Esophageal Dysmotility Research Articles

Additional Diagnostic Yield of the Rapid Drink Challenge in Chicago Classification Version 4.0 Compared With Version 3.0.

Chicago classification version 4.0 enhances the diagnosis of esophageal motility disorders using position change and provocative tests such as multiple rapid swallows and a rapid drink challenge. This study investigates the diagnostic role of the rapid drink challenge based on Chicago classification 4.0 using a functional luminal imaging probe to estimate the cutoff value. This study included 570 patients who underwent esophageal manometry with a rapid drink challenge between January 2019 and October 2022. The diagnostic flow was analyzed according to Chicago classification 4.0. Ninety-nine patients (38, achalasia; 11, esophagogastric junction outflow obstruction; 7, ineffective esophageal motility; 1, hypercontractile esophagus; and 42, normal esophageal function) failed the rapid drink challenge. Among the 453 participants, 50 and 86 were diagnosed with achalasia and esophagogastric junction outflow obstruction, respectively, using Chicago classification 4.0. In 249/453 (55.0%) patients initially diagnosed with esophagogastric junction outflow obstruction using Chicago classification 3.0, the diagnosis was changed to achalasia (n = 28), hypercontractile esophagus (n = 7), ineffective esophageal motility (n = 7), or normal esophageal function (n = 121) using Chicago classification 4.0. Rapid drink challenge-integrated relaxation pressure's diagnostic cutoff value was 19 mmHg. Nine patients had diagnoses changed after the rapid drink challenge, including 3 with panesophageal pressurization. Chicago classification 4.0 increased the diagnostic yield of the rapid drink challenge by 2.0% (9/453 patients). However, the rapid drink challenge had a failure rate of 17.9% (99/552 patients). Given the relatively low diagnostic yield and high failure rate of the rapid drink challenge, we recommend adopting an individualized approach to manometry.

Peroral endoscopic myotomy as a new approach in the treatment of patients with achalasia cardia

Background. Achalasia cardia is a disease characterized by an esophageal motility disorder in which the lower esophageal sphincter fails to fully relax in response to swallo-wing and esophageal motility progressively decreases. The aim of the work is to improve the outcomes of endoscopic treatment for esophageal achalasia by modifying peroral endoscopic myotomy (POEM) in stage II–III achalasia cardia. Materials and methods. In 2019–2023, 35 (100.0 %) patients with stage II–III achalasia cardia were examined and underwent POEM at the multidisciplinary surgical department in the Educational and Scientific Medical Center “University Clinic” of the Zaporizhzhia State Medical and Pharmaceutical University. The diagnosis was made based on the following data: complaints, clinical course, esophagram, endoscopic diagnosis, and computed tomography. The average age of the patients was 50.0 ± 15.7 years. Analyzing the frequency of this disease according to the WHO age classification, there were 16 (45.7 %) young, 8 (22.9 %) middle-aged, 10 (28.6 %) elderly, and 1 (2.8 %) senile patient. The group was equal in terms of gender: 16 men (45.7 %) and 19 women (54.3 %), p = 0.9654. Inclusion criteria: age over 18 years, stage II–III achalasia cardia, informed consent of a patient. Exclusion criteria: achalasia cardia stage I, as we believe that at this stage only conservative treatment and balloon dilatation are indicated; achalasia cardia stage IV for which only Heller myotomy with Dor fundoplication are indicated. Results. All patients of the study group were operated by the method of POEM under total intravenous ane-sthesia with artificial lung ventilation. Esophageal myotomy was performed along the posterior wall, considering the risk of recurrence and preservation of the anterior wall for repeated peroral myotomy or laparoscopic Heller myotomy. The length of the myotomy was determined depending on the stage of achalasia. So, for stage II, the beginning of the endoscopic tunnel formation and myotomy was determined from the first persistent esophageal spasm, which most often corresponded to a distance of 25.0 ± 2.0 cm from the incisors and, accordingly, myotomy of the esophagus, lower esophageal sphincter, and gastric myotomy averaged 15.0 ± 3.0 cm. We noted that in all operated patients six months after the operation, a propulsive activity appeared during contrast X-ray of the esophagus, and its volume decreased from 5.6 ± 0.4 cm at stage II to 2.3–4.1 cm (p = 0.0324) and from 7.4 ± 0.2 cm at stage III to 3.6 ± 0.8 cm (p = 0.0267), which indicates a decreased pressure in the lower esophageal sphincter after surgery. Based on the evaluation of the results of multichannel esophageal gastric pH monitoring, reflux was confirmed in 1 (6.7 %) patient with achalasia stage II and in 1 (5.0 %) case with achalasia stage III. Conclusions. Peroral endoscopic myotomy is an effective minimally invasive method of treating patients with achalasia cardia stage II–III. According to the Eckardt symptom score, reliable results were achieved already one month after surgery: 7.6 ± 1.1 points before surgery and 2.1 ± 0.4 points when evaluated one month after surgical treatment (p < 0.0001, U = 475.0). Given the effectiveness and minimal trauma, the low risk of postoperative complications, a significant reduction in hospital stay and rapid postoperative rehabilitation, POEM can be an alternative to laparoscopic Heller myotomy.

