Type II Achalasia Is Increasing in Prevalence.

BACKGROUND: Three manometric subtypes of achalasia were defined in the Chicago Classification approximately 10 years ago: type I (aperistalsis), type II (pan-pressurization), and type III (spastic). Since the widespread use of this classification scheme, the evolving prevalence of these subtypes has not been elucidated. We aim to determine the prevalence of each subtype a decade after the adoption of the Chicago Classification. METHODS: This is a retrospective cohort analysis of patients diagnosed with achalasia on high-resolution manometry (HRM) at two major academic medical centers between 2015 and 2018. Patients were excluded if they had a diagnosis of another esophageal motility disorder, previously treated achalasia, or foregut surgery. Demographic data, manometric subtype, and esophageal dilatation grade on endoscopy were obtained. Prevalence of achalasia subtypes was compared with a published historical control population (2004-2007). Fischer's exact and t tests were used for analysis. RESULTS: Of 147 patients in the contemporary cohort and 99 in the historical control cohort, the prevalence of type I achalasia was 8% versus 21%, type II 63% versus 50%, and type III 29% versus 29%, respectively (p = 0.01). The mean age in our population was 58 years compared to 57 years in the historical control, and the proportion of men 48% versus 47%, respectively (p = 0.78). Mean endoscopic dilatation grade in the contemporary cohort was 1.5 for type I patients, 0.9 for type II, and 0.4 for type III, compared with 1.5, 0.6, and 0.4, respectively. Overall mean dilatation grade was 0.8 in our cohort versus 0.7 in the historical control (p = 0.58). CONCLUSION: The prevalence of type II achalasia was significantly greater and prevalence of type I significantly less in our patient population compared to our predefined historical control. Other characteristics such as age and sex did not appear to contribute to these differences. Histopathological evidence has suggested that type II achalasia may be an earlier form of type I; thus, the increased prevalence of type II achalasia may be related to earlier detection of the disease. The adoption of HRM, widespread use of the Chicago Classification, and increased disease awareness in the past decade may be contributing to these changes in epidemiology.

The evaluation of the dilation-degree classification of esophageal achalasia patients from the viewpoint of esophageal clearance.

PURPOSE: To examine whether or not we could propose a more appropriate dilation-degree classification suitable for the pathological condition of patients with esophageal achalasia. METHODS: In accordance with the current dilation-degree classification, the maximum dilated diameter was measured based on the esophageal barium swallow. The relationship between the pathophysiology and dilation-degree classification was examined. Furthermore, the current dilatation-degree classification from the viewpoint of esophageal clearance was evaluated to examine whether or not a more appropriate dilatation-degree classification could be proposed. RESULTS: Because the clearance ratio tended to decrease at a maximum expansion diameter of 80 mm, when the maximum dilated diameter was divided into units of 10 mm, they were classified into two groups, with 80 mm as the boundary. As a result, the illness period was significantly prolonged (p = 0.0045) and the frequency of sigmoid type was high (p < 0.001) for lengths of ≥ 80 mm. With regard to the esophageal clearance rate, the clearance rate was significantly decreased in patients with a diameter of ≥ 80 mm at 5 min after taking barium (p = 0.0229). CONCLUSIONS: From the viewpoint of esophageal clearance, classification into 2 groups with a boundary of 80 mm may reflect the pathological condition.

Plasma levels of TNF-α, IL-6, IFN-γ, IL-12, IL-17, IL-22, and IL-23 in achalasia, eosinophilic esophagitis (EoE), and gastroesophageal reflux disease (GERD).

