What is the appropriate management for a patient presenting with esophageal dyskinesia?

Management of Esophageal Dyskinesia

Patients presenting with esophageal dyskinesia require endoscopy with biopsies as the initial diagnostic step to exclude structural and mucosal disease, followed by high-resolution manometry to characterize the specific motility disorder and guide treatment decisions. 1, 2

Initial Diagnostic Approach

Endoscopy First

• Upper endoscopy with esophageal biopsies at two levels is mandatory to exclude malignancy, peptic strictures, eosinophilic esophagitis, and other mucosal diseases before attributing symptoms to a motility disorder 1, 3
• Biopsies should be obtained even when mucosa appears normal, as eosinophilic esophagitis is one of the most common causes of dysphagia in adults and children 3
• Document stricture location, length, mucosal appearance, and any features that increase perforation risk (angulation, diverticula, hiatus hernia) 1

Barium Swallow Studies

• Biphasic esophagram (combining double-contrast and single-contrast views) provides superior diagnostic accuracy compared to single-contrast alone, with 80-89% sensitivity for detecting motility disorders 1
• Particularly valuable when strictures prevent endoscope passage or when evaluating long, tight, or complex strictures 1
• Prone single-contrast views are essential for detecting lower esophageal rings and strictures, which are missed on upright views due to inadequate distention 1

High-Resolution Manometry

When to Perform

• Manometry is indicated after normal endoscopy findings in patients with persistent dysphagia to diagnose the specific motility disorder and guide treatment 1
• Essential for confirming achalasia diagnosis and determining subtype, which predicts clinical outcomes 1
• Localizes the lower esophageal sphincter for potential pH monitoring and evaluates peristaltic function preoperatively 1

Diagnostic Criteria

• High-resolution manometry using Chicago Classification provides standardized diagnosis with hierarchical approach: first assess for esophagogastric junction obstruction (achalasia), then evaluate esophageal body function 1, 4
• Achalasia defined by integrated relaxation pressure (IRP) >15 mm Hg with 98% sensitivity and 96% specificity 1
• Spasm diagnosed when ≥20% of swallows have distal latency <4.5 seconds 1

Treatment Based on Specific Disorder

Achalasia

• Patients with suspected achalasia require manometry to confirm diagnosis and exclude occult carcinoma through careful endoscopic assessment of the gastroesophageal junction 1
• CT scanning and endosonography may be needed when malignancy is suspected 1
• For stage 4 achalasia with severe dilation (>6-7 cm) and sigmoid deformation, esophagectomy should be considered as primary treatment to address irreversible structural changes and prevent aspiration risk 5
• POEM may be attempted in select cases where esophagectomy is not feasible, but with tempered expectations due to limited data 5

Minor Motility Disorders

• Minor disorders of peristalsis (ineffective esophageal motility, fragmented peristalsis) have excellent prognosis, with 70% of patients becoming asymptomatic at 5-year follow-up 1
• These findings rarely progress over time and identification is a good prognostic indicator 1
• Consider provocative testing with rapid drink challenge or standardized meals if water swallows show no major disorder but symptoms persist 1

Diffuse Esophageal Spasm

• Patients may occasionally require lower esophageal sphincter dilation when conservative measures fail 1
• Manometry guides the extent of any surgical myotomy by defining anatomic and functional boundaries of the motor disorder 6

Critical Pitfalls to Avoid

Don't Skip Structural Evaluation

• Never proceed directly to manometry without first excluding structural and mucosal disease through endoscopy, as this risks missing malignancy or eosinophilic esophagitis 1, 3
• Proximal dysphagia may harbor high-risk pathology (pharyngeal pouch, post-cricoid web) requiring experienced endoscopist 1

Recognize Treatment Failures

• Patients with dysphagia unresponsive to twice-daily PPI therapy require endoscopy with biopsies to evaluate for alternative diagnoses 1
• If endoscopy is normal, proceed to manometry to evaluate for subtle presentations of major motor disorders 1
• Ambulatory pH monitoring (off PPI for 7 days) is indicated when both endoscopy and manometry are unrevealing 1

Pre-Procedure Considerations

• Patients with achalasia are particularly prone to esophageal stasis and may require prolonged fasting (>4-6 hours) or esophageal lavage before procedures 1, 5
• Active esophageal perforation is an absolute contraindication to dilation 1
• Severe coagulopathy and anticoagulation should be corrected before any dilation procedure 1

Monitoring for Complications

High-Risk Scenarios

• Stage 4 achalasia patients face high risk of pulmonary aspiration, chest infections, persistent dysphagia, and malnutrition significantly affecting morbidity and mortality 5
• Malignant strictures carry greater perforation risk during dilation compared to benign disease 1
• Post-POEM patients require indefinite acid suppression and surveillance endoscopy due to high reflux risk 5