Letter to the editor: safety and efficacy of EsoFLIP dilation in patients with esophageal dysmotility: a systematic review.

Letter to the editor: safety and efficacy of EsoFLIP dilation in patients with esophageal dysmotility: a systematic review.

Clinical characteristics of patients with eosinophilic esophagitis and eosinophilic esophageal myositis based on esophageal motility.

Eosinophilic esophagitis (EoE) presents with various esophageal motility disorders, and some cases of hypercontractile esophagus (HE) are associated with eosinophilic esophageal myositis (EoEM). This study aimed to compare the clinical characteristics of patients with EoE and EoEM according to their esophageal motility. The 28 patients with EoE and 2 patients with EoEM were divided into three groups based on esophageal motility: normal motility group, hypomotility group, and spastic contraction group. The clinical characteristics of the three groups were retrospectively compared. Among the 28 patients with EoE, there were 15 with normal esophageal motility, 9 with hypomotility (2 with absent contractility, 7 with ineffective esophageal motility), and 4 with spastic contractions (1 with type III achalasia, 1 with HE, 2 with unclassifiable multipeak contractions). The two patients with EoEM had HE. Most patients in the normal and hypomotility groups had typical endoscopic findings of EoE, whereas these typical findings were less common in the spastic contraction group (P < 0.001). Four of the five patients with esophageal stricture were in the hypomotility group (P = 0.036). The therapy method significantly differed between the three groups: the normal group had more patients that responded to a proton pump inhibitor or potassium-competitive acid blocker, the hypomotility group had more patients that responded to steroids, and the spastic contraction group contained two patients treated with per-oral endoscopic myotomy (P = 0.021). The endoscopic findings and therapy methods differ between patients with EoE and EoEM based on the esophageal motility.

FOUR-DIMENSIONAL IMPEDANCE MANOMETRY IN ESOPHAGEAL MOTILITY DISORDERS.

Four-dimensional high-resolution impedance manometry (4D HRM) uses impedance to estimate esophageal luminal cross-sectional area and track nadir impedance to measure intrabolus pressure (IBP). We aimed to determine whether 4D HRM metrics could define abnormal esophagogastric junction (EGJ) opening between Chicago Classification v4.0 (CCv4.0) motility disorders and compare 4D HRM with functional lumen imaging probe (FLIP) metrics. Symptomatic adult patients who completed high-resolution impedance manometry and FLIP were included and compared to an asymptomatic control group. 4D HRM analysis used custom-built software to measure IBP, maximum EGJ diameter and distensibility index (DI) on supine test swallows. 4D HRM metrics were compared to FLIP EGJ metrics. Ninety patients (31 normal motility; 16 ineffective esophageal manometry; 9 absent contractility; 8 conclusive EGJ outflow obstruction (EGJOO); 12 type I achalasia; 14 type II achalasia; 12 type III achalasia and 34 asymptomatic controls) were included. Phase 2 and 3 IBP was higher in type II and III achalasia compared to controls and normal motility groups (p<0.03). Maximum EGJ diameter and EGJ-DI in the conclusive EGJOO and achalasia groups were significantly lower than in controls and normal motility groups (p<0.03). 4D HRM identified 37 out of 44 (84%) subjects with normal EGJ opening and 29 out of 39 (74%) subjects with reduced EGJ opening on FLIP. 4D HRM metrics correlated with expected clinical observations across a spectrum of esophageal motility disorders and defined EGJ obstruction. 4D HRM metrics may have value in defining EGJ obstruction in equivocal cases related to EGJOO or absent peristalsis.