An elevation of serum inflammatory biomarkers in achalasia patients compared with controls recently was demonstrated. It has not been determined whether the elevation of inflammatory cytokines is unique to achalasia or occurs with other diseases involving the esophagus. The primary aim of our study was to compare the differences in plasma immunological profiles (TNF- α receptor, IL-6, IFN-γ, IL-12, IL-17, IL-22, and IL-23) of patients with achalasia, eosinophilic esophagitis (EoE), and gastroesophageal reflux disease (GERD). A secondary aim of this study was to classify these same plasma cytokine profiles in the three achalasia subtypes. METHODS: Plasma from 53 patients with achalasia, 22 with EoE, and 20 with GERD (symptoms plus esophagitis or + reflux study) were analyzed. EXCLUSION CRITERIA: malignancy, autoimmune condition, immunodeficiency disorder, and treatment with steroids/immune modulating drugs. Cytokine levels were assayed via multiplex enzyme-linked immunosorbent assay (ELISA). RESULTS: Our key finding revealed significant elevations in IL- 6 (p = 0.0158) in achalasia patients compared with EoE patients. Overall, plasma inflammatory biomarker patterns were not different in the three subtypes of achalasia. CONCLUSION: There were no differences between the cytokine levels of any of the measured biomarkers between the achalasia and GERD groups suggesting that luminal stasis does increase biomarker levels for any of the cytokines examined in our study. While these results are an early first step towards clarifying some aspects of the pathogenesis of achalasia, they bring about many more questions that require further investigation and expansion. Further investigation with a larger cohort and a broader panel of biomarkers is needed.

Superior clinical outcomes of peroral endoscopic myotomy compared with balloon dilation in all achalasia subtypes.

BACKGROUND AND AIM: Optimal treatment modalities for each of the three subtypes of achalasia are still under debate. Differences in prognosis and long-term outcomes between peroral endoscopic myotomy (POEM) and balloon dilation (BD) are also unclear. We aimed to compare the treatment outcomes of BD and POEM in each subtype of achalasia by using information from the manometry database of a tertiary referral center in Korea. METHODS: Data from 5207 esophageal manometry procedures performed between 1989 and 2016 were analyzed. The medical records and results of esophagography and esophagogastroduodenoscopy were also reviewed. RESULTS: We identified 264 patients (116 men and 148 women) with diagnosis of achalasia during the study period. POEM and BD were carried out on 64 and 177 patients, respectively. There was a significant difference in the time to relapse between the POEM group and the BD group (P = 0.002). At the 24-month follow-up, the clinical success rates of POEM and BD were 91.8% and 68.0%, respectively. The hazard ratio of symptom return was 6.54 for BD compared with POEM (95% confidence interval 2.12-20.22, P = 0.001). After a follow-up period of 24 months, the success rate of POEM was significantly higher than that of BD for all subtypes of achalasia. However, only that of types I and II was statistically significant (type 1: 92.0% vs 51.1%, P = 0.004; type 2: 92.3% vs 59.8%, P = 0.007; and type3: 91.7% vs 55.6%, P = 0.051). CONCLUSIONS: Peroral endoscopic myotomy was more effective than BD in providing mid-long-term remission in patients with all manometric subtypes of achalasia.

Effect of Peroral Endoscopic Myotomy vs Pneumatic Dilation on Symptom Severity and Treatment Outcomes Among Treatment-Naive Patients With Achalasia: A Randomized Clinical Trial.

