Sigmoid Esophagus Research Articles

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.

Surgical Options for End-Stage Achalasia.

Achalasia is one of the most commonly described primary esophageal motility disorders worldwide, but there is significant controversy regarding ideal management of end-stage disease. This article reviews the definition of end-stage achalasia and summarizes past and present surgical treatment. Myotomy of the lower esophageal sphincter remains the mainstay of treatment of achalasia, even in advanced disease. Esophagectomy may have benefit as a primary treatment modality in end-stage achalasia with sigmoid esophagus, but international guidelines recommend consideration of laparoscopic or endoscopic approaches initially in most patients. Novel peroral esophageal plication techniques may provide alternative treatment options in patients with significant esophageal dilation that fail myotomy or esophagectomy. End-stage achalasia is characterized by progressive tortuosity and dilation of the esophagus as a failure of primary peristalsis. Up to 20% of patients with achalasia will progress to end-stage disease. In most cases, laparoscopic or endoscopic myotomy is recommended as initial approach to surgical management.

431. RARE CASE OF CONTAINED ESOPHAGEAL PERFORATION IN LONG STANDING ACHALASIA CARDIA AND ITS SUCCESSFUL MANAGEMENT

Abstract 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.

Conventional versus oblique fiber-sparing endoscopic myotomy for achalasia cardia: a randomized controlled trial (with videos)

GERD is common after peroral endoscopic myotomy (POEM). Selective sparing of oblique fibers may reduce the incidence of reflux esophagitis after POEM. In this study, we compared the incidence of GERD between conventional myotomy (CM) versus oblique fiber-sparing (OFS) myotomy in patients with achalasia. Eligible patients with type I and II achalasia who underwent POEM from January 2020 to October 2020 were randomized into 2 groups (CM and OFS myotomy). Exclusion criteria were type III achalasia, sigmoid esophagus, and history of Heller's myotomy. The primary study outcome was incidence of reflux esophagitis (at least grade B) in the 2 groups. Secondary outcomes were reflux symptoms, esophageal acid exposure, clinical success, and adverse events. One hundred fifteen patients were randomized into CM (n= 58) and OFS myotomy (n= 57) groups. POEM was technically successful in all patients. Overall, reflux esophagitis was found in 56 patients (48.7%). The incidence of at least grade B esophagitis was similar in both groups (CM vs OFS myotomy: 25.9% vs 31.6%, P= .541). The mean number of reflux episodes (48.2 ± 36.6 vs 48.9 ± 40.3, P= .933), increased esophageal acid exposure >6% (45.5% vs 31.7%, P= .266), and high DeMeester scores (38.6% vs 41.5%, P= .827) were similar in both groups. There was no difference in the rate of symptomatic reflux (GERD questionnaire score >7) or use of proton pump inhibitors at 1 year. Sparing of sling fibers has no significant impact on the incidence of significant reflux esophagitis after POEM. Novel strategies need to be explored to prevent reflux after POEM. (Clinical trial registration number: NCT04229342.).

A286 Resolution of sigmoid esophagus from pseudoachalasia associated with prior laparoscopic gastric band placement

A286 Resolution of sigmoid esophagus from pseudoachalasia associated with prior laparoscopic gastric band placement

A prediction model and nomogram for technical difficulty of peroral endoscopic myotomy.

Peroral endoscopic myotomy (POEM) is a promising endoscopic technique for achalasia. We aimed to establish a regression model and develop a simple nomogram to predict the technical difficulty of POEM in a single center with large volume cases. 3385 achalasia patients treated with POEM were included, and the technical difficulty was systemically evaluated. All of them were randomized into the training cohort (n = 1693) or internal validation cohort (n = 1692). Then, the prediction model and nomogram were proposed based on multivariate logistic regression analysis in the training cohort and assessed in the validation cohort. Of 3385 patients, technical difficulty happened in 417 (12.32%) cases. In the training stage, six factors were weighted based on the β coefficient from the regression model, including age, disease duration, sigmoid esophagus, mucosal edema, submucosal fibrosis, and tunnel length. The patients were categorized into low-risk (< 0.1), medium-risk (0.1-0.25), and high-risk (> = 0.25) groups. Our score model performed satisfying discrimination with the areas under the receiver-operating characteristic curve (AUC) of 0.743 (95% confidence interval (CI), 0.701-0.785) and calibration with goodness of fit in the Hosmer-Lemeshow test (P = 0.088) in internal validation. The prediction model and nomogram demonstrated good performance in predicting the technical difficulty of POEM.

