Achalasia Type Research Articles

Treatment outcomes and esophageal cancer incidence by disease type in achalasia patients undergoing peroral endoscopic myotomy: Retrospective study.

This retrospective study aimed to compare treatment outcomes and postoperative courses, including the incidence of esophageal cancer (EC), according to disease types, in 450 achalasia patients who underwent peroral endoscopic myotomy (POEM). Data from consecutive POEM procedures performed from September 2011 to January 2023 at a single institution were reviewed. Achalasia was classified into straight (St), sigmoid (S1), and advanced sigmoid (S2) types using esophagography findings. Regarding efficacy, POEM was considered successful if the Eckardt score was ≤3. A statistical examination of the incidence and trend of EC occurrence across the disease type of achalasia was conducted using propensity score matching. Of the 450 patients, 349 were diagnosed with St, 80 with S1, and 21 with S2. POEM efficacy was 97.9% at 1 year and 94.2% at 2 years postprocedure, with no statistical difference between disease types. Using propensity score matching, the incidence of EC in each disease type was as follows: St, 1% (1/98); S1, 2.5% (2/77); S2, 10% (2/18). While no statistical significance was observed between St (1.0%: 1/98) and all sigmoid types (4.0%, 4/95; P = 0.3686). However, a trend test revealed a tendency for EC to occur more frequently in the order of S2, S1, and St type with a statistically significant difference (P = 0.0413). Outcomes of POEM are favorable for all disease types. After POEM, it is important not only to monitor the improvement of achalasia symptoms but also to pay attention to the occurrence of EC, especially in patients with sigmoid-type achalasia.

Peroral Endoscopic Myotomy: Short Versus Long Esophageal Myotomy for Achalasia Cardia: A Randomized Controlled Noninferiority Trial.

The appropriate length of esophageal myotomy in peroral endoscopic myotomy (POEM) for achalasia cardia remains unclear. This study aimed to compare the outcome of short (≤3cm) and long (≥6cm) esophageal myotomy in patients with type I and II achalasia cardia. This single-blinded, randomized controlled noninferiority trial was conducted at a tertiary center between July 2021 and December 2021. Patients with achalasia types I and II were randomized into short (≤3cm) and long (≥6cm) esophageal myotomy groups. The primary outcome of the study was clinical success (Eckardt score ≤3) 1 year after the procedure. The secondary outcomes included a comparison of technical success, operating duration, occurrence of intraoperative adverse events, alterations in integrated relaxation pressure (IRP), change in barium column height after 5 minutes (1mo), and gastroesophageal reflux disease (3mo) between the groups. Fifty-four patients were randomized into the short (n=27) or long (n=27) esophageal myotomy groups. Technical success rates were 100% (27/27) and 96.3% (26/27) in short myotomy (SM) and long myotomy (LM) groups, respectively. The clinical success rates were 96.3% (26/27) and 96.2% (25/26) in the SM and LM groups, respectively ( P =0.998). The mean (±SD) length of the esophageal myotomy was 2.75±0.36cm in the SM and 6.69±1.35cm in the LM groups ( P <0.001). The mean (±SD) procedure time for the SM and LM groups was 61.22±8.44 and 82.42±14.70 minutes ( P <0.001), respectively. The mean integrated relaxation pressure (IRP), Eckardt score, adverse events, reflux esophagitis, symptomatic gastroesophageal reflux disease, and esophageal acid exposure (>6%) did not differ significantly between the 2 groups following POEM treatment. Short myotomy is noninferior to long myotomy in terms of clinical success, gastroesophageal reflux disease, and intraoperative adverse events at the short-term follow-up ( P >0.05). Short myotomy resulted in a reduced operative time ( P <0.05).

