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Biliary achalasia — Clinical Guidelines

Overview

Achalasia is a primary motor disorder characterized by impaired esophageal peristalsis, elevated lower esophageal sphincter (LES) pressure, and failure of LES relaxation, leading to dysphagia, regurgitation, and weight loss 11.

Diagnosis

Key Diagnostic Criteria: Incomplete LES relaxation, absence of esophageal peristalsis, and abnormal barium swallow findings 11.

Recommended Tests: Esophageal manometry, barium swallow, and endoscopy to confirm diagnosis 11 27.

Grading: Eckardt symptom score often used to assess severity and treatment response 2 11.

Management

First-Line Treatments:

- Peroral Endoscopic Myotomy (POEM): Effective for symptom relief with favorable outcomes 1 5 7 8. - Laparoscopic Heller Myotomy (LHM): Long-term efficacy demonstrated in comparative trials 2 4.

Adjunctive Treatments:

- Pneumatic Dilation: Useful, especially in pediatric populations, with predictive factors for success identified 10. - Fundoplication: Often combined with myotomy to prevent reflux post-myotomy 25.

Special Populations

Pediatrics: Pneumatic dilation effective; predictive factors for treatment failure identified 10.

Elderly: Thoracoscopic and gasless laparoscopic techniques offer minimally invasive options with favorable outcomes 17 18.

Comorbidities: Careful attention to clinical features to avoid misdiagnosis and inappropriate interventions (e.g., fundoplication in achalasia) 19.

Key Recommendations

Primary Treatment Options: POEM or LHM should be considered based on patient preference and operator expertise (Evidence: Strong 1 2 7 8).

Long-Term Follow-Up: Recommend long-term follow-up to assess durability of treatment effects, with LHM showing sustained benefits over pneumatic dilation 2 (Evidence: Strong).

Avoiding Complications: Careful clinical evaluation to prevent misdiagnosis and inappropriate interventions, such as fundoplication in achalasia patients 19 (Evidence: Moderate).

Pediatric Considerations: Pneumatic dilation can be effective in pediatric achalasia, with careful monitoring for predictive factors of treatment failure 10 (Evidence: Moderate).

Minimally Invasive Techniques: Thoracoscopic and gasless laparoscopic approaches are viable options for elderly patients, offering quick recovery and minimal complications 17 18 (Evidence: Moderate).