Delayed diagnosis and treatment of achalasia: a case report

BackgroundAchalasia is characterized as an esophageal motility disorder with incomplete relaxation of the lower esophageal sphincter. Achalasia can be associated with abnormal peristalsis and symptoms of dysphagia, acid reflux, and chest pain. The exact pathophysiology of achalasia remains unclear, but it is hypothesized to be due to degeneration of the myenteric plexus.Case presentationIn this case, a 46-year-old Hispanic man presented to the emergency room with a 12-year history of progressive discomfort with swallowing solids and liquids. Due to many years of incomplete follow-up care and lack of understanding of the course of his disease, this patient’s symptoms escalated to complete intolerance of oral intake and significant weight loss. He was diagnosed with achalasia during his hospital stay and treated successfully with laparoscopic Heller myotomy.ConclusionsThis case discussion illustrates the importance of follow-up care and patient education so that diagnosis and treatment of achalasia are not delayed.

Comparison of measurement of integrated relaxation pressure by esophageal manometry with analysis of swallowing sounds with artificial intelligence in patients with achalasia.

Esophageal motility disorders are mainly evaluated with high-resolution manometry (HRM) which is a time-consuming and uncomfortable procedure with potential adverse events. Acoustic characterization of the swallowing has the potential to be an alternative noninvasive procedure. We compared the findings on HRM and swallowing sounds in 43 patients who were referred for evaluation of dysphagia. The sound analysis was done with empirical mode decomposition method and with artificial intelligence (AI) and the estimated integrated relaxation pressure (IRP) from a two-layer neural network method was compared to measured IRP on HRM. The model then was tested in five patients. IRP was estimated with high accuracy using the model developed with two-layer neural network method. The analysis of acoustic properties of swallowing has the potential to be used for evaluation of esophageal motility disorders, this needs to be further evaluated in larger studies.

Sedation and Endoscopy-Assisted High-Resolution Manometry (SEA-HRM) in Patients Who Previously Failed Standard Esophageal Manometry

Objectives: Esophageal high-resolution manometry (HRM) is the gold standard for diagnosing esophageal motility disorders, but it may be poorly tolerated and unsuccessful. We sought to evaluate a protocol for sedation and endoscopy-assisted (SEA) HRM in patients who previously failed standard HRM and assess patient perspectives towards it. Methods: Adult patients who previously failed HRM were prospectively enrolled. Under propofol sedation, an upper endoscopy was performed during which the HRM catheter was advanced under endoscopic visualization. If the catheter did not reach the stomach on its own, the endoscope itself or a snare was used to help it traverse the esophagogastric junction (EGJ). Results: Thirty patients participated (mean age 67.8, 70% female). The technical success of SEA-HRM was 100%. Twenty-two (73.3%) were diagnosed with a motility disorder including thirteen (43.3%) with achalasia. Eighteen (60%) had previously failed HRM due to discomfort/intolerance, while twelve (40%) failed due to catheter coiling in the esophagus. Subjects in the coiling group were more likely to need endoscopic assistance to traverse the EGJ (91.7% vs. 27.7%, p = 0.001) and have a motility disorder (100.0% vs. 55.6%, p = 0.010), including achalasia (75.0% vs. 22.2%, p = 0.004), compared to the discomfort/intolerance group. All patients preferred SEA-HRM and rated it higher than standard HRM (9.5 ± 1.3 vs. 1.9 ± 2.1, p = &lt;0.001, on a scale of 1–10). Conclusions: SEA-HRM is a highly successful and well-tolerated option in patients who previously failed standard HRM. This should be the recommended approach in cases of failed HRM rather than secondary tests of esophageal motility.