Importance: Case series suggest favorable results of peroral endoscopic myotomy (POEM) for treatment of patients with achalasia. Data comparing POEM with pneumatic dilation, the standard treatment for patients with achalasia, are lacking. Objective: To compare the effects of POEM vs pneumatic dilation as initial treatment of treatment-naive patients with achalasia. Design, Setting, and Participants: This randomized multicenter clinical trial was conducted at 6 hospitals in the Netherlands, Germany, Italy, Hong Kong, and the United States. Adult patients with newly diagnosed achalasia and an Eckardt score greater than 3 who had not undergone previous treatment were included. The study was conducted between September 2012 and July 2015, the duration of follow-up was 2 years after the initial treatment, and the final date of follow-up was November 22, 2017. Interventions: Randomization to receive POEM (n = 67) or pneumatic dilation with a 30-mm and a 35-mm balloon (n = 66), with stratification according to hospital. Main Outcomes and Measures: The primary outcome was treatment success (defined as an Eckardt score ≤3 and the absence of severe complications or re-treatment) at the 2-year follow-up. A total of 14 secondary end points were examined among patients without treatment failure, including integrated relaxation pressure of the lower esophageal sphincter via high-resolution manometry, barium column height on timed barium esophagogram, and presence of reflux esophagitis. Results: Of the 133 randomized patients, 130 (mean age, 48.6 years; 73 [56%] men) underwent treatment (64 in the POEM group and 66 in the pneumatic dilation group) and 126 (95%) completed the study. The primary outcome of treatment success occurred in 58 of 63 patients (92%) in the POEM group vs 34 of 63 (54%) in the pneumatic dilation group, a difference of 38% ([95% CI, 22%-52%]; P < .001). Of the 14 prespecified secondary end points, no significant difference between groups was demonstrated in 10 end points. There was no significant between-group difference in median integrated relaxation pressure (9.9 mm Hg in the POEM group vs 12.6 mm Hg in the pneumatic dilation group; difference, 2.7 mm Hg [95% CI, -2.1 to 7.5]; P = .07) or median barium column height (2.3 cm in the POEM group vs 0 cm in the pneumatic dilation group; difference, 2.3 cm [95% CI, 1.0-3.6]; P = .05). Reflux esophagitis occurred more often in the POEM group than in the pneumatic dilation group (22 of 54 [41%] vs 2 of 29 [7%]; difference, 34% [95% CI, 12%-49%]; P = .002). Two serious adverse events, including 1 perforation, occurred after pneumatic dilation, while no serious adverse events occurred after POEM. Conclusions and Relevance: Among treatment-naive patients with achalasia, treatment with POEM compared with pneumatic dilation resulted in a significantly higher treatment success rate at 2 years. These findings support consideration of POEM as an initial treatment option for patients with achalasia. Trial Registration: Netherlands Trial Register number: NTR3593.

Experience in treatment of patients with esophageal achalasia.

OBJECTIVE: Introduction: Esophageal achalasia is a neuromuscular disease, which is characterized by persistent impaired reflex opening of esophageal sphincter. Disease incidence is 0.6-2.0 per 100 000 population with no tendency to decrease. The aim of the study was to improve the results of treatment in patients with achalasia of cardia by differentiated approach in the choice of therapy method. PATIENTS AND METHODS: Materials and methods:148 patients with esophageal achalasia aged 18 - 67 were studied between 2003 and 2018. The following degrees of the disease were determined (by Petrovskyi's classification): first - in 8, second - in 59, third - in 70, fourth - in 11 patients. Drug therapy was administered in achalasia of first degree, and cardiodilatation courses - in second and third degree of the disease. Indications for surgical treatment were: impossibility to perform cardiodilatation, S-shaped esophageal deformation (stage IV), complications associated with cardiodilatation, achalasia combined with other surgical pathology, recurrence of disease. RESULTS: Review: Cardiodilatation therapy resulted in sustained clinical effect in stages I and II of the disease. In case of unsatisfactory results of cardiodilatation patients underwent elective operations. 18 patients underwent Heller-Dor plastic surgery, 4 - Heller-Petrovskiy operation, 1 - Besley plastic repair, 2 - Nissen fundoplication. In early postoperative period no failure or perforation of the esophagus occurred. The following complications developed: seroma - in 3 patients, postoperative wound infection - in 1 case. In one patient (4%) the recurrence of disease was due to scar deformation of esophagogastric junction after Heller-Petrovskiy operation on the cardia. CONCLUSION: Conclusions: Cardiodilatation is an effective method of conservative therapy in stages I and II of the disease, while in stages III and IV it is warranted as preoperative preparation measure. We think Heller-Dor operation to be the best method of plastic repair in esophageal achalasia.

Validation of new Japanese classification system for esophageal achalasia.

