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Gastroenterology

Achalasia

Confirm suspected achalasia with high-resolution manometry after excluding mechanical obstruction, then select pneumatic dilation, laparoscopic Heller myotomy with fundoplication, or POEM by subtype, anatomy, reflux tradeoff, prior therapy, and local expertise.

Clinical question: How should physicians confirm achalasia, exclude pseudoachalasia, choose definitive therapy, and evaluate persistent symptoms after treatment?

Diagnosis

Confirm achalasia and exclude pseudoachalasia before LES-directed therapy

Do not treat a manometric pattern as idiopathic achalasia until structural obstruction has been assessed.

Obtain esophagogastroduodenoscopy in every patient with suspected achalasia before definitive therapy. A retained saliva-filled esophagus and a puckered, tightly closed gastroesophageal junction support the diagnosis, but the critical procedural purpose is exclusion of pseudoachalasia from an obstructing mass. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedAn Overview of Achalasia and Its Subtypes - PMC

Confirm primary achalasia with high-resolution manometry (HRM): impaired esophagogastric junction relaxation together with absent organized esophageal peristalsis establishes the diagnosis. HRM is the current gold standard and separates clinically relevant subtypes. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedAn Overview of Achalasia and Its Subtypes - PMC

Use a timed barium esophagram when HRM is equivocal, when anatomy needs characterization before intervention, or when symptoms recur after therapy. In a symptomatic patient, aperistalsis, esophageal dilation, and distal "bird-beak" narrowing are characteristic radiographic findings. Wolters KluwerACG Clinical Guideline: Diagnosis and Management... : American Journal of GastroenterologyPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedACG clinical guideline: diagnosis and management of achalasia - PubMed

Tests that change the next diagnostic or therapeutic step in suspected achalasia. Wolters KluwerACG Clinical Guideline: Diagnosis and Management... : American Journal of GastroenterologyPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedAn Overview of Achalasia and Its Subtypes - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
TestActionable findingWhat it changes
Upper endoscopyRetained saliva with puckered gastroesophageal junction supports achalasia; obstructing lesion raises pseudoachalasia. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedAn Overview of Achalasia and Its Subtypes - PMCExclude mechanical obstruction before LES-disrupting therapy; investigate a detected mass rather than proceeding directly to dilation or myotomy. PubMedAn Overview of Achalasia and Its Subtypes - PMC
High-resolution manometryImpaired EGJ relaxation plus absent organized peristalsis confirms achalasia and identifies subtype. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedAn Overview of Achalasia and Its Subtypes - PMCUse subtype in procedure selection; type III favors POEM. PubMedAn Overview of Achalasia and Its Subtypes - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Timed barium esophagramDilated, aperistaltic esophagus with distal bird-beak narrowing in a symptomatic patient. Wolters KluwerACG Clinical Guideline: Diagnosis and Management... : American Journal of GastroenterologyPubMedACG clinical guideline: diagnosis and management of achalasia - PubMedDefines anatomic retention and provides an objective comparator when evaluating post-treatment symptoms. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMC

Definitive treatment

Choose LES-disrupting therapy by subtype, durability, reflux risk, and procedural context

All established therapies are palliative and reduce outflow resistance at the nonrelaxing lower esophageal sphincter.

For treatment-naive type I or II achalasia, offer pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), or peroral endoscopic myotomy (POEM) as definitive options. The selection should explicitly weigh patient preference, local procedural expertise, anticipated need for retreatment, and risk of postprocedural reflux. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

POEM is the preferred treatment for type III achalasia. Its endoscopic myotomy can be extended to address the spastic esophageal segment, whereas outcomes with conventional LHM and PD have historically been more limited in type III disease. jtcvsPeroral endoscopic myotomy provides effective palliation in type III ...ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

When LHM is selected, perform an anterior fundoplication with the myotomy. This operative approach is intended to relieve dysphagia while mitigating the reflux consequence of an unopposed surgical myotomy. JAMALaparoscopic Heller Myotomy and Anterior Fundoplication for ...Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford Academic

Discuss reflux explicitly when choosing POEM. Comparative evidence shows more post-treatment GERD symptoms and more endoscopic esophagitis after POEM than after LHM; in a large randomized comparison, the odds ratio for endoscopic esophagitis at 24 months was 2.00. Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford Academic Post-POEM reflux may be endoscopic rather than symptom-detected, so absence of heartburn does not exclude clinically relevant reflux injury. Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford AcademicScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

Practical selection among established achalasia interventions. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectOxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford AcademicPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCjtcvsPeroral endoscopic myotomy provides effective palliation in type III ...ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
InterventionBest-fit clinical contextKey tradeoff
POEMPreferred for type III achalasia; also an effective option for type I or II disease. jtcvsPeroral endoscopic myotomy provides effective palliation in type III ...ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaHigher postprocedure GERD symptoms and endoscopic esophagitis than LHM in comparative studies; arrange reflux surveillance. Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford AcademicScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Laparoscopic Heller myotomy with anterior fundoplicationEffective definitive option for type I or II achalasia when surgical therapy is appropriate. JAMALaparoscopic Heller Myotomy and Anterior Fundoplication for ...PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaRequires laparoscopy but includes an antireflux procedure; comparative data favor lower reflux burden than POEM. JAMALaparoscopic Heller Myotomy and Anterior Fundoplication for ...Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford Academic
Pneumatic dilationEffective definitive option for type I or II disease, particularly when an endoscopic, nonmyotomy strategy is preferred. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaMay require repeat treatment; outcomes are less favorable than POEM in some comparisons and are not preferred for type III disease. ScienceDirectManage Consent PreferencesjtcvsPeroral endoscopic myotomy provides effective palliation in type III ...ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Botulinum toxin injectionTemporary palliation when definitive treatment is unsuitable. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectEffective in about two-thirds of patients but typically lasts only months. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirect

Persistent or recurrent symptoms

Re-evaluate objectively before retreatment after dilation or myotomy

Persistent dysphagia is not synonymous with recurrent LES obstruction.