6567 Novel Indication of GLP-1 Receptor Agonists Beyond Weight Loss: Addressing Post-Gastric Bypass Late Dumping Syndrome

Abstract Disclosure: G. Arevalo: None. S. Gra Menendez: None. D. Soliman: None. R.J. Galindo: Consulting Fee; Self; Novo Nordisk, Eli Lilly &amp; Company, Sanofi, Bayer, Inc., Boehringer Ingelheim, Pfizer, Inc., Abbott Laboratories. Grant Recipient; Self; National Institute of Diabetes and Digestive and Kidney Diseases (2P30DK111024), National Institute of Diabetes and Digestive and Kidney Diseases (K23DK123384). Other; Self; Novo Nordisk, Eli Lilly &amp; Company, Dexcom. Background: Late dumping syndrome, characterized by postprandial hyperinsulinemic hypoglycemia (PHH), represents a complex clinical challenge in post-bariatric patients. GLP-1 receptor agonists are being explored as an effective therapeutic option. Clinical Case: A 47-year-old overweight (BMI of 29.86 kg/m²) woman, with a medical history of scleroderma and esophageal dysmotility, underwent a gastric sleeve gastrectomy later requiring conversion to Roux-en-Y gastric bypass to improve esophageal motility. Eleven months post-bypass, she began experiencing recurrent episodes of hypoglycemia (venous glucose 49-70 mg/dL, n &amp;gt;70 mg/dL) accompanied by sweating, palpitations, drowsiness, and weakness. Initial tests were consistent with postprandial hyperinsulinemic hypoglycemia: serum insulin (13.5 µIU/mL, n &amp;lt; 3 µIU/mL), and c-peptide (4.1 ng/mL, n &amp;lt; 0.6 ng/mL). The Sigstad score and the Arts dumping severity score were positive for late dumping syndrome with a score of 26 and 14, respectively. During glucagon administration, the patient's blood glucose level increased by up to 50 mg/dL within 10 minutes of administration. Both sulfonylurea screening and insulin antibody tests were negative. Real-time CGM measurements showed patterns of postprandial hyperglycemic peaks, up to 160-180 mg/dL (n &amp;lt;140 mg/dL), approximately 30 to 60 min after meals, followed by rapidly declining glucose (1-2 hours later), resulting in hypoglycemic events as low as 49 mg/dL. Initial management with dietary changes and pharmacological treatment was unsuccessful due to poor medication tolerability of acarbose and diazoxide, and low adherence to the prescribed diet due to her esophageal dysmotility. The CGM patterns and symptom presentation raised suspicion that late dumping syndrome was triggered by rapid hyperglycemic responses after oral ingestions of typical meals, resulting in the desynchronization of hormonal responses (insulin and GLP-1), and post-prandial hypoglycemia. Hence, the consideration for a GLP-1 receptor agonist emerged. A weekly subcutaneous injection of a long-acting GLP-1 receptor agonist, semaglutide 0.25 mg exhibited marked improvement in her glycemic profile: fewer episodes of postprandial hyperglycemia, and the subsequent descents in blood glucose were less pronounced. The patient demonstrated a favorable response with no severe hypoglycemic episodes reported during a follow-up period of three months. Conclusion: This is the first case to demonstrate semaglutide's efficacy as a standalone treatment for PHH in post-bariatric patients. In this case, semaglutide effectively controlled glycemia and improved the patient's quality of life, presenting a promising therapeutic option for such complex cases. Presentation: 6/1/2024

7148 Fake Fingerstick: Artifactual Hypoglycemia in a Hospitalized patient with Systemic Sclerosis

Abstract Disclosure: S. Shteyman: None. F. Mirza: None. Introduction: Artifactual hypoglycemia is a problem in hospitalized patients. It often leads to unnecessary use of resources and creates anxiety for the patient and medical team. We describe a case of a 60-year-old female with history of systemic sclerosis (Raynaud, telangiectasias, esophageal dysmotility, interstitial lung disease, pulmonary hypertension), rheumatoid arthritis, autoimmune hepatitis and hypothyroidism who initially presented to the hospital emergency department with bilateral lower extremity radiculopathy, and new onset urine incontinence. She underwent spine surgery initially, followed by emergent left upper extremity thrombectomy two days later. Postoperatively, she was noted to have fingerstick glucose of 34 mg/dL. Dextrose and juice were given, and endocrinology service was consulted. The patient was monitored with 2 hourly fingerstick glucose and was noted to have frequent hypoglycemia on fingerstick monitoring. Serum glucose in AM labs was consistently within normal limits. Patient denied both adrenergic and neuroglycopenic symptoms during periods of “hypoglycemia”. She was asked to fast till fingerstick glucose was noted to be at 43 mg/dl, when blood was drawn for serum glucose, insulin, proinsulin, C-peptide, B-hydroxybutyric acid, IGF-2, insulin AB, and sulfonylurea panel, prior to giving her any dextrose. She was asymptomatic at the time. All labs were within normal limits, with serum glucose at 119 mg/dL. At that point, artifactual hypoglycemia was suspected. Serum glucose was checked twice again when fingerstick glucose was noted to be &amp;lt;45mg/dL, and corresponding serum glucose was noted to be normal at both times (&amp;gt;100 mg/dL). Decision of artifactual hypoglycemia was made and fingerstick glucose monitoring was discontinued in this non-diabetic patient, with recommendation to check serum glucose only if symptoms of hypoglycemia occur, as fingerstick glucose values were not reliable. Fasting serum glucose continued to be normal for the remainder of the hospitalization. Discussion: There have been a handful cases of artifactual hypoglycemia reported in patients with CREST, systemic sclerosis, and Raynaud’s disease. It is thought to be due to abnormal small vasculature causing delayed transit of blood through capillaries, and increased uptake of glucose into the interstitial tissue. Some recommend using earlobe sticks as a replacement for fingerstick glucose monitoring in these patients. Future research can explore if continuous glucose monitoring can be used reliably in these patients. Presentation: 6/1/2024

