A 4-year-old male with Klinefelter syndrome (KS), speech delay, and intermittent history of coughing and choking during meals was referred for evaluation. Prior evaluation with computed tomography showed a dilated esophagus at the gastroesophageal junction. The patient was unable to tolerate a barium swallow. Upper endoscopy was performed, and an intraoperative esophagogram, demonstrated a \"birds beak\" appearance suggestive of achalasia. There is no documented relationship between achalasia and KS. However, we utilized TriNetX (a large-scale data clearinghouse) to demonstrate a higher prevalence of achalasia in patients with KS as compared to the general population.

Gastroesophageal reflux disease (GERD) is the most prevalent gastrointestinal disorder posing diagnostic and therapeutic challenges. Diagnosis should be objectively defined with endoscopy and pH testing, while novel metrics may augment diagnosis for inconclusive GERD cases, including the postreflux swallow-induced peristaltic wave index and esophageal mucosal impedance. Conditions that overlap with or mimic GERD should be considered such as achalasia, rumination, and eosinophilic esophagitis. Genetic testing for proton pump inhibitor metabolism is an option for precision therapy in complex persistent GERD. Proton pump inhibitor refractory GERD may require medical, surgical, or endoscopic therapies. The presence of GERD should be objectively evaluated in achalasia patients treated with peroral endoscopic myotomy, and further studies are needed to determine timing of this evaluation. Patients with scleroderma are at a high risk for GERD owing to abnormal esophageal motility and should be managed with aggressive medical therapy and lifestyle changes given the high prevalence of esophagitis and Barrett\'s esophagus in this population. Further studies are needed to understand the complex mechanisms of GERD in idiopathic pulmonary fibrosis and lung transplantation.

Immune-mediated gastrointestinal (GI) diseases, including achalasia, celiac disease, and inflammatory bowel diseases, pose significant challenges in diagnosis and management due to their complex etiology and diverse clinical manifestations. While genetic predispositions and environmental factors have been extensively studied in the context of these conditions, the role of viral infections and virome dysbiosis remains a subject of growing interest. This review aims to elucidate the involvement of viral infections in the pathogenesis of immune-mediated GI diseases, focusing on achalasia and celiac disease, as well as the virome dysbiosis in IBD. Recent evidence suggests that viral pathogens, ranging from common respiratory viruses to enteroviruses and herpesviruses, may trigger or exacerbate achalasia and celiac disease by disrupting immune homeostasis in the GI tract. Furthermore, alterations in the microbiota and, specifically, in the virome composition and viral-host interactions have been implicated in perpetuating chronic intestinal inflammation in IBD. By synthesizing current knowledge on viral contributions to immune-mediated GI diseases, this review aims to provide insights into the complex interplay between viral infections, host genetics, and virome dysbiosis, shedding light on novel therapeutic strategies aimed at mitigating the burden of these debilitating conditions on patients\' health and quality of life.

UNASSIGNED: Solid pseudopapillary tumor is a low-grade malignancy of the pancreas and predominantly affects young women. This neoplasm is a rare pancreatic entity with vague clinical presentation. Diagnosis is often incidental through imaging or even during surgical approach for another condition.
UNASSIGNED: A 22-year-old Brazilian female with gastrointestinal symptoms was diagnosed with achalasia and underwent Heller myotomy. Intraoperatory findings included an enlarging mass in the distal pancreas. During follow-up for the surgical approach of achalasia, a hypothesis of Frantz\'s tumor was stated, and spleen-preserving distal pancreatectomy was performed.
UNASSIGNED: The pathological pathways of Frantz\'s tumor is still unclear, and its connection with chromosomal abnormalities is under investigation. Although the tumor has been reclassified over the years to solid pseudopapillary tumor, surgical resection remains the standard treatment.
UNASSIGNED: Despite a surgical challenge, surgery presents a great prognosis in these patients and long-term survival. High suspicion and proper investigation are fundamental to diagnosis and early treatment.

OBJECTIVE: POEM is a mature procedure endorsed by societal guidelines as a first line therapy for achalasia and spastic esophageal disorders. Nonetheless, several questions remain, including expanding indications for POEM, periprocedural evaluation and management, and the optimal POEM technique to enhance clinical success while mitigating risk for reflux.
RESULTS: There is uncertainty regarding several technical aspects of the POEM myotomy; though aggregating evidence supports the use of real-time impedance planimetry to guide the myotomy. While post-POEM reflux remains a concerning long term sequela, there is an increasing focus on the potential role of endoscopic anti-reflux interventions. Lastly, with the widespread adoption of POEM, we continue to witness ongoing efforts to standardize post-procedural care and training in this procedure. POEM is no longer a novel but rather established procedure. Yet, this technique has continued to evolve, with the aim of optimizing treatment success while reducing adverse events and risk for post-procedural reflux.

