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Objectives: High‐resolution manometry (HRM) and esophagography are used for achalasia diagnosis; however, achalasia phenotypes combining esophageal motility and morphology are unknown. Moreover, predicting treatment outcomes of peroral endoscopic myotomy (POEM) in treatment‐naïve patients remains an unmet need. Methods: In this multicenter cohort study, we included 1824 treatment‐naïve patients diagnosed with achalasia. In total, 1778 patients underwent POEM. Clustering by machine learning was conducted to identify achalasia phenotypes using patients' demographic data, including age, sex, disease duration, body mass index, and HRM/esophagography findings. Machine learning models were developed to predict persistent symptoms (Eckardt score ≥3) and reflux esophagitis (RE) (Los Angeles grades A–D) after POEM. Results: Machine learning identified three achalasia phenotypes: phenotype 1, type I achalasia with a dilated esophagus (n = 676; 37.0%); phenotype 2, type II achalasia with a dilated esophagus (n = 203; 11.1%); and phenotype 3, late‐onset type I–III achalasia with a nondilated esophagus (n = 619, 33.9%). Types I and II achalasia in phenotypes 1 and 2 exhibited different clinical characteristics from those in phenotype 3, implying different pathophysiologies within the same HRM diagnosis. A predictive model for persistent symptoms exhibited an area under the curve of 0.70. Pre‐POEM Eckardt score ≥6 was the greatest contributing factor for persistent symptoms. The area under the curve for post‐POEM RE was 0.61. Conclusion: Achalasia phenotypes combining esophageal motility and morphology indicated multiple disease pathophysiologies. Machine learning helped develop an optimal risk stratification model for persistent symptoms with novel insights into treatment resistance factors. [ABSTRACT FROM AUTHOR]

Objectives: Peroral endoscopic myotomy (POEM) is an effective treatment for esophageal motility disorders including achalasia and its variants. However, some surgeons have encountered challenging cases. This study aimed to develop a risk‐scoring system to predict challenging cases of POEM. Methods: Consecutive patients who underwent POEM between April 2015 and March 2020 at our hospital were included in this single‐center retrospective study. Challenging cases of POEM were defined as patients with any of the following: (i) procedure time ≥90 min; (ii) mucosal perforation; (iii) pneumothorax; and (iv) major bleeding. A risk‐scoring system for predicting challenging cases was developed based on multivariate logistic regression and internal validation was performed using the bootstrap method. Clinical usefulness was evaluated using a decision curve analysis. Results: Of the 467 patients, 59 (12.6%) had challenging POEM. A risk‐scoring system consisted of four variables: duration of symptoms ≥5 years (assigned score, 1 point), antithrombotics use (1 point), manometric diagnosis of achalasia variants (2 points), and dilation grade 3 (2 points). Our scoring system showed satisfactory discrimination (area under the receiver operating characteristic curve, 0.69; 95% confidence interval [CI] 0.61–0.77) and calibration (slope, 0.99; 95% CI 0.65–1.35). The decision curve analysis demonstrated its clinical usefulness. Conclusions: We established a risk‐scoring system to predict challenging cases of POEM. This scoring system may aid the selection of patients who require treatment from experienced surgeons. [ABSTRACT FROM AUTHOR]

Objectives: Indications for peroral endoscopic myotomy (POEM) and endoscopic submucosal dissection (ESD) in patients with achalasia concomitant with esophageal carcinoma (EC) are unclear. This study aimed to clarify the role of POEM in cases of achalasia concomitant with EC and to elucidate the indications for ESD and efficient surveillance for EC. Methods: We conducted a multicenter cohort study at 14 hospitals in Japan, including 3707 cases of achalasia‐related esophageal motility disorders (EMDs). Factors contributing to EC risk, the characteristics of EC, and clinical outcomes of POEM/ESD were analyzed. Results: In patients undergoing POEM, screening and surveillance endoscopy throughout a 1‐year period resulted in diagnosis of 72.1% new EC cases. Of 62 patients with 123 ECs, 40.3% had multiple or metachronous lesions within 37.5 months. EC was predominantly observed in the middle thoracic esophagus (58.5%) and posteriorly (73.2%). POEM had comparable safety and efficacy in cases of concomitant EC even after ESD. Endoscopic en bloc resection was performed in 95.8% and 89.3% of ECs diagnosed before and after POEM, respectively (P = 0.351); however, ESD on the POEM‐line was impaired by fibrosis. Multivariate analysis revealed risk factors for EC, including regular alcohol consumption, a history of smoking, advanced age, and extended disease duration. Alcohol intake and smoking had a synergistic effect on EC development. Conclusions: Screening and surveillance of POEM help in detecting EC. ESD is feasible in achalasia, although on the POEM‐line is challenging. Surveillance endoscopy for EC is recommended for cases with specific risks and a history of ECs. [ABSTRACT FROM AUTHOR]

