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Objectives: Per-oral endoscopic myotomy (POEM) is a recommended treatment modality for achalasia, but there is little published data for its use in children. The objective of the present study was to evaluate whether POEM is clinically effective and safe for children., Methods: International multicenter retrospective study conducted in 14 tertiary centers that included consecutive children who underwent POEM between January 2012 and August 2018. Outcomes, such as clinical response were assessed whenever available. Adverse events and factors associated with clinical failure were also investigated., Results: A total of 117 patients (mean ± SD age: 14.2 ± 3.7 years) underwent POEM for achalasia (type I, n = 36; type II n=66; type III, n=8). Among these, 30 (26%) were pretreated (botulinum injection and/or pneumatic dilatation). Mean ± SD baseline Eckardt score was 7.5 ± 2.0. Clinical success was achieved in 90.6% of cases (95%CI [83.8%;95.2%]) in the intention-to-treat analysis. The mean ± SD Eckardt score post-POEM was 0.9 ± 1.2 (P < 0.001). The mean duration of follow-up time 545 days (range: 100-1612). A total of 7 adverse events occurred (4 mucosotomies, 2 subcutaneous emphysema, 1 esopleural fistula). Gastroesophageal reflux symptoms were seen in 17 patients (15%); missing data for 10 patients (9%). There was a trend towards more frequent clinical failure in achalasia associated with genetic disorders (40% vs 8%, P = 0.069)., Conclusions: POEM in pediatric patients appears to be effective and safe, although there was a trend towards more frequent clinical failure achalasia associated with genetic disorders. Further studies are needed to assess the long-term outcomes, especially the consequences of GERD.

Background and Aims: The optimal orientation of the myotomy during peroral endoscopic myotomy (POEM) is unknown. This meta-analysis aims to compare anterior and posterior myotomy regarding clinical success and safety., Methods: PubMed, Embase, Cochrane Library, WOK, and clinicaltrials.gov were searched to identify randomized clinical trials (RCTs) comparing anterior and posterior myotomy during POEM for the treatment of achalasia. The primary outcome was clinical success. Secondary outcomes included postprocedural gastroesophageal reflux disease (GERD), adverse events (AEs), manometric findings, and procedure-related parameters. Random-effects models were used for the primary analysis., Results: Four RCTs enrolling 488 patients were included. Overall clinical success 3-12 months after POEM was 97% (95% confidence interval [CI] 93-100%) and did not differ between anterior and posterior myotomy (Relative risk [RR] 0.98, 95%CI: 0.96-1.01; I2: 0%). Incidence of GERD after POEM based on 24-hour pH monitoring (RR 0.98, 95%CI: 0.75-1.28), endoscopy (RR 1.04, 95%CI: 0.78-1.38), and symptoms (RR 0.89, 95%CI: 0.55-1.42) was similar. Posterior myotomy was associated with fewer AEs (RR 0.63, 95%CI: 0.42-0.94), lower risk of mucosotomy (RR 0.42, 95%CI: 0.27-0.66) and shorter incision closure time (mean difference: -2.28 minutes, 95%CI: -3.46 to -1.10). Anterior myotomy was associated with a shorter length of hospitalization (mean difference: 0.31 days, 95%CI: 0.05-0.57), although the clinical relevance of this finding is negligible. No significant differences were found regarding manometric outcomes, total operation and myotomy time., Conclusions: Anterior and posterior myotomy are equally effective for the treatment of achalasia, without significant differences in postprocedural GERD. Posterior POEM was associated with fewer AEs and a shorter incision closure time.

Background and Study Aims: Detecting esophageal achalasia remains a challenge. We describe the diagnostic utility of corona appearance, a novel endoscopic finding specific to esophageal achalasia., Patients and Methods: Corona appearance and seven conventional endoscopic findings were compared for sensitivity and consistency (κ-value) among 53 untreated esophageal achalasia patients who underwent endoscopy at our hospital. The following criteria had to be met during lower esophageal sphincter examination using the attached ST Hood short-type for positive corona appearance: A) congestion inside the hood, B) ischemic change around the hood, and C) palisade vessels outside the hood., Results: Corona appearance had the highest sensitivity (91%; κ-value, 0.71). Other findings in descending order of sensitivity included 1) functional stenosis of the esophagogastric junction (EGJ; 86%; κ-value, 0.58), 2) mucosal thickening and whitish change (71%; κ-value, 0.27), 3) abnormal contraction of the esophageal body (59%; κ-value, 0.32), 4) dilation of the esophageal lumen (58%; κ-value, 0.53), 5) liquid remnant (57%; κ-value, 0.51), 6) Wrapping around EGJ (49%; κ-value, 0.14), and 7) food remnant (30%; κ-value, 0.88). Even in 22 patients with poor (grade 1) intraluminal expansion, corona appearance had highest sensitivity (88%) compared to other endoscopic findings (κ-value, 0.63)., Conclusions: Among endoscopic findings using a ST Hood short-type to diagnose esophageal achalasia, corona appearance had the highest sensitivity and its consistency (κ-value) among endoscopists was substantial compared to other endoscopic findings. Similar results were obtained for esophageal achalasia cases with poor expansion. Endoscopic diagnosis of esophageal achalasia with hood attached is useful., Competing Interests: The authors have declared that no competing interests exist.

