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High-resolution esophageal manometry [HRM] has become the gold standard for the evaluation of esophageal motility disorders. It is unclear whether there are HRM differences in diagnostic outcome based on regional or geographic distribution. The diagnostic outcome of HRM in a diverse geographical population of Mexico was compared and determined if there is variability in diagnostic results among referral centers. Consecutive patients referred for HRM during 2016–2020 were included. Four major referral centers in Mexico participated in the study: northeastern, southeastern, and central (Mexico City, two centers). All studies were interpreted by experienced investigators using Chicago Classification 3 and the same technology. A total of 2293 consecutive patients were included. More abnormal studies were found in the center (61.3%) versus south (45.8%) or north (45.2%) P  < 0.001. Higher prevalence of achalasia was noted in the south (21.5%) versus center (12.4%) versus north (9.5%) P  < 0.001. Hypercontractile disorders were more common in the north (11.0%) versus the south (5.2%) or the center (3.6%) P.001. A higher frequency of weak peristalsis occurred in the center (76.8%) versus the north (74.2%) or the south (69.2%) P  < 0.033. Gastroesophageal junction obstruction was diagnosed in (7.2%) in the center versus the (5.3%) in the north and (4.2%) in the south p.141 (ns). This is the first study to address the diagnostic outcome of HRM in diverse geographical regions of Mexico. We identified several significant diagnostic differences across geographical centers. Our study provides the basis for further analysis of the causes contributing to these differences. [ABSTRACT FROM AUTHOR]

Esophageal manometry is utilized for the evaluation and classification of esophageal motility disorders. EndoFlip has been introduced as an adjunctive test to evaluate esophagogastric junction (EGJ) distensibility. Treatment options for achalasia and EGJ outflow obstruction (EGJOO) include pneumatic dilation, myotomy, and botulinum toxin. Recently, a therapeutic 30 mm hydrostatic balloon dilator (EsoFLIP, Medtronic, Minneapolis, MN, USA) has been introduced, which uses impedance planimetry technology like EndoFlip. We performed a systematic review to evaluate the safety and efficacy of EsoFLIP in the management of esophageal motility disorders. A systematic literature search was performed with Medline, Embase, Web of science, and Cochrane library databases from inception to November 2022 to identify studies utilizing EsoFLIP for management of esophageal motility disorders. Our primary outcome was clinical success, and secondary outcomes were adverse events. Eight observational studies including 222 patients met inclusion criteria. Diagnoses included achalasia (158), EGJOO (48), post-reflux surgery dysphagia (8), and achalasia-like disorder (8). All studies used 30 mm maximum balloon dilation except one which used 25 mm. The clinical success rate was 68.7%. Follow-up duration ranged from 1 week to a mean of 5.7 months. Perforation or tear occurred in four patients. EsoFLIP is a new therapeutic option for the management of achalasia and EGJOO and appears to be effective and safe. Future comparative studies with other therapeutic modalities are needed to understand its role in the management of esophageal motility disorders. [ABSTRACT FROM AUTHOR]

In recent years, new translational evidence, diagnostic techniques, and innovative therapies have shed new light on esophageal achalasia and revamped the attention on this relatively rare motility disorder. This narrative review aims to highlight the most recent progress and the areas where further research is needed. The four senior authors identified five topics commonly discussed in achalasia management: i.e. pathogenesis, role of functional lumen imaging probe in the diagnostic flow chart of achalasia, how to define the outcome of achalasia treatments, how to manage persistent chest pain after the treatment, and if achalasia patients' may benefit from a regular follow-up. We searched the bibliographic databases to identify systematic reviews, meta-analyses, randomized control trials, and original research articles in English up to December 2023. We provide a summary with the most recent findings in each of the five topics and the critical points where to address future research, such as the immune-genetic patterns of achalasia that might explain the transition among the different phenotypes, the need for a validated clinical definition of treatment success, the use of neuromodulators to manage chest pain, and the need for identifying achalasia patients at risk for cancer and who may benefit of long-term follow-up. Although undoubtedly, progress has been made on the definition and management of achalasia, unmet needs remain. Debated aspects range from mechanistic insights, symptoms, objective measure relationships, and accurate clinical responses to therapeutic interventions. Translational research is eagerly awaited to answer these unresolved questions. [ABSTRACT FROM AUTHOR]

Evaluating clinical care through quality-related metrics is increasingly common. There are now numerous quality statements and indicators related to the medical management of benign and pre-malignant esophageal diseases. Expert consensus leveraging evidence-based recommendations from published society guidelines has been the most frequently used basis for developing esophageal quality statements. While surgical care of patients with esophageal malignancies, including squamous cell carcinoma, has also been developed, those related to benign esophageal disease now include domains of diagnosis, treatment, and monitoring for gastroesophageal reflux disease, eosinophilic esophagitis (EoE), achalasia, and Barrett's esophagus (BE). Several recent studies evaluating adherence to quality metrics affirm substantial variation in practice patterns with opportunities for improvement in care across esophageal diseases. In particular, patient education regarding treatment options in achalasia, frequency of esophageal biopsies among patients with dysphagia to evaluate for EoE, and endoscopic evaluation within a BE segment are areas identified to have need for improvement. As the management of esophageal diseases becomes more complex and interdisciplinary, adherence to quality metrics may be a source of standardization and improvement in delivery and ultimately patient outcomes. Indeed, the development of national quality databases has resulted in a significant growth in the use of these metrics for quality improvement activities and may form the basis for future inclusion in quality reporting and payment programs. [ABSTRACT FROM AUTHOR]

Achalasia is a rare esophageal disorder characterized by abnormal esophageal motility and swallowing difficulties. Pain and/or spasms often persist or recur despite effective relief of the obstruction. A survey by UK charity 'Achalasia Action' highlighted treatments for achalasia pain/spasms as a key research priority. In this patient-requested systematic review, we assessed the existing literature on pharmacological therapies for painful achalasia. A systematic review of the literature using Medline, Embase and Cochrane databases was performed to identify studies evaluating pharmacological therapies for achalasia. Methodological quality of included randomized controlled trials was assessed using the Cochrane Risk of Bias tool. In total, 70% (40/57) of survey respondents reported experiencing pain/spasms. A range of management strategies were reported. Thirteen studies were included in the review. Seven were randomized controlled trials. Most studies were >30 years old, had limited follow-up, and focussed on esophageal manometry as the key endpoint. Generally, studies found improvements in lower esophageal pressures with medications. Only one study evaluated pain/spasm specifically, precluding meta-analysis. Overall risk of bias was high. The achalasia patient survey identified that pain/spasms are common and difficult to treat. This patient-requested review identified a gap in the literature regarding pharmacological treatments for these symptoms. We provide an algorithm for investigating achalasia-related pain/spasms. Calcium channel blockers or nitrates may be helpful when esophageal obstruction and reflux have been excluded. We advocate for registry-based clinical trials to expand the evidence base for these patients. [ABSTRACT FROM AUTHOR]

This case study presents a female patient with progressive dysphagia for solids, heartburn, and obesity that proved refractory to clinical management. Imagenological diagnosis revealed esophageal stenosis and achalasia. Furthermore, metabolic syndrome was established. We proposed intervention through esophagogastric reconstruction due to stenosis, revision of cardiomyotomy and robotic gastric bypass revealing scar tissue and fibrosis on the anterior aspect of the stomach resulting from prior fundoplication surgery. The patient underwent esophagogastric reconstruction due to adhesion bands which conditioned partial angulation of the gastroesophageal junction, cardiomyotomy revision, anterior and posterior hiatal plasty, and Roux Y Gastric Bypass assisted by a robot without complications. The intervention resulted in significant improvement in postoperative symptoms. This case highlights the importance of considering the probability of mechanical obstruction due to postsurgical adhesions in the initial evaluation of recurrent and persistent dysphagia, with surgical reintervention being the ideal option for resolution. [ABSTRACT FROM AUTHOR]

Achalasia is a rare disease with significant diagnostic delay and association with false diagnoses and unnecessary interventions. It remains unclear, whether atypical presentations, misinterpreted symptoms or inconclusive diagnostics are the cause. The aim of this study was the characterization of typical and atypical features of achalasia and their impact on delays, misinterpretations or false diagnoses. A retrospective analysis of prospective database over a period of 30 years was performed. Data about symptoms, delays and false diagnoses were obtained and correlated with manometric, endoscopic and radiologic findings. Totally, 300 patients with achalasia were included. Typical symptoms (dysphagia, regurgitation, weight loss and retrosternal pain) were present in 98.7%, 88%, 58.4% and 52.4%. The mean diagnostic delay was 4.7 years. Atypical symptoms were found in 61.7% and led to a delay of 6 months. Atypical gastrointestinal symptoms were common (43%), mostly 'heartburn' (16.3%), 'vomiting' (15.3%) or belching (7.7%). A single false diagnosis occurred in 26%, multiple in 16%. Major gastrointestinal misdiagnoses were GERD in 16.7% and eosinophilic esophagitis in 4%. Other false diagnosis affected ENT-, psychiatric, neurologic, cardiologic or thyroid diseases. Pitfalls were the description of 'heartburn' or 'nausea'. Tertiary contractions at barium swallows, hiatal hernias and 'reflux-like' changes at endoscopy or eosinophils in the biopsies were misleading. Atypical symptoms are common in achalasia, but they are not the sole source for diagnostic delays. Misleading descriptions of typical symptoms or misinterpretation of diagnostic studies contribute to false diagnoses and delays. [ABSTRACT FROM AUTHOR]