BACKGROUND: The fourth edition of New Japanese classification system for esophageal achalasia was revised after a long interval of 30 years in 2012. In this new system, achalasia is morphologically classified into 3 types, based on its X-ray findings. However, the system has been limitedly used in Japan and has not been fully validated in terms of its predictive capability of postoperative outcomes. The purpose of this study was to clarify the validity of new Japanese classification system for esophageal achalasia, as an index of patient characteristics and as a predictor of operative and mid/long-term postoperative outcomes. PATIENTS AND METHODS: Fifty-nine cases of achalasia underwent laparoscopic Heller-Dor surgery between 2005 and 2018. We evaluated retrospectively patient characteristics, intraoperative findings, esophageal manometry, 24-h pH monitoring and postoperative course. RESULTS: There were 34 St and 25 Sg/aSg cases. Age of St group was lower than Sg group. Preoperative duration of disease of St group was shorter than Sg. There were no differences in the results of surgical outcomes and prognoses. CONCLUSION: The new Japanese classification system may give additional insight and information in understanding epidemiology of esophageal achalasia; however, our study failed to demonstrate "inter-disease type" differences in surgical outcomes and prognoses.

[Esophageal achalasia]. / Akhalaziia kardii.

Prevalence, clinical manifestations and of modern classifications of achalasia are reviewed in the article. Diagnosis and treatment of this pathology were analyzed by using of 58 references.

Outcomes of pneumatic dilation in achalasia: Extended follow-up of more than 25 years with a focus on manometric subtypes.

BACKGROUND AND AIM: Pneumatic dilation (PD) is the most popular nonsurgical treatment for achalasia. This study investigated predicting factors, including manometric subtypes for symptom recurrence in the long term, in patients with achalasia treated with a single PD. METHODS: Between 1983 and 2013, a total of 107 patients were treated initially with a single PD and included in this longitudinal cohort study. Outcomes were correlated with demographics, symptoms (Eckardt score), and esophagographic and manometric features. Manometric tracings were retrospectively classified according to the three subtypes of the Chicago classification. RESULTS: Ninety-one (85%) patients were successfully treated after the first PD. The median follow-up was 13.8 years (interquartile range 7-20). During follow-up, 54% of the patients experienced a clinical relapse. The overall cumulative success rates at 2, 5, 10, 15, 20, and 25 years were 64%, 53%, 49%, 42%, 36%, and 36%, respectively. Age < 40 years, lower esophageal sphincter pressure > 15 mmHg, a cardia width < 5 mm, and an esophageal barium column height > 1 cm 4 to 12 weeks post-dilation significantly correlated with symptom recurrence, whereas achalasia subtypes did not significantly correlate with the treatment results. CONCLUSION: Pneumatic dilation in achalasia is an effective therapy in the short term, but its effect wanes in the very long term. Young age at presentation, a high lower esophageal sphincter pressure, a narrow cardia, and an esophageal barium column of > 1 cm after PD are predictive factors for the need of repeated treatment.

The Chicago Classification 3.0 Results in More Normal Findings and Fewer Hypotensive Findings With No Difference in Other Diagnoses.