OUTCOME OF PER ORAL ENDOSCOPIC MYOTOMY (POEM) IN SIGMOID ACHALASIA AT A MEDIAN FOLLOW UP OF 17 MONTHS.

Advanced achalasia cardia (AC) represents the end stage in the natural history of AC. Role of per oral endoscopic myotomy (POEM) in this technically difficult subset is emerging. Retrospective review of the patients who had undergone POEM for advanced AC with sigmoid esophagus. We assessed the technical success, clinical success and adverse event rate. Pre and post POEM Eckardt score (ES), integrated relaxation pressure-4sec (IRP-4), lower oesophageal sphincter pressure (LESP) and height and width of barium column at 5 minutes were noted. Of the 85 patients who underwent POEM for AC, 10 patients had advanced AC with sigmoid esophagus of which eight were sigmoid and two were advanced sigmoid. The clinical and technical success was 100% with significant reduction of ES, IRP-4, LESP and height and width of barium column at 5 minutes. One patient had a minor adverse event in the form of mucosal injury that was closed with hemoclips. At a median follow up of 17 months there was no recurrence. Our study demonstrates POEM to be a safe and effective modality of treatment in this technically difficult subset of AC with sigmoid morphology.

Management of End-Stage Achalasia

Background: End-stage Achalasia correspond to the final stage in the evolution of this disease, characterized by the presence of severe dysphagia and weight loss despite aggressive treatment, associated to massive esophageal dilatation and tortuosity of the esophagus, with loss of its axis and adopting a sigmoid-shape or L-shape distal esophagus. It is also called advanced sigmoid esophagus. The diagnosis is based on radiological evaluations, but different authors use different criteria to define end-stage. This fact makes difficult to compare the results, due to a great heterogeneity of the patients. Methods: Total of 65 reports contently treatment of patients with end stage achalasia were carefully review. Results: Until the year 2000 subtotal esophagectomy with gastric or long-colon interposition was employed as primary treatment or after several medical and endoscopic failed treatments. The mean mortality rate range 3% to 5%, with a high morbidity rate and 10 to 12 days of hospital stay. From 2000 to 2015 Laparoscopic Heller Myotomy (LHM) was employed as the primary treatment, with a mortality rate of 0%, low postoperative morbidity (6%-8%) and 2 days of Hospital stay. The long-term follow up was satisfactory in near 2/3 of the patients with end-stage esophagus. In the last years POEM (Peroral endoscopic Myotomy) has been employed mainly by Asian authors, reporting 0% mortality, high rate of postoperative morbidity (over 20%), a hospital stay of 5 to 7 days and a very short follow up, usually less than 2 years. Although the control of dysphagia is high, the main problem with this technique is the marked increase in pathologic acid reflux to the esophagus, with values near to 50% of the patients. It is not known the late effect 10 to 15 years after this procedure of this severe reflux in an aperistaltic esophagus, with retention of food and acid inside the esophageal lumen. Conclusion: Up to now Laparoscopic Heller Myotomy seems the best option for these patients with severe end-stage esophagus. If there is a failure after this procedure, thoracoscopic esophagectomy would be the best option.

Laparoscopic Heller-Dor is an effective long-term treatment for end-stage achalasia

BackgroundThe end-stage achalasia is a difficult condition to treat, for the esophageal diameter and conformation of the gullet, that may progress to a sigmoid shape. The aim of this study was to examine the outcome of Laparoscopic Heller-Dor in patients with end-stage achalasia, comparing them with patients who had mega-esophagus without a sigmoid shape.MethodsFrom 1992 to 2020, patients with a diagnosis of sigmoid esophagus, or radiological stage IV achalasia (the SE group), and patients with a straight esophagus larger than 6 cm in diameter, or radiological stage III achalasia (the NSE group), were all treated with LHD. The two groups were compared in terms of patients’ symptoms, based on the Eckardt score, and on barium swallow, endoscopy and manometry performed before and after the treatment. The failure of the treatment was defined as an Eckardt score > 3, or the need for further treatment.ResultsThe study involved 164 patients: 73 in the SE group and 91 in the NSE group. No intra- or postoperative mortality was recorded. The median follow-up was 51 months (IQR 25–107). The outcome was satisfactory in 71.2% of patients in the SE group, and in 89% of those in the NSE group (p = 0.005).ConclusionsSE is certainly the worst condition of the disease and the final outcome of LHD, in term of symptom control, is inferior compared to NSE. Despite this, almost 3/4 of the SE patients experienced a significant relieve in symptoms after LHD, which may therefore still be the first surgical option to offer to these patients, before considering esophagectomy.

