert scale and a Wilcoxon matched-pairs test to assess patients’ symptoms before and after laparoscopic myotomy, Cowgill and colleagues found significant postsurgery decreases in the frequency and severity scores for dysphagia, chest pain, vomiting, regurgitation, choking, and heartburn. They concluded that "[t]he symptoms of achalasia are durably ameliorated by laparoscopic Heller myotomy during long-term follow-up evaluation."