B-104 Correlation of Anti-Centromere Antibodies (ACA) Detection by Indirect Immunofluorescence (IFA) on HEp-2 Cells and by ELISA Demonstrates High Concordance but Highlights Advantage of IFA

Abstract Background Anti-centromere antibodies (ACA), a type of anti-nuclear antibody (ANA) first identified as a discrete speckled pattern on indirect immunofluorescence assay (IFA), are a hallmark of systemic sclerosis (SSc), especially in its limited cutaneous form (lcSSc) or CREST (calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly and telangiectasia). These mitosis-related autoantibodies mainly recognize three centromere proteins (CENP), CENP-A, CENP-B, and CENP-C, that comprise the kinetochore important to chromosome segregation, but more recently, at least three other CENP antigens have been identified (CENP-D, CENP-E and CENP-F). Anti-CENP-B is considered the main autoantibody, and many, if not most, ELISA utilize recombinant CENP-B such that antibodies to CENP antigens other than CENP-B are not detected. Here, an ELISA using both CENP-B and CENP-A is compared to the reference method, HEp-2 cell IFA, highlighting its high, but still less than 100%, sensitivity due to multiple centromere subcomponents. Methods Anti-centromere antibodies are determined in patient serum using the reference method, IFA on the HEp-2 cell substrate (Sprinter XL/EUROpattern®, Euroimmun, NJ) which is also the current method for standalone ACA testing as well as screening by selected ANA profiles designed for the rheumatology specialist. Here ACA IFA- positive and negative serum samples were further analyzed by enzyme immunosorbent assay (ELISA) utilizing recombinant CENP-A and CENP-B (QuantaLyzer® 3000, Werfen, CA). Results When compared, samples between ACA pattern by HEp-2 cell IFA and anti- centromere antibodies by ELISA yielded an agreement of 93.2%. McNemar’s Test Chi Square of p&amp;gt;0.999, with a relative sensitivity of 89.7% and a specificity of 100.0%. Conclusions This study demonstrated very high concordance between ELISA and accurate ACA pattern identification on HEp-2 IFA. The ELISA studied here identifies autoantibodies to both CENP-A and CENP-B where many other ELISA identify only anti-CENP-B. Although ACA are relatively specific for SSc, they have also been reported in systemic lupus erythematosus (SLE), primary biliary cholangitis (PBC), rheumatoid arthritis (RA), Sjogren Syndrome, Raynaud's phenomenon and in cancer in individuals with or without evidence of systemic autoimmune rheumatic disease (SARD). Of note, many other commercially available ELISA for anti-centromere antibodies identify only anti-CENP-B, but there at least four other known CENP antigen targets for autoantibodies causing centromere pattern on IFA. Furthermore, almost all laboratories use the ELISA for ACA on ANA panel (or other connective tissue disease) screening. Hence, it should be duly noted that these testing strategies will not detect autoantibodies to all anti-centromere antibodies. Only specialized ANA profiles designed for the difficult-to-diagnose autoimmune patient begin screening using the IFA method for anti-centromere and therefore, are capable of detecting autoantibodies to all CENP antigens. Laboratorians should advise clinicians of these diagnostic limitations especially as our knowledge about centromere subcomponents and their clinical associations increases.