OBJECTIVES: High-resolution manometry (HRM) is the preferred method for the evaluation of motility disorders. Recently, an update of the diagnostic criteria (Chicago 3.0) has been published. The aim of this study was to compare the performance criteria of Chicago version 2.0 (CC2.0) vs. 3.0 (CC3.0) in a cohort of healthy volunteers and symptomatic patients. METHODS: HRM studies of asymptomatic and symptomatic individuals from several centers of Spain and Latin America were analyzed using both CC2.0 and CC3.0. The final diagnosis was grouped into hierarchical categories: obstruction (achalasia and gastro-esophageal junction obstruction), major disorders (distal esophageal spasm, absent peristalsis, and jackhammer), minor disorders (failed frequent peristalsis, weak peristalsis with small or large defects, ineffective esophageal motility, fragmented peristalsis, rapid contractile with normal latency and hypertensive peristalsis) and normal. The results were compared using McNemar's and Kappa tests. RESULTS: HRM was analyzed in 107 healthy volunteers (53.3% female; 18-69 years) and 400 symptomatic patients (58.5% female; 18-90 years). In healthy volunteers, using CC2.0 and CC3.0, obstructive disorders were diagnosed in 7.5% and 5.6%, respectively, major disorders in 1% and 2.8%, respectively, minor disorders in 25.2% and 15%, respectively, and normal in 66.4% and 76.6%, respectively. In symptomatic individuals, using CC2.0 and CC3.0, obstructive disorders were diagnosed in 11% and 11.3%, respectively, major disorders in 14% and 14%, respectively, minor disorders in 33.3% and 24.5%, respectively, and normal in 41.8% and 50.3%, respectively. In both groups of individuals, only an increase in normal and a decrease in minor findings using CC3.0 were statistically significant using McNemar's test. DISCUSSIONS: CC3.0 increases the number of normal studies when compared with CC2.0, essentially at the expense of fewer minor disorders, with no significant differences in major or obstructive disorders. As the relevance of minor disorders is questionable, our data suggest that CC3.0 increases the relevance of abnormal results.

Chicago Classification of Esophageal Motility Disorders: Lessons Learned.

PURPOSE OF REVIEW: High-resolution manometry (HRM) is increasingly performed worldwide, to study esophageal motility. The Chicago classification is subsequently applied to interpret the manometric findings and facilitate a diagnosis of esophageal motility disorders. This review will discuss new insights regarding the diagnosis and management using the Chicago classification. RECENT FINDINGS: Recent studies have demonstrated that high-resolution manometry is superior to conventional manometry, and has a higher sensitivity to diagnose achalasia. Furthermore, the subclassification of achalasia as used in the Chicago classification has prognostic value and can be used to direct treatment. Diagnosis of esophageal spasm has been improved by using the distal latency as diagnostic criterion. Recently, criteria for minor disorders of peristalsis have been sharpened, leading to a lower rate of patients with abnormal results, thereby increasing the relevance of a diagnosis. High-resolution manometry is now considered the gold standard for diagnosis of esophageal motility disorders. The Chicago classification provides a standardized approach for analysis and categorization of abnormalities that has led to a significant increase in our knowledge regarding the diagnosis and management of motility disorders. Further refinement of the classification will be required.

Diagnosis and Treatment Strategy of Achalasia Subtypes and Esophagogastric Junction Outflow Obstruction Based on High-Resolution Manometry.

BACKGROUND: Based on Chicago Classification version 3.0, the disorders of esophagogastric junction outflow obstruction (EGJOO) include achalasia (types I, II and III) and EGJOO. Although no curative treatments are currently available for the treatment of the disorders of EGJOO, medical treatments, endoscopic pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), and per-oral endoscopic myotomy (POEM) are usually the sought-after modes of treatment. Since the etiology and pathogenesis might vary depending on the types of EGJOO disorders, treatment strategies should be considered based on those subtypes. SUMMARY: Based on the accumulated evidences, the treatment strategies of our institution are as follows: effects of medical treatments on achalasia are limited. Either PD or LHM/POEM can be considered a first-line in achalasia type I, according to the patient's wish. PD and POEM can be considered first-line in achalasia types II and III, respectively. Conversely, In EGJOO, medical treatments including drugs like acotiamide and/or diltiazem can be tested as a first-line, and PD and POEM will be considered second and third-line treatments, respectively. Key Messages: The classification of subtypes based on high-resolution manometry will help us consider which treatment option can be selected as a first-line treatment depending upon the subtypes of disorders of EGJOO. Acotiamide has the potential to cure patients with EGJOO.

Ling classification applied in the preoperative safety and effectiveness assessment of POEM.

