Serra doria procedure as an alternative treatment for end stage achalasia. A case report.

The standard of care for achalasia is laparoscopic Heller's cardiomyotomy. This procedure achieves satisfactory and long-standing results in over 85 % of patients. However, disease progression occurs in some patients leading to end-stage achalasia, occasionally requiring oesophagectomy. In a recent systematic review and meta-analysis of 1307 patients who underwent oesophagectomy for end-stage achalasia, the pooled prevalence of pneumonia, anastomotic leakage and mortality were 10 %, 7 % and 2 %, respectively. We present a Serra Doria procedure as an alternative 'esophagus-preserving' procedure in a 58-year-old female patient with end-stage achalasia. This advancement highlights the crucial role of personalized care and the ongoing research necessary to enhance outcomes for those suffering from this challenging condition.

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.

LONG-TERM CLINICAL EVALUATION OF A DECADE WITH PERORAL ENDOSCOPIC MYOTOMY AT A SINGLE EUROPEAN TERTIARY CENTER.

BACKGROUND AND AIMS: Peroral endoscopic myotomy (POEM) is established treatment for achalasia with compelling midterm efficacy. The aim was to analyze long-term development of efficacy, search for predictors of failure and comprehensively assess post-POEM reflux. METHODS: Retrospective analysis of prospective database of patients undergoing POEM at high-volume center 12/2012-6/2023. Patients with completed 3-month follow-up were included. Efficacy was assessed at 3, 6 and 8 years by Eckardt Score (ES). ES ≤2 was considered treatment success. At 3-months, gastroscopy, manometry, 24h pH-monitoring and fluoroscopy were performed, gastroscopy was repeated at 2-3years. At each visit patients were assessed for reflux symptoms and quality of life. RESULTS: From 496 patients included, 3, 6 and 8-year follow-up was completed in 302, 115 and 40 patients. The treatment success rate according to Kaplan-Meier analysis was 90.2%(87.2-93.3), 82.3%(77.3-87.6) and 73.5%(64.8-83.5). Year of procedure was the only predictor of outcome. Fifty-three (10.7%) patients encountered failure/recurrence. Treatment success of redo-POEM (n=20) at 1year was significantly lower 62.9%(44.3-89.2) vs 96.9%(95.3-98.5), p<0.001. At 3-months, reflux esophagitis was present in 40.4% patients, acid exposure time >4,5% in 43.6%, 39.2% patients were using PPIs but only 24.2% had reflux symptoms. At 2-3-years, 50.8% of patients took PPIs, while 39.6% were symptomatic. Reflux esophagitis decreased to 20.2% at 6-years. CONCLUSIONS: Treatment success of POEM declines over time but remains sufficiently high in long-term perspective. Experience with the procedure improves the outcome. Gastroesophageal reflux affects almost half of patients early after POEM and decreases substantially over time together with rise in PPI-use.

Esophageal achalasia compressing the left atrium and mimicking an extracardiac tumor on the transthoracic echocardiography: A case report.

Achalasia is an uncommon disorder affecting esophageal motility. Occasionally, the appearance of a dilated esophagus in achalasia may resemble an extracardiac tumor when observed through transthoracic echocardiography. Left atrial compression due to extensive esophageal dilation is also rare, potentially leading to hemodynamic compromise. Here, we present a rare case involving left atrial compression caused by esophageal dilation in achalasia, with echocardiographic findings mimicking those of an extracardiac tumor.

Long-term outcomes of treatment for achalasia: Laparoscopic Heller myotomy versus POEM.

Achalasia is a rare esophageal motility disorder characterized by nonrelaxation of the lower esophageal sphincter. Laparoscopic Heller myotomy (LHM) is the gold standard treatment for achalasia. Peroral endoscopic myotomy (POEM), a less invasive treatment, is performed extensively, and the selection of the intervention method remains debatable to date. In addition to the availability of extensive studies on short-term outcomes, recent studies on the long-term outcomes of LHM and POEM have shown similar clinical success after 5 y of follow-up. However, gastroesophageal reflux disease (GERD) was more common in patients who had undergone POEM than in those who had undergone LHM. Moreover, existing studies have compared treatment outcomes in various disease states. Some studies have suggested that POEM is superior to LHM for patients with type III achalasia because POEM allows for a longer myotomy. Research on treatment for sigmoid types is currently in progress. However, the long-term results comparing LHD and POEM are insufficient, and the best treatment remains controversial. Further research is needed, and treatment options should be discussed with patients and tailored to their individual needs and pathologies.