Comprehensive Approaches to the Treatment of Esophageal Achalasia

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

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

Although per oral endoscopic myotomy (POEM) has shown to be beneficial forthe treatment ofachalasia, it can be difficult to predict who will have a robust and long-lasting response. Historically, it hasbeen shownthat higher lower esophageal sphincter pressures havebeen associatedwith poorer responses to alternative endoscopic therapies such as Botox therapy and pneumatic dilation. This studywas designedto evaluate if modern preoperative manometric data could similarly predict response totherapyafter POEM. This was a retrospective study of 237 patients who underwent POEM at a single institution over a period of 13years (2011-2023) and who had a high-resolution manometry performed preoperativelyandan 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 postoperativelyaswell asthe degree of Eckardt score reduction using a linear regression model. 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 forfurtherinterventions, however, it was predictive of agreaterreduction in postoperative Eckardt scores (p = 0.03)asshown by the non-zero regression slope. In this study, achalasia type was not a predictive factor in the need for further interventions or the degree of symptom relief.Although IRPwas not predictive ofthe need for further interventions, a higher IRPdid predictbetter 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 POEMwhichprovides significant symptomatic relief postoperatively.

Does preoperative symptom duration affect outcomes following per oral endoscopic myotomy (POEM)?

Per oral endoscopic myotomy (POEM) is a safe therapy for the treatment of achalasia. Long-term effects of untreated achalasia include worsening dysmotility and disruptions in esophageal anatomy, i.e., tortuosity and dilation. We hypothesize that long-standing achalasia prior to intervention will have worse outcomes following POEM than in patients with symptoms for shorter duration. We retrospectively analyzed achalasia patients who underwent POEM at our institution from 2011 to 2023, categorizing them into symptom duration cohorts (< 1year, 1-3years, 4-10years, > 10years). Inclusion criteria comprised patients with documented achalasia diagnosis who received POEM treatment at our facility. Exclusion criteria encompassed individuals lacking data pertaining to achalasia diagnosis, the time frame before intervention, or those missing pre and postoperative Eckardt scores. POEM failure was defined as symptom recurrence, necessity for repeat intervention, or high postoperative Eckardt score. We compared demographic, preoperative, and postoperative outcomes across these cohorts, and employed multivariable logistic regression to explore the link between symptom duration and POEM response. During the study period, in our increased cohort 234 patients met inclusion criteria. 75 patients had symptoms for < 1year, 78 patients had symptoms from 1 to 3years, 47 patients had symptoms from 4 to 10years, and 34 patients had symptoms > 10years. Patient demographics such as age, sex, BMI, Charleson-Deyo-Comorbidity-Index, and diabetes did not differ amongst cohorts. High-resolution manometry data, including achalasia type, Median IRP, LES residual pressure, and Basal LES pressure did not differ between groups. Preoperative Eckardt scores ranged from 4 to 5 across groups (p 0.24). Patients endorsed an average of three total preoperative symptoms across groups (p 0.13). Patients with symptoms greater than 4years had significantly more endoscopic interventions prior to POEM (37% vs, 68% p .001). There was no significant difference in post-procedure mean Eckardt scores between cohorts. All cohorts experienced the same number of post-POEM symptoms. Post-POEM manometric measurements remained consistent across cohorts. Similarly, there were no significant differences in terms of symptom recurrence, requirement for repeat interventions, or repeat POEM among the cohorts. Multivariable logistic regression analysis determined achalasia symptoms greater than a decade did not result in increased odds of having a higher postoperative Eckardt score, worse dysphagia, regurgitation, or weight loss. In this increased cohort, this data once again suggests that longer symptom duration is not associated with increased rates of POEM failure.

Association of gastric myotomy length in peroral endoscopic myotomy (POEM) with gastro-esophageal junction distensibility measured by Endoluminal Functional Lumen Imaging Probe (EndoFLIP).