Esophageal high-resolution manometry (HRM) is the gold standard for diagnosing esophageal motility disorders. As this is performed without sedation, it may be poorly tolerated by patients. Additionally, advancing the catheter tip until the stomach may also be difficult, especially if there is a disease affecting the esophagus or esophagogastric junction which may lead to coiling of the catheter. Currently, there are no guidelines as to who can perform HRM. An expert opinion has recommended minimum numbers of cases for the operator to perform in order to obtain and maintain competency. Several studies have reported on the rates of flawed HRM studies with approximately 20% being imperfect, including about 3% with critical flaws. The presence of a large hiatal hernia and achalasia have been identified as predictors of technical failure. Studies with minor flaws are usually still interpretable and clinically useful. Reports have also described several techniques for sedation-assisted placement of the HRM catheter, and this appears to be a promising alternative in cases of failed HRM, especially as sedation does not appear to significantly affect HRM measurements. An algorithm on how to proceed after a technically imperfect HRM study is proposed. Further research in this field is warranted including confirmation of the recommended competency numbers, assessing for quality measures in performing HRM such as technical success rate, identifying additional predictors of technical failure, standardization of the terminology, and qualifying the benefit of sedation-assisted HRM. [ABSTRACT FROM AUTHOR]

Peroral endoscopic myotomy (POEM) is a safe and effective minimally invasive treatment for achalasia. Postoperative reflux rates remain high. The functional luminal imaging probe (FLIP) allows intraoperative measurement of lower esophageal distensibility during POEM. In theory, this enables a tailoring of myotomies to ensure adequate distensibility while minimizing postoperative reflux risk. Two prospectively collected POEM databases were analyzed from two UK tertiary upper GI centers. The operators in each center used FLIP measurements to ensure adequate myotomy. Outcome measures included Eckardt score (where <3 indicated clinical success) and proton-pump inhibitor use (PPI), collected at the first postoperative appointment. Length of stay was recorded as were complications. In all, 142 patients underwent POEM between 2015 and 2019. Overall, 90% (128/142) had postoperative Eckardt scores of <3 at 6 weeks. Clinical success improved to 93% (66/71) in the latter half of each series with a significantly higher rate of complete symptom resolution (53 versus 26%, P  = 0.003). In all, 79% of the poor responders had previous interventions compared with 55% of responders (P  = 0.09). Median post-myotomy distensibility index was 4.0 mm2/mmHg in responders and 2.9 in nonresponders (P  = 0.16). Myotomy length of <7 cm was associated with 93% clinical success and 40% post op PPI use compared with 60% PPI use with longer myotomies. There were two type IIIa, two type IIIb, and one IV Clavien–Dindo complications. This is the largest series of endoluminal functional lumen imaging probe (EndoFLIP)-tailored POEM in the UK to date. The shorter myotomies, allowed through EndoFLIP tailoring, remained clinically effective at 6 weeks. Complete symptom response rates improved in the latter half of each series. More data will be needed from high-volume collaborations to decipher optimal myotomy profiles based on EndoFLIP parameters. [ABSTRACT FROM AUTHOR]

Due to the unclear quality of the current guidelines, users may be confused about how to diagnose and treat achalasia. The objective of this work is to systematically evaluate the methodological quality of the current guidelines for diagnosing and treating achalasia and to determine the heterogeneity among recommendations. We systematically searched literature databases to retrieve relevant guidelines for the diagnosis and treatment of achalasia. The Appraisal of Guidelines for Research and Evaluation II tool was used to evaluate the quality of the included guidelines. Key recommendations in the guidelines were extracted, and the reasons for the heterogeneity of the key recommendations between different guidelines were further analyzed. Seven guidelines on the diagnosis and treatment of achalasia are included in this study. The overall score of three guidelines exceeded 60%. The average score in domain 5 was the lowest, at 41.8%. The average scores in domain 2, domain 3, and domain 6 were also low, at 45.4%, 57.1% and 56.9%, respectively. The main recommendations and quality of evidence for different guidelines vary greatly, mainly due to the different emphases among different guidelines, the lack of systematic retrieval, or the unfairness of evidence use in some guidelines. There are considerable differences in the methodological quality of diagnosis and treatment guidelines for achalasia. Additionally, the differences in the main recommendations and evidence support among guidelines are also obvious. Guideline developers should improve the above related factors to decrease the heterogeneity, and they should further formulate or update the guidelines for the diagnosis and treatment of achalasia. [ABSTRACT FROM AUTHOR]

High-resolution manometry (HRM) with the Chicago Classification (CC) is the standard paradigm to define esophageal motility disorders. Functional lumen imaging probe (FLIP) panometry utilizes impedance planimetry to characterize esophageal compliance and secondary peristalsis. The aim of this study was to explore the clinical impact of FLIP panometry in addition to HRM. A retrospective chart review was performed on FLIP panometry cases utilizing the 322N catheter. Cases with prior foregut surgeries or botulinum injection within 6 months of FLIP panometry were excluded. EGJ-diameter and distensibility index (DI) and secondary contraction patterns at increasing balloon volumes were recorded. An EGJ-DI of ≥2.8 mm2/mm Hg at 60 mL was considered as a normal EGJ distensibility. CC diagnosis, Eckhardt score, Brief Esophageal Dysphagia Questionnaire, and clinical outcomes were obtained for each FLIP case. A total of 186 cases were included. Absent contractility and achalasia types 1 and 2 showed predominantly absent secondary contraction patterns, while type 3 had a variety of secondary contractile patterns on FLIP panometry. Among 77 cases with EGJ outflow obstruction (EGJOO), 60% had a low EGJ-DI. Among those with no motility disorder or ineffective esophageal motility on HRM, 27% had a low DI and 47% had sustained contractions on FLIP, raising concern for an esophageal dysmotility process along the achalasia and/or spastic spectrum. FLIP panometry often confirmed findings on HRM in achalasia and absent contractility. FLIP panometry is useful in characterizing EGJOO cases. Spastic features on FLIP panometry may raise concern for a motility disorder on the spastic spectrum not captured by HRM. Further studies are needed on FLIP panometry to determine how to proceed with discrepancy with HRM and explore diagnoses beyond the CC. [ABSTRACT FROM AUTHOR]

The efficacy of peroral endoscopic myotomy (POEM) for achalasia has potential associations with Chicago classification by high-resolution manometry (HRM). Type II achalasia demonstrates the best response to POEM of all subtypes, while there remain controversies between type I and type III. Moreover, previous treatment history might cause discrepancy in direct comparison. We aimed to compare the clinical outcome of POEM for type I vs type III in treatment-naive patients. In total, 82 patients with type I or type III achalasia (45 type I, 37 type III) from February 2015 to December 2018 were enrolled and POEM was carried out as the initial treatment. Clinical success, change of Eckardt scores and HRM parameters were analyzed and compared between type I and type III group. About, 43 (95.6%) patients and 34 (91.9%) patients in type I and type III group acquired the clinical success (P  = 0.821). Eckardt score and HRM results after POEM treatment decreased significantly in either group (P <0.01). Compared to type III group, higher reduction rates of Eckardt score (type I vs type III, 78.6 vs 66.9%, P  = 0.034) and basal LES pressure (type I vs type III, 58.9 vs 40.4%, P  = 0.040) were observed in type I group. Type I achalasia patients showed better response to POEM with more favorable clinical remission in Eckardt score and HRM outcomes than type III. [ABSTRACT FROM AUTHOR]

Background Achalasia is a primary esophageal motility disorder with potential molecular pathogenesis remaining uncertain. This study aimed to identify the differentially expressed proteins and potential pathways among achalasia subtypes and controls to further reveal the molecular pathogenesis of achalasia. Methods Paired lower esophageal sphincter (LES) muscle and serum samples from 24 achalasia patients were collected. We also collected 10 normal serum samples from healthy controls and 10 normal LES muscle samples from esophageal cancer patients. The 4D label-free proteomic analysis was performed to identify the potential proteins and pathways involved in achalasia. Results Analysis of Similarities showed distinct proteomic patterns of serum and muscle samples between achalasia patients and controls (both P  <   0.05). Functional enrichment analysis suggested that these differentially expressed proteins were immunity-, infection-, inflammation-, and neurodegeneration-associated. The mfuzz analysis in LES specimens showed that proteins involved in the extracellular matrix–receptor interaction increased sequentially between the control group, type III, type II, and type I achalasia. Only 26 proteins altered in the same directions in serum and muscle samples. Conclusions This first 4D label-free proteomic study of achalasia indicated that there were specific protein alterations in both the serum and muscle of achalasia, involving immunity, inflammation, infection, and neurodegeneration pathways. Distinct protein clusters between types I, II, and III revealed the potential molecular pathways associated with different disease stages. Analysis of proteins changed in both muscle and serum samples highlighted the importance of further studies on LES muscle and revealed potential autoantibodies. [ABSTRACT FROM AUTHOR]

Background: To date, the ideal endoscopic knife for peroral endoscopic myotomy (POEM) with good performance and cost-effectiveness is still under investigation. The present study was aimed to evaluate the efficacy, safety, and cost-effectiveness of snare-assisted POEM, compared with the conventional endoscopic knife approach. Methods: From May 2017 to December 2018, patients with achalasia presenting for POEM without previous endoscopic or surgical therapy were prospectively recruited in this randomized controlled trial. Patients were randomly allocated to receive POEM using either the snare (snare group) or HookKnife (conventional group). The primary outcome was clinical success (Eckardt score ≤ 3) at 12-month follow-up, powered for noninferiority with a margin of −15%. The secondary outcomes included adverse events (AEs), procedure-related parameters, clinical outcomes, and cost-effectiveness. Results: A total of 75 patients with similar baseline characteristics between the snare (N  = 37) and conventional (N  = 38) groups were included. Clinical success at 12-month follow-up was achieved in 94.6% of patients in the snare group and 92.1% of patients in the conventional group (difference, 2.5% [95% CI, −8.7% to 13.7%]; P  < 0.001 for noninferiority). No severe AEs occurred in both groups. The use of snare is associated with comparable procedure time (40.6 minutes vs. 42.5 minutes, P  = 0.337), a lower frequency of hemostatic forceps use (27.0% vs. 68.4%, P <  0.001), and lower hospital costs ($4271.1 vs. $5327.3, P  < 0.001). The cost-effectiveness plane revealed that 96.9% of snare-assisted POEM procedures offered more cost-savings and health utility benefits. Conclusions: The snare-assisted POEM was noninferior to the conventional endoscopic knife approach in terms of clinical efficacy, with comparable safety outcomes and cost-effective benefits. [ABSTRACT FROM AUTHOR]