BACKGROUND AND STUDY AIMS: At present, peroral endoscopic myotomy (POEM) has been considered as the recommended treatment for achalasia, as it causes trauma to a smaller area and has fewer short-term serious complications than other treatments. However, due to the different morphology of esophagus, not all the patients with achalasia are eligible to receive POEM surgery. And the purpose of our study was to apply Ling classification, which proser Linghu put forward in 2011, in the preoperative assessment of POEM, expecting to reduce the occurrence rate of complications during or post-POEM. PATIENTS AND METHODS: The clinical data of 341 achalasia patients were collected and classified, according to the endoscopic pictures related to the middle and the lower parts of the esophagus. The correlation between Ling classification and LESP or Eckardt score was analyzed for the efficiency estimation, and the correlation of Ling classification and incidence of complications was counted for the safety evaluation. RESULTS: Ling classification was correlated with the LESP of 95 patients and Eckardt score of 131 patients. Compared to preoperative data, post-POEM LESP and Eckardt score both decreased significantly in all types of Ling classification (p < 0.05), while no significant difference in the decrease degree of either LESP or Eckardt score was found among different types of Ling classification (p > 0.05). This means Ling classification may not directly affect the postoperative efficacy of POEM. By analyzing the correlation between Ling classification and intraoperative complications, we found that the differences of total complications, gas-related complication and mucosal injury were all significant among all types of Ling classification (p < 0.05). CONCLUSIONS: Ling classification can be applied in the preoperative assessment of the safety and efficacy of POEM, and it might provide a guideline for how to reduce the complications during POEM surgery.

The relationship between manometric subtype and outcomes of surgical treatment for patients with achalasia : Achalasia: manometric subtypes.

BACKGROUND: The Chicago Classification describes three distinct subtypes of achalasia and it appears to be a promising tool in predicting results of treatment with standard Heller Myotomy. The aim of this study is to analyze the outcomes of surgical treatment for achalasia using an extended Heller myotomy for each subtype and to identify additional parameters that may predict the success of therapy. METHODS: 72 consecutive patients with achalasia were evaluated at the University of Washington between 2008 and 2013. Symptom duration, patient age, and the degree of esophageal dilation (stage 1-3) as assessed by radiography were determined. We defined treatment failure as no improvement in symptoms and/or need for a second therapy within 1 year. Long-term follow-up data of 25 patients were available in the form of a survey evaluating overall satisfaction with the operation. RESULTS: The distribution of patients according to subtype included 13 with type I, 54 with type II, and 5 with type III. All of the type I patients had some degree of esophageal dilation on radiography, whereas no dilation was found in the type III group. All patients underwent uneventful laparoscopic-extended Heller myotomy. Two patients were classified as failures, including one with type I and one with type II achalasia; however, further investigation revealed the cause of both failures to be the development of peptic stricture. Only one of the 25 patients with long-term follow-up reported dissatisfaction with the treatment result and indicated persistent chest pain without dysphagia. CONCLUSIONS: Laparoscopic-extended Heller myotomy is a highly successful treatment for patients with achalasia and outcomes do not appear to vary significantly according to the manometric subtype. Failures may result from reflux in patients who develop esophagitis or stricture. Chest pain is not always responsive to esophagogastric myotomy despite relief of dysphagia.

Impact of esophageal flexion level on the surgical outcome in patients with sigmoid esophageal achalasia.

PURPOSE: Esophageal achalasia can be roughly divided into non-sigmoid and sigmoid types. Laparoscopic surgery has been reported to be less than optimally effective for sigmoid type. The aim of this study was to examine the impact of the esophageal flexion level on the clinical condition and surgical outcomes of patients with sigmoid esophageal achalasia. METHODS: The subjects were 36 patients with sigmoid esophageal achalasia who had been observed for >1 year after surgery. The subjects were divided into sigmoid type (Sg) and advanced sigmoid type (aSg) groups based on the flexion level of the lower esophagus to compare their clinical parameters and surgical outcomes. RESULTS: The Sg and aSg groups included 26 (72%) and 10 subjects, respectively. There were no marked differences in the clinical parameters or surgical outcomes between the two groups. However, the clearance rate calculated using the timed barium esophagogram was lower in the aSg group than in the Sg group. No differences were found in the postoperative symptom scores between the two groups, and both reported a high level of satisfaction. CONCLUSIONS: Although laparoscopic surgery for symptoms of sigmoid esophageal achalasia was highly successful regardless of the flexion level, the improvement in esophageal clearance was lower when the flexion level was higher.

