Hypercontractile esophagus responsive to potassium-competitive acid blockers: a case report.

BACKGROUND: Hypercontractile esophagus is a rare hypercontractile esophageal motility disorder. The etiology of hypercontractile esophagus is unknown but an association between acid reflux and hypercontractile esophagus has been suggested. We present the first report on the use of potassium-competitive acid blockers in the treatment of hypercontractile esophagus. CASE PRESENTATION: A 43-year-old man presented with dysphagia, chest pain and regurgitation for a period of 1 year. Initial workup showed a twisted lumen with abnormal contractions in the distal esophagus during upper gastrointestinal endoscopy and abnormal acid exposure under 24-h esophageal pH monitoring. The use of standard-dose proton pump inhibitors didn't relieve his symptoms. Subsequent high-resolution esophageal manometry made a diagnosis of hypercontractile esophagus. Treatment with vonoprazan resulted in symptomatic resolution and abnormal contractions were no longer detected on follow-up high-resolution manometry. CONCLUSIONS: Potassium-competitive acid blockers like vonoprazan offer an alternative therapeutic method for patients with hypercontractile esophagus who are refractory to proton pump inhibitor therapy. The use of potassium-competitive acid blockers in hypercontractile esophagus warrants further research and may provide evidence for an acid-related etiology of hypercontractile esophagus.

High-Pressure Tactics: Jackhammer Esophagus-Diagnosing Is Easier than Treating.

Diagnosis of esophageal disorders is well ahead of available treatment options. With HRM, for example, one can identify numerous conditions and their variants, which may lose meaning if the clinical and therapeutic implications of these subclassifications are limited. We report an exemplary case of a patient with hiatal hernia complaining of reflux, dysphagia, and chest pain refractory to medical treatment. Jackhammer esophagus was diagnosed and a hybrid approach consisting of POEM and concomitant crural repair and Dor fundoplication is proposed.

Characteristics of patients with esophageal motility disorders on high-resolution manometry and esophagography-a large database analysis in Japan.

BACKGROUND: With the development of high-resolution manometry (HRM) and peroral endoscopy, more patients with esophageal motility disorders (EMDs) including achalasia are diagnosed and treated. The characteristics of Japanese patients with EMDs are unknown and should be elucidated. METHODS: A large-scale database analysis was performed at seven high-volume centers in Japan. EMDs between 2010 and 2019 were analyzed. RESULTS: A total of 1900 patients were diagnosed with treatment naïve achalasia on esophagography. A long disease history was related to the sigmoid and dilated esophagus, and patients' symptom severity declined as achalasia progressed to the sigmoid type. Among 1700 patients received starlet HRM, 1476 (86.8%) completed the examination. Long disease history and sigmoid achalasia were identified as risk factors for the failure of HRM examination. Type I achalasia was the most common type found on starlet HRM, and 45.1% of patients with achalasia had lower esophageal sphincter (LES) pressure within the normal range. Type III had a high age of onset and mild symptom severity, compared to the other two subtypes. Type III achalasia, esophagogastric outflow obstruction (EGJ-OO), jackhammer esophagus (JE), and diffuse esophageal spasm (DES) were relatively rare compared to type I-II achalasia. The clinical characteristics of EGJ-OO, JE, and DES were generally close to those of achalasia. CONCLUSION: This first large-scale database analysis indicates that more Japanese patients with achalasia are type I and have a normal range of LES pressure on starlet HRM. Failure of HRM is not rare; therefore, esophagography continuously has a complementary role in achalasia diagnosis.

Manometrically jackhammer esophagus with fluoroscopically/endoscopically distal esophageal spasm: a case report.