Endoluminal Functional Lumen Imaging Probe (EndoFLIP) is a device that measures gastro-esophageal junction (GEJ) distensibility. However, it is not demonstrated that GEJ distensibility increases proportionally with varying gastric myotomy length in peroral endoscopic myotomy (POEM). This study aimed to investigate the association between gastric myotomy length in POEM and intraoperative EndoFLIP findings. This single-center, retrospective cohort study included patients who underwent POEM with intraoperative EndoFLIP from December 2019 to January 2023. Using EndoFLIP, minimal balloon diameter and its distensibility index (DI) were measured pre- and post-myotomy. Primary and secondary outcomes were the post-myotomy EndoFLIP findings at 30ml and 40ml volume fills. The study included 44 patients (mean age 53.1years, 50% female). Chicago classification included achalasia type I (39%), II (41%), III (9%), hypercontractile esophagus (2%), and EGJOO (9%). The mean esophageal myotomy length was 7.5 ± 2.2cm and gastric myotomy was 2.1 ± 0.6cm. Simple linear regression analyses indicated that for each 1cm increase in gastric myotomy length, the DI at 30ml volume fill was estimated to increase by 2.0mm2/mmHg (p < 0.05, R2 = 0.41), the minimal diameter at 30ml volume fill was estimated to increase by 2.4mm (p < 0.05, R2 = 0.48), and the minimal diameter at 40ml volume fill was estimated to increase by 1.3mm (p < 0.05, R2 = 0.09). This study demonstrates a significant linear relationship between gastric myotomy length and GEJ distensibility measured by EndoFLIP during POEM. These findings may be useful in clinical practice by enabling EndoFLIP to help calibrate a desired gastric myotomy length to achieve optimal DI and minimal diameter.

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

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. 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. As many as118 cases (41.2 ± 13.9years, 61% males) with achalasia and 200 controls (43.4 ± 11.9years, 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. Achalasia patients demonstrate a notably higher risk of malnutrition compared to individuals with FGIDs. Nutritional status significantly improves after POEM. (NCT05161923).

Peroral Endoscopic Myotomy in Patients with Type III Achalasia and Non Achalasia Esophageal Motility Disorders.

Background : Achalasia is a disease characterized by dysphagia, regurgitation, chest pain, and weight loss. It is now classified into three subtypes according to the Chicago classification. Type III achalasia is characterized by lower esophageal sphincter obstruction with premature or spastic distal esophageal contractions. The aim of this study is to evaluate the efficiency of peroral endoscopic myotomy (POEM) in patients with type III achalasia as well as non achalasia esophageal motility disorders. Methods : This pilot prospective observational study was carried out on 12 patients aged from 18 to 75 years old, both sexes, with achalasia type III and non-achalasia esophageal motility disorders. Follow up after 3 months of Peroral Endoscopic Myotomy was evaluated by Eckardt score. Results : There is significant decrease in mean Eckardt score after Peroral Endoscopic Myotomy (9.83 vs 1.17, P &lt; 0.001) as t-test showed (t-test 13.23 with 95% CI 7.23- 10.11). Thus, the effect of POEM on reducing Eckardt score is statistically significant. During the operative procedure, the average length of myotomy measured 20.42 cm, with a mean operative duration of 55.33 minutes. Following a mean follow-up period of 15.83 months, the mean Eckardt score decreased to 1.17. Bleeding occurred during the procedure in three cases (25%), while mucosal injury was observed in only one case. Additionally, one case experienced postoperative chest infection. Conclusions : Peroral Endoscopic Myotomy is safe and effective intervention in the management of achalasia type III as well as non-achalasia esophageal motility disorders

Automatic three-dimensional reconstruction of the oesophagus in achalasia patients undergoing POEM: an innovative approach for evaluating treatment outcomes

Background and aimsPeroral endoscopic myotomy (POEM) is a standard treatment option for achalasia patients. Treatment response varies due to factors such as achalasia type, degree of dilatation, pressure and distensibility...

Two onset types of achalasia and the long-term course to diagnosis.

