Diagnosis
In addition to careful symptomatic evaluation, the following tests should be routinely performed: Barium swallow usually shows narrowing at the level of the gastroesophageal junction ("bird beak") and various degrees of esophageal dilatation. Endoscopy is important to rule out the presence of a peptic stricture or cancer and gastroduodenal pathology. Esophageal manometry is the key test for establishing the diagnosis. The classic manometric findings are: (a) absence of esophageal peristalsis and (b) hypertensive or normotensive lower esophageal sphincter (LES) that fails to relax completely in response to swallowing.
Prolonged pH monitoring may be helpful preoperatively in patients who have previously failed treatment with pneumatic dilatation, Botulinum toxin (Botox), or surgical myotomy, for whom a myotomy is planned. Demonstration of reflux clearly indicates the need for a fundoplication in addition to the myotomy.
In patients older than 60 years of age with recent onset of dysphagia and excessive weight loss, secondary or pseudo-achalasia should be ruled out. Because a cancer of the gastroesophageal junction is the most common cause of pseudo-achalasia, an endoscopic ultrasound or a computed tomography (CT) scan of the gastroesophageal junction can help to establish the diagnosis.
Treatment
Treatment is palliative, and it is directed toward elimination of the outflow resistance at the level of the gastroesophageal junction. The following treatment modalities are available to achieve this goal:
Pneumatic dilatation has a success rate between 70 and 80%. Gastroesophageal reflux occurs after dilatation in 25 to 35% of patients. Up to 5% of patients may sustain a perforation at the time of a dilatation. These patients may require open surgery to close the perforation and perform a myotomy.
Intrasphincteric injection of botulinum toxin results in initial relief of symptoms in about 60% of patients, but this is transitory and symptoms will return in the majority of patients within a year. Subsequent injections are less effective and the benefit is of briefer duration. In addition, this treatment may cause an inflammatory reaction at the level of the gastroesophageal junction, which obliterates the anatomic planes. Consequently, a myotomy is more difficult, a mucosal perforation occurs more frequently, and the relief of dysphagia is less predictable. Because of these shortcomings, botulinum toxin should be reserved for elderly or high-risk patients who are poor candidates for dilatation or surgery.
Traditionally, pneumatic dilatation has been the first line of treatment for esophageal achalasia, while surgery was reserved for patients who had persistent dysphagia after multiple dilatations or who had suffered a perforation during dilatation.
Today, minimally invasive surgery has completely changed this treatment algorithm and a laparoscopic Heller myotomy and partial fundoplication is preferred by most gastroenterologists and surgeons as the primary treatment modality. Critical details of the operation include a generous myotomy of the lower esophagus, extending well onto the gastric wall. Because of the lack of esophageal peristalsis, a partial (Dor or Toupet), rather than a total fundoplication is frequently added to prevent reflux. Patients can usually eat the morning of the first postoperative day and can be discharged home after one or two days.
The need for esophagectomy for achalasia is very uncommon, even in the presence of a dilated esophagus, and should be reserved for failures after myotomy.
All patients undergoing treatment for achalasia should be followed by surveillance endoscopy, because they are at increased risk for development of both squamous and adenocarcinoma.
Expected Outcomes
About 90% of patients have long-term relief of dysphagia after a myotomy, with a low incidence of symptomatic acid reflux. Patients should undergo 24-hour pH testing routinely after surgery, as reflux is often asymptomatic, and should be treated with proton pump inhibitors if abnormal acid reflux is present.
Qualifications for Performing Operations for Achalasia
At a minimum, surgeons who are certified or eligible for certification by the American Board of Surgery, the Royal College of Physicians and Surgeons of Canada, or their equivalent should perform operations for achalasia. The qualifications of a surgeon performing any operative procedure should be based on training, experience, and outcomes.
