Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content + Print Section ++ For further information, see CMDT Part 15-20: Esophageal Motility Disorders + Key Features Print Section ++ ++ Essentials of Diagnosis ++ Gradual, progressive dysphagia for solids and liquids Regurgitation of undigested food Barium esophagogram shows "bird's beak" distal esophagus Esophageal manometry confirms diagnosis ++ General Considerations ++ Idiopathic motility disorder characterized by loss of peristalsis in the distal two-thirds (smooth muscle) of the esophagus and impaired relaxation of the lower esophageal sphincter Cause unknown Type III is a spastic variant with less favorable treatment outcomes (66%) than types I (81%) or II (96%) ++ Demographics ++ Increased incidence with advancing age Can be seen in persons as young as 25 years + Clinical Findings Print Section ++ ++ Symptoms and Signs ++ Gradual onset of dysphagia for solid foods and, in the majority, liquids also Symptoms persist for months to years Substernal chest pain, discomfort, or fullness Regurgitation of undigested food Nocturnal regurgitation Coughing or aspiration Weight loss is common Physical examination unhelpful ++ Differential Diagnosis ++ Chagas disease Primary or metastatic tumors at the gastroesophageal junction Diffuse esophageal spasm Scleroderma esophagus Peptic stricture + Diagnosis Print Section ++ ++ Imaging Studies ++ Chest radiographs: air-fluid level in an enlarged, fluid-filled esophagus Barium esophagography Esophageal dilation Loss of esophageal peristalsis Poor esophageal emptying A smooth, symmetric "bird's beak" tapering of the distal esophagus Five minutes after ingestion of 8 oz of barium, a column height of > 2 cm has a sensitivity and specificity of > 85% in differentiating achalasia from other causes of dysphagia ++ Diagnostic Procedures ++ Endoscopy to exclude a distal stricture or carcinoma Esophageal manometry confirms the diagnosis; characteristic features include Complete absence of normal peristalsis Incomplete lower esophageal sphincteric relaxation with swallowing + Treatment Print Section ++ ++ Medications ++ Calcium channel blockers (nifedipine) may provide temporary symptomatic improvement for some esophageal disorders ++ Surgery ++ Modified Heller cardiomyotomy of the lower esophageal sphincter and cardia Performed by a laparoscopic approach and preferred to an open surgical approach Results in good to excellent symptomatic improvement in over 90% of patients Because gastroesophageal reflux develops in up to 20% of patients after myotomy, most surgeons also perform an antireflux procedure (fundoplication) All patients are prescribed a once-daily proton pump inhibitor Symptoms recur following cardiomyotomy in > 25% within 10 years but usually respond to pneumatic dilation Pneumatic dilation may be less effective in young males (age < 45 years), thus surgical myotomy may be preferred for them Surgical myotomy may also be preferred for patients with the type III variant Per-oral endoscopic myotomy (POEM) Success rates of over 90% are reported, including in patients with type III achalasia Long-term proton pump inhibitor therapy is required in many patients with gastroesophageal reflux because a fundoplication is not performed In expert centers, POEM may be the preferred treatment modality for type III achalasia (where a longer myotomy of the distal esophagus is indicated) and an appropriate option in patients with types I and II achalasia who do not wish to undergo laparoscopic surgery Complete esophagectomy or percutaneous gastrostomy may be required in patients with megaesophagus, in whom dilation and myotomy are less effective ++ Therapeutic Procedures ++ Botulinum toxin injection Endoscopically guided injection of botulinum toxin directly into the lower esophageal sphincter results in improvement in 65–85% Symptom relapse occurs in > 50% within 6–9 months May cause submucosal scarring that may make subsequent surgical myotomy more difficult 75% of initial responders to botulinum toxin injection who relapse improve with repeated injections Pneumatic dilation; goal is to disrupt lower esophageal sphincter About 90% of patients experience good to excellent relief of dysphagia after 1–3 sessions Symptoms recur in up to 35% within 10 years but usually respond to repeated dilation + Outcome Print Section ++ ++ Follow-Up ++ No follow-up is necessary unless symptoms recur ++ Complications ++ Perforations occur in < 3% of pneumatic dilations, may require operative repair ++ When to Refer ++ All patients with achalasia should be evaluated by a gastrointestinal specialist + References Print Section ++ + +Blonski W et al. Timed barium swallow: diagnostic role and predictive value in untreated achalasia, esophagogastric outflow obstruction, and non-achalasia dysphagia. Am J Gastroenterol. 2018 Feb;113(2):196–203. [PubMed: 29257145] + +Chrystoja CC et al. Achalasia-specific quality of life after pneumatic dilation or laparoscopic Heller myotomy with partial fundoplication: a multicenter, randomized controlled trial. Am J Gastroenterol. 2016 Nov;111(11):1536–45. [PubMed: 27619832] + +Haito-Chavez Y et al. Comprehensive analysis of adverse events associated with per oral endoscopic myotomy in 1826 patients: an international multicenter study. Am J Gastroenterol. 2017 Aug;112(8):1267–76. [PubMed: 28534521] + +Kahrilas PJ et al. Clinical practice update: the use of per-oral endoscopic myotomy in achalasia: expert review and best practice advice from the AGA Institute. Gastroenterology. 2017 Nov;153(5):1205–11. [PubMed: 28989059] + +Li QL et al. Outcomes of per-oral endoscopic myotomy for treatment of esophageal achalasia with a median follow-up of 49 months. Gastrointest Endosc. 2018 Jun;87(6):1405–12.e3. [PubMed: 29108981] + +Ngamruengphong S et al. Long-term outcomes of per-oral endoscopic myotomy in patients with achalasia with a minimum follow-up of 2 years: an international multicenter study. Gastrointest Endosc. 2017 May;85(5):927–33. [PubMed: 27663714] + +Pannala R et al. ASGE Technology Status Evaluation Report: Per-oral myotomy (with video). Gastrointest Endosc. 2016 Jun;83(6):1051–60. [PubMed: 27033144]