Esophageal Dysmotility Research Articles

BN SO47 - Can Peroral Endoscopic Myotomy (POEM) be the new gold standard for Achalasia? – as it is safe and effective

Abstract Background Laparoscopic Heller’s cardiomyotomy is the gold standard for treatment of achalasia. POEM has emerged as a revolutionary minimal invasive procedure in the management of achalasia and related oesophageal motility disorders. POEM effectively utilises an endoscopic approach by creating a submucosal tunnel to perform a myotomy at the lower oesophageal sphincter (LES) , achieving symptomatic relief. Being firstly introduced in 2008, POEM offers an excellent alternative option to surgical intervention and conservative approaches. The aim of this study is to evaluate the clinical efficacy and safety of POEM since its introduction in our unit. Method POEM procedures performed in adult patients are recruited prospectively from May 2020 to January 2024 and data are collected using electronic trust records. Eckardt score (a commonly used scoring system to characterise severity of achalasia) is recorded before and after the procedure. A total of 46 patients are identified however three patients are excluded in this study due to unavailable clinical information. This study presents a cohort of patients with median age of 60 and an equal gender distribution. Results Out of 43 patients, 69.8% were treatment naïve and 30.2% had previous treatments. Mean Eckardt score pre-procedure was 8.2. The average length of hospital stay was 1.7 days and immediate post treatment complications were 6.9% (2.3% mucosotomy, 2.3% oesophageal leak, 2.3% infection). No mortality was recorded within 30 days post-procedure. A 93% clinical success rate (Eckardt score ≤3) was achieved in the initial follow-up period (three to six months) with a mean Eckardt score of 1.1. 41.9% of patients experienced reflux symptoms which require proton pump inhibitor use within four weeks post-procedure. 18.4% of patients had recurrent achalasia symptoms over their follow-up period. Conclusion The clinical success rate (Eckardt score ≤3) and recurrence rate post POEM in this study are comparable with the international cohort quoted in literature. Post POEM reflux rate is prevalent, which is a well-known complication. The substantial improvement in clinical outcomes, evidenced by the Eckardt score reduction and the low incidence of post-operative complications, underscores the procedure’s efficacy and safety. Overall, POEM represents a significant advancement in the treatment of oesophageal motility disorders, especially achalasia. This study presents POEM as a safer and less invasive treatment alternative compared to Heller’s cardiomyotomy.

BN SO18 - Considering Physiology Surveillance in Patients with Oesophageal Atresia

Abstract Background Oesophageal atresia (OA) is a congenital malformation found between one in every 2,400 to 4,500 new-borns per year world-wide [1] who require immediate oesophageal surgery and lifelong endoscopic surveillance for complications of gastroesophageal reflux, oesophagitis, Barrett's oesophagus, anastomotic strictures, oesophageal dysmotility and dysphagia [2, 3]. This study investigates whether ad-hoc oesophageal physiology surveillance would be clinically beneficial for patients with post OA surgical history. Method Retrospective study was conducted on patients with surgical history for OA and undergone oesophageal physiology studies between 2010 and 2023. 11 patients were found (6 females) who had oesophageal physiology at mean age of 29 years (95% CI 26.7 to 35.3 years) or correspondingly post-surgery for OA. Standard high-resolution manometry assessment was re-interpreted with reference Chicago Classification(version 4.0)[4]. This followed by stimulation with multiple water swallowing and solid bolus swallows as adjunctive testing [5, 6, 7]. This project was approved by the NHS institution and the local research ethics committee (REC 18/NW/0120)(IRAS 333800). Results Major dysmotility was observed in 7 patients (63.6%)(achalasia 3, absent contractility(AC) 4) and minor dysmotility was apparent in 4 patients (ineffective oesophageal motility [IOM]). Development of major dysmotility was at a later onset post-surgery to minor dysmotility (30.4 years vs 26.5 years, p=0.214). As was achalasia to AC (32.3 years vs 29.0 years, p=0.379). Adjunctive testing augmented oesophageal smooth muscle contractility in all patients with IOM and invoked remaining neuromuscular functioning in 2 patients with AC. The achalasia diagnosis in the 3 patients remained unchanged during the adjunctive testing. Conclusion Oesophageal physiology surveillance in patients with post OA surgery would enable identifying the staging of the motility disorder and which may direct further surgical management. This study revealed patients were still suitable for partial fundoplication post OA surgery (54.5%) and surgical myotomy for achalasia (27.3%). The prevalence of achalasia (27.3%) and absent contractility (36.4%) in post OA surgery observed this study are significantly higher compared to the non-OA treatment-naïve patients found in the general population living with achalasia and absent contractility disorders.