Defining lower esophageal sphincter physiomechanical states among esophageal motility disorders using functional lumen imaging probe panometry.

BACKGROUND: Functional lumen imaging probe (FLIP) panometry assesses esophageal motility in response to controlled volumetric distension. This study aimed to describe the physiomechanical states of the lower esophageal sphincter (LES) in response to serial filling/emptying regimes for esophageal motility disorders. METHODS: Fourty-five patients with absent contractile response on FLIP and diagnoses of normal motility (n = 6), ineffective esophageal motility (IEM; n = 8), scleroderma (SSc; n = 10), or nonspastic achalasia (n = 21) were included, as were 20 patient controls with normal motility on FLIP and manometry. LES diameter and pressure were measured after stepwise FLIP filling at 60 mL, 70 mL, and emptying to 60 mL with relative changes used to define physiomechanical states. KEY RESULTS: Passive dilatation after FLIP filling occurred in 63/65 (97%) patients among all diagnoses. After FLIP emptying, passive shortening occurred in 12/14 (86%) normal motility/IEM, 10/10 (100%) SSc, 9/21(43%) achalasia, and 16/20 (80%) controls, with auxotonic relaxation seen in 2/14 (14%) normal motility/IEM, 12/21 (57%) achalasia, and 4/20 (20%) controls. After achalasia treatment (LES myotomy), 21/21 (100%) achalasia had passive shortening after FLIP emptying. CONCLUSIONS & INFERENCES: Physiomechanical states of the LES can be determined via response to FLIP filling and emptying regimes. While passive shortening was the general response to FLIP emptying, auxotonic relaxation was observed in achalasia, which was disrupted by LES myotomy. Further investigation is warranted into the clinical impact on diagnosis and treatment of esophageal motility disorders.

Lessons in clinical reasoning - pitfalls, myths, and pearls: a case of persistent dysphagia and patient partnership.

OBJECTIVES: Diagnostic excellence underscores the patient-centered diagnosis and patient engagement in the diagnostic process. In contrast to a patient-centered diagnosis, a doctor-centered diagnosis with a lack of patient engagement may inhibit the diagnostic process due to the lack of responsibility, disrupted information, and increased effect of cognitive biases, particularly in a situation where multiple physicians are involved. In this paper, we suggest a promising idea to enhance patient engagement in the diagnostic process by using written information by a patient about their perspective and experience, which can fill the gaps needed for diagnosis that doctors cannot find alone. CASE PRESENTATION: A 38-year-old woman developed chest pain, which gradually worsened during the following two years. For two years, she was evaluated in multiple departments; however, no definitive diagnosis was made, and her condition did not improve. During this evaluation, she searched her symptoms and image findings online. She reached a possible diagnosis of 'esophageal achalasia.' Still, she could not tell her concerns to any physicians because she felt that her concerns were not correctly recognized, although she showed her notes that her symptoms were recorded. She finally consulted the department of internal medicine, where her notes and previous test results were thoroughly reviewed. The final diagnosis of esophageal achalasia was confirmed. CONCLUSIONS: Doctors must organize an environment where patients can freely express their thoughts, emotions, and ideas regarding their diagnosis. Cogenerating visit notes using patient input through written communication can be a promising idea to facilitate patient engagement in the diagnostic process.

Complementary pneumatic dilations are an effective and safe treatment when laparoscopic myotomy fails: A 30-year experience at a single tertiary center.

BACKGROUND: In the last 3 decades, laparoscopic Heller myotomy (LHM) has represented the treatment of choice for esophageal achalasia, solving symptoms in most patients. Little is known about the fate of patients relapsing after LHM or their treatment. In this study, we aimed at evaluating the results of complementary pneumatic dilations (CPDs) after ineffective LHM. METHODS: We evaluated the patients who underwent LHM with Dor fundoplication (LHD) from 1992 to 2022 and were submitted to CPD for persistent or recurrent symptoms. The patients were followed clinically and with manometry, barium swallow, and endoscopy when necessary. An Eckardt score (ES) of > 3 was used as threshold for failure. RESULTS: Of 1420 patients undergoing LHD, 120 (8.4%) were considered failures and were offered CPD. Ten patients refused further treatment; in 5 CPD was not indicated for severe esophagitis; 1 patient had surgery for a misshaped fundoplication and 1 patient developed cancer 2 years after LHD; that leaves 103 patients who underwent a median 2 CPDs (IQR, 1-3), at a median of 15 (IQR, 8-36) months after surgery, with 3.0- to 4.0-cm Rigiflex dilator (Boston Scientific, Massachusetts, USA). No perforations were recorded. Only 6 patients were lost to follow-up. Thus, 97 were followed for a median of 37 months (IQR, 6-112) after the last CPD: 70 (72%) were asymptomatic, whereas 27 (28%) had significant persistent dysphagia (ES > 3). The only differences between the 2 groups were the ES after surgery (P < .01) and the number of required CPD. Overall, the combination of LHD + CPD provided a satisfactory outcome in 96.5% of the patients. CONCLUSION: CPDs represent an effective and safe option to treat patients after a failed LHD: when the postsurgery ES consistently remains high and the number of CPDs required to control symptoms exceeds 2, this may suggest the need for further invasive treatments.

