BACKGROUND: 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.

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).
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.
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).
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.

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.
METHODS: 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.

UNASSIGNED: 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.
METHODS: 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.
UNASSIGNED: 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.
CONCLUSIONS: 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.

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.

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.

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.
CONCLUSIONS: 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.

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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.

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.
METHODS: 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.