579. LONG TERM RESULTS OF THE PULL-DOWN HELLER-DOR TECHNIQUE FOR ACHALASIA PATIENTS WITH SIGMOID ESOPHAGUS

Abstract The outcome of achalasia treatment in patients with sigmoid esophagus is worse than that achieved for fusiform achalasia. We compared two groups of sigmoid achalasia patients, in which we performed the standard Heller-Dor procedure (HD) and the Heller-Dor plus a technique aimed to restore the vertical axis of the intraabdominal portion of the esophagus pull-down Heller-Dor (PD-HD). Long-term results were evaluated. We considered 48 patients affected by primitive esophageal sigmoid achalasia operated upon consecutively (1979-2021) in first instance. After 1987, we routinely isolated 360° of the gastro-esophageal junction and the lower esophagus and applied U stitches at the right side of the lower esophagus to pull down and rotate the gastro-esophageal junction toward the right. Twenty-five patients underwent the standard HD and 23 patients underwent the PD-HD technique. Postoperative follow-up included barium swallow, clinical interview investigating dysphagia and reflux symptoms and endoscopy. The overall outcome was graded from excellent to poor according to the severity of symptoms and esophagitis. The median follow-up period was 11 years (IQR 6-22) for HD group and 9 (IQR 6-14) for PD-HD (p=0.22). At postoperative barium swallow, patients in the PD-DH group had smaller esophageal diameter [4 cm (IQR 4-4.7) vs 4.7 cm (IQR 4-5), p=0.04] and lower residual barium column [10 cm (IQR 6-13) vs 6 cm (IQR 0-8), p=0.001] than those in the HD group. Postoperative moderate-severe (D2-D3) dysphagia rates were 40% for HD and 13% for PD-HD group (p=0.003). The outcome of treatment revealed satisfactory (excellent, good, fair) results in 68% and 91.4% of HD and PD-HD group patients, respectively. The Heller-Dor operation is effective in the presence of sigmoid achalasia. The clinical and instrumental evaluations showed better results with the PD-HD technique. This technique may be the first choice for end-stage achalasia with null or low risk for cancer.

Development of Dilated Esophagus, Sigmoid Esophagus, and Esophageal Diverticulum in Patients With Achalasia: Japan Achalasia Multicenter Study.

Background/AimsPatients with achalasia-related esophageal motility disorders (AEMDs) frequently present with dilated and sigmoid esophagus, and develop esophageal diverticulum (ED), although the prevalence and patients characteristics require further elucidation.MethodsWe conducted a multicenter cohort study of 3707 patients with AEMDs from 14 facilities in Japan. Esophagography on 3682 patients were analyzed.ResultsStraight (n = 2798), sigmoid (n = 684), and advanced sigmoid esophagus (n = 200) were diagnosed. Multivariate analysis revealed that long disease duration, advanced age, obesity, and type I achalasia correlate positively, whereas severe symptoms and integrated relaxation pressure correlate negatively with development of sigmoid esophagus. In contrast, Grade II dilation (3.5-6.0 cm) was the most common (52.9%), while grade III dilation (≥ 6 cm) was rare (5.0%). We found early onset, male, obesity, and type I achalasia correlated positively, while advanced age correlated negatively with esophageal dilation. Dilated and sigmoid esophagus were found mostly in types I and II achalasia, but typically not found in spastic disorders. The prevalence of ED was low (n = 63, 1.7%), and non-dilated esophagus and advanced age correlated with ED development. Patients with right-sided ED (n = 35) had a long disease duration (P = 0.005) with low integrated relaxation pressure values (P = 0.008) compared with patients with left-sided ED (n = 22). Patients with multiple EDs (n = 6) had lower symptom severity than patients with a single ED (P = 0.022).ConclusionsThe etiologies of dilated esophagus, sigmoid esophagus, and ED are considered multifactorial and different. Early diagnosis and optimal treatment of AEMDs are necessary to prevent these conditions.