Aim: To elucidate the impact of the coronavirus disease 2019 (COVID‐19) on the practice of high‐resolution manometry (HRM) and peroral endoscopic myotomy (POEM) in Japan. Methods: We utilized a large‐scale database involving 14 high‐volume centers in Japan to investigate changes in the numbers of HRM and POEM procedures performed and outcomes of POEM between 2019 and 2020. A questionnaire survey was also conducted to analyze pandemic‐associated changes in the HRM and POEM protocols. Results: Compared to that in 2019, the number of HRM and POEM procedures decreased by 17.2% (1587–1314) and 20.9% (630–498), respectively. These declines were prominent during the state of emergency from April to May 2020, particularly in pandemic areas. HRM and POEM in nonpandemic areas were relatively unaffected. From 2019 to 2020, there was a 0.4% (254–248) decrease in POEM cases within the prefecture, but the number outside the prefecture decreased by 33.6% (372–247). During the pandemic, the safety and efficacy of POEM were maintained. The implementation of personal protective equipment (PPE) measures varied among facilities, and PPE for POEM was relatively insufficient compared to that for HRM. Conclusion: The COVID‐19 pandemic influenced HRM and POEM practices in Japan. It is necessary to establish a sufficient system for HRM and POEM in each hospital as well as countrywide to overcome the effects of the pandemic. [ABSTRACT FROM AUTHOR]

Background and Aim: Achalasia is a rare disease, with an incidence of one in 100 000. Genetic factors and autoimmune involvement have been reported in its etiology, and their involvement is strongly suspected, especially in patients with familial achalasia and those with comorbid hereditary or autoimmune diseases. However, these special types of achalasia are rare, and their frequency and clinical characteristics remain unclear. Methods: This retrospective, multicenter cohort study included Japanese patients with a diagnosis of achalasia, treated between 2010 and 2019 across six tertiary centers in Japan. The frequency and clinical characteristics of special types of achalasia, namely, familial achalasia, achalasia with a comorbid hereditary disease, and achalasia with a comorbid autoimmune disease, were retrospectively investigated using a large‐scale multicenter database. Results: During the study period, 1115 patients were treated for achalasia at six tertiary centers. Familial achalasia, achalasia with a comorbid hereditary disease, and achalasia with a comorbid autoimmune disease occurred in 7 (0.63%), 11 (0.99%), and 27 (2.4%) patients, respectively. Familial achalasia had a slightly younger age of onset (37.6 ± 12.1 years old) and a higher incidence in male patients (six patients; 85.7%). Down's syndrome was the most common hereditary comorbidity, and thyroid disease was the most common autoimmune comorbidity. Conclusions: We clarified the frequency and clinical characteristics of special types of achalasia. Although special types of achalasia are rare, these comorbidities should be considered when treating patients with achalasia. [ABSTRACT FROM AUTHOR]

Achalasia is a rare benign esophageal motility disease caused by the impaired relaxation of the lower esophageal sphincter, which results from nerve damage. Patients with achalasia are known to have a high risk of esophageal cancer. Here, we present the case of a patient with achalasia and esophageal cancer in whom the Lugol‐voiding areas (LVAs) could be improved by pneumatic dilation and the extending area of esophagus cancer could become clear. In achalasia patients, LVAs are modified by inflammation and appear wider than their actual size. Moreover, some parts of LVAs in achalasia patients might be reversible by treatments that improve delayed emptying. When the spread of esophagus cancer is unclear due to the detection of numerous LVAs by Lugol chromoendoscopy, the treatments that improve delayed emptying first may be effective in accurately diagnosing the extending area of esophagus cancer. [ABSTRACT FROM AUTHOR]

Although treatment for esophageal motility disorder improves dysphagia and increases body weight, the effect of the treatment on body composition is unclear. This study aimed to assess the change in body composition between before and after treatment, the preoperative predictors of muscle increase, and the association between muscle increase and quality of life. Sixty-one patients (achalasia, n  = 55; spastic esophageal disorder n  = 6) who underwent per-oral endoscopic myotomy were analyzed in a single-arm prospective observational study. Appendicular skeletal muscle mass was measured with dual X-ray absorptiometry before and 3 months after per-oral endoscopic myotomy. For subgroup analysis, patients with postoperative appendicular skeletal muscle mass increase were defined as the muscle-increase group and the rest as the non-muscle-increase group. Preoperative factors related to the muscle-increase group were clarified via multivariate analysis. Further, the improvement after per-oral endoscopic myotomy in health-related quality-of-life score (Short Form-36) was compared between the muscle-increase and non-muscle-increase groups. Appendicular skeletal muscle mass increased significantly 3 months after per-oral endoscopic myotomy (P =  0.0002). The patients who underwent effective treatment (postoperative Eckardt score < 3) showed a significant improvement in appendicular skeletal muscle mass compared to those who did not (P =  0.04). In the stepwise logistic regression analysis, the preoperative Eckardt score (odds ratio: 1.95, 95% confidence interval 1.30–3.26, P =  0.0005) and preoperative serum prealbumin (odds ratio: 0.83, 95% confidence interval 0.70–0.97, P =  0.02) were identified as independent factors related to postoperative muscle increase. The improvements in the Short Form-36 domains of General Health (P =  0.0007) and Vitality (P =  0.003) were significantly higher in the muscle-increase group. The findings show that effective treatment improved the body composition of patients with esophageal motility disorder and that the Eckardt score and serum prealbumin may aid the prediction of increased appendicular skeletal muscle mass after treatment, resulting in a better quality of life. [ABSTRACT FROM AUTHOR]