BN SO30 - One hundred consecutive Laparoscopic Heller's Cardiomyotomy & Fundoplication – a personal series

Abstract Background Oesophageal Achalasia (OA) is an uncommon primary oesophageal motility disorder, characterised by the loss of inhibitory neurons in the oesophageal myenteric plexus. The primary goal of treating OA is to alleviate dysphagia caused by the functional obstruction of the distal oesophagus due to the inability of the lower oesophageal sphincter (LOS) to relax appropriately after swallowing. Heller's cardiomyotomy (HCM) remains a standard treatment for OA. The aim of this study was to assess the symptomatic improvement and post-operative outcomes following HCM with fundoplication at our centre. Method All patients underwent HCM through laparoscopic blunt muscle separation technique, between January 2005 and March 2024, by a single surgeon. Retrospective review of electronic and compiled paper patient records were analysed to assess patient demographics, preoperative symptoms, operative details, length of stay (LOS), complications, post-operative symptoms, gastroscopy and pneumatic dilation (PD) as well as revisional surgery. Primary outcomes assessed were binary changes in key symptoms of weight loss, dysphagia, retrosternal pain and regurgitation. Comparison of pre and post-operative outcomes were performed using paired t-test. A p-value of <0.05 was considered statistically significant. Results 100 procedures involving 99 patients (average age 50 years, 43% female), 1 conversion to open and 1 revision at 3 years. ASA grades were 1 (27%), 2 (58%), and 3 (15%). Median LOS was one day, median follow-up 99 months. Post-operatively; weight loss persisted in 11% of patients from 48%, dysphagia reduced from 98% to 42%, retrosternal chest pain from 39% to 20% and regurgitation from 57% to 24% (all p<0.05). 2 patients required (PD) ≤30 days (CD≥3). 36 patients underwent PD at median of 13 months, 20 needed repeat PD. None of the patients developed cancer. Conclusion As demonstrated in this large series of 100 consecutive patients, HCM remains one of the best treatment modalities for OA. Key symptoms of OA significantly reduced following HCM. Our technique avoided the use of energy devices, ensured a short hospital stay and durable symptom improvement, lends support to its continued application in managing this condition.

MS O10 - BN Case Report: Aberrant Right Subclavian Artery As A Rare Differential For Dysphagia

Abstract Background Dysphagia lusoria (DL) is characterised by swallowing difficulty due to external oesophageal compression from congenital aortic arch malformations. It is clinically rare and affects less than 2.5% of the population. The most prevalent form of DL is the aberrant right subclavian artery (ARSA) where approximately 10% of patients experience dysphagia. Despite AL being congenital, dysphagia often manifests later in life, likely due to age-related atherosclerotic changes in the vasculature and decreased oesophageal flexibility. Method Presented is a case report of a female in her seventies where, despite repeat hiatal hernia repair and maximal medical management, her index symptoms of dyspepsia and retrosternal discomfort persisted when reviewed in specialist upper gastrointestinal clinic. She underwent extensive investigation; however, her symptoms were unremitting, manifesting in numerous emergency department presentations to exclude acute coronary syndrome. This case report details the anatomical differences via diagrams and documents the comprehensive investigation and multi-disciplinary approach to the management of dysphagia in ARSA. Results Prior to referral to our team, the patient had a laparoscopic hiatus hernia repair and Nissen fundoplication undertaken. Oesophageal manometry revealed weak oesophageal motility and 24-hour pH monitoring did not demonstrate pathological acid-reflux. Recurrence of hiatus hernia was observed on oral contrast swallow and gastroscopy. Subsequently, the patient underwent laparoscopic revision of hiatus hernia and conversion of Nissen to a Dor. Post-operatively her symptoms required complete fundoplication reversal. Following reversal, assessment demonstrated improved distal oesophageal emptying. Her CT emphasised an ARSA causing significant compression and deviation of the proximal oesophagus. The patient was referred onwards to vascular surgery. Conclusion Initial diagnostic workup for patients presenting with dysphagia in the upper gastrointestinal clinic seeks to encompass several common aetiologies including oesophageal motility disorders, malignant compression and benign strictures. Clinicians must consider less common causes of extrinsic compression, such as ARSA, particularly in patients with unrelenting and persistent symptoms despite appropriate implementation of both medical and surgical interventions. Treatment approaches for ARSA reflect symptom severity, with both conservative and surgical options considered.