The Functional Lumen Imaging Probe Detects Esophageal Contractility Not Observed With Manometry in Patients With Achalasia.

BACKGROUND & AIMS: The functional lumen imaging probe (FLIP) could improve the characterization of achalasia subtypes by detecting nonocclusive esophageal contractions not observed with standard manometry. We aimed to evaluate esophageal contractions during volumetric distention in patients with achalasia using FLIP topography. METHODS: Fifty-one treatment-naive patients with achalasia, defined and subclassified by high-resolution esophageal pressure topography, and 10 asymptomatic individuals (controls) were evaluated with the FLIP during endoscopy. During stepwise distension, simultaneous intrabag pressures and 16 channels of cross-sectional areas were measured; data were exported to software that generated FLIP topography plots. Esophageal contractility was identified by noting periods of reduced luminal diameter. Esophageal contractions were characterized further by propagation direction, repetitiveness, and based on whether they were occluding or nonoccluding. RESULTS: Esophageal contractility was detected in all 10 controls: 8 of 10 had repetitive antegrade contractions and 9 of 10 had occluding contractions. Contractility was detected in 27% (4 of 15) of patients with type I achalasia and in 65% (18 of 26, including 9 with occluding contractions) of patients with type II achalasia. Contractility was detected in all 10 patients with type III achalasia; 8 of these patients had a pattern of contractility that was not observed in controls (repetitive retrograde contractions). CONCLUSIONS: Esophageal contractility not observed with manometry can be detected in patients with achalasia using FLIP topography. The presence and patterns of contractility detected with FLIP topography may represent variations in pathophysiology, such as mechanisms of panesophageal pressurization in patients with type II achalasia. These findings could have implications for additional subclassification to supplement prediction of the achalasia disease course.

New endoscopic classification of the cardiac orifice in esophageal achalasia: Champagne glass sign.

BACKGROUND AND AIM: Endoscopy, barium esophagram and manometry are used in the diagnosis of achalasia. In the case of early achalasia, characteristic endoscopic findings are difficult to recognize. As a result, the diagnosis of achalasia is often made several years after symptom onset. Therefore, we examined the endoscopic findings of the cardiac orifice in achalasia and propose a new classification. METHODS: A total of 400 patients with spastic esophageal motility disorders who underwent peroral endoscopic myotomy (POEM) at our hospital between March 2014 and August 2015 were screened for this study. Champagne glass sign (CG) was defined as when the distal end of the lower esophageal sphincter relaxation failure (LESRF) was proximal to the squamocolumnar junction (SCJ) and the SCJ was dilated in the retroflex view. Specifically, CG-1 was defined as a distance from the SCJ to the lower end of LESRF of <1 cm, and CG-2 was defined as a distance ≥1 cm. RESULTS: CG-0 was seen in 73 patients (28.0%), whereas the CG sign was seen in 186 patients (71.3%), of whom 170 (65.1%) were CG-1 and 16 (6.1%) were CG-2. CONCLUSIONS: The CG sign is often observed in esophageal achalasia patients. CG-0 (equal to Maki-tsuki) was observed in 28.0% of achalasia patients only. Its absence with dilated SCJ cannot be used to rule out achalasia. Barium esophagram and manometry should be done if esophageal achalasia is strongly suspected.

High resolution manometry sub-classification of Achalasia: does it really matter? Does Achalasia sub-classification matter?