Background & Aims: In patients with persistent symptoms after Heller myotomy (HM), treatment options include repeat HM, pneumatic dilation, or peroral endoscopic myotomy (POEM). We evaluated the efficacy and safety of POEM in patients with achalasia with prior HM vs without prior HM., Methods: We conducted a retrospective cohort study of 180 patients with achalasia who underwent POEM at 13 tertiary centers worldwide, from December 2009 through September 2015. Patients were divided into 2 groups: those with prior HM (HM group, exposure; n = 90) and those without prior HM (non-HM group; n = 90). Clinical response was defined by a decrease in Eckardt scores to 3 or less. Adverse events were graded according to criteria set by the American Society for Gastrointestinal Endoscopy. Technical success, clinical success, and rates of adverse events were compared between groups. Patients were followed up for a median of 8.5 months., Results: POEM was technically successful in 98% of patients in the HM group and in 100% of patients in the non-HM group (P = .49). A significantly lower proportion of patients in the HM group had a clinical response to POEM (81%) than in the non-HM group (94%; P = .01). There were no significant differences in rates of adverse events between the groups (8% in the HM group vs 13% in the non-HM group; P = .23). Symptomatic reflux and reflux esophagitis after POEM were comparable between groups., Conclusions: POEM is safe and effective for patients with achalasia who were not treated successfully by prior HM. Although the rate of clinical success in patients with prior HM is lower than in those without prior HM, the safety profile of POEM is comparable between groups., (Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Peroral endoscopic myotomy (POEM) is an established treatment for primary achalasia. It has gained endorsement from the American Society for Gastrointestinal Endoscopy with increasing clinical acceptance since the first procedure, performed in Japan in 2008. The first successful POEM in the UK was performed in November 2013 at King's College Hospital and this article presents the first UK case series. Prospective data were collected at 3 and 12-24 months for consecutive patients undergoing POEM. Post-POEM gastro-oesophageal reflux health-related quality of life scale (GORD-HRQoL) score was recorded. Statistical comparisons were made using paired non-parametric testing. In an initial series of 33 consecutive prospectively followed patients (12 female; 49.5±13 years; median follow-up 9 (3-28) months; 58% having had previous intervention), a 91% success rate has been achieved at 3 months. To date, 16 patients have reached the 12-month time point, with 13 (81%) sustaining response. This case series compares well with international cohorts and demonstrates excellent long-term safety and favourable efficacy., (© Royal College of Physicians 2017. All rights reserved.)

Background and Study Aims: Endoscopic diagnosis of esophageal achalasia lacking typical endoscopic features can be extremely difficult. The aim of this study was to identify simple and reliable early indicator of esophageal achalasia., Patients and Methods: This single-center retrospective study included 56 cases of esophageal achalasia without previous treatment. As a control, 60 non-achalasia subjects including reflux esophagitis and superficial esophageal cancer were also included in this study. Endoscopic findings were evaluated according to Descriptive Rules for Achalasia of the Esophagus as follows: (1) esophageal dilatation, (2) abnormal retention of liquid and/or food, (3) whitish change of the mucosal surface, (4) functional stenosis of the esophago-gastric junction, and (5) abnormal contraction. Additionally, the presence of the longitudinal superficial wrinkles of esophageal mucosa, "pinstripe pattern (PSP)" was evaluated endoscopically. Then, inter-observer diagnostic agreement was assessed for each finding., Results: The prevalence rates of the above-mentioned findings (1-5) were 41.1%, 41.1%, 16.1%, 94.6%, and 43.9%, respectively. PSP was observed in 60.7% of achalasia, while none of the control showed positivity for PSP. PSP was observed in 26 (62.5%) of 35 cases with shorter history < 10 years, which usually lacks typical findings such as severe esophageal dilation and tortuosity. Inter-observer agreement level was substantial for food/liquid remnant (k = 0.6861) and PSP (k = 0.6098), and was fair for abnormal contraction and white change. The accuracy, sensitivity, and specificity for achalasia were 83.8%, 64.7%, and 100%, respectively., Conclusion: "Pinstripe pattern" could be a reliable indicator for early discrimination of primary esophageal achalasia.