Role of Peroral Endoscopic Myotomy in Advanced Achalasia Cardia With Sigmoid and/or Megaesophagus: A Systematic Review and Metanalysis.

Background/AimsSigmoid esophagus and/or megaesophagus are considered as an advanced stage in the natural history of achalasia cardia. The role of peroral endoscopic myotomy (POEM) in these subset of patients is emerging. We performed a systematic review and metanalysis to study the efficacy of POEM in advanced achalasia cardia with sigmoid and megaesophagus.MethodsA literature search in PubMed and Embase was done from inception till August 3, 2021 to look for studies reporting exclusively on the role of POEM in advanced achalasia cardia with sigmoid and/or megaesophagus. The random effect method with inverse variance approach was used for the computation of pooled prevalence. For 2 groups’ analysis of continuous outcome standardized mean difference was used as the summary measure.ResultsEleven studies with 428 patients were included for analysis. The pooled technical and clinical success was 98.27% (95% CI, 96.19-99.22; I2 = 0%) and 89.38% (95% CI, 84.49-92.86; I2 = 26%) and on subgroup analysis into sigmoid and megaesophagus it was (98.06% [95% CI, 95.41-99.19; I2 = 0%], 98.47% [95% CI, 92.72-99.69; I2 = 0%] and 87.92% [95% CI, 80.68-92.70; I2 = 37%], 88.36% [95% CI, 62.62-97.17; I2 = 77%]) respectively. The clinical success at < 1 year and 1-3 year follow-up was 89.37% (95% CI, 82.82-93.61; I2 = 0%) and 88.66% (95% CI, 81.65-91.22; I2 = 46%) respectively. There was a significant reduction in the post-POEM scores with standardized mean difference for Eckardt score (4.81), for integrated relaxation pressure at 4 seconds (1.93), and for lower esophageal sphincter pressure (2.06).ConclusionsPOEM is an effective modality of treatment even in the subset of patients of advanced achalasia cardia with sigmoid and megaesophagus.

Geriatric patients with esophageal motility disorders benefit more from minimally invasive peroral endoscopic myotomy: a multicenter study in Japan.

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 ≥ 75years; n = 321) and non-geriatric (age < 75years; 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.

558 OUTCOMES OF PER ORAL ENDOSCOPIC MYOTOMY IN ACHALASIA IN PATIENTS WITH END-STAGE ESOPHAGUS

Abstract Per oral endoscopic myotomy (POEM) has emerged as a favorable alterative to Heller myotomy for achalasia. However data is limited on the effectiveness in palliation of symptoms in patients who have an esophagus with a sigmoid/tortuous configuration, or a mega-esophagus with width more than 5 cm, due to long-standing achalasia. Our objective is to investigate the outcomes after POEM procedure in patients diagnosed with achalasia and considered end-stage. Methods A retrospective cohort study was conducted in a large hospital between April 2014 and July 2019. A total of 504 patients were identified with 218 undergoing POEM during the study time period of which 57 patients had a sigmoid or tortuous esophagus, and/or had a barium column width of more than 5 cm on timed barium esophagograms (TBE). Primary outcome was symptom relief during follow-up, defined as a post-operative Eckardt score ≤ 3. We also explored integrated resting pressure (IRP) on manometry, barium column height and width on TBE, complications and procedure related parameters. Statistical analyses were performed using R. Results Significant decrease in median Eckardt scores were observed, 7.5 [6,8.8] pre-operatively to 0 [0,1] at 2 months (p &amp;lt; 0.001). At 2 years, 12 of 14 patients (85%), reported scores of ≤3. Improvements at 2 months were noted in median IRP (25.4 mmHg [15.5, 35.9] vs. 4.0 mmHg [2.2,9.0]), 1 min TBE height (11.5 cm [7.5,15.0] vs. 6.8 cm [4.7,9.0]) and width (5.0 cm [4.1,6.1] vs. 2.8 cm [1.9,3.1]), p &amp;lt; 0.001. At 2 years, they were 9.5 cm [5,12.8] and 3.0 cm [2.4,4.5], respectively. Post-operative pH studies in 35(61%) patients demonstrated a DeMeester score ≥ 14.72 in 17(49%) patients. Almost all patients reported return to activities of daily living within 7 days. Conclusion POEM is an effective and safe modality in achalasia patients with end-stage esophagus. Patients in this cohort historically have had poor outcome to interventions and our data shows excellent short-to-medium term outcome with low morbidity after POEM. Given its efficacy, minimal invasiveness and short return to function, POEM is a reasonable first and gold standard palliative therapy in this difficult cohort and should be attempted before considering esophagectomy.