Do children with dysphagia and normal esophageal motility according to Chicago Classification always have "normal" esophageal motility?

Internationally adopted Chicago Classification (CC) criteria based on adult normative data have been used to diagnose children with esophageal motility disorders undergoing high-resolution esophageal manometry (HREM). The aim of this study was to compare HREM parameters of children without dysphagia and children with dysphagia and normal findings according to CC. HREM metrics of 41 children (13.2 (9-18) years; 20 female) without dysphagia and 41 children (13.7 (8-18) years; 30 female) with dysphagia and normal diagnosis according to CC were compared. Analyzed data included resting and integrated relaxation pressures (IRP) of upper (UES) and lower (LES) esophageal sphincters, esophageal peristaltic contractile integrals, transition zone (TZ) gaps, distal latency (DL), and manometric esophageal length to height ratio (MELH). 95%ile normative cutoffs were calculated from the cohort without dysphagia. Proximal contractile integral (PCI), UES and LES mean resting and IRP were not significantly different between the cohorts (p > 0.3). On the contrary, distal contractile integral (DCI), TZ gap and MELH were notably different with p = 0.0002, p = 0.027, and p = 0.033 respectively. According to 95%ile normative cutoffs of DCI, TZ gap and MELH, in cohort with dysphagia 27%, 15%, and 22% of patients respectively were not normal. First study ever to compare HREM parameters of children without dysphagia to children with dysphagia. Considerable proportion of children with dysphagia may be underdiagnosed according to the adult criteria. This emphasizes the need for universally accepted child-specific diagnostic protocols and norms.

Lymphocytic Esophagitis—A Novel Clinicopathologic Variant of Chronic Esophagitis: A Review Paper

Background/Objectives: Lymphocytic esophagitis is a rare inflammatory disease of the esophagus, first described in 2006. Since that time, interest in this disease entity has steadily increased, yet specific data on the etiology of the disease are still lacking. Lymphocytic esophagitis co-occurs with esophageal motility disorders, Crohn’s disease, hypersensitivity (allergy, asthma), the use of certain medications, or immune disorders. Risk factors include smoking and alcohol abuse. The disease is more prevalent in women, developing predominantly within the sixth decade of life. The most important symptom is dysphagia, with odynophagia, epigastric, and chest pain or heartburn being less common. No particular endoscopic presentation is pathognomonic for lymphocytic esophagitis, the findings not infrequently resembling those of eosinophilic esophagitis. Conclusions: There is great need for an international consensus defining strict histopathological criteria for the diagnosis of lymphocytic esophagitis as well as relevant diagnostic and therapeutic management. This review paper presents the current state of knowledge on lymphocytic esophagitis.

Esophageal achalasia presenting as recurrent pneumonia in children: A case series.

Esophageal achalasia (EA) is a rare primary esophageal motility disorder that is considered a rare etiology of dysphagia among infants and children. The proposed primary pathophysiology is related to the loss of ganglion cells in the distal esophageal sphincters, particularly in the Auerbachian muscle layer, which then leads to the dysmotility and failure of lower esophageal sphincter relaxation. Dysphagia, vomiting, poor weight gain, cough, and recurrent aspiration pneumonia are the most common presenting complaints. Herein, we report 3 cases of EA who presented with chronic cough and recurrent aspiration pneumonia. This study reviewed 3 pediatric patients with typical symptoms of EA. All the patients were admitted and referred to the pediatric pulmonology service for evaluation of recurrent pneumonia and suspected aspiration syndrome. All patients underwent a barium esophagogram as a part of the aerodigestive workup of recurrent vomiting, dysphagia, and aspiration pneumonia. Additionally, all the patients underwent workup for other associated congenital anomalies, which included echocardiography, brain magnetic resonance imaging, and an abdominal ultrasound. All patients had EA and presented with recurrent pneumonia. All patients had isolated EA, and none had any evidence of Allgrove syndrome. Pneumatic balloon dilatation was performed for all patients at the same time as the upper gastrointestinal endoscopy. Later, all the patients underwent a laparoscopic Heller myotomy and had no postoperative complications, and their symptoms resolved. EA is a rare condition in children, yet it can be a serious and life-threatening condition if left untreated. Our cases emphasize the significance of considering achalasia in children who experience esophageal dysphagia and recurrent pneumonia. Several pediatric cases have been reported in which respiratory involvement was the primary manifestation of achalasia. These cases highlight the importance of considering gastrointestinal disorders, particularly EA, in the differential diagnosis of children who experience recurrent pneumonia. Early diagnosis and treatment with laparoscopic Heller myotomy can lead to good outcomes for children with achalasia.