OBJECTIVE: Peroral endoscopic myotomy (POEM) has become the first line treatment for achalasia, but controversies remain about the prevalence of gastro-esophageal reflux disease (GERD) after the procedure. The aim of this study was to evaluate post-POEM GERD by a retrospective analysis of a single center cohort.
METHODS: Achalasia patients aged 18 or above, who underwent POEM between 2012 and 2021, were included, provided they had an endoscopic control of reflux at least one year after POEM. GERD symptoms based on GerdQ questionnaire, and proton pomp inhibitors (PPI) consumption were also evaluated.
RESULTS: Among a consecutive cohort of 422 patients treated by POEM, 254 patients were included. Endoscopic results were available after a mean follow-up of 1.9 ± 1.5 years. 71/254 patients (28 %) had erosive esophagitis (86 % Los Angeles Grade A or B). At the last follow-up (mean 4.5 ± 2.2 years), clinical success of POEM (Eckardt score ≤ 3) was achieved in 79.5 % of patients. 44.5 % of patients were on PPI. Mean GerdQ score was 2.2 ± 2.7, with only 13 patients (6.5 %) with a score ≥ 8.
CONCLUSIONS: In this cohort of achalasia patients with an endoscopic follow-up at least 1 year after POEM, GERD did not appear a major threat concern: clinical symptoms were mild in most cases, as was the degree of erosive esophagitis. Furthermore, at the time of last follow up, less than half of patients required treatment with PPI.

UNASSIGNED: This case emphasizes the need for early recognition and accurate diagnosis of achalasia in young adults to avoid exacerbation of the condition and misdiagnosis as GERD. Patient outcomes and quality of life are greatly enhanced by suitable diagnostic techniques, appropriate therapy, interdisciplinary care, and comprehensive patient education along with frequent follow-ups.
UNASSIGNED: Achalasia results from the degeneration of inhibitory ganglion cells within the esophageal myenteric plexus and the lower esophageal sphincter (LES), leading to a loss of inhibitory neurons and resulting in the absence of peristalsis with failure of LES relaxation. Its origins are multifactorial, potentially involving infections, autoimmune responses, and genetics, with equal incidence in males and females. The hallmark symptoms include progressive dysphagia for solids and liquids, along with regurgitation, heartburn, and non-cardiac chest pain. A 22-year-old female patient initially diagnosed with gastroesophageal reflux disease (GERD) received proton pump inhibitors and antacid gel for persistent dysphagia and regurgitation. Subsequent tests including barium esophagogram and manometry indicated Type II Achalasia Cardia. The patient showed clinical improvement with relief of dysphagia, regurgitation, and heartburn symptoms after pneumatic balloon dilatation (PBD). She was advised to follow up after 6 months with upper gastrointestinal (UGI) endoscopy and manometry in the outpatient clinic for regular endoscopic surveillance as there is a risk of transformation to esophageal carcinoma. Diagnosing achalasia in young adults poses challenges due to its diverse presentation and resemblance to other esophageal disorders like GERD. Diagnosis relies on clinical symptoms and imaging studies such as barium esophagogram revealing a bird\'s beak appearance and esophageal manometry showing absent peristalsis. UGI endoscopy is needed to rule out malignancy. Treatment options include non-surgical approaches like medication and Botox injections, as well as surgical methods such as pneumatic balloon dilation, laparoscopic Heller myotomy, and per-oral endoscopic myotomy (POEM). The treatment options depend upon the patient\'s condition at presentation and their individual choices. This case report emphasizes that it is crucial to consider achalasia as a potential differential diagnosis in young adults with dysphagia, especially if conventional treatments for acid peptic disorder do not alleviate symptoms. Prompt diagnosis and appropriate management can lead to significant clinical improvement and better patient outcomes.