Recently, the incidence of achalasia has been increasing, but its cause remains unknown. This study aimed to examine the initial symptoms and the course of symptoms and to find new insights into the cause and course of the disease. Altogether, 136 patients diagnosed with achalasia by high-resolution manometry (HRM) were enrolled. Questionnaires and chart reviews were conducted to investigate the initial symptoms, time from onset to diagnosis, and comorbidities, as well as the relationship between HRM results, time to diagnosis, and symptom severity. In total, 67 of 136 patients responded to the questionnaire. The median ages of onset and diagnosis were 42 and 58years, respectively. The median time from onset to diagnosis was 78.6months, with 25 cases (37.3%) taking > 10years to be diagnosed. The symptom onset was gradual and sudden in 52 (77.6%) and 11 (16.4%) patients, respectively. Of the 11 patients with acute onset, three (27.3%) developed anhidrosis at the same time. There was no correlation between the time from onset to diagnosis and esophageal dilatation, resting LES pressure, or mean integrated relaxation pressure (IRP). No correlation was also found between the degree of symptoms and resting LES pressure or IRP. Esophageal achalasia can have acute or insidious onsets. This finding may help to elucidate the cause of achalasia.

Heller Cardiomyotomy in end stage of achalasia with mega esophagus: Results in five cases

Achalasia is a motility disorder of esophagus with poor emptying and, characterised by a lack of peristalsis and impaired relaxation of the lower esophageal sphincter with high LES pressure,diameter of esophsgous can increase, and some patients progress to develop end-stage achalasia as megaesophagus or sigmoid esophagus and significant form dilation and tortuosity.These omplications occurs in between 5% and 20% of patients with acha­lasia. This group of patients if untreated are at risk of aspiration, aspiration pneumonia, and malnutrition. The management of this type of achalasia, aims to palliate symptoms by improving esophagus emptying and reducing the pressure gradient. In this group of patients, pneumatic dilatation (PD), surgical myotomy, or peroral endoscopic myotomy (POEM) may be less or not effective. Esophagectomy for the treatment of end-stage achalasia remains a controversial topic and has been recommended by some authors. We describe 5 patients with end-stage achalasia who was been successfully managed with laparotomy heller cardiomyotomy . In 4,12,18,24 months follow- up the condition of patients was good.

Manometric findings in children with eosinophilic esophagitis and persistent post-remission dysphagia.

Dysphagia is a frequent symptom of active eosinophilic esophagitis (EoE), but at times it persists despite attaining histologic healing and lack of fibro-stenotic changes. We aimed to describe the manometric findings in this subset of patients. A retrospective review of charts between 2013 and 2023 at a tertiary pediatric gastroenterology center, treating roughly 1500 EoE patients per year. We included children with EoE referred to high-resolution impedance manometry (HRIM) for persistent dysphagia despite histologic healing (i.e., <15 eosinophils/high-power field [Eos/hpf]). Data including initial EoE diagnosis, endoscopy reports, esophageal biopsies, treatment regimens, and HRIM were retrospectively collected. The estimated prevalence of post-remission dysphagia in our cohort was exceedingly rare (<0.05%). Four patients met the eligibility criteria of histologic remission and absence of fibro-stenotic features on endoscopic evaluation and thus, were included in this case series. Patients achieved remission with steroids, proton-pump inhibitor, or both within a median time of 5 months from diagnosis. Peak Eosinophil count at remission was ≤5 Eos/hpf in three patients and ≤10 Eos/hpf in one. On HRIM, all four patients had a hypomotile esophagus and abnormal bolus clearance. Lower esophageal sphincter integrated relaxation pressure values were normal in three patients and elevated in one. Two patients were diagnosed with ineffective esophageal motility, one with aperistalsis and one with achalasia type 1. Post-remission dysphagia is rare in EoE. Esophageal dysmotility with a hypomotile pattern may contribute to the persistent dysphagia in children with EoE. HRIM should be considered in patients with EoE in whom symptoms persist despite histologic remission.

Cardiovascular Autonomic Deficits in Different Types of Achalasia.