Geriatric patients with existing studies on the safety and efficacy of peroral endoscopic myotomy (POEM) for achalasia involve small sample sizes and single institutions. However, multi-center, large-scale data analyses are lacking. The study aimed to clarify the characteristics of geriatric patients with esophageal motility disorders (EMDs) and determine the procedure-related outcomes and clinical course following POEM. This cohort study included 2,735 patients with EMDs who were treated at seven Japanese facilities between 2010 and 2019. The patients' characteristics and post-POEM clinical courses were compared between the geriatric (age ≥ 75 years; n  = 321) and non-geriatric (age < 75 years; n  = 2,414) groups. Compared with the non-geriatric group, the geriatric group had higher American Society of Anesthesiologists physical status scores; more recurrent cases; lower incidence of chest pain; and higher incidence of type III achalasia, distal esophageal spasm, and Jackhammer esophagus. Furthermore, the incidence of sigmoid esophagus was higher, although esophageal dilation was not severe in this group. POEM was safe and effective for geriatric patients with treatment-naïve and recurrent EMDs. Furthermore, compared with the non-geriatric group, the geriatric group had lower post-POEM Eckardt scores, fewer complaints of refractory chest pain, and a lower incidence rate of post-POEM reflux esophagitis. Geriatric patients are characterized by worse clinical conditions, more spastic disorders, and greater disease progression of EMDs, which are also the indications for minimally invasive POEM. POEM is more beneficial in geriatric patients as it has lowering symptom scores and incidence rates of reflux esophagitis. [ABSTRACT FROM AUTHOR]

Peptic esophagitis can occur as a complication of laparoscopic Heller-Dor surgery (LHD) among patients with esophageal achalasia. The goal of this study was to identify the characteristics of patients who have developed peptic esophagitis following LHD surgery along with the risk factors associated with the occurrence of peptic esophagitis. Among the 447 cases consisting of esophageal achalasia patients who underwent LHD as the primary surgery, we compared the patient background, pathophysiology, symptoms, and surgical outcomes according to whether or not peptic esophagitis occurred following surgery. We also attempted to use univariate and multivariate analyses to identify the risk factors for peptic esophagitis occurring following surgery. Esophagitis following surgery was confirmed in 67 cases (15.0%). With respect to the patient backgrounds for cases in which peptic esophagitis had occurred, a significantly higher number were male patients, with a significantly high occurrence of mucosal perforation during surgery in terms of surgical outcomes, along with a high occurrence of esophageal hiatal hernias in terms of postoperative course (P = 0.045, 0.041, and 0.022, respectively). However, there were no significant differences in terms of age, BMI, disease duration, preoperative symptoms, esophageal manometric findings, esophageal barium findings, and esophageal clearance. A multivariate analysis indicated independent risk factors for the occurrence of peptic esophagitis following LHD as being male, the occurrence of mucosal perforation during surgery, and the occurrence of esophageal hiatal hernias. Peptic esophagitis occurred following LHD in 15% of cases. Independent risk factors for the occurrence of peptic esophagitis following LHD included being male, the occurrence of mucosal perforation during surgery, and the occurrence of esophageal hiatal hernias following surgery. [ABSTRACT FROM AUTHOR]

Peroral endoscopic myotomy (POEM) in patients with achalasia who are status post bariatric surgery may be technically challenging due to postsurgical scarring and altered anatomy. The aim of the study was to assess the efficacy and safety of POEM for achalasia in patients with prior bariatric surgery. A review of prospectively maintained databases at three tertiary referral centers from January 2015 to January 2021 was performed. The primary outcome of interest was clinical success, defined as a post-treatment Eckardt score ≤ 3 or improvement in Eckardt score by ≥ 1 when the baseline score was <3, and improvement of symptoms. Secondary outcomes were adverse event rates and symptom recurrence. Sixteen patients status post Roux-en-Y gastric bypass (n  = 14) and sleeve gastrectomy (n  = 2) met inclusion criteria. Indications for POEM were achalasia type I (n  = 2), type II (n  = 9), and type III (n  = 5). POEM was performed either by anterior or posterior approach. The pre-POEM mean integrated relaxation pressure was 26.2 ± 7.6 mm Hg. The mean total myotomy length was 10.2 ± 2.7 cm. The mean length of hospitalization was 1.4 ± 0.7 days. Pre- and postprocedure Eckardt scores were 6.1 ± 2.1 and 1.7 ± 1.8, respectively. The overall clinical success rate was 93.8% (15/16) with mean follow-up duration of 15.5 months. One patient had esophageal leak on postprocedure esophagram and managed endoscopically. Dysphagia recurred in two patients, which was successfully managed with pneumatic dilation with or without botulinum toxin injection. POEM appears to be safe and effective in the management of patients with achalasia who have undergone prior bariatric surgery. [ABSTRACT FROM AUTHOR]

Objective: CAVI encodes caveolin-1, a major protein of plasma membrane microdomains called caveolae, involved in several signaling pathways. Caveolin-1 is also located at the adipocyte lipid droplet. Heterozygous pathogenic variants of CAVI induce rare heterogeneous disorders including pulmonary arterial hypertension and neonatal progeroid syndrome. Only one patient was previously reported with a CAV1 homozygous pathogenic variant, associated with congenital generalized lipodystrophy (CGL3). We aimed to further delineate genetic transmission, clinical, metabolic, and cellular characteristics of CGL3. Design/Methods: In a large consanguineous kindred referred for CGL, we performed next-generation sequencing, as well as clinical, imagery, and metabolic investigations. We studied skin fibroblasts from the index case and the previously reported patient with CGL3. Results: Four patients, aged 8 months to 18 years, carried a new homozygous p.(His79Glnfs*3) CAVI variant. They all displayed generalized lipodystrophy since infancy, insulin resistance, low HDL-cholesterol, and/or high triglycerides, but no pulmonary hypertension. Two patients also presented at the age of 15 and 18 years with dysphagia due to achalasia, and one patient had retinitis pigmentosa. Heterozygous parents and relatives (n = 9) were asymptomatic, without any metabolic abnormality. Patients' fibroblasts showed a complete loss of caveolae and no protein expression of caveolin-1 and its caveolin-2 and cavin-1 partners. Patients' fibroblasts also displayed insulin resistance, increased oxidative stress, and premature senescence. Conclusions: The CAVI null variant investigated herein leads to an autosomal recessive congenital lipodystrophy syndrome. Loss of caveolin-1 and/or caveolae induces specific manifestations including achalasia which requires specific management. Overlapping phenotypic traits between the different CAV7-related diseases require further studies. [ABSTRACT FROM AUTHOR]

Allgrove syndrome (AS), or Triple-A syndrome, is a multi-system disorder characterized by alacrima (a decrease or absence of tear production), adrenal insufficiency and achalasia (absence of esophageal muscle peristalsis and failure to relax the lower esophageal sphincter). This syndrome may affect the autonomic nervous system, in which case it is called a 4A syndrome. It is a rare autosomal recessive inheritance, and early identification is difficult due to the rarity and wide phenotypic variation even among members of the same family. Endocrinologists, gastroenterologists, ophthalmologists, neurologists and surgeons are needed to coordinate care for these patients. We describe a case of AS that took several years to complete the diagnosis. She was diagnosed with alacrima at the age of 1-year-old, adrenal insufficiency at the age of 9 and achalasia at the age of 16. This case demonstrates the difficulty and delay in the diagnosis of AS. [ABSTRACT FROM AUTHOR]

Introduction: Achalasia may evolve to sigmoid megaesophagus in 10–15% of patients and is usually treated with esophagectomy, which has high morbi-mortality. Many surgeons debate the applicability of the Heller myotomy for treating sigmoid megaesophagus. This study intents to analyze the effectiveness of myotomy for treating patients with sigmoid megaesophagus. Methods: A systematic review and meta-analysis was conducted in PubMed, Cochrane, Lilacs and Embase alongside manual search of references. The inclusion criteria were clinical trials, cohort, case-series; patients with sigmoid megaesophagus and esophageal diameter ≥ 6 cm; and patients undergoing primary myotomy. The exclusion criteria were reviews, case reports, cross-sectional studies, editorials, letters, congress abstracts, full-text unavailability; previous surgical treatment for achalasia; and pediatric or animal model studies. No restrictions on language and date of publication, and no filters were applied. Subgroups analyses were performed to assess the laparoscopic myotomy perioperative outcomes. Besides, subgroup analyses were performed to assess the long-term outcomes of the studies with a follow-up time > 24 months. To verify heterogeneity, the I2 test was used. The random effects were applied, and the fixed model was evaluated as sensitivity analysis. To assess risk of bias and certainty of evidence, the tools ROBINS-I and GRADE were used, respectively. Registration number: CRD42020199667. Results: Sixteen articles were selected, encompassing 350 patients. The mean age ranged from 36 to 61 years old, and the mean follow-up ranged from 16 to 109 months. Complications rate was 0.08 (CI: 0.040–0.153; P  = 0.01). Need for retreatment rate was 0.128 (CI: 0.031–0.409; P  = 0.01). The probability of good or excellent outcomes after myotomy was 0.762 (CI: 0.703–0.812; P  < 0.01). Postoperative mortality rate was 0.008 (CI: 0.004–0.015; P  < 0.01). Conclusion: Surgical myotomy is an option for avoiding esophagectomy in achalasia, with a low morbi-mortality rate and good results. It is effective for most patients and only a minority will demand retreatment. [ABSTRACT FROM AUTHOR]