The patient characteristics of esophageal carcinoma following laparoscopic myotomy for esophageal achalasia.

It is common knowledge that esophageal achalasia patients have a high risk of developing esophageal carcinoma. The present study assessed the characteristics of esophageal carcinoma patients following laparoscopic Heller-Dor surgery (LHD) for esophageal achalasia. Among 622 cases which were esophageal achalasia patients and underwent LHD as the primary surgery, we compared the patient background, pathophysiology, symptoms, and surgical outcomes according to whether or not esophageal carcinoma occurred following surgery. Six cases (0.96%) of postoperative esophageal carcinoma were confirmed. The characteristics of the cases in which esophageal carcinoma occurred were older age, longer disease duration (p = 0.0362 and 0.0028, respectively), decreased sphincter pressure of the lower esophagus, a high rate of sigmoid esophagus, and a long esophagus lateral diameter (p = 0.0214, 0.001, and 0.0416, respectively). Moreover, no differences in surgical outcomes were confirmed and there were no differences in symptoms from before and following surgery. The characteristics of esophageal carcinoma patients with achalasia following laparoscopic myotomy were an older age, longer disease duration, and greater progression of disease pathophysiology.

An Unexpected Thoracic Finding in a Patient With COVID-19

Question: A 75-year old woman presented to the emergency department with a history of epigastric discomfort and rapidly worsening thoracic pain. She had no fever or cough. On physical examination, she appeared overweight (102 kg, height 162 cm). Laboratory examination revealed normal cardiac troponin levels and slight increase in inflammatory markers (C-reactive protein and neutrophil-lymphocyte ratio). As a nasal screening swab was positive for coronavirus 2019 (COVID-19) infection, she was transferred to the COVID isolation ward. A subsequent computed tomography scan of the chest showed no interstitial lung disease. However, the presence of a monstrous esophageal dilatation with sigmoid shape and dislodgment of the surrounding structures was seen. The esophagus appeared full of solid content compatible with accumulated undigested food residues (Figure A–C). The patient was then referred to our gastroenterology unit. Upper endoscopy was performed under general anesthesia with endotracheal intubation. Endoscopic esophageal findings are shown in Figure D. What is the most likely diagnosis and how should it be managed? Look on page 1112 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The patient was suffering from type II achalasia, which was refractory to previous endoscopic and surgical treatment. In detail, she had undergone three endoscopic pneumatic dilation sessions in 2005 and ultimately laparoscopic Heller-Dor myotomy in 2015. In 2018, an esophageal high-resolution manometry was unsuccessful, probably because the catheter did not reach the stomach in the sigmoid dilated esophagus. We treated our patient with prolonged fasting and nasoesophageal tube placement at 35 cm from the incisors teeth. Multiple daily irrigations with saline solution were performed without apparent benefit. Three endoscopic sessions were then performed to clean the entire esophagus from the huge amount of feces-like food residues. We believe that 2 reasons were behind this ominous presentation. First, the patient suffered from poorly controlled hypothyroidism for which she was taking levothyroxine per os. Laboratory examination revealed high levels of thyrotropin (103 μU/mL) and low levels of free thyroxine (<2.5 pg/mL) and free tri-iodothyronine (1.5 pg/mL). Her severe hypothyroidism was probably due to the inability of oral levothyroxine to reach the stomach, thus decreasing the subjective awareness of her own clinical condition. Second, she was edentulous and this affected the chewing phase, favoring episodes of food impaction and accumulation of food in the esophagus. Surprisingly, she did not experience weight loss and her general nutritional status was good. Achalasia is an uncommon esophageal motility disorder, usually characterized by dysphagia to solids and liquids, chest pain, regurgitation, and weight loss. High-resolution esophageal manometry is the gold standard test for the diagnosis. Diagnostic findings include distal esophageal aperistalsis and integrated relaxation pressure of >15 mm Hg. Achalasia is classified as type 1 classic, type 2 compartmentalized high pressure in esophageal body, and type 3 spastic.1Weusten B.L.A.M. Barret M. Bredenoord A.J. et al.Endoscopic management of gastrointestinal motility disorders - part 1: European Society of Gastrointestinal Endoscopy (ESGE) Guideline.Endoscopy. 2020; 52: 498-515Crossref PubMed Scopus (41) Google Scholar In the last few years, peroral endoscopic myotomy is the most frequent endoscopic intervention for achalasia leading to outcomes that are comparable with those of laparoscopic Heller myotomy. Additional interventions are represented by pneumatic dilation and botulinum toxin injection. Esophagectomy is the last option to manage end-stage achalasia.2Familiari P. Greco S. Volkanovska A. et al.Achalasia: current treatment options.Expert Rev Gastroenterol Hepatol. 2015; 9: 1101-1114Crossref PubMed Scopus (11) Google Scholar,3Hammad A. Lu V.F. Dahiya D.S. et al.Treatment challenges of sigmoid-shaped esophagus and severe achalasia.Ann Med Surg (Lond). 2020; 61: 30-34Crossref PubMed Scopus (5) Google Scholar Peroral endoscopic myotomy was not considered for our patient because of the patent cardia (Figure E), the lack of manometric data, and the poor expected outcome in sigmoid esophagus. She was referred to the endocrinologist for further therapeutic workup. Moreover, dental prosthesis implantation was recommended.