Background and objective Achalasia cardia is a primary esophageal motility disorder, and the etiopathology of this disease's progression is not known. Moreover, autonomic dysfunction has not been studied in different types of achalasia. In light of this, we aimed to address this lack of data in this study. Methods The diagnosis of achalasia was done using high-resolution esophageal manometry (HRM)-based Chicago classification v4.0. Autonomic function tests (AFT) such as the head-up tilt test, deep breathing test (DBT), Valsalva maneuver (VM), handgrip test (HGT), and cold pressor test (CPT),as well as the heart rate variability (HRV) test, were performed among the cohort and the results were compared with those of 39 age- and sex-matched healthy controls. Results AFT and HRV tests were done on 62 patients (30 achalasia type I, 28 type II, and 4 type III) and compared with 39 age- and sex-matched healthy controls. The mean duration of symptoms, high Eckardt score, and dysphagia were most common in type I achalasia, followed by type II and III. The results of AFT showed a generalized loss of parasympathetic and baroreflex-independent sympathetic reactivity in all types of achalasia. However, baroreflex-dependent cardiovascular adrenergic reactivity was normal. Regarding cardiac autonomic tone, there was a loss of parasympathetic and sympathetic influence, but sympathovagal balance was maintained. The severity of the loss of autonomic functions was higher in type I, followed by type II. Conclusions In all types of achalasia, parasympathetic reactivity, baroreflex-independent sympathetic reactivity, and cardiac autonomic tone were lower compared to healthy controls, and the severity of dysfunction increased during the progression of the disease from type II to type I.

Histological changes of the esophageal mucosa in patients with achalasia

Background. Morphological changes in the esophageal mucosa (EM) depend on the type of esophageal achalasia (ЕA). In types I and II of ЕA, morphological changes are subtle, but in types III and IV, dystrophic changes are clearly visible. ЕA can transform from one type to another. This mainly concerns the pathological transition from inflammation to fibrosis. The purpose is to investigate the histological and morphometric differences of the EM depending on the type of ЕA. Materials and methods. We studied 51 biopsy specimens of the EM in patients with ЕA. The distribution of biopsies for histo-morphometric characterisation of changes in the EM in ЕA was used according to the radiological classification of the Japanese Society of the Esophagus (2017). To study the general histological structure of the EM, biopsy sections of 5–7 μm thickness were cut using a rotary microtome RM60-ECA, stained with hematoxylin-­eosin according to the standard method, and staining for fibrosis was performed according to the Mallory-Slinchenko method. For morphometric study, the sections were photographed using a light microscope XSZ-21 (Ukraine) and measured with the Image J.45S software (USA). Results. In histological examination, the development of structural changes in the EM on the background of ЕA in 24.1 % of cases of type III was accompanied by migration of eosinophils into the lamina propria, and in 100 % — by epithelial atrophy. Dense lymphoplasmacytic infiltration was noted in all patients with types III and IV of ЕA, as well as in 93.3 % of patients with type II, whereas in type I of ЕA, this sign was diagnosed in 20 % of cases (p = 0.005 and p = 0.0001 compared with types II and III of ЕA). Ballooning degeneration was detected in 20 % of patients with EA type II, in 57.1 % with type III and in 100 % of patients with type IV. Fibrosis in the EM was observed only in ЕA type IV. Thickening of the basal layer of the squamous epithelium of the EM was noted in all patients with type IV ЕA and 86.2 % with type III ЕA, while in types I and II, it was observed in only 20 and 13.3 % of cases, respectively (p = 0.007 and p &lt; 0.001 compared with type III ЕA). Expansion of the intercellular space was detected in 100 % of patients with types II, III and IV, and in only 40 % with type I (p = 0.009 and p = 0.0017 compared with types II and III ЕA). According to the morphometric study, the squamous epithelial height was largest in type IV ЕA — 343.40 μm versus 325.45 μm in type I ЕA (p = 0.028). The height of the basal layer was the lo­west in type II ЕA (about 40 µm) and the largest in type IV ЕA (about 50 µm) (p = 0.074). An increase in the intercellular space by 41.6 % was noted with the progression of ЕA (p = 0.001). The height of the papillae is the lowest in type II ЕA — 125.05 μm and the largest in type IV ЕA — 134.70 μm (p = 0.006). Conclusions. All types of AE are accompanied by esophagitis, histological examination of which revealed an increase in the height of the basal layer, papillae height, expansion of the intercellular space, balloon degeneration, fibrous changes in the lamina propria, lymphoplasmacytic and eosinophilic infiltration of the EM that progress depending on the type of ЕA. Morphometric studies of the EM in terms of increasing the height of the epithelium (p = 0.028), the height of the papillae (p = 0.006), the height of the basal layer (p = 0.074), and the expansion of the intercellular space (p = 0.001) allow to detect the type of ЕA.