BACKGROUND: Jackhammer esophagus is a rare esophageal motility disorder that can result in dysphagia, chest pain, and gastro-esophageal reflux symptoms. High-resolution manometry is the gold standard for diagnosis, while corkscrew esophagus on upper gastrointestinal endoscopy is an uncommon manifestation. CASE PRESENTATION: 72-year-old man who presented with progressive dysphagia for three months without symptoms of chest pain or heartburn. Initial workup showed a corkscrew esophagus on upper gastrointestinal endoscopy; subsequently, high-resolution manometry revealed an esophago-gastric junction outflow obstruction with hypercontractile (jackhammer) esophagus. Treatment with calcium channel blockers and proton pump inhibitors was successful and relieved his symptoms near completion. CONCLUSIONS: Even though the corkscrew esophagus is typically for distal esophageal spasm, the hypercontractile (jackhammer) esophagus can appear. The high-resolution manometry can help to distinguish each specific motility disorder.

Differences in clinical characteristics between conservative-treatment-response group and refractory (surgical-treatment) group in patients with jackhammer esophagus.

BACKGROUND: Jackhammer esophagus (JE) is a rare disease with unclear clinical features. The objective of this study was to retrospectively compare the clinical characteristics of patients with JE whose symptoms were controlled with conservative treatment, such as observation or medication, versus those who were required surgical treatment, such as surgical myotomy or per oral endoscopic myotomy. METHODS: Eighteen patients with JE were included in this study. The patients were divided into two groups: patients who responded to conservative treatment (C group) and patients who were refractory to conservative treatment and underwent surgery (S group). Patient age, sex, disease duration before treatment, symptoms, esophagogastroduodenoscopic (EGD) findings, esophagographic findings, esophageal wall thickness on computed tomography, number of swallows with hypercontractile peristalsis in 10 water swallows, and maximum distal contractile integral (DCI) were compared between the groups. RESULTS: Thirteen of 18 patients (72%) were in the C group and five of 18 (28%) were in the S group. There were no significant differences in age, sex, disease duration before treatment, symptoms, EGD findings, esophagographic findings, esophageal wall thickness, or number of swallows with hypercontractile peristalsis between the groups. On Starlet high-resolution manometry, the median maximum DCI value was significantly higher in the S group (32,651 mmHg-s-cm) than in the C group (17,926 mmHg-s-cm) (P = 0.0136). CONCLUSIONS: JE treatment should be carefully considered because some patients require surgery, whereas others are controlled with conservative treatment alone. A higher DCI value in patients with JE may predict resistance to conservative treatment.

Clinical Symptom Presentation of Hypercontractile Peristalsis in the Era of High-Resolution Manometry: A Single-Center Experience.

BACKGROUND: Hypercontractile motility of the esophagus is occasionally noted on high-resolution manometry (HRM), but its clinical correlations are unclear. We compared symptom severity and clinical presentation of patients with hypercontractile motility of the esophagus. METHODS: This was a retrospective cohort study. We queried a prospectively maintained database for patients who underwent esophageal function testing from October 1, 2016, to October 30, 2018. We included patients with jackhammer esophagus (JE; ≥2 swallows with distal contractile integral [DCI] ≥8,000 mm Hg∙cm∙s), nutcracker esophagus (NE; mean DCI 5,000-8,000 mm Hg∙cm∙s without meeting JE criteria), or esophagogastric junction outflow obstruction ([EGJOO]: abnormal median integrated relaxation pressure (>15 mm Hg) without meeting achalasia criteria, with JE [EGJOO-h], or normal motility [EGJOO-n]). HRM, endoscopy, barium esophagram, ambulatory pH studies, and symptom questionnaires were reevaluated for further analysis. Clinical parameters were analyzed using Spearman Rho correlation. Categorical variables were assessed with Fisher exact or chi-square test. RESULTS: Altogether, 85 patients met inclusion criteria. They were divided into 4 subgroups: 28 with JE, 18 with NE, 15 with EGJOO-h, and 24 with EGJOO-n. Patients with EGJOO-h were the most symptomatic overall. No correlation was seen between symptoms and mean DCI (p ≥ 0.05 all groups) or number of hypercontractile swallows (≥8,000 mm Hg∙cm∙s, p ≥ 0.05). A significant correlation was noted between dysphagia and lower esophageal sphincter pressure (LESP) and LESP integral (p ≤ 0.05). CONCLUSION: The number of hypercontractile swallows and mean DCI were not associated with patient-reported symptoms. Elevated LESP may be a more relevant contributor to dysphagia.

