GI NOTES

Achalasia

1. Presentation

• Dysphagia to solids and liquids from the start (mechanical obstruction usually begins with solids only).
• Regurgitation of undigested food, often at night; aspiration and cough.
• Retrosternal chest pain, especially in type III. Weight loss.
• Often misdiagnosed as GERD for years.

Eckardt score (0 to 12) adds dysphagia, regurgitation, chest pain (each 0 to 3) and weight loss (0 to 3). A score above 3 after treatment means treatment failure.

2. Diagnostic work-up

Test	Typical finding
EGD	Dilated esophagus with retained food or fluid; tight LES that the scope passes with a gentle pop. Look hard at the cardia for tumor.
Barium esophagram	Dilated esophagus with a smooth “bird’s beak” taper. A timed barium esophagram (column height at 1 and 5 minutes) is useful to measure emptying and follow response.
High-resolution manometry	Elevated integrated relaxation pressure (IRP) and 100% failed peristalsis
FLIP (functional lumen imaging probe)	Reduced EGJ distensibility; helpful when manometry is inconclusive

Chicago Classification v4.0 subtypes

Type	Manometry	Notes
Type I (classic)	No contractility, no pressurization	Often a dilated esophagus
Type II	Panesophageal pressurization in 20% or more of swallows	Best response to any treatment
Type III (spastic)	Premature (spastic) contractions in 20% or more of swallows	Chest pain is common; POEM preferred because the myotomy can be tailored in length

Red flags for pseudoachalasia: age above about 55, symptoms for less than a year, rapid weight loss. Consider CT or endoscopic ultrasound.

3. Treatment

Option	Best for	Key facts
Pneumatic dilation (30, 35, then 40 mm balloon)	Types I and II	Graded dilations; perforation about 1 to 2%. Often repeated over time.
Laparoscopic Heller myotomy with partial fundoplication (Dor or Toupet)	Types I and II	Fundoplication reduces post-operative reflux. Durable results.
POEM (peroral endoscopic myotomy)	All types; preferred in type III	Equivalent to Heller for types I and II. Post-procedure reflux is common (up to about half develop esophagitis), so plan PPI and endoscopic follow-up.
Botulinum toxin injection	Poor candidates for definitive therapy	Effect fades over 6 to 12 months; repeated injections can make later myotomy harder.
Calcium channel blockers or nitrates	Bridge only	Least effective; side effects common.

Choose a definitive option based on subtype, local expertise, patient preference, and reflux risk. Discuss the reflux trade-off of POEM openly.

4. Follow-up

• Assess symptoms (Eckardt score) and objective emptying with a timed barium esophagram after treatment.
• Recurrence is treated with repeat dilation, POEM after failed Heller, or redo surgery.
• End-stage, massively dilated (sigmoid) esophagus may need esophagectomy.
• Long-standing achalasia carries an increased risk of squamous cell carcinoma; any new symptoms deserve a low threshold for endoscopy.

5. High-yield pearls

• Liquid dysphagia from the start points to a motility problem, not a stricture.
• A normal-looking EGD does not rule out achalasia.
• Chagas disease can cause an identical picture; ask about time spent in endemic areas of Latin America.
• Type II has the best and type III the worst outcomes with dilation.

References

• Vaezi MF, Pandolfino JE, Yadlapati RH, Greer KB, Kavitt RT. ACG Clinical Guidelines: Diagnosis and Management of Achalasia. Am J Gastroenterol. 2020;115(9):1393-1411.
• Yadlapati R, Kahrilas PJ, Fox MR, et al. Esophageal motility disorders on high-resolution manometry: Chicago Classification version 4.0. Neurogastroenterol Motil. 2021;33(1):e14058.
• Khashab MA, Vela MF, Thosani N, et al. ASGE guideline on the management of achalasia. Gastrointest Endosc. 2020;91(2):213-227.
• Werner YB, Hakanson B, Martinek J, et al. Endoscopic or Surgical Myotomy in Patients with Idiopathic Achalasia. N Engl J Med. 2019;381(23):2219-2229.

For educational purposes only. This note does not replace clinical judgment or local protocols. Check doses against current labeling and institutional guidance.