Using impedance planimetry (EndoFLIP) to determine ideal distensibility ranges for esophageal motility disorders

ObjectiveUse of impedance planimetry (EndoFLIP) has shown distensibility index ranges associated with improved patient-reported outcomes after antireflux surgery. Questions remain whether the previously described ideal distensibility index range can be used for patients with esophageal motility disorders. We hypothesized that patients with esophageal motility disorders would have a different ideal distensibility range for optimal outcomes. MethodsA retrospective review of a prospectively maintained gastroesophageal database was performed for all patients undergoing Toupet and Nissen fundoplication and impedance planimetry. Demographic data, perioperative outcomes, and quality-of-life indicators (Reflux Symptom Index, Gastroesophageal Reflux Disease-Health Related Quality of Life Questionnaire, and gas/bloat and dysphagia scores) were analyzed and compared between patients by use of the χ2 and Wilcoxon rank-sum tests. ResultsFrom 2015 to 2024, 475 patients underwent laparoscopic fundoplication and impedance planimetry evaluation. Of those, 160 had a final distensibility index score in the ideal range, 165 with a final distensibility index score <2.5, and 150 with a final distensibility index >3.6. In the ideal-range cohort, there were no statistically significant differences between those with normal and abnormal motility in regards to outcomes or quality of life indicators. In the low distensibility index cohort, patients with abnormal motility had worse Gastroesophageal Reflux Disease-Health Related Quality of Life Questionnaire, gas/bloat, and dysphagia scores at 1 year postoperatively compared with those with normal motility. More patients in the low distensibility index cohort required dilations postoperatively, and more patients in the high distensibility index cohort had recurrences compared with those in the ideal range cohort. ConclusionThe previously described ideal distensibility index range of 2.5–3.6 for patients undergoing laparoscopic fundoplication may be used for patients with certain esophageal motility disorders.

S587 Esophageal Distensibility Changes as Measured by EndoFLIP in Different Esophageal Motility Disorders

S587 Esophageal Distensibility Changes as Measured by EndoFLIP in Different Esophageal Motility Disorders

Esophageal Function Testing Patterns in the Evaluation and Management of Lung Transplantation: Results of a National Survey.

We surveyed esophageal motility laboratories affiliated with adult pulmonary transplant centers to determine esophageal function testing (EFT) practices. Gastroesophageal reflux and esophageal dysmotility are associated with worse lung transplant outcomes, yet no consensus guidelines for EFT exist in this population. A deidentified online survey was sent to gastrointestinal motility laboratory directors of 49 academic and community-affiliated medical centers that perform lung transplants. Practice characteristics, including annual lung transplant volume and institutional EFT practices pre-lung transplantation and post-lung transplantation were queried. Respondents were categorized by transplant volume into small and large programs based on median annual volume. Among 35 respondents (71% response rate), the median annual transplant volume was 37, and there were 18 large programs. Institutional EFT protocols were used pretransplant by 24 programs (68.6%) and post-transplant by 12 programs (34.2%). Among small and large programs, 52.9% and 72.2% always obtained high-resolution manometry before transplant, respectively. Endoscopy before transplant was performed more often in small programs (n=17, 100%) compared with large programs (n=15,83.3%). Pretransplant endoscopy ( P =0.04), barium esophagram ( P <0.01), and high-resolution manometry ( P =0.04) were more common than post-transplant. In contrast, post-transplant reflux monitoring off-therapy was more common than pretransplant ( P =0.01). In general, pulmonologists direct referrals for EFT and gastroenterology consultation (n=28, 80.0%), with symptoms primarily prompting testing. In the absence of established guidelines, substantial variability exists in pretransplant and post-transplant EFT, directed by pulmonologists. Standardized EFT protocols and gastroenterologist-directed management of esophageal dysfunction has potential to improve lung transplant outcomes.

The Relationship between Upper Esophageal Sphincter Manometry Function and Esophageal Motility Disorders.