Provocative testing in patients with jackhammer esophagus: evidence for altered neural control.

Jackhammer esophagus (JE) is a hypercontractile disorder, the pathogenesis of which is incompletely understood. Multiple rapid swallows (MRS) and rapid drink challenge (RDC) are complementary tests used during high-resolution manometry (HRM) that evaluate inhibitory and excitatory neuromuscular function and latent obstruction, respectively. Our aim was to evaluate esophageal pathophysiology using MRS and RDC in 83 JE patients (28 men; median age: 63 yr; IQR: 54-70 yr). Twenty-one healthy subjects (11 men; median age: 28 yr; range: 26-30 yr) were used as a control group. All patients underwent solid-state HRM with ten 5-ml single swallows (SS) and one to three 10-ml MRS; 34 patients also underwent RDC. Data are shown as median (interquartile range). Abnormal motor inhibition was noted during at least one MRS test in 48% of JE patients compared with 29% of controls ( P = 0.29). Mean distal contractile integral (DCI) after MRS was significantly lower than after SS [6,028 (3,678-9,267) mmHg·cm·s vs. 7,514 (6,238-9,197) mmHg·cm·s, P = 0.02], as was highest DCI ( P < 0.0001). Consequently, 66% of JE patients had no contraction reserve. At least one variable of obstruction during RDC (performed in 34 patients) was outside the normal range in 25 (74%) of JE patients. Both highest DCI after SS and pressure gradient across the esophagogastric junction (EGJ) during RDC were higher in patients with dysphagia versus those without ( P = 0.04 and 0.01, respectively). Our data suggest altered neural control in JE patients with heterogeneity in inhibitory function. Furthermore, some patients had latent EGJ obstruction during RDC, which correlated with the presence of dysphagia. NEW & NOTEWORTHY Presence of abnormal inhibition was observed during multiple rapid swallows (MRS) in some but not all patients with jackhammer esophagus (JE). Unlike healthy subjects, JE patients were more strongly stimulated after single swallows than after MRS. An obstructive pattern was frequently observed during rapid drink challenge (RDC) and was related to presence of dysphagia. MRS and RDC during high-resolution manometry are useful to show individual pathophysiological patterns in JE and may guide optimal therapeutic strategies.

Is POEM the Answer for Management of Spastic Esophageal Disorders? A Systematic Review and Meta-Analysis.

BACKGROUND AND AIMS: Spastic esophageal disorders (SEDs) include spastic achalasia (type III), diffuse esophageal spasm (DES), and nutcracker/jackhammer esophagus (JH). Per-oral endoscopic myotomy (POEM) has demonstrated efficacy and safety in the treatment of achalasia. Recently, POEM has been indicated for the treatment of SEDs. We conducted a systematic review and meta-analysis to determine the clinical success and safety of POEM in SEDs. METHODS: We searched several databases from 01/01/2007 to 01/10/2016 to identify studies (with five or more patients) on POEM for the treatment of SEDs. Weighted pooled rates (WPRs) for clinical success and adverse events (AEs) were calculated for all SEDs. Clinical success was defined as Eckardt scores of ≤3 and/or improvement in severity of dysphagia based on achalasia disease-specific health-related quality of life questionnaire. The WPRs for clinical success and AEs were analyzed using fixed- or random-effects model based on heterogeneity. The proportionate difference in clinical success and post-procedure adverse event rates among individual types of SEDs was also calculated. RESULTS: A total of eight observational studies with 179 patients were included in the final analysis. Two studies were of good quality and six were of fair quality based on the National Institutes of Health quality assessment tool. The WPR with 95% confidence interval (CI) for cumulative clinical success of POEM in all SEDs was 87% (78, 93%), I 2 = 37%. The total number of patients for individual disorders, i.e., type III achalasia, JH, and DES, was 116, 37, and 18, respectively. The WPRs for clinical success of POEM for type III achalasia, DES, and JH were 92, 88, and 72%, respectively. Proportion difference of WPR for clinical success was significantly higher for type III achalasia in comparison with JH (20%, P = 0.01). The WPR with 95% CI for AEs of POEM in all SEDs was 14% (9, 20%), I 2 = 0%. The WPRs for post-procedure adverse events for type III achalasia, DES, and JH were 11, 14, and 16%, respectively. There was no difference in safety of POEM among individual SEDs. CONCLUSION: POEM is an effective and safe therapeutic modality for the treatment of spastic esophageal disorders.