BACKGROUND: Peroral endoscopic myotomy (POEM) is a standard procedure for achalasia, an esophageal motility disorder, characterized by a thickened lower esophageal sphincter muscularis propria (LEMP). Visualization of this anatomical stratification has been made possible by the advent of endoscopic ultrasound (EUS). However, the effect of LEMP thickness on treatment outcomes remains unclear. The objective of this study was to investigate whether LEMP thickness affects treatment response post-POEM.
METHODS: This was a single-center, prospective cohort study of patients who underwent POEM between 2014 and 2021. Patients who underwent EUS to evaluate the LEMP before POEM were included in the study. We divided the patients into two groups according to muscle thickness measured by EUS (≥ 2.80 mm; group 1, < 2.80 mm; group 2). The pre- and post-procedural clinical parameters were compared between the two groups.
RESULTS: Among 278 patients, 189 were enrolled. There were no significant differences in the pre- and post-Eckardt scores, integrated relaxation pressure, and distensibility index between the two groups divided by muscle thickness. Furthermore, there was no statistically significant difference in symptom recurrence, as measured by an Eckardt score > 3, post-procedural complications, or post-POEM GERD symptoms. However, patients with thicker lower esophageal sphincter muscle showed a greater decrease in Eckardt scores (ΔES ≥ 3) which was statistically significant (P = 0.002).
CONCLUSIONS: POEM is an effective and safe treatment method for achalasia, regardless of LES muscle thickness. There was a statistically significant difference in the decrease of Eckardt scores of 3 or greater (ΔES ≥ 3) after POEM in the thicker LEMP group suggesting greater alleviation of symptoms in POEM patients with thicker lower esophageal muscle.

BACKGROUND: Peroral endoscopic myotomy (POEM) is the standard treatment for achalasia. Functional luminal imaging probe (FLIP) technology enables objective measurement of lower esophageal sphincter (LES) geometry, with literature linking specific values to improved post-POEM outcomes. Our study assesses FLIP\'s intraoperative use in evaluating myotomy extent in real-time.
METHODS: Retrospective data from all patients undergoing POEM with intraoperative FLIP measurements were extracted from June 2020 to January 2023. The primary endpoint was intraoperative FLIP measurements, management changes, and symptom improvement (Eckardt score).
RESULTS: Fourteen patients (age 56 ± 14 years, BMI 28 ± 7 kg/m2) were identified. Most patients were female (64%). Predominantly, patients presented with type II achalasia (50%). FLIP measurements were taken before and after myotomy, demonstrating increases in mean distensibility index (DI) 1.6 ± 1. 4 to 5.4 ± 2.1 mm2/mmHg (p < 0.05) and mean diameter (Dmin) 6 ± 1.8 to 10.9 ± 2.3 mm (p < 0.05) at 50 ml balloon fill. Additional myotomy was performed in one patient when an inadequate increase in FLIP values were noted. Mean operative time was 98 ± 28 min, and there were no intraoperative complications. At the 30-day follow-up, median Eckardt score decreased from mean a preoperative score of 7 ± 2 to a post-operative mean of 2 ± 3, with 10 patients (78%) having a score ≤ 2. In total, four patients experienced symptom recurrence, with repeat FLIP values revealing a significant decrease in DI from 7 ± 2.2 post-POEM to 2.5 ± 1.5 at recurrence. FLIP technology identified LES pathology in 3 out of 4 (75%) patients, facilitating referral to LES-directed therapy.
CONCLUSIONS: Our study adds to the literature supporting the use of FLIP technology during the POEM procedure, with most patients achieving ideal values after a standard-length myotomy. This suggests the potential benefits of shorter myotomies guided by FLIP to achieve comparable outcomes and reduce postoperative GERD risk. Collaborative standardization of study designs and outcome measures is crucial for facilitating prospective trials and cross-setting outcome comparisons.

BACKGROUND: The routine use of post-operative esophogram has come under evaluation for multiple upper GI surgeries such as with bariatric surgery and gastric resections. A major complication following Per Oral Endoscopic Myotomy (POEM) is a leak from the myotomy site. A post-operative contrast esophogram is often utilized to evaluate the presence of a leak, however it is not a standardized care practice for all patients. Presently it is selectively performed depending on physician assessment intra-operatively. This project will evaluate the necessity of post-operative contrast esophogram following POEM.
METHODS: We retrospectively reviewed 277 patients diagnosed with achalasia who underwent POEM by two surgeons from 2011 to 2022. 173 patients met the inclusion criteria. A post-operative esophogram was used for the evaluation of a leak. Post-operative esophagram were selectively performed on day 1 following surgery using a water-soluble material. Data was evaluated using Stata.
RESULTS: There were 3 detected leaks in the group that underwent esophagrams compared to the non-esophagram group in the early post-operative period. The overall complication rate was 5.5% in the non-esophagram versus 7.9% in the esophagram group. Length of stay was 1.48 days in the non-UGI vs 1.76 days in the esophagram group. Readmission rate was 10.9% in non-esophagram versus 8.7% in esophagram group.
CONCLUSIONS: There was no statistically significant difference in outcomes in patients undergoing POEM who received post-operative esophagram verses patients who did not receive post-operative esophagram. The routine use of a contrast esophogram to detect a leak following POEM may not be justified. This study suggests that esophagrams should be performed depending on the clinical signs/symptoms post-operatively that would warrant imaging and intervention.