High-resolution manometry (HRM) provides measures of esophageal function which are used to classify esophageal motility disorders based on the Chicago Classification system. Upper esophageal sphincter (UES) measures are obtained from HRM, but are not included in the classification system, rendering the relationship between UES measures and esophageal motility disorders unclear. Furthermore, changes in the acceptable amount of esophageal dysfunction between versions of this classification system has created controversy. The objective of this study was to determine the relationship between UES measures and esophageal function. Cross-sectional study. Referral centre. HRM studies from the Calgary Gut Motility Center were reviewed for UES mean basal pressure, mean residual pressure, relaxation time-to-nadir, relaxation duration, and recovery time. Patients were grouped by number of failed swallows according to different iterations of the Chicago Classification: 0 to 4 (Group 1), 5 to 7 (Group 2), and 8 to 10 (Group 3). 2114 patients (65.1% female, median age 56 y) were included. There were significant increases in UES mean basal pressure (P < .001), mean residual pressure (P < .001), relaxation duration (P < .001), and recovery time (P < .001) between groups. Positive correlations existed between number of failed swallows and UES mean basal pressure (r = 0.143; P < .001), mean residual pressure (r = 0.201; P < .001), relaxation duration (r = 0.145; P < .001), and recovery time (r = 0.168; P < .001). Differences in UES measures exist among patients with failed swallows, with a positive correlation between UES dysfunction and increasing dysmotility. Our findings illustrate that UES measures are closely related to esophageal function, and that even minor esophageal dysfunction is related to UES dysfunction.

Effect of hiatal hernia and esophagogastric junction morphology on esophageal motility: Evidence from high-resolution manometry studies.

High-resolution Manometry (HRM) is the most sensitive and specific test available for clinical assessment of hiatal hernia (HH), a common condition defined as the separation between the Lower Esophageal Sphincter (LES) and crural diaphragm (CD). While the link between HH and Gastroesophageal Reflux Disease (GERD) is established, the potential association of HH with esophageal dysmotility, independently from GERD, is uncertain. This study aimed to analyze if HH, with or without GERD, can associate with esophageal motility disorders. Consecutive patients without previous esophageal surgery who underwent HRM between 2018 and 2022 were enrolled. All patients with symptoms suggestive of GERD underwent impedance-pH testing off-therapy. HH was defined as a separation >1 cm between LES and CD, and esophagogastric junction (EGJ) morphology was classified as: Type I, when there was no separation between LES and CD; Type II, in case of minimal separation (>1 and <3 cm); Type III, when ≥3 cm of separation was present. Demographic and clinical characteristics were collected at baseline, including Age, Gender, Alcohol-, Coffee- and Smoke-habits, GERD diagnosis and symptoms' duration. Two cohorts of patients, with and without HH, were retrospectively individuated, and their association with Ineffective Peristalsis, Hypercontractile Esophagus and Outflow Obstruction was analyzed with univariate and multivariate Logistic regressions using the statistical software R. 848 consecutive patients were enrolled, and 295 cases of HH (34.8%), subdivided into 199 (23.5%) Type II- and 96 (11.3%) Type III-EGJ patients, were identified. Ineffective peristalsis was diagnosed in 162 (19.1%) subjects, Hypercontractile esophagus in 32 (3.8%), and Outflow Obstruction in 91 (10.7%), while GERD was present in 375 (44.2%) patients. HH was significantly associated with Ineffective Peristalsis (p < 0.001) and GERD (p < 0.001). Furthermore, HH resulted to be a risk factor for Ineffective peristalsis (OR 2.0, 95% CI 1.4-2.8, p < 0.001) both when the analysis was conducted in all the 848 subjects, independently from GERD, and when it was carried out in patients without GERD (OR 2.3, 95% CI 1.02-5.3, p = 0.04). The risk for Ineffective Peristalsis increased 1.3 times for every centimeter of HH. No statistically significant association was found between HH and Outflow obstruction or Hypercontractile Esophagus. An increasing separation between the LES and CD may lead to a gradual and significant elevation in the risk of Ineffective Peristalsis. Interestingly, this association with HH is true in patients with and in those without GERD, suggesting that the anatomical alteration seems to play a major role in motility change.