References

1 Yang D, Bechara R, Dunst CM, Konda VJA. AGA Clinical Practice Update on Advances in Per-Oral Endoscopic Myotomy (POEM) and Remaining Questions-What We Have Learned in the Past Decade: Expert Review. Gastroenterology 2024. link 2 Boeckxstaens G, Elsen S, Belmans A, Annese V, Bredenoord AJ, Busch OR et al.. 10-year follow-up results of the European Achalasia Trial: a multicentre randomised controlled trial comparing pneumatic dilation with laparoscopic Heller myotomy. Gut 2024. link 3 Kuipers T, Ponds FA, Fockens P, Bastiaansen BAJ, Pandolfino JE, Bredenoord AJ. Focal Distal Esophageal Dilation (Blown-Out Myotomy) After Achalasia Treatment: Prevalence and Associated Symptoms. The American journal of gastroenterology 2024. link 4 Oude Nijhuis RAB, Zaninotto G, Roman S, Boeckxstaens GE, Fockens P, Langendam MW et al.. European guidelines on achalasia: United European Gastroenterology and European Society of Neurogastroenterology and Motility recommendations. United European gastroenterology journal 2020. link 5 Dacha S, Wang L, Li X, Jiang Y, Philips G, Keilin SA et al.. Outcomes and quality of life assessment after per oral endoscopic myotomy (POEM) performed in the endoscopy unit with trainees. Surgical endoscopy 2018. link 6 Obradović D, Sević BJ, Stojanović M, Ilić M, Ivanov I. "Stray" achalasia: From gastroenterologist to pulmonologist and back. Srpski arhiv za celokupno lekarstvo 2016. link 7 Sanaka MR, Jegadeesan R, Thota PN, Navaneethan U, Lopez R, Murthy SC et al.. Two-Person Technique of Peroral Endoscopic Myotomy for Achalasia with an Advanced Endoscopist and a Thoracic Surgeon: Initial Experience. Canadian journal of gastroenterology & hepatology 2016. link 8 Dunst CM, Kurian AA, Swanstrom LL. Endoscopic myotomy for achalasia. Advances in surgery 2014. link 9 Tan JT, Dudi-Venkata NN, Neelankavil SI, Sanders LH. A bleeding esophagopulmonary fistula: rare complication of stasis ulcer in refractory achalasia. Surgical laparoscopy, endoscopy & percutaneous techniques 2014. link 10 Di Nardo G, Rossi P, Oliva S, Aloi M, Cozzi DA, Frediani S et al.. Pneumatic balloon dilation in pediatric achalasia: efficacy and factors predicting outcome at a single tertiary pediatric gastroenterology center. Gastrointestinal endoscopy 2012. link 11 Eckardt AJ, Eckardt VF. Current clinical approach to achalasia. World journal of gastroenterology 2009. link 12 Mikaeli J, Islami F, Malekzadeh R. Achalasia: a review of Western and Iranian experiences. World journal of gastroenterology 2009. link 13 Ahmed WU, Qureshi H, Maher M, Arif A. Achalasia in a gastroenterology unit of Karachi. JPMA. The Journal of the Pakistan Medical Association 2008. link 14 Yi A, Shin JH, Song HY, Jung HY, Lee GH, Yoon CJ et al.. Esophageal achalasia: comparison of fluoroscopically-guided double vs. endoscopically-guided single balloon dilation. Abdominal imaging 2008. link 15 Kennedy R, Hunt S, Ahmad J, Menezes C, Clements WB, Kennedy JA. Wernicke's encephalopathy after laparoscopic cardiomyotomy for achalasia. JPEN. Journal of parenteral and enteral nutrition 2007. link 16 Shah J, Rockall T, Darzi A. Robot-assisted laparoscopic Heller's cardiomyotomy. Surgical laparoscopy, endoscopy & percutaneous techniques 2002. link 17 Foley R, Brough W. Thoracoscopic cardiomyotomy for achalasia of the cardia: early results. Annals of the Royal College of Surgeons of England 1995. link 18 Azurin DJ, Go LS, Schuricht AL. Gasless laparoscopic esophagomyotomy. Surgical endoscopy 1995. link 19 Marshall JB. Pneumatic dilatation for achalasia after fundoplication. Journal of clinical gastroenterology 1989. link 20 Carlsson-Nordlander B. Acute upper airway obstruction in a patient with achalasia. Archives of otolaryngology--head & neck surgery 1987. link 21 Cox J, Buckton GK, Bennett JR. Balloon dilatation in achalasia: a new dilator. Gut 1986. link 22 Efrati Y, Mares AJ. Infantile achalasia associated with deficient tear production. Journal of clinical gastroenterology 1985. link 23 Zimmerman FH, Rosensweig NS. Achalasia in a father and son. The American journal of gastroenterology 1984. link 24 Kenney RD. Achalasia in an adolescent with behavioral features compatible with anorexia nervosa. Journal of adolescent health care : official publication of the Society for Adolescent Medicine 1984. link80134-5) 25 Pinotti HW, Sakai P, Ishioka S. Cardiomyotomy and fundoplication for esophageal achalasia. The Japanese journal of surgery 1983. link 26 Blok C, Van Elk PJ. The treatment of benign strictures in the distal portion of the oesophagus. Archivum chirurgicum Neerlandicum 1977. link 27 Edwards DA. Achalasia: symptoms and radiology. Postgraduate medical journal 1974. link

Biliary achalasia