OBJECTIVE: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States.
METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM.
RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017).
CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

A 14-year-old girl with a history of asthma was hospitalized because of sudden-onset back pain around her thoracic region that spread to her chest and abdomen. She had been experiencing dysphagia and breathing difficulties for two years, especially after overeating, which often resulted in vomiting undigested food. CT imaging revealed a severely dilated esophagus narrowing at the gastroesophageal junction, suggestive of type 1 achalasia. Further testing confirmed the diagnosis, with an esophageal manometry showing a lack of esophageal contractions and sphincter relaxation. She then underwent a laparoscopic Heller myotomy with relief to her symptoms. This case underscores the rarity of pediatric-onset achalasia with significant esophageal dilation and secondary airway compression, presenting with unusual musculoskeletal and respiratory symptoms. Timely diagnosis and treatment are crucial to prevent worsening and complications.

Pseudoachalasia is a condition in which symptoms, manometry, and imaging findings highly resemble primary achalasia but has a secondary etiology. The majority of patients with pseudoachalasia have the condition as the result of a malignancy, most often at the gastroesophageal junction. There may be issues with timely identification of this malignancy as symptoms are often obscure with diagnostic testing yielding nonspecific results. We describe a case of a 65-year-old diabetic female smoker with a four-month history of intractable vomiting, abdominal pain, and weight loss who was belatedly found to have an adenocarcinoma at the gastric cardia necessitating a total gastrectomy and chemotherapy administration. The case educates clinicians on the clinical alarm symptoms related to malignant pseudoachalasia and stresses the paramount importance of performing a timely esophagogastroduodenoscopy in all cases of achalasia, even with seemingly normal imaging, to rule out pseudoachalasia related to malignancy.

High-resolution manometry (HRM) is a diagnostic tool for surgeons, gastroenterologists and other healthcare professionals to evaluate esophageal physiology. The Chicago Classification (CC) system is based on a consensus of worldwide experts to minimize ambiguity in HRM data acquisition and diagnosis of esophageal motility disorders. The most updated version, CCv4.0, was published in 2021; however, it does not provide step-by-step guidelines (i.e., for beginners) on how to assess the most important HRM metrics. This paper aims to summarize the basic guidelines for conducting a high-quality HRM study including data acquisition and interpretation, based on CCv4.0, using Manoview ESO analysis software, version 3.3 (Medtronic, Minneapolis, MN).

BACKGROUND: Previous studies have demonstrated that 50% of patients with normal high-resolution manometry (HRM) findings or ineffective esophageal motility (IEM) may have abnormal functional luminal imaging probe (FLIP) results. However, the specific HRM findings associated with abnormal FLIP results are unknown. Herein, we investigated the relationship between nonspecific manometry findings and abnormal FLIP results.
METHODS: We retrospectively analyzed 684 patients who underwent HRM at a tertiary care center in Seoul, Korea, based on the Chicago Classification version 4.0 protocol.
RESULTS: Among the 684 patients, 398 had normal HRM findings or IEM. Of these 398 patients, eight showed esophageal wall thickening on endoscopic ultrasonography or computed tomography; however, no abnormalities were seen during esophagogastroduodenoscopy. Among these eight patients, seven showed repetitive simultaneous contractions (RSCs) in at least one of the two positions: 61% (±29%) in 10 swallows in the supine position and 51% (±30%) in five swallows in the upright position. Four patients who underwent FLIP had a significantly decreased esophagogastric junction distensibility index (1.0 ± 0.5 m m 2 mmHg - 1 at 60 mL). Two of these patients underwent per-oral endoscopic myotomy (POEM) due to a lack of response to medication. Esophageal muscle biopsy revealed hypertrophic muscle with marginal eosinophil infiltration.
CONCLUSIONS: A subset of patients (2%) with normal HRM findings or IEM and RSCs experienced dysphagia associated with poor distensibility of the thickened esophageal wall. FLIP assessment or combined HRM and impedance protocols may help better define these patients who may respond well to POEM.