Robotic Heller's myotomy using the new Hugo™ RAS system: first worldwide report.

Achalasia is a rare disorder of the esophagus characterized by motor dysfunction in the esophagus and relaxation failure of the lower esophageal sphincter (LES). Currently, surgical myotomy procedures are considered the standard of care. Robotic Heller's myotomy (RHM) with fundoplication has been gaining popularity due to documented advantages in the precision of myotomy as well as avoiding the potential reflux following per-oral endoscopic myotomy (POEM). To the best of our knowledge, RHM has thus far has been performed exclusively by the da Vinci surgical system. The new Hugo RAS™ system offers a unique modular design and an open console which offers better maneuverability and docking options. In this study, we present the first worldwide series of patients undergoing RHM using the new Hugo RAS™ platform. Our objective is to propose optimal operating configuration and setup to fully harness the advantages of the unique modular design of this system. Ten consecutive achalasia patients underwent Robotic Heller's myotomy (RHM) with the Hugo RAS™ system. We prospectively collected patient data, including demographics, comorbidities, ASA class, Eckardt scores, pre-operative manometric data, and EndoFlip parameters. Additionally, we recorded the docking and total operative times. Between December 2022 and August 2023, 10 patients underwent RHM with the Hugo™ RAS system. Patients had a median age of 42.5 years, 60% were female, and mean BMI was 23.2. Fifty percent had achalasia type 2 and 50% type 1. The median pre-operative integrated relaxation pressure (IRP) was 24.9. Median docking time was 10 min and overall operative time was 129.5 min. All patients, except one with acute coronary syndrome, had an uneventful peri-operative course and were discharged on post-operative day 2. The Hugo™ RAS system is well designed for robotic Heller myotomy. The operative and clinical results are similar to the currently used robotic system; however, the modular design of the system has some differences. These translate to better docking angles and maneuverability as well as console surgeon's ergonomics. Further experience is needed to explore the advantages of the system's modular design and function.

Esophageal anatomy and physiology vary across spastic and non-spastic phenotypes of disorders of esophagogastric junction outflow.

Pathophysiologic mechanisms of disorders of esophagogastric junction (EGJ) outflow are poorly understood. We aimed to compare anatomic and physiologic characteristics among patients with disorders of EGJ outflow and normal motility. We retrospectively evaluated adult patients with achalasia types 1, 2, 3, EGJ outflow obstruction (EGJOO) or normal motility on high-resolution manometry who underwent endoscopic ultrasound (EUS) from January 2019 to August 2022. Thickened circular muscle was defined as ≥1.6 mm. Characteristics from barium esophagram (BE) and functional lumen imaging probe (FLIP) were additionally assessed. Of 71 patients (mean age 56.2 years; 49% male), there were 8 (11%) normal motility, 58 (82%) had achalasia (5 (7%) type 1, 32 (45%) classic type 2, 21 (30%) type 3 [including 12 type 2 with FEPs]), and 7 (7%) had EGJOO. A significantly greater proportion of type 3 achalasia had thickened distal circular muscle (76.2%) versus normal motility (0%; p < 0.001) or type 2 achalasia (25%; p < 0.001). Type 1 achalasia had significantly wider mean maximum esophageal diameter on BE (57.8 mm) compared to type 2 achalasia (32.8 mm), type 3 achalasia (23.4 mm), EGJOO (15.9 mm), and normal motility (13.5 mm). 100% type 3 achalasia versus 0% type 1 achalasia/normal motility had tertiary contractions on BE. Mean EGJ distensibility index on FLIP was lower for type 3 achalasia (1.2 mmHg/mm2 ) and EGJOO (1.2 mmHg/mm2 ) versus type 2 (2.3 mmHg/mm2 ) and type 1 achalasia (2.9 mmHg/mm2 ). Our findings suggest distinct pathologic pathways may exist: type 3 achalasia and EGJOO may represent a spastic outflow phenotype consisting of a thickened, spastic circular muscle, which is distinct from type 1 and 2 achalasia consisting of a thin caliber circular muscle layer with more prominent esophageal dilation.