Peroral Endoscopic Myotomy Is Safe and Highly Effective Treatment for Advanced Achalasia With Sigmoid Esophagus.

In advanced achalasia patients with sigmoid esophagus, peroral endoscopic myotomy (POEM) might be technically challenging and its outcomes are not well known in western population. Hence, our study aims were to assess and compare the safety and efficacy of POEM in achalasia patients with and without sigmoid esophagus. Medical records of achalasia patients who had POEM at our institution between April 2014 and December 2019 were reviewed. Patients who underwent work-up comprising timed barium esophagram (TBE) and high-resolution esophageal manometry (HREM) before POEM along with 2-month post-POEM esophageal pH study, TBE and HREM were included in the final analysis. Patients were categorized as either sigmoid or no sigmoid esophagus based on the morphology of esophagus on TBE as per Japan Esophageal Society guidelines. Treatment success was defined as reduction of post-POEM Eckardt score to ≤3. A total of 168 patients (sigmoid esophagus=20; no sigmoid esophagus=148) were included. Esophageal width on TBE at 1 minute was significantly higher in sigmoid esophagus group compared with no sigmoid esophagus group (4.9 vs. 3.0 cm, P<0.001). Procedural outcomes and complications were similar in both groups. At 2-month follow-up, both groups had significant improvement in Eckardt scores, TBE, and HREM parameters. Treatment success was high and similar in both sigmoid and no sigmoid groups (94.4% vs. 93.2%, P=0.84). The rates of gastroesophageal reflux disease were also similar in both groups. Our study findings suggest that POEM is safe and highly effective treatment for achalasia patients with sigmoid esophagus similar to those without sigmoid esophagus in western population. POEM should be considered as the treatment of choice in advanced achalasia with sigmoid esophagus.