For persistent or recurrent dysphagia after PD, LHM, or POEM, reassess symptoms with the Eckardt score and obtain objective testing rather than selecting repeat therapy from symptoms alone. Timed barium esophagram and repeat HRM help identify persistent esophagogastric junction outflow obstruction, while endoscopy evaluates retained contents, inflammatory injury, structural complications, and alternative pathology. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

After failed LHM, POEM is a reasonable salvage option. In a multicenter randomized study with 5-year follow-up after failed LHM, treatment success was 40% with POEM versus 11% with PD; grade B through D reflux esophagitis occurred in 11% and 9%, respectively. ScienceDirectLong-Term Outcomes of Peroral Endoscopic Myotomy vs Pneumatic Dilation After Prior Laparoscopic Heller Myotomy for Achalasia

After POEM, actively monitor for GERD, including endoscopic esophagitis. Persistent esophagitis or reflux-like symptoms despite proton pump inhibitor therapy should trigger testing for etiologies other than pathologic acid exposure and adjustment of reflux management rather than assuming inadequate acid suppression is the sole explanation. ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

Post-treatment symptoms: test-directed next steps. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaScienceDirectLong-Term Outcomes of Peroral Endoscopic Myotomy vs Pneumatic Dilation After Prior Laparoscopic Heller Myotomy for Achalasia
Clinical problemObjective assessmentManagement implication
Persistent dysphagia after any interventionEckardt score, timed barium esophagram, repeat HRM, and endoscopy as indicated. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaDistinguish persistent obstruction from reflux injury, anatomic progression, or another disorder before retreatment. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Failure after laparoscopic Heller myotomyConfirm ongoing symptom burden and objective abnormality before salvage therapy. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectLong-Term Outcomes of Peroral Endoscopic Myotomy vs Pneumatic Dilation After Prior Laparoscopic Heller Myotomy for AchalasiaPOEM produced higher 5-year treatment success than PD in a randomized post-LHM population. ScienceDirectLong-Term Outcomes of Peroral Endoscopic Myotomy vs Pneumatic Dilation After Prior Laparoscopic Heller Myotomy for Achalasia
Esophagitis or reflux symptoms after POEMEndoscopic assessment and further reflux evaluation when symptoms or esophagitis persist despite PPI therapy. ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaOptimize reflux control and investigate nonacid or alternative causes when appropriate. ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

Long-term care

Monitor treatment response, reflux injury, and progressive esophageal disease

Follow-up should target dysphagia recurrence and complications created by chronic stasis or LES-disrupting treatment.

At follow-up, document the Eckardt score and determine whether dysphagia, regurgitation, chest pain, or weight loss has recurred. Pair clinically meaningful symptom recurrence with timed barium esophagram, HRM, and/or endoscopy according to the suspected mechanism rather than using symptoms alone to define procedural failure. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

Maintain particular surveillance for reflux complications after POEM because esophagitis can occur without proportionate reflux symptoms. Persistent esophagitis warrants reassessment and management aimed at demonstrable reflux or an alternate cause. Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford AcademicScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia

Achalasia is associated with esophageal squamous cell carcinoma, but routine endoscopic cancer screening is not recommended by ACG and ASGE guidance because absolute cancer yield is low; one estimate suggested more than 400 endoscopies would be required to detect one cancer. New progressive symptoms, unexplained weight loss, or suspicious endoscopic findings should instead prompt diagnostic evaluation for malignancy. Wolters KluwerAchalasia: A Risk Factor for Esophageal Squamous Cell... : Official journal of the American College of Gastroenterology | ACGPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMC

Follow-up priorities after definitive achalasia treatment. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectWolters KluwerAchalasia: A Risk Factor for Esophageal Squamous Cell... : Official journal of the American College of Gastroenterology | ACGOxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford AcademicPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Follow-up targetAssessmentAction when abnormal
Recurrent symptom burdenEckardt score with targeted timed barium esophagram, HRM, or endoscopy. ScienceDirectPharmacologic Therapy in Treating Achalasia - ScienceDirectPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaIdentify the mechanism before repeating dilation or myotomy. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Post-POEM reflux injurySymptoms plus endoscopic assessment because esophagitis may not parallel symptoms. Oxford AcademicPeroral endoscopic myotomy compared to laparoscopic Heller myotomy and pneumatic dilation in the treatment of achalasia: a systematic review | Diseases of the Esophagus | Oxford AcademicScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasiaTreat and investigate persistent esophagitis or symptoms despite PPI therapy. ScienceDirectBlown-out myotomy: an adverse event of laparoscopic Heller myotomy and peroral endoscopic myotomy for achalasia
Cancer concernDiagnostic endoscopy for concerning new findings; do not use routine screening solely for achalasia. Wolters KluwerAchalasia: A Risk Factor for Esophageal Squamous Cell... : Official journal of the American College of Gastroenterology | ACGPubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCEvaluate for esophageal malignancy or pseudoachalasia when clinical concern is present. PubMedACG Clinical Guidelines: Diagnosis and Management of Achalasia - PMCPubMedAn Overview of Achalasia and Its Subtypes - PMC

References

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