Objectives: Although histologic features in biopsies suggesting a possibility of achalasia would be helpful diagnostically, such features remain unknown. The goal of this study was to explore the prevalence, histologic features, and immunophenotype of lymphocytic esophagitis (LyE) in achalasia biopsies.Methods: The study group consisted of 57 patients with achalasia. Controls comprised 52 patients with severe gastroesophageal reflux disease (GERD) and normal esophageal motility. CD4/CD8 immunophenotype of lymphocytes was analyzed by immunohistochemistry.Results: LyE was identified in 30% (17/57) of patients with achalasia and 6% (3/52) of patients with GERD, indicating a strong association with achalasia (odds ratio, 6.94; 95% confidence interval, 1.90-25.38). LyE was focal in 59% (10/17) of the cases and diffuse in 41% (7/17). CD4 T-cell predominance over CD8 T cells was observed in 88% of patients with achalasia and LyE. T helper 1 (Th1) cells, but not T helper 2 cells, were expanded in CD4 T cells; in the absence of evident infection, this was compatible with the role of Th1 cells in organ-specific autoimmunity.Conclusions: Achalasia should be considered in the differential diagnosis of clinical entities associated with CD4-predominant LyE. Additional studies to explore the significance of Th1 cells in achalasia-associated LyE are warranted. [ABSTRACT FROM AUTHOR]

Esophageal strictures commonly cause dysphagia and require treatment with endoscopic dilation using balloons or bougies. We aimed to determine whether biopsy forceps disruption of strictures at time of dilation increases time to repeat intervention or duration of intervention-free follow-up. We performed a retrospective analysis of 289 adults (age 61.0 ± 0.8 years, 66.4% female) who underwent dilation of an esophageal stricture at our tertiary care center between 2014 and 2016. Exclusions consisted of endoscopic intervention within the preceding 6 months, prior foregut neoplasia, achalasia, radiofrequency ablation, endoscopic mucosal resection, endoscopic submucosal dissection, or foregut surgery. Demographics, clinical presentation, dilation technique, and follow-up were abstracted from electronic medical records. We compared time to repeat dilation and duration of intervention-free follow-up between treatment subgroups. Balloon dilation was performed more often than bougie dilation (76.8 vs. 17.6%); biopsy forceps disruption was performed in 23.2%. Over a median follow-up of 52.9 months, 135 patients (46.7%) underwent repeat dilation. Age, body mass index, gender, and use of antisecretory medications did not influence need for repeat dilation (P  = ns for each). Bougie dilation with biopsy forceps disruption prolonged time to repeat dilation in all patients (P  ≤ 0.02), particularly in those with gastroesophageal reflux disease (P  ≤ 0.03), compared with bougie dilation alone and balloon dilation with or without disruption. On Kaplan–Meier analysis, bougie dilation with biopsy forceps resulted in longer intervention-free follow-up compared with dilation alone (P  = 0.03). We conclude that stricture disruption with biopsy forceps increases time to repeat intervention with bougie but not balloon dilation. [ABSTRACT FROM AUTHOR]

Per oral endoscopic myotomy (POEM) is a safe and effective treatment for esophageal motility disorder in treatment-naïve patients as well as salvage therapy. Though type II achalasia, compared to other subtypes, is reported to have a more favorable outcome with pneumatic dilation (PD) or Heller myotomy (HM), it is unclear whether achalasia subtype predicts symptom response to POEM. We aimed to evaluate whether type II achalasia is associated with a comparably favorable outcome following POEM. We performed a retrospective review of patients with esophageal motility disorder who were referred for POEM from April 2014 to June 2017. The main outcome was clinical success based on Eckardt score ≤3 and its association with subtype and safety. A total of 63 patients (mean age 51 years [SD 15]; 63% male) underwent a total of 68 POEMs with median of 263 days follow-up. Of these, 45 (71.3%) patients were type II achalasia. In all, 29 (46%) patients were treatment-naïve and 34 (54%) patients had previous endoscopic or surgical therapy including botulinum toxin injection in 16 (25%), PD in 10 (16%), both botulinum toxin injection and PD in 8 (13%) and HM in 3 (5%). Technical success was 100% and clinical success was achieved in 51 (81%) patients. The rate of clinical success was higher in patients with type II achalasia compared to the other subtypes (88.9% vs. 61.1% [ P  = 0.028]) and type II achalasia patients required fewer redo POEM (2.2% vs. 22.2% [ P  = 0.021]). Multivariate logistic regression analysis demonstrated the positive prediction of clinical success for type II achalasia following POEM (P  = 0.046). As observed with PD and HM, type II achalasia was associated with a favorable clinical outcome following POEM. [ABSTRACT FROM AUTHOR]

The Chicago Classification of esophageal motility disorders improved the differentiation of achalasia subtypes and tailored treatment. Heller myotomy (HM) and pneumatic dilation are two established treatments for achalasia. Peroral endoscopic myotomy (POEM) has become a third definitive option and is on the rise. Using the National Inpatient Sample (NIS) database, we evaluated patients hospitalized with achalasia and associated surgical and endoscopic interventions from 2013 to 2017 and compared patients undergoing HM versus POEM. The NIS database was queried to include patients with achalasia. Patients who underwent HM, POEM (only 2017 due to lack of distinct procedure code in 2013), pneumatic dilation, or esophagectomy were identified. Adverse events during the hospitalization were also queried using diagnosis codes. From 2013 to 2017, patients hospitalized with achalasia increased from 16 850 to 19 485. There were reductions in the number of esophageal dilations (10.6–5.4%, P  < 0.001) and HM (18.7–13.1%, P  < 0.001). In 2017, 580 POEMs were performed. Compared with patients undergoing HM in 2017, patients who had POEM had higher mean age > 64 (P  = 0.004), Charlson comorbidity index (P  < 0.001), disease severity (P  < 0.001), and likelihood of mortality (P  < 0.001). There were no differences in length of stay, mortality, or total costs between the HM and POEM groups. Patients hospitalized with achlasia increased from 2013 to 2017, possibly due to the growth and accessibility of high-resolution esophageal manometry. As expertise in POEM increases, the number of POEM performed is anticipated to rise with possible further reductions in other treatment modalities for achalasia. [ABSTRACT FROM AUTHOR]

Peroral endoscopic myotomy (POEM) is a novel minimally invasive intervention, which has shown to be effective and safe for treating achalasia in adults. Presently, POEM was also reported to be effective for achalasia in children. So we conducted this study to explore the clinical outcomes of POEM for pediatric achalasia. A systematic literature search in PubMed, Embase, and Cochrane databases was performed, which covered the period from January 2009 to June 2020. Selecting studies and collecting data was independently by two reviewers according to predefined criteria. The statistical analysis was carried out using Comprehensive Meta-Analysis software version 2 and Review Manager 5.3. A total of 11 studies with 389 children were identified in the final analysis. Pooled technical success of POEM treatment achalasia was achieved in 385 children (97.4%; 95% confidence interval [CI], 94.7%–98.7%), and the pooled clinical success was achieved in 348 children (92.4%; 95% CI, 89.0%–94.8%). After POEM, the Eckardt score was significantly decreased by 6.76 points (95% CI, 6.18–7.34, P  < 0.00001), and the lower esophageal sphincter pressure was significantly reduced by 19.38 mmHg (95% CI, 17.54–21.22, P  < 0.00001). The pooled major adverse events rate related to POEM was 12.8% (95% CI, 4.5%–31.5%) and the gastroesophageal reflux rate was 17.8% (95% CI, 14.2%–22.0%). Our current study demonstrated that the POEM was an effective and safe technique for treating achalasia in children. Further randomized comparative studies of POEM and other therapeutic methods are warranted to determine the most effective treatment modality for achalasia in children. [ABSTRACT FROM AUTHOR]

Peroral endoscopic myotomy (POEM) has been increasingly accepted as the first-line treatment of achalasia. However, laparoscopic Heller myotomy or esophagectomy still remains as the mainstay treatment for advanced achalasia featured with megaesophagus and/or sigmoid-shaped esophagus. Although the efficacy of POEM for advanced achalasia with sigmoid-shaped esophagus has been described, little is known about the role of POEM for patients with megaesophagus. The aim of our study is to evaluate the efficacy and safety of POEM for advanced achalasia with megaesophagus. Patients who received initial examinations and underwent POEM in our hospital from June 2013 to December 2018 were identified and data were retrospectively analyzed. The advanced achalasia was defined as megaesophagus with a diameter of ≥6 cm. The primary outcome was clinical success. The secondary outcomes were technical success and adverse events. A total of 112 patients (50 females, 44.6%) were included with a mean age of 44.8 years. The median symptom duration was 6.5 years (IQR:3.0–13.0 years). Modified POEM techniques were used in 27.7% (31/112) of patients. Technical success rate was 99.1% (112/113) per procedure. Clinical success was seen in 93.1% patients with median Eckardt score decreasing from 8.0 to 1.0 (P  < 0.001) during a median 31.0 months of follow-up. The mean LES pressure decreased from 29.5 mmHg to 14.2 mmHg after POEM (P  < 0.05). Procedure-related adverse events occurred in 8.9% patients. Reflux disease was observed postoperatively in 26.7% of patients. POEM is also indicated for patients with advanced achalasia with a favorable safety and efficacy. [ABSTRACT FROM AUTHOR]

