Diagnosis and Management of Achalasia Cardia
Diagnostic Workup
All patients suspected of having achalasia must undergo a comprehensive diagnostic evaluation including clinical history with medication review, upper endoscopy, timed barium esophagram, and high-resolution manometry (HRM) to confirm the diagnosis and guide treatment decisions. 1
Essential Diagnostic Tests
Upper endoscopy (EGD) is mandatory to identify findings suggestive of poor esophageal clearance (frothy retained secretions, puckered gastroesophageal junction) and to exclude pseudoachalasia from occult malignancy through careful retroflexed examination of the gastroesophageal junction 1
Timed barium esophagram confirms outflow obstruction, demonstrates structural changes, and can reveal more subtle narrowing at the esophagogastric junction; administration of a 13-mm barium tablet may elicit additional evidence 1
High-resolution manometry (HRM) remains the gold standard for diagnosis and is crucial for defining achalasia subtype according to the Chicago Classification system, which has critical therapeutic implications 1
Endoscopic functional luminal impedance planimetry (FLIP) serves as a useful adjunct test, particularly when diagnosis is equivocal, by demonstrating impaired esophagogastric junction opening through low distensibility index 1
Excluding Malignancy
Computed tomography scanning and endoscopic ultrasound may be necessary when neoplasm is suspected and other modalities fail to confirm the diagnosis, as pseudoachalasia from malignancy must be excluded 1
Careful endoscopic assessment with biopsies of any irregularities at the gastroesophageal junction is essential before proceeding with treatment 1
Management Strategy by Achalasia Subtype
Type I and Type II Achalasia
For type I and type II achalasia, POEM (per-oral endoscopic myotomy), laparoscopic Heller myotomy (LHM), and pneumatic dilation (PD) are all effective therapies; the decision should be based on shared decision-making considering patient characteristics, preferences, and local expertise. 1
Pneumatic dilation has less morbidity and cost compared to surgical options but requires anticipation of repeat dilations over years 1
Laparoscopic Heller myotomy with partial fundoplication and pneumatic dilation both have excellent outcomes demonstrated in high-level randomized controlled trials 1
POEM has been found superior to pneumatic dilation and noninferior to laparoscopic Heller myotomy in separate multicenter randomized controlled trials, though only short-term data are available 1
Graded pneumatic dilation to between 13-20 mm provides good relief in 85-93% of cases with benign strictures 1
Type III Achalasia (Spastic Achalasia)
POEM should be considered the preferred treatment for type III achalasia because it allows unlimited proximal extension of myotomy tailored to the extent of esophageal body spasm, which is critical for optimal outcomes in this subtype. 1
Type III achalasia is characterized by spastic body contractions and symptoms are best palliated with myotomy extended to the proximal extent of esophageal body spasm rather than confined to the lower esophageal sphincter alone 1
The myotomy length should be calibrated to the spastic segment imaged on HRM or thickened segment on endoscopic ultrasound 1
POEM provides the advantage of unlimited proximal extension compared to laparoscopic approaches 1
Esophagogastric Outflow Obstruction (EGJOO)
Patients with EGJOO alone and/or nonachalasia spastic disorders should undergo comprehensive evaluation with symptom correlation, and POEM should only be considered on a case-by-case basis after other less invasive approaches have been exhausted, as evidence is limited. 1
Many EGJOO cases can resolve spontaneously or represent early/incomplete achalasia (12-40% of cases) 1
Further imaging of the esophagogastric junction (EUS or CT) may clarify diagnosis and exclude secondary causes 1
POEM-Specific Considerations
Technical Aspects
POEM should be performed by experienced physicians in high-volume centers as an estimated 20-40 procedures are needed to achieve competence 1
POEM can be performed via either anterior or posterior tunnel orientation with comparable efficacy, safety, and postprocedure reflux rates; endoscopist preference and patient's surgical history should guide tunnel orientation 1
A single dose of antibiotics at the time of POEM may be sufficient for antibiotic prophylaxis 1
The optimal length of myotomy in the esophagus and cardia remains to be determined; adjunct techniques including real-time intraprocedure FLIP may be considered to tailor or confirm adequacy 1
Post-POEM Management
Pharmacologic acid suppression should be strongly considered in the immediate post-POEM setting given the increased risk of postprocedure reflux and esophagitis 1
Post-POEM patients should be considered high risk to develop reflux esophagitis and advised of management considerations including potential indefinite proton pump inhibitor therapy and/or surveillance endoscopy before undergoing the procedure 1
Same-day discharge can be considered in select patients meeting discharge criteria; patients with advanced age, significant comorbidities, poor social support, and/or limited access to specialized care should be considered for hospital admission 1
The clinical impact of routine esophagram or endoscopy immediately post-POEM remains unclear but can be considered based on local practice preferences and when intraprocedural events warrant further evaluation 1
Alternative Treatment Options
Pneumatic Dilation
Graded approach with weekly dilatation until easy passage of greater than 14 mm dilator is common strategy for tight strictures 1
Perforation risk is 0-7% (mostly 3-4%) with mortality <1%; most perforations occur during the first dilatation 1, 2
Post-dilatation reflux may occur but is usually mild and readily controlled with acid suppression 1
Medical Management
- Botulinum toxin injection and pharmacologic therapy (nitrates, calcium channel blockers) are reserved for patients unable to undergo more effective therapies due to inferior long-term outcomes 3, 4
Critical Complications to Monitor
Immediate Post-Procedure Complications
Suspect perforation when patients develop pain, breathlessness, fever, or tachycardia after any intervention 1, 2
Urgent chest x-ray and water-soluble contrast study should be performed if perforation is suspected; normal chest x-ray does not exclude perforation 1, 2
Perforation is a medical emergency requiring assessment by experienced physician and surgeon to formulate management plan 1
Long-Term Complications
Monitor for pulmonary aspiration, chest infections, persistent dysphagia, and weight loss as these are the most significant complications affecting morbidity and mortality 2
Pulmonary aspiration is a major complication due to food and fluid retention in the esophagus, particularly in patients with esophageal stasis 2
Regular monitoring of weight and nutritional status is essential as weight loss is common due to reduced oral intake 2
Gastroesophageal reflux commonly develops after successful treatment and can lead to reflux esophagitis and stricture formation if untreated 2
Special Populations
Patients on Anticoagulation
Patients at low risk of thromboembolic events should discontinue anticoagulants prior to dilatation with preprocedure prothrombin time performed 1
High-risk patients should discontinue oral anticoagulants and transition to intravenous heparin, which should be stopped 4-6 hours before and resumed 4-6 hours after the procedure 1
High-Risk Patients
Elderly patients and those with significant comorbidities (ASA grades III-V) are at higher risk of complications and cardiorespiratory events during procedures 1, 2
Patients with achalasia are particularly prone to esophageal stasis and may require prolonged fasting or esophageal lavage before procedures 1