166. ROBOTIC-ASSISTED BILATERAL EPIPHRENIC DIVERTICULECTOMY WITH HELLER MYOTOMY AND DOR FUNDOPLICATION: A SURGICAL TECHNIQUE

Abstract Background Epiphrenic diverticula are rare esophageal diseases often associated with motility disorders such as achalasia. We present a case of bilateral epiphrenic diverticula in a patient with achalasia type III who presented with dysphagia. This video demonstrates the surgical technique of robotic-assisted bilateral epiphrenic diverticulectomy with Heller myotomy and Dor fundoplication, highlighting the six essential surgical steps. Methods A 72-year-old female presented with dysphagia for 3 years. An esophagogastroduodenoscopy revealed two epiphrenic diverticula without food content retention. A barium swallow revealed two epiphrenic diverticula, measuring 3.3 cm and 1.2 cm in diameter, on right and left side walls of distal esophagus, respectively. Esophageal manometry showed the lower esophageal sphincter (LES) pressure was 25.6 mmHg (normal &amp;lt;15 mmHg). Subsequently, both the acid exposure time and the DeMeester score indicated normal levels of acid exposure. The patient underwent robotic-assisted surgery. The diverticulectomy was performed using stapling device under endoscopic guidance, followed by standard myotomy. Additionally, Dor fundoplication was performed to complete procedure. Results Postoperatively, the patient recovered well. An upper gastrointestinal study demonstrated an adequate myotomy without evidence of leak or residual epiphrenic diverticula. Further postoperative evaluation, including esophageal manometry, showed normal LES pressure of 12.8 mmHg, the postoperative acid exposure time of 0.0%, and the DeMeester score of 0.20, that indicating normal levels of acid exposure. The patient resumed a liquid diet on postoperative day 4 and a soft diet on postoperative day 7. Based on these results, the patient’s dysphagia was resolved, and there was an absence of gastroesophageal reflux symptoms. Conclusion Bilateral epiphrenic diverticula are extremely rare, and require complex procedure. Robotic-assisted surgery has the potential to enhance the facilitation of surgical treatment for this condition. The robotic tools are precise and maneuverable, making it possible to perform intricate surgical procedures in tight angles and confined spaces, such as the hiatal area. This technique offers several advantages over conventional laparoscopic surgery, and should therefore be considered for the treatment of bilateral epiphrenic diverticula.

Effect of peroral endoscopic myotomy on growth and esophageal motility for pediatric esophageal achalasia

Peroral endoscopic myotomy (POEM), a novel minimally invasive treatment for esophageal achalasia, has been shown to be effective and safe for both adult and pediatric patients. However, studies on its application in children in Taiwan and its impact on growth and esophageal motility are lacking. We conducted a retrospective study on consecutive pediatric patients who were diagnosed with esophageal achalasia at National Taiwan University Hospital and underwent POEM during 2015-2022. Disease characteristics and treatment outcomes were analyzed. Ten patients (age 16.9±3.1 years), nine newly diagnosed and one previously treated with pneumatic dilatation, underwent POEM for achalasia (type I/II/III: 3/7/0). Average symptom duration before diagnosis was 19.4±19.9 months, mean POEM procedure time was 83.6±30.7min, and clinical success (Eckardt score ≤3) was achieved in all patients. Eight patients experienced mild adverse events during POEM, but none required further endoscopic or surgical intervention. Over a mean follow-up period of 3.7±1.6 years, mean Eckardt score decreased significantly from 5.7±2.4 to 1.1±0.7 (p=0.0001). The BMI z-score also increased significantly after POEM (p=0.023). Five patients received follow-up high-resolution impedance manometry (HRIM), and all had improved lower esophageal sphincter resting pressures (p=0.011), body contractility, and bolus transit (p=0.019). POEM is an effective and safe treatment for pediatric achalasia in Taiwan. Early diagnosis and treatment with POEM may help to restore esophageal function and nutrition status in children.