Background In the last 3 decades, laparoscopic Heller myotomy (LHM) has represented the treatment of choice for esophageal achalasia (EA), solving symptoms in most patients. Little is known about the fate of patients who relapsed after LHM, or about their most appropriate treatment. In this study we aimed at evaluating the results of complementary pneumatic dilations (CPD) after ineffective LHM. Methods We evaluated the patients with EA who underwent LHM plus Dor fundoplication (LHD) from 1992 to 2021 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) > 3 was used as threshold for failure both after LHD and after LHD + CPD. Results Out of 1420 patients undergoing LHD in the study period, 115 (8.1%) were considered failures and were offered CPD. Ten patients refused further treatment, in 5 CPD was not indicated for severe reflux esophagitis, 1 patient had surgery for a misshaped fundoplication and 1 last patient developed a cancer 2 years after LHD; that leaves 103 patients who underwent a median 2 CPD (IQR:1–3), at a median of 16 (IQR:8–36) months after surgery, with 3.0 to 4.0 cm Rigiflex dilator. No perforations were recorded. Only 6 patients were lost to follow up. The remaining 97 were followed for a median of 37 months (IQR:6–112) after the last CPD: 70 saw their symptoms healed, whereas 27 still complained of symptoms (ES > 3). The only differences between the 2 groups were the ES before CPD, that was 3 (IQR:3–4) in the former and 4 (IQR:4–5) in the latter (p < 0.05), and the number of required CPD, that was 2 (IQR:1–2) and 3 (IQR:1–3), respectively (p < 0.05). All other parameters were similar between the 2 groups. Of the un-responding patients, 17 still require repeated CPD, 7 underwent re-myotomy, 1 POEM and 1 esophagectomy for end-stage disease. Conclusion CPD represent an effective and safe option to treat patients after a failed LHD: when the post-surgery ES consistently remains high, and the number of CPD exceeds 3, this may suggest the need for further invasive treatments. [ABSTRACT FROM AUTHOR]

Background Esophageal perforation is the most feared complication after any intervention in achalasia cardia, especially after pneumatic dilation. We present a case of contained perforation after manometry in a long-standing case of achalasia cardia and its successful management. Methods A 64-year-old lady, known case of long-standing Rheumatoid arthritis on steroids and methotrexate, presented to us with complains of dysphagia to solids and liquids and 15 kgs weight loss in the past 3 years. On evaluation, she was dehydrated and cachexic. Upper GI endoscopy showed lot of food residue with erosions in body of esophagus and tight LES. Barium swallow showed sigmoid esophagus with typical bird beak appearance. Patient was admitted for hydration and nutritional optimization was done with nasojejunal feeding. She was further subjected to manometry to characterize the achalasia, which couldn't be completed despite multiple forceful attempts. After 5 days, she was admitted for elective surgery. A CT scan of chest was done to evaluate the anatomy thoroughly before surgery, which showed contained mucosal perforation in lower esophagus on right side with minimal right pleural effusion. We planned for surgery next day. Results Laparoscopic Heller's cardiomyotomy with Dor's fundoplication was done with thoracoscopic lavage. Intra-operatively, upper GI endoscopy with minimal insufflation was done to confirm adequate cardiomyotomy and to look for the site of perforation. The perforation was not found. Hence, right thoracoscopy was done, which showed reactive pleural effusion without any food contents. Lavage was given and ICD was placed. Feeding jejunostomy was done laparoscopically. Patient was kept NBM for 7 days and JT feeds were started on POD 1 and gradually increased. ICD removed on POD 7. Oral gastrograffin contrast study was done on POD 8, which showed delayed passage at 5 minutes and no extravasation. Oral liquids were started and escalated gradually. Supplemental high protein nutrition was given through JT feeding. Patient was discharged on POD 15. At 1 month follow up, patient had 2 kgs weight gain and tolerated semisoft diet adequately. Conclusion Successful management of contained perforation in long standing achalasia cardia was achieved by targeting the source pathology in an already immunocompromised patient. [ABSTRACT FROM AUTHOR]

Achalasia is a primary motor disorder of the oesophagus. End-stage achalasia is characterised by a grossly dilated sigmoid-shaped oesophagus with food stasis leading to chronic inflammation. Despite treatment, approximately 5% of patients progress to this late stage, needing oesophagectomy. Current guidelines recommend oesophagectomy as the last resort after failure of first-line endoscopic treatment or cardiomyotomy. We review a case of a 70 years old female with end-stage achalasia who successfully underwent cardiomyotomy but required a looser wrap for oesophageal emptying. Patient first presented in her 50s with dysphagia and Oesophago-Gastro-Duodenoscopy (OGD) showed a dilated oesophagus with food stasis and no contractions. Barium swallow confirmed the diagnosis of achalasia. She underwent pneumatic dilatations with minimal symptom improvement and eventually defaulted follow-up. Several years later she presented with haematemesis due to severe oesophagitis and Eckardt score of 7. Both OGD and CT scans showed a grossly dilated megaoesophagus. High-resolution manometry confirmed Type I achalasia with IRP (integrated relaxation pressure) 25.8 and complete absence of peristalsis. She was initiated on nasogastric feeding and was also diagnosed with pulmonary embolism (PE). In view of PE and patient's comorbidities, she was not fit for oesophagectomy. Peroral endoscopic myotomy (POEM) was also deemed challenging in the presence of severe, chronic oesophagitis and patient's need for anticoagulation with potential difficulty in endoscopic haemostasis. She underwent laparoscopic cardiomyotomy with anterior 180-degree fundoplication. However, post-operation contrast swallow showed hold up of contrast despite intraoperative OGD showing smooth passage of scope through LES. Decision was made for revision of fundoplication to anterior 90-degree to further promote oesophageal emptying. Post-operation contrast swallow showed satisfactory drainage into stomach and patient tolerated post-fundoplication diet regime. This case illustrates the role of cardiomyotomy as first-line in patients with end-stage achalasia who are not fit for oesophagectomy. However, an anterior 180-degree fundoplication may not allow adequate drainage from a megaoesophagus and a less tight wrap should be considered in such cases. Post-operation, reported Eckardt score of our patient was 1 with no reflux symptoms, indicating marked alleviation of symptoms. Thus, cardiomyotomy with a lesser 90-degree fundoplication is feasible as first-line treatment in end-stage achalasia. [ABSTRACT FROM AUTHOR]

Achalasia Quality of Life (ASQ) and Eckardt scores are two patient-reported instruments widely used to assess symptom severity in achalasia patients. ASQ is validated and reliable. Although Eckardt is commonly used, it has not been rigorously assessed for validity or reliability. This study aims to evaluate (i) the accuracy of Eckardt and ASQ for assessing improvement post-treatment (predictive validity), (ii) accuracy of Eckardt and ASQ for assessing improvement post-treatment with pneumatic dilatation (PD) versus surgical myotomy (predictive validity), and (iii) convergent validity of Eckardt and ASQ tools. Patients with achalasia treated between 2011 and 2018 were eligible. Both instruments were administered by telephone. Treatment failure was determined by the review of medical records by two clinicians. The predictive ability of ASQ and Eckardt instruments in identifying treatment successes and failures was determined using receiver operating characteristics analysis and summarized as area under the curve (AUC). A total of 106 patients met inclusion criteria with 39 PD, 51 Heller myotomy, and 16 per-oral endoscopic myotomy. A review of medical records and esophageal testing revealed 13 failures (12%). AUC for Eckardt was 0.96 (95% confidence interval [CI] 0.87–0.99] and ASQ 0.97 (95% CI 0.92–0.99). The Eckardt cutoff 4, and ASQ, cutoff 15, were 94% and 87% accurate in identifying treatment successes versus failures, respectively. The correlation coefficient between the two tools was 0.85. In conclusions, (i) ASQ and Eckardt scores are valid and reliable tools to assess symptom severity in achalasia patients, (ii) both instruments accurately classify treatment successes versus failures, and (iii) the choice of tool should be informed by the physicians and patients' values and preferences and repeat physiologic testing may be reserved for treatment failures with either instrument and patients classified, as treatment successes may be spared routine physiologic testing in the long term. [ABSTRACT FROM AUTHOR]

Background There is evidence that idiopathic achalasia has an autoimmune component and a significant association with several autoimmune comorbidities has been described. However, data regarding the prevalence of autoimmune diseases in achalasia are not well established, and few studies have explored this association. Objective Our primary aim was to prospectively investigate the type and frequency of autoimmune comorbidities in a large cohort of consecutive achalasia patients. Our secondary aim was to investigate the effects of autoimmune comorbidities on achalasia phenotype (clinical features and manometric pattern). Methods The study population consisted of 375 consecutive patients (215 females—median age 55 ± 17 years), referred at our tertiary referral center from January 2008 to January 2018, with clinical and instrumental (EGDS, barium esophagogram, and manometry) diagnosis of idiopathic achalasia. Gender- and age-matched subjects undergoing manometry and pH-impedance monitoring for typical gastroesophageal reflux (GERD) complaints served as controls. In all patients a detailed history taking was carried out, recording the presence and type of autoimmune comorbidities. Results The overall prevalence of autoimmune comorbidities was two times higher in achalasia than in control patients (12.3 vs. 5%, respectively). The presence of comorbidities did not significantly affect disease's phenotype, as the age of disease onset was similar in achalasia patients with and without comorbidities (50.13 ± 14.47 and 48.3 ± 18.71, respectively, P  = NS). Conclusions Although larger epidemiologic studies are needed to confirm our data, our findings likely suggest that achalasia has a complex multifactorial pathophysiology with an autoimmune component. [ABSTRACT FROM AUTHOR]