Elevated preoperative lower esophageal sphincter pressure predicts improved clinical outcomes after per oral endoscopic myotomy (POEM).

BACKGROUND: Although per oral endoscopic myotomy (POEM) has shown to be beneficial for the treatment of achalasia, it can be difficult to predict who will have a robust and long-lasting response. Historically, it has been shown that higher lower esophageal sphincter pressures have been associated with poorer responses to alternative endoscopic therapies such as Botox therapy and pneumatic dilation. This study was designed to evaluate if modern preoperative manometric data could similarly predict response to therapy after POEM. METHODS: This was a retrospective study of 237 patients who underwent POEM at a single institution over a period of 13 years (2011-2023) and who had a high-resolution manometry performed preoperatively and an Eckardt symptom score performed both preoperative and postoperatively. The achalasia type and integrated relaxation pressures (IRP) were tested for potential correlation with the need for any further achalasia interventions postoperatively as well as the degree of Eckardt score reduction using a linear regression model. RESULTS: The Achalasia type on preoperative manometry was not predictive for further interventions or degree of Eckardt score reduction (p = 0.76 and 0.43, respectively). A higher IRP was not predictive of the need for further interventions, however, it was predictive of a greater reduction in postoperative Eckardt scores (p = 0.03) as shown by the non-zero regression slope. CONCLUSION: In this study, achalasia type was not a predictive factor in the need for further interventions or the degree of symptom relief. Although IRP was not predictive of the need for further interventions, a higher IRP did predict better symptomatic relief postoperatively. This result is opposite that of other endoscopic treatment modalities (Botox and pneumatic dilation). Therefore, patients with higher IRP on preoperative high-resolution manometry would likely benefit from POEM which provides significant symptomatic relief postoperatively.

Nutritional Management of Pediatric Gastrointestinal Motility Disorders.

Normal and optimal functioning of the gastrointestinal tract is paramount to ensure optimal nutrition through digestion, absorption and motility function. Disruptions in these functions can lead to adverse physiological symptoms, reduced quality of life and increased nutritional risk. When disruption or dysfunction of neuromuscular function occurs, motility disorders can be classified depending on whether coordination or strength/velocity of peristalsis are predominantly impacted. However, due to their nonspecific presenting symptoms and overlap with sensory disruption, they are frequently misdiagnosed as disorders of the gut-brain interaction. Motility disorders are a prevalent issue in the pediatric population, with management varying from medical therapy to psychological therapy, dietary manipulation, surgical intervention or a multimodal approach. This narrative review aims to discuss the dietary management of common pediatric motility disorders including gastroesophageal reflux, esophageal atresia, achalasia, gastroparesis, constipation, and the less common but most severe motility disorder, pediatric intestinal pseudo-obstruction.

Esophageal Per-Oral Endoscopic Myotomy (E-POEM): Future Directions and Perspectives.

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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.

Efficacy and safety of per-oral endoscopic myotomy in non-achalasia esophageal motility disorders: a systematic review and meta-analysis.