The clinical significance of hypercontractile peristalsis: comparison of high-resolution manometric features, demographics, symptom presentation, and response to therapy in patients with Jackhammer esophagus versus Nutcracker esophagus.

The Chicago Classification version 3.0 (CC v 3.0) defines hypercontractile peristalsis as Jackhammer esophagus (JE); Nutcracker esophagus (NE) is no longer recognized. Data regarding patient characteristics and treatment response for JE versus NE are limited. We aimed to compare demographic characteristics, high resolution manometry (HRM) features, clinical presentation, management strategies, and treatment outcomes in patients with JE versus NE. We performed a retrospective analysis of adult patients diagnosed with NE (CC v 2.0) or JE (CC v 3.0) by HRM from January 2012 to August 2015. Demographics, symptoms, treatments, and response to therapy (none or partial/complete) were ascertained by chart review, for statistical comparisons. In 45 patients with JE and 29 with NE, there was no significant difference in rate of dysphagia (73% and 59%) or chest pain (44% and 59%). Treatment data were available in 29 JE (smooth muscle relaxants in 4, pain modulators in 3, botulinum toxin injection (BTX) in 10, endoscopic dilation in 5, multimodal treatment in 7), and 20 NE patients (smooth muscle relaxants in 2, pain modulators in 2, (BTX) in 6, endoscopic dilation in 3, multimodal treatment in 7). Follow-up data on 26/29 JE and 20/20 NE patients showed similar treatment response (96.4% vs. 82.1%, p= 0.08) after mean follow-up of 11.2 and 11 months, respectively. There were no major differences for JE versus NE in demographics, symptoms, or type of and response to therapy. Larger prospective, controlled trials are needed to clarify the clinical significance and response to treatment in JE and NE.

An innovative fast track solution for food bolus impaction due to Jackhammer esophagus in an emergency department: the "Nitro-Push Blind Technique" case report.

BACKGROUND: In the medical literature are described only few clinical cases of esophageal food bolus impaction due to esophageal motility disorders. Moreover, the management of this condition is highly variable with no evidence in the literature to strongly support a clear defined intervention. CASE PRESENTATION: In this paper we describe for the first time a case of 53-year-old male with food bolus impaction due to Jackhammer esophagus referred to emergency department. On the basis of the known esophageal past medical history as well as the absence of bones in the bolus, the patient was submitted to a new conservative treatment, the "Nitro-Push Blind Technique". CONCLUSIONS: The new technique performed with naso-gastric tube thrust after nitrates medication in definite clinical case supported by known functional disease, represents a safe and successful method, with short observational period to minimize exposure to potential morbidity and reduce the inpatient stay in emergency department. It should be recommended, once validated in a larger cohort, as the initial treatment of choice in the selected patients with food boneless bolus impaction in the emergency settings. Indeed, this management provides only minimal deviation from the current practice and is hence technically easy to learn and perform.

Jackhammer esophagus: high-resolution manometry and therapeutic approach using peroral endoscopic myotomy (POEM).