Sa1312 CLINICAL OUTCOME OF PERORAL ENDOSCOPIC MYOTOMY IN ADVANCED ACHALASIA TREATMENT STRATIFIED BY MORPHOLOGICAL SUBTYPES

Peroral endoscopic myotomy (POEM) has been first-line treatment of achalasia. However, the efficacy and safety of POEM on advanced achalasia remains to be clarified. Our purpose was to evaluate the feasibility and clinical outcome of POEM for advanced AC with various esophageal morphological changes. From June 2015 to March 2019, a total of 50 patients were enrolled with advance achalasia (megaesophagus type in 23 patients, sigmoid type in 19 patients and both type in 8 patients). Primary endpoint was symptomatic relief during follow-up, defined as an Eckardt score ≤3. Secondary endpoints were intra-procedural details such as operation time, length of myotomy, post-treatment adverse events, hospital stay, as well as delayed AE, named gastroesophageal reflux disease (GERD). All patients successfully received endoscopic myotomy, with a mean procedure time of 38.5±26.3 (range, 10.7-162.6) min. In 10 patients (20%) adverse events were encountered, including mild (n=8) and moderate (n=6) without conversion to surgery and ICU admission. During a mean follow-up of 23.8 months, despite 41 (89.1%) patients achieved treatment success with a decrease in mean Eckardt score from 7.2 to 1.7 (P=6.7×10-9), only 4 (57.1%) patients in subgroup 3 (dilation and sigmoid esophagus) obtained clinical success. Symptomatic reflux occurred in 13 patients (13/46, 28.3%), and all reflux symptoms were easily controlled by intermittent oral medication with PPIs. In advanced achalasia patients with various esophageal morphological changes, POEM was a technically feasible, safe and effective option. POEM appears to be less effective in patients with significant dilated and distorted esophagus, which should be paid more attention in future managements.

Unusual achalasic sigmoid esophagus

Achalasia cardia is a rare primary esophageal motility disorder with an incidence of about 1 in 100,000 individuals. If left untreated or inappropriately treated, the disease may progress to end-stage achalasia characterized by megaesophagus or sigmoid esophagus. The presentation as severe malnutrition, anemia, hypoalbuminemia, and asymptomatic tracheal compression has not been described in the setting of the sigmoid esophagus, in absence of underlying malignancy. Here we report an interesting case of a young male, who presented with the above features and managed excellently by open Heller’s myotomy under epidural anesthesia due to the poor performance status.

O89 TWENTY-FIVE YEARS OF LAPAROSCOPIC TREATMENT FOR ESOPHAGEAL ACHALASIA: OUR EXPERIENCE ON 1001 LAPAROSCOPIC HELLER-DOR OPERATIONS

Abstract Background In the last decade of the past century, primary Laparoscopic Heller-Dor (LHD) for Achalasia progressively became the procedure of choice in the new millennium. The aim of this study was to assess the long-term outcome of LHD to treat Achalasia at a single high-volume institution during the past 25 years. Methods 1000 patients underwent LHD from 1992-2017 by 6 staff surgeons alternatively. Patients who had already been treated with surgical or endoscopic myotomy were ruled out. Symptoms were collected and scored using a detailed questionnaire; barium-swallow, endoscopy, manometry were performed, before and after surgery while, 24-hour pH monitoring were performed 6 months after surgery. Results LHD was the primary treatment for 1000 patients (M:F=536:464); the median age was 46 (IQR 36-54), 183 (18.3%) had a history of endoscopic treatments (pneumatic dilation or botox injections, or both). The surgical procedure was completed laparoscopically in all but 7 patients (0.7%) and there was one perioperative death for heart attack. There were 25 perforations (2.5%): 22 were recognized and repaired during the operation, 3 were detected by postoperative contrast swallow. The outcome was positive in 902 patients (90.2%). In patients who had a previous treatment the failures were 25/183 (13.7%) while in the primary treatment group the failures were 73/817 (8.9%) (p=0.055). All the 98 patients whose LHD failed subsequently underwent one or more endoscopic pneumatic dilations, which ameliorated their recurrent symptoms in all but 11 patients (10 of whom required reoperation). The overall success rate of the combination of LHD and endoscopic dilations (where necessary) was 98.4%. At univariate analysis, manometric pattern (p=0.001), sigmoid megaesophagus (p=0.003) and a chest pain score (p=0.002) were the only factors predictive of a positive final results. At multivariate analysis, these three factors were independently associated to good outcome. Postoperative 24-hour pH-monitoring was abnormal in 50/590 patients (8.5%) Two patients developed an esophageal cancer during the follow-up time. Conclusions In a university tertiary referral center LHD can durably relieve achalasia symptoms. Preoperative manometric pattern, a presence of a sigmoid esophagus and the chest pain score represent the strongest predictor of outcome.