Prevalence and gender - specific analysis of a systemic sclerosis cohort in Latvia

BackgroundSystemic sclerosis (SSc) is considered by many to be one of the most severe autoimmune rheumatic diseases with lower prevalence observed in Northern Europe. No previous studies on the prevalence of SSc in Latvia have been conducted and the aim was to study the demographic and clinical data of patients with SSc in northeastern Europe country.MethodsThis study was conducted in two main Latvian hospitals for adults and includes patients with SSc who were consulted between 2016 and 2021.ResultsDuring the study period, 159 patients with SSc were consulted. The point prevalence on 1 January 2021 was 84.0 per million. Female to male ratio was 4.67:1, and highest gender ratio was observed in the age group 70–79-year (6.75:1). Antinuclear antibodies were present in 82.58% of patients, without gender difference. Centromere pattern was more frequently observed in females (40.19% vs. 19.04%), in contrast to speckled pattern (50.98% vs. 57.14%). At disease onset females tended to be younger (46.51 ± 13.52) than males (50.5 ± 16.64). Males had more diffuse cutaneous subtype, interstitial lung disease, pulmonary hypertension and esophageal dysmotility. More than half of patients received treatment with glucocorticoids at any point of the disease (68.31%), without gender difference.ConclusionsSystemic sclerosis is less common in Latvia than in other countries and regions. Due to its location, the data from Latvia are consistent with a north-south gradient in Europe. Gender ratio differences persisted in older age groups as well. Antinuclear antibodies presence did not differ between genders, but in female’s centromere pattern was much more likely to be present. Males had more severe disease course, but in both genders more than half of patients received treatment with GCs at any point of the disease.

Beyond visualizing the bird beak: esophagram, timed barium esophagram and manometry in achalasia and its 3 subtypes.

Achalasia is a rare esophageal motility disorder characterized by lack of primary peristalsis and a poorly relaxing lower esophageal sphincter. This disease process can be examined several ways and these evaluations can offer complementary information. There are three manometric subtypes of achalasia, with differing appearances on esophagram. Differentiating them is clinically important, because treatment for the subtypes varies. Timed barium esophagram (TBE) is a simple test to quantitatively evaluate esophageal emptying. TBE can be used to diagnose achalasia and assess treatment response. Considerable variation in the TBE protocol exist in the literature. We propose a standardized approach for TBE to allow for comparison across institutions.

Comprehensive Approaches to the Treatment of Esophageal Achalasia

Introduction and purpose Achalasia is the most prevalent primary esophageal motility disorder, marked by improper relaxation of the lower esophageal sphincter (LES), increased resting pressure in the LES, and a lack of peristalsis in the esophagus. Symptoms include difficulty swallowing (dysphagia), regurgitation, chest pain, and weight loss. Due to the nonspecific nature of these symptoms, diagnosis can be delayed by several years. Since the underlying cause of achalasia is still unknown, treatment options focus on alleviating symptoms by targeting the lower esophageal sphincter. Materials and methods This paper is based on a literature review sourced from PubMed, using the following keywords: achalasia, POEM, laparoscopic cardiomyotomy, pneumatic dilation, and botulinum toxin injection. Results Various treatment options are available for achalasia, each offering unique features and results. The main approaches include Peroral Endoscopic Myotomy (POEM), Laparoscopic Myotomy (LM), Botulinum Toxin Injection, and Pneumatic Dilation (PD). Conclusion Each treatment differs in its level of invasiveness, duration of symptom relief, risk of complications, and the likelihood of requiring additional interventions. The selection of the most suitable option is influenced by individual patient factors, such as the type and severity of achalasia, overall health, and personal preference

Dacryoadenitis as a Rare Initial Presentation in a Patient with Suspected Crest Syndrome.

Broad spectrum of clinical features: Recognize that although CREST syndrome is primarily characterized by its hallmark features - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia - it can also present with additional clinical features that may indicate the onset or presence of the syndrome.Diagnostic challenges and differentiation: Diagnostic challenges and differentiation: Bilateral dacryoadenitis can be challenging to diagnose due to its overlapping symptoms with other orbital or systemic conditions. The case report may highlight the importance of differentiating it from conditions like orbital cellulitis, sarcoidosis, or lymphoproliferative disorders. Advanced imaging techniques (like magnetic resonance imaging or computed tomography scans) and careful assessment of clinical history (including systemic symptoms like fever or autoimmune conditions) are crucial for accurate diagnosis.Awareness of atypical presentations and follow-up: The case report underscores the need for tailored management strategies for bilateral dacryoadenitis, from conservative treatments in mild cases to more aggressive therapies in severe ones, while also highlighting the importance of monitoring for complications and being vigilant about atypical presentations, such as those seen in patients with CREST syndrome.