We present the first report on peroral endoscopic myotomy (POEM) in the treatment of jackhammer esophagus. A 34-year-old female patient was newly diagnosed with a jackhammer esophagus. After failure of medical treatment, the patient underwent POEM procedure for myotomy of the spastic segment. Postoperatively, a mild emphysema and pneumothorax occurred that required drainage and antibiotic therapy until full recovery. Discharge was possible after 5 days. Six months later, she presented with recurrent but mild pain due to a remnant spastic segment proximal to the myotomy. Endoscopic balloon dilation was performed twice within 6 weeks with full symptomatic relief of pain and mild symptoms of dysphagia.

Botulinum toxin injection for hypercontractile or spastic esophageal motility disorders: may high-resolution manometry help to select cases?

Endoscopic injections of botulinum toxin in the cardia or distal esophagus have been advocated to treat achalasia and spastic esophageal motility disorders. We conducted a retrospective study to evaluate whether manometric diagnosis using the Chicago classification in high-resolution manometry (HRM) would be predictive of the clinical response. Charts of patients with spastic and hypertensive motility disorders diagnosed with HRM and treated with botulinum toxin were retrospectively reviewed at two centers. HRM recordings were systematically reanalyzed, and a patient's phone survey was conducted. Forty-five patients treated between 2008 and 2013 were included. Most patients had achalasia type 3 (22 cases). Other diagnoses were jackhammer esophagus (8 cases), distal esophageal spasm (7 cases), esophagogastric junction outflow obstruction (5 cases), nutcracker esophagus (1 case), and 2 unclassified cases. Botulinum toxin injections were performed into the cardia only in 9 cases, into the wall of the distal esophagus in 19 cases, and in both locations (cardia and distal esophagus) in 17 cases. No complication occurred in 31 cases. Chest pain was noticed for less than 7 days in 13 cases. One death related to mediastinitis occurred 3 weeks after botulinum toxin injection. Efficacy was assessed in 42 patients: 71% were significantly improved 2 months after botulinum toxin, and 57% remained satisfied for more than 6 months. No clear difference was observed in terms of response according to manometric diagnosis; however, type 3 achalasia previously dilated and with normal integrated relaxation pressure (4s-integrated relaxation pressure < 15 mmHg) had the worst outcome: none of these patients responded to the endoscopic injection of botulinum toxin. Endoscopic injections of botulinum toxin may be effective in some patients with spastic or hypercontractile esophageal motility disorders. The manometric Chicago classification diagnosis does not seem to predict the results. Prospective randomized trials are required to identify patients most likely to benefit from esophageal botulinum toxin treatment.

Peroral endoscopic myotomy for hypercontractile (Jackhammer) esophagus: A retrospective multicenter series with long-term follow-up.

INTRODUCTION: Long-term outcome data are limited for non-achalasia esophageal motility disorders treated by peroral endoscopy myotomy (POEM) as a separate group. We investigated a subset of symptomatic patients with hypercontractile esophagus (Jackhammer esophagus). METHODS: Forty two patients (mean age 60.9 years; 57% female, mean Eckardt score 6.2 ± 2.1) treated by primary peroral myotomy for symptomatic Jackhammer esophagus 2012-2018 in seven European centers were retrospectively analyzed; myotomy included the lower esophageal sphincter but did not extend more than 1 cm into the cardia in contrast to POEM for achalasia. Manometry data were re-reviewed by an independent expert. The main outcome was the failure rate defined by retreatment or an Eckardt score >3 after at least two years following POEM. RESULTS: Despite 100% technical success (mean intervention time 107 ± 48.9 min, mean myotomy length 16.2 ± 3.7 cm), the 2-year success rate was 64.3% in the entire group. In a subgroup analysis, POEM failure rates were significantly different between Jackhammer-patients without (n = 22), and with esophagogastric junction outflow obstruction (EGJOO, n = 20) (13.6% % vs. 60%, p = 0.003) at a follow-up of 46.5 ± 19.0 months. Adverse events occurred in nine cases (21.4%). 14 (33.3%) patients were retreated, two with surgical fundoplication due to reflux. Including retreatments, an improvement in symptom severity was found in 33 (78.6%) at the end of follow-up (Eckardt score ≤3, mean Eckardt change 4.34, p < 0.001). EGJOO (p = 0.01) and frequency of hypercontractile swallows (p = 0.02) were predictors of POEM failure. The development of a pseudodiverticulum was observed in four cases within the subgroup of EGJOO. CONCLUSIONS: Patients with symptomatic Jackhammer without EGJOO benefit from POEM in long-term follow-up. Treatment of Jackhammer with EGJOO, however, remains challenging and probably requires full sphincter myotomy and future studies which should address the pathogenesis of this variant and alternative strategies.