Per­oral endoscopic myotomy (POEM) is the preferred endoscopic modality for managing achalasia cardia. However, there are no recommendations on the role of POEM in non­achalasia esophageal dysmotility disorders (NAEMD), including esophagogastric junction outflow obstruction (EGJOO), distal esophageal spasm (DES), and hypercontractile esophagus (HE). The present systematic review and meta-analysis aimed to assess the safety and efficacy of POEM in the treatment of NAEMD. MEDLINE, Embase, and Scopus were searched from inception to August 2023 for studies analyzing the outcome of POEM in NAEMD. Clinical success and adverse events were the main outcomes assessed. The event rates and their 95% confidence interval were calculated using a random effects model. A total of 11 studies with 271 patients were included in the final analysis. The pooled clinical success rate with POEM in NAEMD was 86.9% (82.9-90.9). On subgroup analysis, the pooled clinical success rates of POEM in DES and EGJOO were 97.8% (90.9-100.0) and 92.7% (86.3-95.1), which were significantly higher than in HE 81.2% (73.5-88.8). Data from limited studies showed that the pooled rate of improvement in dysphagia and chest pain was 88.5% (83.0-93.9) and 87.4% (80.5-94.4). The pooled incidence of overall AEs and serious AEs was 12.6% (5.7-19.5) and 0.3% (0.0-1.9), respectively. On follow-up, the pooled incidence of new-onset heartburn was 18.7% (11.1-26.2). POEM is a safe and efficacious treatment modality for the management of NAEMD with a lower clinical success in patients with HE. Further large-scale studies are required to validate the findings of the present analysis.

Treatment of Esophageal Achalasia With Sarcopenic Dysphagia by Rehabilitation and Nutritional Support: A Case Report.

Esophageal achalasia is a disease characterized by esophageal motor dysfunction, leading to various symptoms, including vomiting and chest pain. There is no curative treatment for this disease, and the consensus on nutritional therapy or rehabilitation is unclear. Herein, we present the case of a 90-year-old woman with symptoms of esophageal achalasia, exacerbated by secondary sarcopenia and sarcopenic dysphagia after coronavirus disease 2019 (COVID-19) pneumonia. The patient presented with chest pain and vomiting while on a soft diet, and esophagography revealed typical esophageal achalasia. Her esophageal achalasia symptoms resolved, with improvements in nutritional status, skeletal muscle mass, and physical capacity, when a combination of nutritional and comprehensive rehabilitation therapies was adopted. This case highlights that oral dysphagia is associated with worsening esophageal achalasia symptoms and that nutritional and rehabilitative interventions are effective in relieving the symptoms of achalasia in patients with esophageal achalasia and sarcopenia.

Clinical and nutritional correlates associated with weight changes in achalasia patients and the impact of laparoscopic Heller myotomy.

BACKGROUND: Achalasia is characterized by symptoms of esophageal obstruction, preventing food consumption. However, weight loss is observed only in a subset of patients, and data from literature is conflicting. AIMS: Our study aimed at evaluating predictors of weight loss in achalasia patients and at verifying the impact of treatment on nutritional status. METHODS: 123 achalasia patients, eligible for laparoscopic Heller myotomy, were studied. Demographic, clinical and nutritional data (calorie intake and macronutrient composition) were recorded at baseline and one-year post-treatment. Significant weight loss/gain was considered for variation of 10 % of body weight at baseline and post-treatment, respectively. RESULTS: 57.7 % of patients reported weight loss at presentation. These subjects had shorter disease duration, worse symptoms, lower BMI and consumed fewer calories than patients without weight loss. Post-treatment, we observed a considerable improvement in Eckardt score and BMI values. Almost 50 % of the population reported significant weight gain, particularly in individuals with weight loss at baseline. Caloric intake also rose significantly, positively affecting BMI categories. CONCLUSION: We showed that achalasia-induced weight loss is associated with symptoms' severity and disease duration. Conversely, over 50 % of treated patients were in the overweight/obese category, highlighting the need for individualized nutritional interventions in achalasia patients.

Gastroesophageal reflux after per oral endoscopic myotomy for achalasia: Results of a monocentric cohort.

BACKGROUND AND STUDY AIMS: 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. PATIENTS AND 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. CONCLUSION: 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.

Toll-Like Receptor 7-Expressed Macrophages Are Involved in the Pathogenesis of Esophageal Achalasia and Esophagogastric Junction Outflow Obstruction.