Purpose To report a novel approach for locally advanced tumors located at the gastroesophageal junction (GEJ) using a laparoscopic abdominal phase and open left thoracotomy with the patient in a single right lateral decubitus position. Background The standard open left thoracoabdominal approach offers excellent exposure and access to the GEJ and lower esophagus. It also involves a single position for the procedure, shortening the operation time. The disadvantages are a large incision, division of the costochondral junction, and a low-level thoracotomy. The laparoscopic-assisted left thoracoabdominal esophagectomy (LLTA) is performed with the patient in the same right lateral decubitus position, but initially rolled away from the operator at 45° allowing laparoscopic gastric mobilization and lymphadenectomy. The patient is then tilted back to the lateral position for the thoracic phase. An anterolateral left thoracotomy is performed through the higher fifth intercostal space allowing a high intrathoracic anastomosis, just below the aortic arch. No disruption of the costochondral junction is made. Methods Consecutive patients selectively treated for locally advanced GEJ tumors with an LLTA approach between 2013 and 2019 were analyzed and compared to national standards (NOGCA). Results This series of 74 consecutive patients had a mean age of 63 years. The median operation time was 235 minutes. The median inpatient stay was 10 days (NOGCA 9 [11–17]). The tumors were predominantly adenocarcinoma (95%) and located at the GEJ (92%). The majority were locally advanced T3 or T4 tumors. Postoperative morbidity was low, Clavien–Dindo (C–D) 0 in 52.7% patients, C–D1 (1.4%), C–D2 (31.1%), C–D3a (5.4%), C–D4a (9.5%), and C–D5 (1.4%). The median number of total lymph nodes (LN) excised was 28 (NOGCA >15); LN % yield ≥18 was 90% (NOGCA 82.5%). Positive nodes were located at the lesser-curve (40%), paraesophageal (32.4%), and subcarinal regions (2.7%). Positive circumferential resection margins (<1 mm) were present in 28.4% of resected specimens (NOGCA 25.1%). This is reflective of the high proportion T3/T4 tumors selected for this approach. Hospital and 30-day mortality was 1.4% (NOGCA 2.7%). Recurrence after LLTA was 25.7% (local 5.4%, systemic 17.6%, mixed 2.7%) at a median of 311 days (62–1,158). Conclusion This series demonstrates a novel, safe, and reproducible approach for locally advanced cancer of the GEJ. It offers a better exposure of the hiatus than the right-sided approach and avoids division of the costochondral junction and low thoracotomy seen with the open left thoracoabdominal approach. [ABSTRACT FROM AUTHOR]

Introduction: Alacrimia, achalasia cardia, and adrenocorticotrophic hormone (ACTH)-resistant adrenal insufficiency make up the triple-A syndrome. It is an uncommon disease with an autosomal recessive inheritance pattern. The AAAS gene, which codes for the ALADIN protein and is located on chromosome 12q13, is the cause of Allgrove syndrome. The initial and typical symptoms are achalasia cardia and adrenal insufficiency. The majority of neurological disorders have autonomic signs, and they typically show in later life. The other typical neurological conditions are polyneuropathy, amyotrophy, and optic atrophy. Clinical Case: A 25-year-old female patient presented to our clinic with complaints of weakness, dizziness, and hyperpigmentation of the skin. Her medical history revealed a recent intensive care unit (ICU) stay due to hypotension, during which her condition was managed with steroid therapy. However, she did not continue the steroid treatment after discharge. Notably, the patient had a family history of achalasia with operative intervention in all three siblings. The patient and her family members were also under the care of a gastroenterology specialist. Additionally, the patient reported experiencing dry eyes, for which she was using artificial tears. Physical examination revealed hyperpigmentation of tongue, gums and buccal mucosa. Upon examination, her baseline investigations revealed a hemoglobin of 11.4 g/dL, sodium 137 mmol/L and potassium 4.0 mmol/L. The patient's adrenocorticotropic hormone (ACTH) level was measured at 1250 pg/ml, with a basal cortisol level of 0.5 ug/dL, leading to the diagnosis of adrenal insufficiency. Hydrocortisone treatment was initiated at a dosage of 2×10mg, resulting in a decline of the ACTH level to 31. Given the combination of symptoms including achalasia, alacrima, and adrenal insufficiency, genetic testing was conducted. Both alleles revealed a homozygous c.1432C>T (p.Arg478*) mutation, confirming the diagnosis of Triple A Syndrome. Following the genetic diagnosis, a familial investigation was initiated. This investigation revealed the presence of the homozygous positive mutation in two siblings and two cousins. The constellation of symptoms and the genetic findings confirmed the occurrence of Triple A Syndrome within this family. Conclusion: This case highlights the significance of recognizing Triple A Syndrome as an underlying cause of adrenal insufficiency in the presence of achalasia, alacrima, and a suggestive clinical history. In order to confirm the diagnosis and identify any affected family members, genetic testing is crucial. To avoid potential complications brought on by adrenal insufficiency and other systemic Triple A Syndrome manifestations, early identification and treatment are crucial. [ABSTRACT FROM AUTHOR]

Minimally invasive Heller myotomy is considered the gold standard surgical approach for symptomatic achalasia because it is a safe and effective procedure. Over the last years, several studies comparing the laparoscopic and robotic approach for Heller myotomy have been published. Although the robotic approach appears to have some advantages over standard laparoscopy, data on this topic are still controversial and no definite conclusions have been drawn. This metanalysis has been designed to systematically evaluate and compare the effectiveness and safety of the robot-assisted Heller myotomy as compared to the standard laparoscopic approach. According to Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, a systematic search on both laparoscopic and robotic Heller myotomy was performed in all the major electronic databases (PubMed, Web of Science, Scopus, EMBASE), using the following search string: (achalasia OR Dor) AND robotic. Six articles were included in the final analysis. A metaregression analysis was performed to assess the possible effects of demographic variables (age, gender, body mass indes (BMI)) and previous abdominal surgery or endoscopic intervention on the analyzed outcomes. No statistical difference was observed in operative times (mean difference (MD) = 20.79, P = 0.19, 95% confidence interval (CI) −10.05,51,62), estimated blood loss (MD = −17.10, P = 0.13, 95% CI −40.48,5.08), conversion rate to open surgery (risk difference (RD) = −0.01, P = 0.33, 95% CI −0.05,0.02), length of hospital stay (MD = −0.73, P = 0.15, 95% CI −1.71,0.25) and long-term recurrence (odds ratio (OR) = 0.59, P = 0.45, 95% CI 0.15,2.33). On the contrary, the robotic approach was found to be associated with a significantly significant lower rate of intraoperative esophageal perforations (OR = 0.13, P < 0.001, 95% CI 0.04, 0.45). Our results suggest that the robotic approach is safer than the laparoscopic Heller myotomy, encouraging the use of robot-assisted surgery. However, our analysis is limited because of the exiguous number of comparative studies and because most of the included studies were statistically underpowered, given the small sample size. Moreover, a high degree of heterogeneity was observed in most of published studies. Taking in consideration the additional costs of robot-assisted procedures, larger Randomized Controlled Trials (RCTs) are advocated to confirm the safety and effectiveness of the robotic approach, and its advantages over standard laparoscopic surgery. In conclusion, well-designed prospective trials and RCTs with homogeneous parameters are needed to draw definitive conclusions about the best surgical approach to pursue in treating symptomatic achalasia. [ABSTRACT FROM AUTHOR]

During peroral endoscopic myotomy (POEM), creation of the tunnel is highly technically demanding and mucosal injury is one of the most common potential complications. We explored a method without a submucosal tunnel, which we call open peroral endoscopic myotomy (O-POEM). This study aimed to assess the feasibility and safety of O-POEM. O-POEM was performed on 82 patients with achalasia. Treatment success was defined as an Eckardt score of less than or equal to 3 after the myotomy. Adverse events including operative and postoperative adverse events were recorded. Treatment success and procedure-related adverse events were analyzed. After a median follow-up of 18 months (range: 6–26 months), the treatment success (Eckhart score ≤3) was achieved in 96.3% of cases (mean score pre- vs. post-treatment (7.4 vs. 1.8); P < 0.001) with a recurrence of 3 cases. Ten patients (12.2%) had adverse events consisting of 2 cases of mediastinitis, 1 case of post-O-POEM bleeding, 1 case of subcutaneous emphysema, 6 cases of pleural effusion. Two cases of mediastinitis required intraprocedural drainage, and other patients were managed by endoscopy and conservative medical treatment. There were no deaths. No patients required surgical conversion. Clinical reflux occurred in 15.9% of patients (13/82). O-POEM was reliable and effective for the treatment of achalasia. In addition, O-POEM might be a better option for patients with severe submucosal fibrosis. [ABSTRACT FROM AUTHOR]

View large Download slide View large Download slide OBJECTIVES The risk factors for oesophageal achalasia, a precancerous condition that can lead to epidermoid carcinoma, are unknown. The aim of this study was to determine these factors. METHODS Beginning in 1973, patients presenting with achalasia from 1955 to 2016 periodically underwent clinical assessment, the barium swallow test (the oesophageal diameter and residual barium column were measured) and endoscopy according to a prospective protocol. We included patients with the minimum follow-up duration of 12 months. RESULTS Of 681 cases, 583 patients were considered. The median follow-up time was 147.13 months (interquartile range 70.42–257.82 months), and 17 epidermoid carcinomas and 1 carcinosarcoma were diagnosed. After excluding 4 achalasia patients admitted with a cancer diagnosis, the incidence rate of epidermoid carcinomas was 1.61/1000 persons-year, and the cumulative probability of developing cancer at 56.34 years of follow-up was 13%. Risk factors for cancer were a sigmoid oesophagus [risk ratio (RR) = 17.64, 95% confidence interval (CI) 4.13–75.43], duration of achalasia symptoms >280 months (RR = 19.62, 95% CI 4.59–83.80), duration of follow-up >353 months (RR = 5.96, 95% CI 2.50–14.18), oesophageal diameter at diagnosis >71 mm (RR = 21.07, 95% CI 9.29–47.82), residual barium column at diagnosis >23 cm (RR = 24.27, 95% CI 6.93–85.01) and residual barium column at the last follow-up >10 cm (RR = 8.15, 95% CI 2.40–27.62). Conversely, the risk of epidermoid carcinoma was lower when the residual barium swallow decreased by >57% (RR = 0.10, 95% CI 0.03–0.34). CONCLUSIONS Patients with achalasia carry a substantial risk of developing epidermoid carcinoma. Several factors, such as sigmoid achalasia and dysphagia lasting more than 23 years, are associated with an increased risk of cancer. An effective Heller myotomy may positively influence the carcinogenetic process. [ABSTRACT FROM AUTHOR]