BACKGROUND: Three variants of Achalasia have been described using high-resolution esophageal manometry (HRM). While manometrically distinct, their clinical significance has yet to be established. Our objective was to compare the outcome after myotomy in patients with these Achalasia subtypes. METHODS: A retrospective chart review was performed to identify patients with Achalasia who had HRM and who underwent Heller myotomy or Per oral endoscopic myotomy (POEM). Symptoms and esophageal clearance by timed barium study were compared before and after treatment. RESULTS: We identified 49 patients, 21 males and 28 females, with a median age of 52 years. The primary symptom in all patients was dysphagia, with a median duration of 4 years (range 4 months-50 years). By HRM, ten patients (20 %) were classified as Type I, 30 (61 %) as Type II, and 9 (18 %) as Type III. At a median follow-up of 16 months after myotomy (range 1-63 months), the median Eckardt score was zero and was similar across subtypes. Relief of dysphagia was also similar across subtypes (80 % of Type I, 93 % of Type II and 89 % of Type III). On pre-treatment timed barium study, no patient had complete emptying at 1 or 5 min. After myotomy, complete emptying occurred within 1 min in 50 % (20/40) and within 5 min in 60 % (24/40) and was similar across groups. CONCLUSION: Myotomy for Achalasia results in excellent symptomatic outcome and improvement in esophageal clearance. There was no difference among the described HRM Achalasia variants. This calls into question the clinical utility of Achalasia sub-classification and affirms the benefit of myotomy for this disease.

Diagnostic evaluation of achalasia: from the whalebone to the Chicago classification.

From the earliest description of dysphagia relieved by dilatation with a whalebone in 1674 we have witnessed the evolution of esophageal function testing from the conventional manometry to the high-resolution manometry (HRM) and esophageal topography pressure plotting that have led to the revised Chicago classification for esophageal motility disorders in 2014. The goals of this paper are, therefore, (1) to highlight the historical milestones that have led to the diagnostic definition of achalasia, as we know it today; (2) to describe the evaluation process of patients with suspected achalasia; (3) to describe the diagnostic value of the HRM and the usefulness of the Chicago classification in predicting treatment outcomes. The value of Chicago classification is linked to the ability of the clinician to perform a thorough clinical evaluation to identify and correlate specific clinical phenotypes to specific manometric subtypes and predict treatment outcomes. Chicago classification, however, cannot predict which treatment, pneumatic dilatation, or Heller myotomy, should be selected for those with a specific subtype of achalasia.

Inter-observer agreement for diagnostic classification of esophageal motility disorders defined in high-resolution manometry.

High-resolution esophageal manometry (HRM) is a recent development used in the evaluation of esophageal function. Our aim was to assess the inter-observer agreement for diagnosis of esophageal motility disorders using this technology. Practitioners registered on the HRM Working Group website were invited to review and classify (i) 147 individual water swallows and (ii) 40 diagnostic studies comprising 10 swallows using a drop-down menu that followed the Chicago Classification system. Data were presented using a standardized format with pressure contours without a summary of HRM metrics. The sequence of swallows was fixed for each user but randomized between users to avoid sequence bias. Participants were blinded to other entries. (i) Individual swallows were assessed by 18 practitioners (13 institutions). Consensus agreement (≤ 2/18 dissenters) was present for most cases of normal peristalsis and achalasia but not for cases of peristaltic dysmotility. (ii) Diagnostic studies were assessed by 36 practitioners (28 institutions). Overall inter-observer agreement was 'moderate' (kappa 0.51) being 'substantial' (kappa > 0.7) for achalasia type I/II and no lower than 'fair-moderate' (kappa >0.34) for any diagnosis. Overall agreement was somewhat higher among those that had performed >400 studies (n = 9; kappa 0.55) and 'substantial' among experts involved in development of the Chicago Classification system (n = 4; kappa 0.66). This prospective, randomized, and blinded study reports an acceptable level of inter-observer agreement for HRM diagnoses across the full spectrum of esophageal motility disorders for a large group of clinicians working in a range of medical institutions. Suboptimal agreement for diagnosis of peristaltic motility disorders highlights contribution of objective HRM metrics.