INTRODUCTION: Esophageal achalasia is a typical esophageal motility disorder (EMD). Although viral infections have been hypothesized to play a role in the pathogenesis of esophageal achalasia, its etiology remains unclear. This study used esophageal muscle layer specimens collected during per-oral endoscopic myotomy (POEM) procedures to investigate the association between esophageal achalasia and esophagogastric junction outflow obstruction (EGJOO) and pattern recognition receptors. METHODS: Patients with esophageal achalasia and EGJOO who underwent POEM were allocated to the EMD group. Biopsies of the inner circular muscle were conducted during the POEM procedure. The control group comprised individuals diagnosed with esophageal squamous cell carcinoma who underwent surgical resection. Expression of pattern recognition receptors, including Toll-like receptor (TLR) 7, was examined by polymerase chain reaction. Immunohistochemical staining was performed to determine TLR7 expression sites in the esophageal muscle layer, and the relationship between TLR7 mRNA expression and clinical score was investigated. RESULTS: Our analysis revealed a notable upregulation of TLR7 mRNA levels within the muscle layer of esophageal achalasia and EGJOO, in contrast to those of control specimens. In contrast, the correlation between TLR7 and clinical score was not significant. Immunohistochemical staining revealed increased numbers of TLR7-expressing macrophages between the muscle layers. CONCLUSIONS: TLR7-expressing macrophages are involved in the innate immune response underlying esophageal achalasia and EGJOO. This result will lead to the elucidation of new pathogenetic mechanisms and the development of novel therapeutic targets.

Frantz's tumor as incidental finding during Heller myotomy for achalasia: case report and review of literature.

Introduction: 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. Case presentation: 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. Discussion: 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. Conclusion: 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.

Gastroesophageal reflux following peroral endoscopic myotomy for achalasia: Bumps in the road to success.

Achalasia can significantly impair the quality of life. The clinical presentation typically includes dysphagia to both solids and liquids, chest pain, and regurgitation. Diagnosis can be delayed in patients with atypical presentations, and they might receive a wrong diagnosis, such as gastroesophageal reflux disease (GERD), owing to overlapping symptoms of both disorders. Although the cause of achalasia is poorly understood, its impact on the motility of the esophagus and gastroesophageal junction is well established. Several treatment modalities have been utilized, with the most common being surgical Heller myotomy with concomitant fundoplication and pneumatic balloon dilatation. Recently, peroral endoscopic myotomy (POEM) has gained popularity as an effective treatment for achalasia, despite a relatively high incidence of GERD occurring after treatment compared to other modalities. The magnitude of post-POEM GERD depends on its definition and is influenced by patient and procedure-related factors. The long-term sequelae of post-POEM GERD are yet to be determined, but it appears to have a benign course and is usually manageable with clinically available modalities. Identifying risk factors for post-POEM GERD and modifying the POEM procedure in selected patients may improve the overall success of this technique.

Comprehensive analysis of nutritional parameters in patients with idiopathic achalasia: A prospective study in India.

BACKGROUND AND AIM: Achalasia cardia, a primary motility disorder of the esophagus, poses significant malnutrition risks. This study aims at comprehensively assessing the nutritional status in untreated achalasia patients, contrasting it with functional gastrointestinal disorders (FGIDs) cases and impact of per-oral endoscopic myotomy (POEM) on nutrition at one-year. METHODS: We conducted a prospective study, including consecutive achalasia cases, from December 2021 to April 2022 at a tertiary care centre. Biochemical parameters, anthropometry, subjective global assessment (SGA) and malnutrition universal screening tool were used for nutritional assessment. Cases diagnosed with FGIDs served as controls. RESULTS: As many as 118 cases (41.2 ± 13.9 years, 61% males) with achalasia and 200 controls (43.4 ± 11.9 years, 69% males) were included in the study. Sub-types of achalasia included type I (16.9%), II (76.3%) and III (6.8%). Overall, 38.1% and 6.8% cases were moderately and severely malnourished, respectively. As compared to controls, cases with achalasia had lower pre-albumin (19.4 vs. 25.2; p = 0.001), serum calcium (p = 0.012), vitamin D (p = 0.001), serum iron (p = 0.001), triceps fold thickness (p = 0.002) and hand-grip strength (p = 0.001). On univariate analysis, type-I achalasia, body mass index, % weight loss, lower esophageal sphincter pressures and Eckardt scores were predictors of malnourishment (SGA). On multivariate analysis, type of achalasia, mid arm circumference and low body mass index were significant predictors of malnourishment in cases with achalasia. There was significant improvement in the nutritional status after POEM at one-year follow-up. CONCLUSION: Achalasia patients demonstrate a notably higher risk of malnutrition compared to individuals with FGIDs. Nutritional status significantly improves after POEM. (NCT05161923).

Achalasia in Klinefelter syndrome: A suspected pediatric case as well as prevalence analysis suggesting increased risk in this population.

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.