Background: The introduction of high‐resolution manometry and the Chicago classification has made it possible to diagnose achalasia and predict treatment response accurately. The aim of this study was to compare the effect of the different treatments available on symptomatic outcomes across all achalasia subtypes. Methods: The study was conducted according to PRISMA and MOOSE guidelines. A literature search of PubMed and MEDLINE databases was undertaken to identify all relevant articles reporting clinical outcomes of patients with achalasia after botulinum toxin injection, pneumatic dilatation, laparoscopic Heller myotomy (LHM) and peroral endoscopic myotomy (POEM) based on manometric subtypes. Patients were grouped according to the Chicago classification and the success rate in treating symptoms was measured as the primary endpoint. Results: Twenty studies (1575 patients) were selected, and data on botulinum toxin, pneumatic dilatation, LHM and POEM were extracted. Success rates for LHM in type I, II and III achalasia were 81, 92 and 71 per cent respectively. Those for POEM were 95, 97 and 93 per cent respectively. POEM was more likely to be successful than LHM for both type I (odds ratio (OR) 2·97, 95 per cent c.i. 1·09 to 8·03; P = 0·032) and type III (OR 3·50, 1·39 to 8·77; P = 0·007) achalasia. The likelihood of success of POEM and LHM for type II achalasia was similar. Conclusion: Pneumatic dilatation had a lower but still acceptable success rate compared with POEM or LHM in patients with type II achalasia. POEM is an excellent treatment modality for type I and type III achalasia, although it did not show any superiority over LHM for type II achalasia. Good response rates but longer follow‐up needed [ABSTRACT FROM AUTHOR]

Background The presence of intraepithelial eosinophils (IE) was described in patients with esophageal motor disorders (EMD). Conversely, EMD and even achalasia were reported in patients with eosinophilic esophagitis (EoE). The purpose of this study was to further investigate this possible association. Methods From February to July 2022, patients with achalasia undergoing laparoscopic myotomy were evaluated. Patients who had previous treatment were excluded. Immediately before the operation, the patients underwent endoscopy with the addition of 4 biopsies from the proximal, middle and distal esophagus and from the Z line, respectively. Two additional samplings of the esophageal muscle were taken while performing the myotomy. The specimens were stained with H&E in order to highlight the presence of eosinophils and the number of IE eventually present in a high-power field (hpf) was recorded. Patients were re-evaluated 6 months after the operation. Results A total of 30 patients with a median age of 45.5 years were studied. Their preoperative ES was 6 (5–7) and the median duration of symptoms was 42 months (12–120); there were 18 stage I, 9 stage II, 2 stage III and only one stage IV, respectively; manometric subtype 1 was found in 7 patients, subtype 2 in 18 and subtype 3 in 5. There were no complications. All the patients had a good outcome and the median ES was 0 (0–1). IE were found in 6 patients (20%) [median 6/hpf (2–10)]. Only one patient met the pathologic criteria for EoE, whereas only one additional showed eosinophils in the muscular specimen. No differences in preoperative and postoperative data were found between patients with and without IE, but the maximum diameter of the gullet was narrower in the former than in the latter (3.24 cm vs 22.50 cm, respectively, p < 0.05). Conclusions EoE and achalasia are 2 of the commonest causes of dysphagia and an association between them was recently hypothesized. Our study confirmed that IE are present in some patients with achalasia, but seldom meeting the criteria for EoE. Only a narrower esophagus was found in patients with IE as compared to those without, possibly reflecting an earlier stage of the disease. [ABSTRACT FROM AUTHOR]

Background Achalasia is an incurable neurodegenerative disease of the esophagus. Palliative treatment includes disruption of the lower esophageal sphincter via multiple techniques, including open Heller myotomy (OHM), laparoscopic Heller myotomy (LHM), and per-oral esophageal myotomy (POEM). There is a paucity of data comparing long-term outcomes between OHM, LHM, and POEM. We sought to evaluate the relationship of these methods with clinical outcome parameters using our institution's experience. Methods Patients greater than 3 years removed from myotomy for achalasia were studied. Post-operative Eckardt Symptom (ES) score and daily proton pump inhibitor (PPI) usage was recorded. Patients underwent timed barium esophagram (TBE) as clinically indicated. Retention was defined as a persistent column >5 cm at 5 minutes. Post-operative interventions included Savary dilation, Botox injection, pneumatic dilation, surgical myotomy, and esophagectomy. Groups were stratified by surgery type (OHM, LHM, POEM) and by time since surgery (3–6 years, 6–10 years, >10 years). A logistic regression analysis was performed examining the relationship of surgery type, time, symptomatic/objective outcomes, and post-operative interventions. Results 172 patients had long-term data available. Mean follow up was 7.8 years (3.1–19.5 years). Nearly one-third of patients had a post-operative intervention (31%, 53/172). On regression analysis, OHM was associated with a higher risk of intervention (OR 9.4, p < 0.05). Barium retention was significantly associated with LHM (OR 15.9, p < 0.05) and the 3–6 year post-operative period (OR 11.1, p < 0.05). PPI usage was not associated with surgery type, but was associated with the 3–6 year post-operative period (OR 3.2, p < 0.05). There was no significant association of dysphagia with surgery type or time. Conclusion Post-operative barium retention and interventions are associated with specific myotomy techniques, but not time. PPI usage is associated with the early post-operative period, but not technique. Neither myotomy technique nor time alone are sufficient to predict risk of abnormal ES or dysphagia. [ABSTRACT FROM AUTHOR]

Background Patients with sigmoid shape have an advanced form of the disease and are considered the most difficult to treat. A modified technique (pull-down) has been proposed to straighten the esophageal axis, but there is a limited amount of data available in literature. In this study, we aimed to compare the final outcome of the pull-down technique (PDLHD) with the results of classical myotomy (CLHD) in patients with end-stage achalasia. Methods From 1995–2022, patients with a radiological diagnosis of end-stage achalasia undergoing laparoscopic myotomy were enrolled in the study. Symptoms were quantified using the Eckardt score. Barium-swallow, endoscopy and manometry were performed before and after the treatment. Treatment failure was defined as the persistence or reoccurrence of an Eckardt score > 3, or the need for retreatment. Results Of the 94 patients with end-stage achalasia (M:F = 52:42), 60 patients were treated with CLHD, and 34 patients with PDLHD. The surgical procedures were completed laparoscopically in all patients. During follow-up, two patients (2.1%) developed squamous cell carcinoma (SCC), both in the CLHD group. All patients in both groups had an improvement in their Eckardt score after surgery, and the failure rates were 27.6% (16/58) after CLHD and 5.9% (2/34) after PDLHD (p < 0.01). Amongst the patients who underwent complete post-operative follow-up, an abnormal acid exposure was detected in 2 patients after PDLH, and in 6 after CLHD (p = n.s.). Conclusions Taken into account the intrinsic limitations of the study (different time window, and different follow-up), the results of this study indicate that performing the pull-down technique during Laparoscopic Heller-Dor improves the final outcome in end-stage achalasia patients. [ABSTRACT FROM AUTHOR]

Background Peroral endoscopic myotomy (POEM) is standard treatment for achalasia. Gastroesophageal reflux disease (GERD) after POEM has been a limiting factor with this procedure. Preservation of the sling fiber during POEM was reported to reduce post-POEM GERD in Japan, but there are no reports of this technique in a western population. As such, we investigated the association of sling-fiber preservation during POEM and post-POEM GERD symptoms at our institution, which is a large therapeutic endoscopy referral center in Canada. Methods This is a retrospective, single-center study of patients who underwent POEM from October 2017 to January 2023 at our center. The initial cohort of patients were treated by conventional POEM until June 2021, after which a second cohort underwent POEM with sling-fiber preservation, as the techniques advanced. The primary outcome was the rate of positive GERD symptoms after POEM. The secondary outcomes were procedure time, gastric myotomy length, clinical success rate (Eckard score of less than 3), adverse events rate and use of PPI at follow-up. Results 148 POEM cases (52.5 ± 15.6 y/o, female: 61(43%)) were included in this study. There was no significant difference in patient characteristics between the groups. The mean procedure time (108.6 ± 34.5 vs 109.1 ± 45.7 min, P = 0.93) and rate of adverse events (21% vs 14%, P = 0.36) were similar between the traditional and modified groups. In the sling fiber preservation group, gastric myotomy length was significantly longer (2.2 ± 0.7 vs 1.6 ± 0.8 cm, P < 0.05) yet the GERD symptom rate at follow-up was significantly lower (22% vs 41%, P < 0.05), although PPI use was similar (57% vs 50%, P = 0.47). Finally, the clinical success rate was similar between groups (88% vs 84%, P = 0.6). Conclusions Sling fiber preservation during POEM is safe and reduces post-POEM GERD symptoms, despite the longer gastric myotomy length. As such, sling fiber preservation may be a useful solution to reduce post-POEM GERD in western populations. [ABSTRACT FROM AUTHOR]