Botulinum toxin reduces Dysphagia in patients with nonachalasia primary esophageal motility disorders.

BACKGROUND & AIMS: Endoscopic injection of botulinum toxin (BTX) has shown benefits for patients with diffuse esophageal spasm (DES) and nutcracker esophagus (NE) in small uncontrolled trials. We investigated the effect of BTX on symptoms of patients with DES or NE and assessed manometry findings in a prospective, double-blind, randomized, controlled study. METHODS: We assessed 22 patients with dysphagia-predominant, manometry-confirmed DES or NE (6 men; age, 63 ± 2 y) at a tertiary care medical center. Patients were given injections of BTX (8 × 12.5 U) or saline (8 × 0.5 mL) in 4 quadrants, at 2 and 7 cm above the esophagogastric junction. After 1 month, patients crossed over between groups and received endoscopic injections of BTX or saline. When the study began and 4 weeks after each injection, the patients were assessed by esophageal manometry and completed a symptom questionnaire (to determine solid and liquid dysphagia, chest pain, and regurgitation and heartburn; all scored 0-4). Responders were defined based on modified Vantrappen criteria for achalasia. RESULTS: After BTX injections, patients had significant decreases in total symptom scores (sum of solid and liquid dysphagia and chest pain; from 7.6 ± 0.7 to 4.8 ± 0.8; P = .01); this decrease was not observed in patients who received saline injections. Moreover, BTX injection stabilized unintentional weight loss (weight gain of 0.3 ± 0.3 after BTX injection vs further weight loss of 1.6 ± 0.5 kg after saline injection; P = .01). Fifty percent of patients had a response 1 month after BTX injection, compared with 10% after saline injection (P = .04); 30% still had a response 1 year after BTX injection. BTX injection also caused a significant decrease in the mean esophagogastric junction pressure, compared with baseline (15.8 ± 1.7 vs 24.0 ± 2.8 mm Hg; P = .02). CONCLUSIONS: In a prospective controlled study of patients with DES and NE, injections of BTX reduced symptoms and stabilized unintentional weight loss. TRIAL REGISTRY: http://www.targid.eu, ML2669, ML6294.

Major mixed motility disorders: An important subset of esophagogastric junction outflow obstruction.

BACKGROUND: Esophagogastric junction outflow obstruction (EGJOO) is an esophageal motility disorder characterized by a lack of relaxation of the esophagogastric junction (EGJ), with preserved esophageal body peristalsis. We propose new terminology for the coexistence of EGJOO with hypercontractile esophagus and distal esophageal spasm as a major mixed motility disorder (MMMD), and normal peristalsis or a minor disorder of peristalsis such as ineffective esophageal motility with EGJOO as isolated or ineffective EGJOO (IEGJOO). METHODS: We reviewed prior diagnoses of EGJOO, stratified diagnoses as IEGJOO or MMMD, and compared their symptomatic presentations, high-resolution manometry (HRM) and endoluminal functional lumen imaging probe (EndoFLIP) metrics, and treatment responses at 2-6 months of follow-up. RESULTS: Out of a total of 821 patients, 142 met CCv3 criteria for EGJOO. Twenty-two were confirmed by CCv4 and EndoFLIP as having EGJOO and were clinically managed. Thirteen had MMMD, and nine had IEGJOO. Groups had no difference in demographic data or presenting symptoms by Eckardt score (ES). HRM showed MMMD had greater distal contractile integral, frequency of hypercontractile swallows, and frequency of spastic swallows, and greater DI by EndoFLIP. Patients with MMMD showed greater reduction in symptoms after LES-directed intervention when measured by ES compared with IEGJOO (7.2 vs. 4.0). CONCLUSION: Patients with MMMD and IEGJOO present similarly. Detectable differences in HRM portend different responses to endoscopic therapy. Because patients with MMMD have greater short-term prognosis, they should be considered a different diagnostic classification to guide therapy.

