Abstract
Idiopathic achalasia is a rare esophageal dysmotility disorder of unknown etiology with only palliative treatment available. Many studies have established a significantly increased risk of esophageal cancer in patients with achalasia. However, current guidelines advise against routine surveillance due to low absolute risk and a paucity of high-quality evidence and cost-effectiveness assessments. This review aims to assess the need for routine endoscopic surveillance in achalasia based on a growing body of literature calling in support of it, mainly due to the increased risk of esophageal cancer. We searched PubMed and Google Scholar electronic databases for articles within the last 10 years using the keywords 'achalasia', 'cancer,' 'neoplasms,' 'screening,' and 'surveillance.' After excluding pseudoachalasia/secondary achalasia, other esophageal dysmotility disorders, and associations with malignancies outside the esophagus, we selected 31 articles for this review. Through these articles, we identified areas of focus for ongoing and future research that may result in significant risk reduction of complications, including esophageal cancer and beyond.
Highlights
BackgroundIdiopathic achalasia is a progressive esophageal motility disorder characterized by esophageal aperistalsis and failure of the lower esophageal sphincter (LES) to relax spontaneously [1,2]
This review aims to assess the need for routine endoscopic surveillance in achalasia based on a growing body of literature calling in support of it, mainly due to the increased risk of esophageal cancer
A strong, established association between achalasia and the development of esophageal cancer exists, with recent literature calling for routine endoscopic cancer surveillance to be considered as an individual patient risk, in men, is significantly high
Summary
Idiopathic achalasia is a progressive esophageal motility disorder characterized by esophageal aperistalsis and failure of the lower esophageal sphincter (LES) to relax spontaneously [1,2]. The guidelines on the management of achalasia published in 2020 by the American Society of Gastrointestinal Endoscopy (ASGE) report insufficient evidence to support routine screening in this population They suggest that surveillance endoscopy may benefit patients who have undergone a POEM due to the increased risk of reflux, but recognized that more evidence is needed to support a stronger recommendation [25]. A 2020 review article in the Annals of Esophagus (AOE) journal noting the guidelines of the ASGE and ACG brought attention to the differences in management recommendations for conditions with shared risk factors and commonly seen conditions in achalasia such as BE, where surveillance is recommended [31] This discrepancy is seen in the ASGE’s recommendation for surveillance in post-POEM achalasia patients due to increased reflux, increasing the risk of AC, despite the much higher risk for SCC present from the diagnosis of achalasia alone. No specific surveillance schedule is proposed in the ESGE recommendations, and surveillance strategies across Europe continue to vary
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