BACKGROUND: Esophagogastric junction outflow obstruction (EGJOO) is a condition characterized by poor relaxation of the lower esophageal sphincter (LES), which can manifest as dysphagia and chest pain. The best treatment of EGJOO is unknown as some patients improve without any specific therapy, whereas some patients undergo invasive therapy. Currently, prognostic factors are lacking. We aimed to assess the long-term prognosis and predictors of dysphagia and chest pain by the rapid drink challenge and solid bolus swallows in EGJOO.
METHODS: We retrospectively assessed high-resolution esophageal manometries (HRM) performed at our center between 2015 and 2018. The patients completed a dysphagia and chest pain questionnaire a median of 34 months after the HRM/baseline assessment, including the Impaction dysphagia questionnaire-10 (IDQ-10) complemented with questions regarding chest pain and esophageal treatments. Symptoms were compared with HRM findings.
RESULTS: In all, 980 HRMs were analyzed and 66 (6.5%) were identified as having HRM findings compatible with EGJOO. Of these, 27 patients with EGJOO (41%) completed the follow-up questionnaires and had no exclusion criteria, and 70% of these patients had dysphagia and 44% chest pain at least once a week. Dysphagia at follow-up was more common in patients with elevated integrated relaxation pressure (IRP) on all three HRM metrics (water swallows, solid bolus swallows, and rapid drink challenge) (p = 0.03, odds ratio: 8.4 (95% CI: 1.2-56.0)), but this was not seen for chest pain (p = 0.45). Abnormal motility patterns on rapid drink challenge or solid bolus swallows were not associated with dysphagia or chest pain at follow-up.
CONCLUSIONS: Having a high IRP on three HRM metrics-water swallows, solid bolus swallows, and rapid drink challenge-is associated with a worse prognosis in patients with EGJOO and could potentially be used to select candidates suitable for invasive procedures.

OBJECTIVE: Peroral endoscopic myotomy (POEM) is a safe and effective endoscopic treatment for achalasia and other esophageal motility disorders, and a versatile knife (TriangleTipKnife J [TTJ]; Olympus, Tokyo, Japan) is currently widely used in POEM. Recently, we reported a novel modification of TTJ, which was adjusted to a knife length of 2 mm by attaching a disposable clip (QuickClip Pro; Olympus) sheath to the tip as a hood attachment. This study compares the safety and effectiveness of TTJ and TTJ with a hood attachment (TTJ-H) in POEM.
METHODS: This 1:1 propensity score-matched retrospective cohort study compared the procedure time, myotomy efficiency, number of coagulation forceps usage, adverse events, length of hospital stay after POEM, procedural success, and clinical success between the TTJ and TTJ-H groups.
RESULTS: A total of 682 consecutive patients who underwent POEM between January 2021 and June 2023 were examined. We excluded 134 patients who had already undergone POEM or laparoscopic Heller myotomy as prior myotomy. Finally, 98 propensity score-matched pairs (n = 196) were identified. The mean procedure time was shortened from 93.5 minutes to 80.2 minutes (14% reduction, P = .012) when comparing the TTJ-H group versus the TTJ group. The mean myotomy efficiency was improved from 2.76 min/cm to 2.32 min/cm (16% improvement, P ≤ .001), and usage of coagulation forceps for hemostasis was decreased from 3.87 to .55 (86% reduction, P ≤ .001).
CONCLUSIONS: This study showed that use of TTJ-H could reduce total procedure time, improve myotomy efficiency, and reduce costs compared with TTJ.

BACKGROUND: Esophageal diverticulum (ED) is an uncommon structural disorder with heterogenous manifestations and elusive pathophysiology. Our aim was to investigate esophageal motility and associated symptom profiles in patients with ED based on high-resolution impedance manometry (HRIM).
METHODS: Consecutive patients with ED referred to our motility laboratory between 2015 to 2022 were identified in our electronic database. All patients were evaluated based on an upper endoscopy, HRIM, and standardized symptom questionnaires. Patients with ED were further stratified into upper, middle, and lower (epiphrenic) cases. Esophageal motility was evaluated with HRIM and the updated Chicago Classification v4.0.
RESULTS: Twenty-four patients with ED (9 upper, 4 middle, and 11 epiphrenic) were analyzed. Patients with ED were generally older (mean: 65 ± 13.3 years) and predominantly women (58.3%). Most ED cases were unilaterally located (95.8%) and left-side predominant (62.5%). Mean symptom duration was 20 months (range: 1-120) and the most common symptoms were dysphagia (70.8%) and regurgitation (37.5%). Erosive esophagitis was noted in 16 patients (69.6%), while barium stasis was noted in 5 patients (20.8%). Fourteen patients (58.3%) were diagnosed with esophageal motility disorders using HRIM, with achalasia being the most common diagnosis (n = 5, 20.8%). Patients with epiphrenic diverticulum had significantly higher symptom scores and achalasia prevalence.
CONCLUSIONS: Patients with ED tended to be older and was associated with a high prevalence of EMD. A multi-disciplinary evaluation, including complete anatomical and motility surveys, may help clarify the underlying pathophysiology and tailor further treatment strategies.