Esophageal retention cyst: Esophagogastric junction outflow obstruction (EGJOO) as a potential etiology and management with endoscopic mucosal resection (EMR).

INTRODUCTION AND IMPORTANCE: Esophageal retention cysts are acquired cysts with no known etiology. They are characterized by dilation of the submucosal glands. Symptomatic cysts are traditionally managed by surgical resection. CASE PRESENTATION: We present a case of progressive dysphagia and chest pain secondary to esophageal retention cysts in the mid and distal esophagus with associated esophagogastric junction outflow obstruction (EGJOO) and jackhammer esophagus on high resolution manometry (HRM). The patient underwent staged endoscopic mucosal resection (EMR) with subsequent improvement in her symptoms. However, EGJOO persisted after resection, suggesting it was the primary pathology and not a consequence of the obstruction from the cysts. CLINICAL DISCUSSION: Esophageal retention cysts are rarely reported in the literature with most descriptions coming from incidental post-mortems. The presented case suggests EGJOO as a potential etiology of retention cysts. The proposed mechanism is that a significant rise in esophageal intraluminal pressure creates a state of stasis in the esophagus, ideal for the development of these cysts. Symptomatic or malignant retention cysts should be resected. We demonstrate the feasibility of EMR as an alternative to surgical resection. CONCLUSION: Esophageal retention cyst is a rare entity, which may arise as a result of EGJOO. The natural history and malignant potential of these cysts are unknown, and no formal guidelines have been established for follow-up for patients with asymptomatic retention cysts. Endoscopic mucosal resection can be used to successfully manage these cysts.

Chicago Classification Update (v4.0): Technical review on diagnostic criteria for hypercontractile esophagus.

Hypercontractile esophagus (HE), defined by the Chicago Classification version 3.0 (CCv3.0) as 20% or more hypercontractile peristalsis (Distal Contractile Integral >8000 mmHg·s·cm) on high-resolution manometry (HRM), is a heterogeneous disorder with variable clinical presentations and natural course, leading to management challenges. An update on the diagnostic criteria for clinically relevant HE was needed. Literature on HE was extensively reviewed by the HE subgroup of the Chicago Classification version 4.0 (CCv4.0) Working Group and statements relating to the diagnosis of HE were ranked according to the RAND UCLA Appropriateness methodology by the Working Group, and the quality of evidence was rated using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) framework. An overall emphasis of the CCv4.0 is on clinically relevant esophageal dysmotility, and thus it is recommended that an HE diagnosis requires both conclusive manometric diagnosis and clinically relevant symptoms of dysphagia and non-cardiac chest pain. The Working Group also recognized the subtypes of HE, including single-peaked, multi-peaked contractions (Jackhammer esophagus), and hypercontractile lower esophageal sphincter. However, there are no compelling data currently for formally subdividing HE to these subgroups in clinical practice.

Efficacy and Safety of Peroral Endoscopic Myotomy for Esophageal Achalasia and Achalasia-Related Diseases in Patients Aged 75 Years and Over.