Impaired sliding between the lower esophageal sphincter and crural diaphragm (esophageal hiatus) in patients with achalasia esophagus.

Swallow-related axial shortening of the esophagus results in the formation of phrenic ampulla in normal subjects; whether it is the case in achalasia esophagus is not known. The goal is to study axial shortening of the esophagus and relative movement between the lower esophageal sphincter (LES) and crural diaphragm (CD) in normal subjects and patients with achalasia. A novel method, isoimpedance contour excursion at the lower edger of LES, as a marker of axial esophageal shortening was validated using X-ray fluoroscopy (n = 5) and used to study axial shortening and separation between the LES and CD during peristalsis in normal subjects (n = 15) and patients with achalasia type 2 esophagus (n = 15). Abdominal CT scan images were used to determine the nature of tissue in the esophageal hiatus of control (n = 15) and achalasia patients (n = 15). Swallow-induced peristalsis resulted in an axial excursion of isoimpedance contours, which was quantitatively similar to the metal clip anchored to the LES on X-ray fluoroscopy (2.3 ± 1.4 vs. 2.1 ± 1.4 cm with deep inspiration and 2.7 ± 0.6 cm vs. 2.7 ± 0.6 cm with swallow-induced peristalsis). Esophageal axial shortening with swallows in patients with achalasia was significantly smaller than normal (1.64 ± 0.5 cm vs. 3.59 ± 0.4 cm, P < 0.001). Gray-level matrix analysis of CT images suggests more "fibrous" and less fat in the hiatus of patients with achalasia. Lack of sliding between the LES and CD explains the low prevalence of hiatus hernia, and low compliance of the LES in achalasia esophagus, which likely plays a role in the pathogenesis of achalasia.NEW & NOTEWORTHY Swallow-related axial shortening of the esophagus is reduced, and there is no separation between the lower esophageal sphincter and crural diaphragm (CD) with swallowing in patients with achalasia esophagus. Fat in the hiatal opening of the esophagus appears to be replaced with fibrous tissue in patients with achalasia, resulting in tight anchoring between the LES and CD. The above findings explain low prevalence of hiatus hernia and the low compliance of the LES in achalasia esophagus.

Preoperative manometric data can predict symptom improvement after per oral endoscopic myotomy for treatment of achalasia.

While per oral endoscopic myotomy (POEM) has been shown to be efficacious in the treatment of achalasia, it can be difficult to predict who will have a robust and durable response. Historically, high lower esophageal sphincter pressures have been shown to predict a worse response to endoscopic therapies such as botox therapy. This study was designed to evaluate if modern preoperative manometric data could predict a response to therapy after POEM. This was a retrospective study of 144 patients who underwent a POEM at a single institution by a single surgeon over an 8-year period (2014-2022) who had high-resolution manometry performed preoperatively and had an Eckardt symptom score performed both preoperatively and postoperatively. The achalasia type and integrated relaxation pressures (IRP) were then tested for potential correlation with need for any further achalasia interventions postoperatively as well as the degree of Eckardt score reduction using univariate analysis. The achalasia type on preoperatively manometry was not predictive of need for further interventions or degree of Eckardt score reduction (p = 0.74 and 0.44, respectively). A higher IRP was not predictive of need for further interventions however it was predictive of a greater reduction in postoperative Eckardt scores (p = 0.03) as shown by a nonzero regression slope. In this study, achalasia type was not a predictive factor in need for further interventions or degree of symptom relief. While IRP was not predictive of need for further interventions, a higher IRP did predict better symptomatic relief postoperatively. This result is opposite that of other endoscopic treatment modalities. Therefore, patients with higher IRP on high-resolution manometry would likely benefit from myotomy which provides significant symptomatic relief postoperatively.