Background The clinical manifestation of achalasia is nonspecific (weight loss, vomiting); particularly, making an early diagnosis is often difficult. It often remains unrecognized for a long period or can be initially misdiagnosed as an eating disorder, such as anorexia nervosa, whose main clinical features mimic achalasia. Additionally, several overlooked patients with esophagogastric junction outflow obstruction (EGJOO), including achalasia, are should be identified in the community population. Methods 1. We collected data from 38 patients with achalasia and examined them for diagnostic factors that could lead to a delayed diagnosis. 2. The data of a cumulative total of 489,823 hospital visits at a tertiary medical center were collected. We selected patients with the given suspected diagnoses that could be misdiagnosed, and investigated the kind of imaging tests used in them. Among them, we selected patients with symptoms persisting for >6 months, without a definitive diagnosis, and in whom upper endoscopy, but not barium swallow test, was performed. Subsequently, we performed barium swallow tests in these patients. Results 1. The most important factor that caused a delay in the diagnosis of achalasia was non-performance of a chest CT scan or barium swallow test promptly at first physician contact. 2. We identified 39 people that could have been misdiagnosed, who had undergone upper endoscopy, but not barium swallow testing, with symptoms persisting for >6 months and without a definitive diagnosis. Among them, 16 individuals agreed to undergo the barium swallow test. One of them was confirmed to suffer from EGJOO. Conclusion To avoid a delayed diagnosis of EGJOO, including achalasia, performing a barium swallow test promptly, in addition to routine endoscopy, appears to be extremely important. Furthermore, we successfully identified one patient with EGJOO from among the community population using an extraction algorithm. [ABSTRACT FROM AUTHOR]

Heller myotomy with fundoplication (HF) is one of the established treatments for esophageal achalasia. However, the less invasive Peroral endoscopic myotomy (POEM) is rapidly spreading. Several papers report short-term efficacy and safety of POEM is as well as HF, but GERD is more frequent after POEM. Moreover, the long-term outcome is still unknown. This is a single center retrospective study. In our center, HF was carried out for 59 patients between 1999 and 2014. Among them, 58 cases underwent laparoscopic procedure, 52 were with Dor fundoplication and 7 were with Toupet. From 2015 we performed POEM for 224 patients until Dec 2022. For POEM, grade of dilatation was lower (I/II/III=POEM;76/131/17, HF;6/44/9, p < 0.001) and esophageal myotomy length was longer (8.5 ± 3.4 cm vs 5.7 ± 0.8 cm, P < 0.001). Median IRP and Eckardt score decreased for them (24.3 ± 13.0 to 7.8 ± 4.8 mmHg/5.37 ± 2.09 to 0.54 ± 0.84). HF improved their LES pressure (40.2 ± 20.3 to 18.0 ± 7.1 mmHg, p < 0.001). Postoperative LA grade B GERD was more often for POEM (26.9% vs 4.7%, p < 0.001), however grade C or D were comparable. All of them were controlled by PPIs, except one POEM patient considering further treatment. For HF, median follow-up period was longer (78 vs 19 mo), however, additional treatment was more necessary (6.8% vs 1.3%, p = 0.033). Postoperative grade B GERD was more common for POEM, but almost all of them were controlled by PPIs. POEM was less likely to require additional treatment, though follow-up time was relatively short. Further observation and re-evaluation are warranted. [ABSTRACT FROM AUTHOR]

Background and study aims: The short-term outcomes of Peroral endoscopic myotomy (POEM) for achalasia are well described, however, there is limited long-term data in the literature. This study aims to retrospectively assess the long-term outcomes of the POEM in a Canadian tertiary care centre. Methods All patients who underwent POEM for treatment of Achalasia with minimum follow-up of at least 4 years at a tertiary care centre were enrolled in this study. The primary outcome of the study was clinical success defined as a post-POEM Eckardt score ≤ 3 at ≥48 months. Secondary outcomes included changes in body mass index (BMI), incidence of gastroesophageal reflux disease (GERD), adverse events and length of hospital stay. Result Thirty-eight patient (aged 53.9 ± 17.9) underwent POEM, with a median follow-up period of 61 months (48–79). The mean Eckardt score was significantly reduced from 7.7 ± 1.9 to 1.2 ± 0.6 (P < 0.001). All patients achieved post-POEM Eckardt scores of ≤3 at ≥48 months. BMI increased from 27.4 ± 6.5 pre-POEM to 29.4 ± 6.7 (P < 0.003) post-POEM. Twelve (31.5%) patients developed pathologic reflux. Conclusion POEM is an effective and safe procedure for achalasia with durable long-term outcomes. Larger series with longer follow-up are required to confirm these results. [ABSTRACT FROM AUTHOR]

Situs inversus totalis (SIT) is a rare congenital condition, which is characterized by abnormal placement of the thoracic and abdominal organs. The incidence of this condition is estimated to be from 1/8000 to 1/25,000. There have been minimal reports on SIT patients with esophageal cancer. In this report, we discuss a patient with SIT complicated by middle and lower esophageal cancer who underwent laparoscopic and thoracoscopic esophagectomy with intrathoracic anastomosis, and provide useful information with regards to treatment of this rare condition. [ABSTRACT FROM AUTHOR]

Management of complex acute surgical pathology in austere environments necessitates rapid evaluation and resource appropriate management to avoid time-associated morbidity and potentially mortality. Obstructive upper gastrointestinal (UGI) pathologies can be particularly challenging and associated with significant morbidity. Herein, we present six patients with UGI obstructions encountered over the course of an 8-mo deployment onboard a US Navy Aircraft Carrier. Each patient presented to our medical department with signs and symptoms of obstructive UGI pathology including one gastric volvulus requiring operative management at sea, one with a new diagnosis of achalasia requiring transportation and continental United States outpatient evaluation, and four patients with food impaction requiring urgent endoscopic management. Although UGI pathology is seldom encountered at sea, definitive surgical interventions, including prompt evaluation and management of these acute pathologies, can be performed in an austere environment. We wish to call attention to these potential encounters in order that underway deployed medical units and supporting resources ashore are prepared and equipped to intervene on acute UGI obstructive pathology. [ABSTRACT FROM AUTHOR]

Background: The aim of this national population‐based cohort study was to compare rates of reintervention after surgical myotomy versus sequential pneumatic dilatation for the primary management of oesophageal achalasia. Methods: Patients with oesophageal achalasia diagnosed between 2002 and 2012, and without an intervention in the preceding 5 years were identified from the Hospital Episode Statistics database. Patients were divided into two groups based on the primary treatment, and propensity score matching was used to compensate for differences in baseline characteristics. Results: Some 14 705 patients were diagnosed with oesophageal achalasia, of whom 7487 (50·9 per cent) received interventional treatment: 1742 (23·3 per cent) surgical myotomy, 4534 (60·6 per cent) pneumatic dilatation and 1211 (16·2 per cent) endoscopic botulinum toxin injection. As age increased, the proportion of patients receiving myotomy decreased and the proportion undergoing dilatation increased. Patients who underwent surgical myotomy were younger (mean age 44·8 years versus 58·5 years among those who had pneumatic dilatation; P < 0·001), a greater proportion had a Charlson co‐morbidity index score of 0 (90·1 versus 87·7 per cent; P = 0·003) and they were more commonly men (55·6 versus 51·8 per cent; P = 0·020). Following propensity score matching, the safety of the two initial treatment approaches was equivalent, with no difference in incidence of oesophageal perforation (1·3 and 1·4 per cent after myotomy and dilatation respectively; P = 0·750). However, dilatation was associated with increased need for reintervention (59·6 versus 13·8 per cent; P < 0·001) and frequency of reinterventions (mean 0·34 versus 0·06 per year; P < 0·001). Conclusion: Surgical myotomy was associated with a lower rate of reintervention and could be offered as primary treatment in patients with oesophageal achalasia who are fit for surgery. For those unfit for surgery, pneumatic dilatation may provide symptomatic relief with approximately 60 per cent of patients requiring reintervention. [ABSTRACT FROM AUTHOR]

Esophageal achalasia is a type of motility disorder characterized by incomplete relaxation of lower esophageal sphincter (LES) and absence of esophageal peristalsis. Peroral endoscopic myotomy (POEM) is a new treatment option for achalasia that is less invasive, more effective, and safe as compared to surgery. High-frequency electrotome is commonly used in POEM, but takes longer time to make the tunnel in the esophagus and causes many complications. The thulium laser decreases the risk of bleeding and perforation in endoscopy but has not been reported in digestive diseases, especially in POEM. Therefore, the aim of this study is to evaluate the feasibility of the 1940 nm thulium laser in POEM. FromMarch 2015 to August 2015, five patients with achalasia at the Digestive department, Beijing Friendship Hospital, Capital Medical University, Beijing, China were included. Before the procedure, the patients' gender, age, and duration of symptoms were recorded. Eckardt symptom score and LES thickness, which measured by endoscopic ultrasonography, were recorded. While the subtypes of achalasia (according to the Chicago classification), lower esophagus sphincter resting pressure (LESRP) and integrated relaxation pressure (IRP) were measured by HRM for all patients. Barium esophagram was also used to rule out anatomical lesions, esophageal varices, or neoplasia, which may cause similar symptoms. All examinations were performed one week before POEM. POEM was performed with the 1940 nm thulium laser under general anesthesia. Eckardt score, procedure duration, myotomy length, and complications were recorded one week after POEM. All the patients were followed-up at two weeks and four weeks after POEM. POEM was successfully performed in all five patients. The mean age of the patients was 38.8 years (24-54 years). Achalasia subtypes were type I (n = 1), II (n = 2), and III (n=2). The operation duration was 186, 180, 111, 75, and 126 minutes for the five cases. Pre/postprocedure Eckardt scores were 3/0, 7/0, 5/1, 6/0, and 9/0. Pre/postprocedure LESRP (mmHg) were 45.3/26.4, 18.0/1.1, 25.8/10.4, 16.5/11.2, and 24.2/20.8. Pre/postprocedure IRP (mmHg) were 27.3/15.5, 15.4/4.2, 5.7/6.8, 15.5/10.1, and 13.1/14, respectively. No adverse events occurred during the procedure. After POEM, subcutaneous emphysema occurred in case 1 on the first day, which relieved spontaneously after two days without special intervention. Infection occurred in case 5 on the day of POEM was healed with antibiotics three days later. The 1940 nm thulium laser is feasible for POEM procedure. Further studies are needed to determine whether the 1940 nm thulium laser is better than high-frequency electrotome. [ABSTRACT FROM AUTHOR]