Peroral endoscopic myotomy (POEM) has become a popular treatment for esophageal achalasia and other esophageal motility disorders. However, its efficacy and safety in elderly patients are unclear. To clarify that, we reviewed the medical records of patients who underwent POEM in our hospital. A total of 11 patients who underwent POEM for esophageal achalasia (n = 10) and jackhammer esophagus (n = 1) were included. Procedural success, defined as the completion of an esophageal and gastric myotomy, was 100%. Clinical success, defined as an Eckardt score of 3 or less, without the use of additional treatments at 2 months, was 100%. The median Eckardt score significantly decreased after the POEM (baseline vs. 2 months after POEM; 7 (2-8) vs. 0 (0-1), p < 0.01). In the second and third years, the cumulative treatment effect maintenance rate was 88.9%. All patients taking antithrombotic agents had safe operations with the temporary discontinuation of these agents. There were four adverse events (two pneumoperitoneum, one mucosal injury, and one pneumonia), all of which improved with fasting or antibiotics. In conclusion, POEM is an effective and safe treatment for esophageal achalasia and achalasia-related diseases in patients aged 75 years and over.

Esophageal hypercontractility is abolished by cholinergic blockade.

BACKGROUND: Esophageal hypercontractility (EHC) is considered a major esophageal motor disorder of unclear etiology. Different mechanisms have been proposed, including an imbalance in inhibitory and excitatory esophageal innervation. We hypothesized that patients with EHC suffer from cholinergic hyperactivity. AIM: To interrogate the excitatory and inhibitory neurotransmission in EHC by assessing the esophageal motor response to atropine (ATR) and cholecystokinin (CCK), respectively, in EHC patients. METHOD: We retrospectively reviewed patients who underwent high-resolution manometry (HRM) with pharmacologic challenge in a tertiary referral center between 2007 and 2017. We identified 49 EHC patients who were categorized based on frequency of hypercontractile peristaltic sequence into "frequent" and "infrequent" and motility diagnosis groups. Deglutitive pressure metrics and esophageal motor responses to ATR (12 mcg/kg iv) and CCK (40 ng/kg iv) were analyzed across groups. RESULTS: Atropine abolished hypercontractility across all groups studied, converting nearly half of patients to a motor pattern of ineffective esophageal motility. Abnormal CCK responses primarily occurred in the patient groups with concomitant outflow obstruction. CONCLUSIONS: Hypercontractility is cholinergically mediated in all esophageal motor disorders. Most patients with isolated EHC appear to have excessive cholinergic drive, rather than loss of inhibitory innervation, and might be candidates for treatment with anticholinergic agents.

Chicago Classification update (version 4.0): Technical review on diagnostic criteria for achalasia.

The recommended diagnostic criteria for achalasia have been recently updated by Chicago Classification version 4.0 (CCv4.0), the widely accepted classification scheme for esophageal motility disorders using metrics from high-resolution manometry (HRM). CCv4.0 continued upon prior versions by subtyping achalasia into type I, type II, and type III on HRM. The achalasia subgroup of the CCv4.0 Working Group developed both conclusive and inconclusive statements for the HRM diagnoses of achalasia subtypes. Conclusive achalasia on HRM is defined as an abnormal median integrated relaxation pressure (IRP) in the primary position of wet swallows along with 100% failed peristalsis, with type I achalasia having 100% failed peristalsis without panesophageal pressurization (PEP), type II achalasia with PEP in at least 20% of swallows, and type III achalasia having at least 20% of swallows premature with no appreciable peristalsis. An inconclusive HRM diagnosis of achalasia can arise when there is an integrated relaxation pressure (IRP) that is borderline or at the upper limit of normal in at least one position, there is an abnormal IRP in both positions but evidence of peristalsis with PEP or premature swallows, or there is peristalsis in the secondary position after apparent achalasia in the primary position. In patients with dysphagia and an inconclusive HRM diagnosis of achalasia, supportive testing beyond HRM such as a timed barium esophagram (TBE) for functional lumen imaging probe (FLIP) is recommended. The review recommends a diagnostic algorithm for achalasia, discusses therapeutic options for the disease, and outlines future needs on this topic.