OBJECTIVE: Distal esophageal spasm is an uncommon esophageal motility disorder presenting with non-cardiac chest pain and dysphagia. The main goal of therapy is symptom relief with pharmacologic, endoscopic, and surgical therapies. Pharmacologic treatment is less invasive and is the preferred method of choice. The purpose of this study was to compare the effectiveness of diltiazem versus fluoxetine in the treatment of distal esophageal spasm.
METHODS: A total of 125 patients with distal esophageal spasm diagnosed using endoscopy, barium esophagogram, and manometry were evaluated. Patients were divided into diltiazem and fluoxetine groups and received a 2-month trial of diltiazem + omeprazole or fluoxetine + omeprazole, respectively. Of 125 patients, 55 were lost to follow up and 70 were eligible for final analysis. Clinical signs and symptoms were assessed before and after therapy using four validated questionnaires: Eckardt score, short form-36, heartburn score, and the hospital anxiety and depression scale.
RESULTS: Both regimens significantly relieved symptoms (a decrease in mean Eckardt score of 2.57 and 3.18 for diltiazem and fluoxetine groups, respectively; and a decrease in mean heartburn score by 0.89 and 1.03 for diltiazem and fluoxetine groups, respectively). Patients\' quality of life improved based on short form-36 (an increase in mean score of 2.37 and 3.95 for fluoxetine and diltiazem groups, respectively). There was no relationship between patients\' improvement and severity of symptoms. Psychological findings based on the hospital anxiety and depression scale were inconsistent (a decrease in mean of 0.143 and 0.57 for fluoxetine and diltiazem groups, respectively; p > 0.05).
CONCLUSIONS: Fluoxetine and diltiazem were effective for clinical symptom relief in patients with distal esophageal spasm, but were not promising for improving psychological symptoms. Neither regimen was superior in terms of efficacy. Consequently, it is key to consider side effects and comorbidities when choosing a therapy.

UNASSIGNED: To analyze various adverse events (AEs) related to the peroral endoscopic myotomy (POEM) procedure and to analyze whether these AEs are related to an extended hospital stay.
UNASSIGNED: Patients admitted for POEM for esophageal motility disorders from August 2012 to February 2020 at 5 centers were retrospectively collected. Length of hospital stay, AEs during or after the POEM procedure were analyzed.
UNASSIGNED: Of the 328 patients, 63.1% did not have any AEs, but 2.4% had major AEs, and 33.4% had minor AEs. Major AEs included mucosal injury, bleeding, and hemothorax, accounting for 1.5%, 0.6%, and 0.3%, respectively. Among the minor AEs, pneumoperitoneum was the most common gas-related AEs. Among non-gas-related minor AEs, pneumonia was the most common at 4.6%, followed by pain, fever, and pleural effusion. All major AEs had meaningful delayed discharge and significantly extended hospital stay compared to the no AEs group (median differences range 4.5-9.0 days). Among gas-related minor AEs, except for 4 cases of emphysema, the extended hospital stay was meaningless. All non-gas-related minor AEs was associated with a significant prolongation of hospital stay compared to that in the no AEs group (median differences range 2.0-4.0 days).
UNASSIGNED: In conclusion, most gas-related minor AEs do not significantly affect the patient\'s clinical course. However, subcutaneous emphysema and minor non-gas related AEs such as pneumonia, pain, fever, and pleural effusion can prolong the hospital stay, therefore careful observation is required. Efforts will be made to reduce major AEs that significantly prolong hospitalization.