Background: Per oral endoscopic myotomy (POEM) has been reported to be a new therapeutic option for esophageal achalasia. The possibility that POEM could reduce the risk of developing esophageal squamous cell carcinoma was evaluated., Methods: This was a single-centre, retrospective study. Fifteen consecutive patients with esophageal achalasia who underwent POEM in our institution between August 2010 and January 2012 were enrolled. Ultra-high magnification with endocytoscopy was performed, and both histopathological and immunohistochemical evaluations for Ki-67 and p53 were assessed before and 3 months after POEM., Results: POEM was successfully performed and effectively released the dysphagia symptom in all patients without severe complications. Subjective symptoms (mean Ekcardt score, before 7.4 vs. after 0.5, p<0.05) and manometric pressure studies (mean lower esophageal sphincter pressure), before 82.7 vs. after 22.9 mmHg, p<0.05) showed substantial improvement following POEM. The average numbers of esophageal epithelial nuclei before and after POEM on endocytoscopic images were 128.0 and 78.0, respectively (p<0.05). The mean Ki-67-positive ratio was 26.0 (median 25.4, range, 10.3-33.2) before and 20.7 (median 20.0, 13.1-29.9; p=0.07) after POEM, and the mean p53-positive ratio was 2.35 (median 2.61, 0.32-4.23) before and 0.97 (median 1.49, 0.32-1.56; p<0.05) after POEM. A significant positive correlation was seen between the number of nuclei and the Ki-67-positive ratio (p<0.05)., Conclusions: POEM appears to be an effective and less invasive treatment of choice against achalasia and may reduce the risk of esophageal carcinogenesis. Endocytoscopy can be useful for the assessment of esophageal cellular proliferation.

Background/Objectives: Esophageal achalasia is an archetypal esophageal motility disorder characterized by abnormal peristalsis of the esophageal body and impaired lower esophageal sphincter (LES) relaxation. Methods: In this study, the mRNA expression of docking proteins 1 and 2 (DOK1 and DOK2, respectively) were analyzed and the mechanisms underlying achalasia onset were investigated. Results: DOK1 and DOK2 mRNA levels significantly increased in the LES of patients with achalasia. Moreover, significant correlations were observed between IL-1β and DOK1, IL-1β and DOK2, ATG16L1 and DOK1, and HSV1-miR-H1-3p and DOK2 expression levels. However, a correlation between ATG16L1 and DOK2 or between HSV-miR-H1-3p and DOK1 expression was not observed. In addition, a positive correlation was observed between patient age and DOK1 expression. Microarray analysis revealed a significant decrease in the expression of hsa-miR-377-3p and miR-376a-3p in the LES muscle of patients with achalasia. Conclusions: These miRNAs possessed sequences targeting DOK. The upregulation of DOK1 and DOK2 expression induces IL-1β expression in the LES of achalasia patients, which may contribute to the development of esophageal motility disorder. [ABSTRACT FROM AUTHOR]

Peroral endoscopic myotomy (POEM) has been developed in the context of natural orifice transluminal endoscopic surgery (NOTES) as a minimally invasive endoscopic treatment for symptomatic esophageal achalasia, which is a chronic progressive benign disease with severe morbidity and difficult management. Since September 2008, POEM has been successfully performed in more than 200 consecutive patients with symptomatic achalasia at the Digestive Disease Center of Showa University, Northern Yokohama Hospital, Yokohama, Japan, with excellent short- and long-term results and absence of serious complications. International experience of POEM within clinical studies is also promising. According to these results, POEM is considered as a safe procedure that can be applied to all achalasia patients. However, the low incidence of achalasia (0.3%-1% per 100,000 population), in combination with the potential serious complications related to the technically demanding POEM procedure, has made training difficult. There is therefore an urgent need for an animal model for training to decrease the learning curve. Further, there are other ethical and training issues to address. The pig is the most appropriate animal model for training in POEM due to its anatomy being similar to that of humans. The porcine esophagus has the advantage of easy mobilization due to absence of tight junctions to surrounding organs. A non-survival porcine model would be a simple, inexpensive, and reproducible animal model for training in POEM, without the need for concern about complications. A possible training process might first involve observation of POEM performed by specialists, then training on non-survival and survival porcine models, followed by training in humans under specialist guidance and finally, performance of POEM in humans. [ABSTRACT FROM AUTHOR]