ENDOSCOPIC SEDATION TYPE DURING FLIP PANOMETRY DOES NOT SIGNIFICANTLY IMPACT FLIP MOTILITY CLASSIFICATION RELATIVE TO MANOMETRY

Functional lumen imaging probe (FLIP) Panometry evaluates esophageal motility at the time of sedated endoscopy and often parallels high-resolution manometry (HRM) performed in awake patients. This study aimed to assess the impact of endoscopic sedation on FLIP evaluation of esophageal motility. Adult patients who completed FLIP Panometry during sedated endoscopy and had a conclusive Chicago Classification v4.0 diagnosis on HRM were included in this retrospective study. HRM diagnoses relative to FLIP Panometry motility classifications were compared by sedation type used during FLIP, i.e. conscious sedation (CS) with midazolam and fentanyl or monitored anesthesia care (MAC) with propofol. 454 patients (mean (SD) age 53 (17) years, 62% female) completed FLIP Panometry under CS (n=174, 38%) or MAC (n=280, 62%; 177/280 MAC included fentanyl). On comparison of CS vs MAC, HRM diagnoses within FLIP Panometry motility classifications did not differ (P=0.306 across all 5 FLIP Panometry classifications; P values 0.202-0.856 within specific FLIP classifications). The proportion of HRM diagnoses within each FLIP Panometry classification also did not differ between FLIP completed with CS vs MAC with fentanyl (P=0.098) or MAC without fentanyl (P=0.0261) CONCLUSIONS: Whether CS or MAC was used as sedation during FLIP did not have a clinically significant impact on the relationship between diagnosis on FLIP Panometry and HRM. This supports the validity of diagnosing esophageal motility disorders using FLIP Panometry during sedated endoscopy.

Laparoscopic transhiatal surgery based on high-resolution manometric evaluation of epiphrenic esophageal diverticulum.

Epiphrenic esophageal diverticulum is rare and often associated with abnormalities of esophageal motility. Here, we report a case of a patient diagnosed with high-resolution manometry as having epiphrenic esophageal diverticulum with esophagogastric junction outflow obstruction, which were successfully treated with laparoscopic transhiatal surgery. A 59-year-old woman presented to our hospital for treatment of a symptomatic epiphrenic esophageal diverticulum. An esophagogram revealed a left epiphrenic diverticulum measuring 50mm. High-resolution manometry showed a high integrated relaxation pressure of 35.6mmHg (> 26mmHg) and preserved esophageal peristalsis. A chest computed tomography scan showed no external compression of the distal esophagus. Therefore, we diagnosed an epiphrenic esophageal diverticulum with esophagogastric junction outflow obstruction according to the Chicago Classification v3.0. Laparoscopic transhiatal diverticulectomy, planned and selective myotomy, and Dor fundoplication were performed. We performed myotomy just on the esophageal side and did not perform gastric myotomy. The postoperative course was uneventful, and the postoperative esophagogram showed smooth passage of contrast without leakage or stenosis. High-resolution manometry showed a normal integrated relaxation pressure (11.6mmHg) at three months after surgery. Because an epiphrenic esophageal diverticulum is frequently associated with esophageal motility disorder, not only morphologic but also functional and appropriate treatment must be considered.

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 version 4.0 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 with an asymptomatic control group. 4D HRM analysis used custom-built software to measure IBP, maximum EGJ diameter, and distensibility index on supine test swallows. 4D HRM metrics were compared with 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 with controls and normal motility groups ( P < 0.03). Maximum EGJ diameter and EGJ-distensibility index in the conclusive EGJOO and achalasia groups were significantly lower than in controls and normal motility groups ( P < 0.03). 4D HRM identified 37 of 44 (84%) subjects with normal EGJ opening and 29 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.