Achalasia: Diagnosis and Treatment
Diagnosis
High-resolution manometry (HRM) is the gold standard for diagnosing achalasia and must be performed to confirm the diagnosis and determine the achalasia subtype according to the Chicago Classification system, which is essential for guiding treatment decisions. 1
Comprehensive Diagnostic Workup
The diagnostic evaluation must include the following tests 1:
- Clinical history: Look specifically for dysphagia to both solids and liquids, regurgitation of undigested food, chest pain, weight loss, and pulmonary symptoms (cough, aspiration, recurrent chest infections) 2
- Upper endoscopy (EGD): Identify frothy retained secretions, puckered gastroesophageal junction, and perform careful retroflexed examination to exclude pseudoachalasia 1
- Timed barium esophagram: Demonstrates barium retention, structural changes, and confirms outflow obstruction; administration of a 13-mm barium tablet can reveal subtle EGJ narrowing 1
- High-resolution manometry: Confirms diagnosis and defines subtype (Type I, II, or III) based on esophageal body pressurization patterns and spastic contractions 1
- Functional luminal impedance planimetry (FLIP): Useful adjunct when diagnosis is equivocal; impaired EGJ opening with low distensibility index supports achalasia diagnosis 1
Critical Diagnostic Pitfalls
Be aware that symptoms may be present for 0.5 to 2.8 years before diagnosis, and early disease may have subtle manometric findings that don't meet full diagnostic criteria 2. Late-stage disease may show very low LES pressure mimicking absent contractility rather than achalasia 2.
Treatment
For Type I and Type II achalasia, POEM (per-oral endoscopic myotomy), laparoscopic Heller myotomy (LHM), and pneumatic dilation (PD) are all effective; however, for Type III achalasia, POEM should be considered the preferred treatment because it allows unlimited proximal extension of myotomy tailored to the spastic segment. 1
Treatment Algorithm by Achalasia Subtype
Type I and Type II Achalasia
Treatment selection should be based on shared decision-making considering patient characteristics, local expertise, and patient preferences 1:
POEM: Superior to PD and noninferior to LHM in randomized controlled trials; symptom improvement 89-97% 1
Laparoscopic Heller myotomy with fundoplication: 90% symptom relief with low complication rate (6.3%) 4
- Adding fundoplication reduces postoperative reflux (8.8% with fundoplication vs. 31.5% without) 4
Pneumatic dilation: 68-90% symptom improvement; less morbidity and cost than surgery 1, 4, 5
Type III Achalasia (Spastic Achalasia)
POEM is the preferred treatment because it allows calibration of myotomy length to the proximal extent of the spastic segment identified on HRM or thickened segment on EUS 1. Laparoscopic approaches cannot achieve this extended myotomy effectively 1.
Post-Treatment Management
All patients undergoing POEM must be counseled about high reflux risk and the likely need for indefinite proton pump inhibitor (PPI) therapy 1, 3:
- Prescribe 8 weeks of PPI therapy immediately post-POEM to decrease acid secretion and aid mucosal healing 3
- Consider indefinite PPI therapy given the increased risk of reflux esophagitis 1, 3
- Surveillance endoscopy may be necessary 1
Treatment for Special Populations
For esophagogastric junction outflow obstruction (EGJOO) alone or nonachalasia spastic disorders, POEM should only be considered on a case-by-case basis after other less invasive approaches have been exhausted 1. EGJOO is not pathognomonic for any single diagnosis and should not justify permanent intervention in isolation 1.
Obsolete Treatments
Botulinum toxin injection should be reserved only for patients who cannot undergo balloon dilation and are not surgical candidates, as it provides inferior symptom relief (40.6%) and requires more frequent additional therapy (46.6%) compared to pneumatic dilation 4, 5. Calcium channel blockers and nitrates offer only mild, transient improvement and are reserved for patients who are not candidates for definitive therapy 5, 6, 7.