What are the symptoms and treatment options for achalasia?

Achalasia: Symptoms and Treatment

Symptoms of Achalasia

Achalasia presents with progressive dysphagia to both solids and liquids, bland regurgitation, chest pain, and weight loss. 1, 2, 3

• Dysphagia is the hallmark symptom, affecting both solid and liquid food equally, distinguishing it from mechanical obstruction 1, 2
• Regurgitation of undigested food and saliva occurs frequently, often unresponsive to proton pump inhibitor therapy 2, 3
• Chest pain is common and can be a prominent feature, particularly in type III (spastic) achalasia 1, 4
• Weight loss is present in many patients due to impaired esophageal emptying 1, 3
• Pulmonary aspiration and chest infections can occur from regurgitation of retained esophageal contents 5

Diagnostic Workup

All patients suspected of having achalasia must undergo upper endoscopy, timed barium esophagram, and high-resolution manometry to confirm the diagnosis and define the achalasia subtype. 5

• Upper endoscopy (EGD) is mandatory to exclude pseudoachalasia from occult malignancy through careful retroflexed examination of the gastroesophageal junction 6, 5
• Timed barium esophagram confirms outflow obstruction and may reveal a dilated esophagus with distal tapering ("bird's beak" appearance) 6, 2
• High-resolution manometry (HRM) is the gold standard for diagnosis and crucial for defining achalasia subtype (I, II, or III) according to Chicago Classification, which has critical therapeutic implications 6, 7, 5
• Functional luminal imaging probe (FLIP) serves as an adjunct when diagnosis is equivocal, assessing impaired EGJ opening through low distensibility index 6, 7

Treatment Algorithm by Achalasia Subtype

Type I and Type II Achalasia

For type I and II achalasia, pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), and per-oral endoscopic myotomy (POEM) are all effective primary treatments with comparable outcomes; the decision should be based on shared decision-making considering patient preferences and local expertise. 6, 7, 5

• Pneumatic dilation has less morbidity and cost but requires anticipation of repeat dilations over years 6, 7

  • Start with a 30mm balloon, advance to 35mm at 2-28 days if symptoms persist 6, 5
  • Effectiveness is 90% at 1 year, 86% at 2 years, with long-term success rates of 97% at 5 years and 93% at 10 years with repeat dilations 6, 5
  • Perforation risk is 0-7% (mostly 3-4%) with mortality <1% 5

• Laparoscopic Heller myotomy (LHM) with partial fundoplication provides excellent long-term symptom control 6

  • The myotomy must divide circular and longitudinal muscle layers of the lower esophageal sphincter 8
  • Concomitant fundoplication is necessary to prevent postoperative reflux 8

• POEM has been demonstrated superior to PD and noninferior to LHM in multicenter randomized controlled trials 6

  • Requires 20-40 procedures to achieve competence and should only be performed by experienced physicians in high-volume centers 6, 7, 5
  • Technical advantages include no abdominal incisions, more rapid recovery, and ability to perform longer myotomies 6, 7

Type III (Spastic) Achalasia

POEM is the definitive preferred treatment for type III achalasia because it allows unlimited proximal extension of myotomy tailored to the extent of esophageal body spasm, achieving 92% response rates. 6, 7, 5

• Type III achalasia is characterized by spastic body contractions capable of luminal obliteration 6
• Symptoms are best palliated with a myotomy extending to the proximal extent of esophageal body spasm rather than confined to the LES alone 6
• As opposed to laparoscopic approach, POEM provides unlimited proximal extension capability 6, 5

Post-Treatment Management

All patients require proton pump inhibitor (PPI) therapy after treatment, particularly after POEM, which has the highest reflux risk. 6, 7, 8, 5

• Post-POEM patients should be considered high risk to develop reflux esophagitis and may require indefinite PPI therapy and/or surveillance endoscopy 6, 7, 5
• Gastroesophageal reflux disease affects up to 58% of patients post-myotomy 8
• Erosive esophagitis develops in 23-48% of cases without adequate acid suppression 8
• PD has a 10-40% rate of symptomatic GORD or ulcerative esophagitis after treatment 6, 5

Critical Complications to Monitor

Suspect perforation when patients develop pain, breathlessness, fever, or tachycardia after any intervention. 5

• Most perforations occur during the first dilatation 5
• Monitor for pulmonary aspiration, chest infections, persistent dysphagia, and weight loss as these are the most significant complications affecting morbidity and mortality 5

Special Considerations

Patients with esophagogastric outflow obstruction (EGJOO) alone should not undergo permanent intervention without comprehensive evaluation and correlation with symptoms. 6, 7

• EGJOO is a manometric finding associated with multiple alternative causes including obesity, hiatal hernia, and opioid effects 6
• 20-40% of EGJOO cases resolve spontaneously, but 12-40% end up being treated as achalasia 6
• Evidence for POEM for EGJOO and nonachalasia spastic disorders is limited and should only be considered case-by-case after less invasive approaches have been exhausted 6

Patients with achalasia have a 4.6-fold higher risk for esophageal cancer, providing support for endoscopic surveillance. 7