Achalasia Research Articles

Endoscopic closure of 5-cm long esophageal perforation in an elderly patient with achalasia.

Chronic esophageal achalasia can lead to significant morphological changes in the esophagus, posing challenges during endoscopic examinations. Here, we report a case of iatrogenic esophageal perforation in an elderly patient with achalasia. An 80-year-old woman presented with severe dysphagia and marked weight loss, indicating chronic malnutrition. She had a 50-year history of achalasia, previously treated with a Heller-Dor operation. Endoscopic evaluation was performed to assess the esophageal condition; however, during the procedure, an iatrogenic perforation occurred. The patient underwent urgent management under general anesthesia. Two drainage tubes were placed at the base of the right thoracic cavity. Saline infusion via the perforation site enabled endoscopic lavage of the thoracic cavity. The perforation was initially closed with the thread-and-cliptechnique. After 30days, the perforation site had developed into a fistula approximately 1cm in size. Attempts to close it using argon plasma coagulation ablation and over-the-scope clip were unsuccessful. It was presumed that the significant accumulation of fluid in the esophagus due to achalasia was hindering fistula closure. Consequently, peroral endoscopic myotomy was performed, ultimately leading to the closure of the fistula. Despite requiring prolonged hospitalization for recovery and nutritional rehabilitation, the patient was eventually discharged in stable condition, able to consume meals without difficulty. This case underscores the highrisk of esophageal perforation during endoscopy in elderly patients with advanced achalasia and subsequentmalnutrition. Furthermore, it highlights the potential for successful management of severe complications like esophageal perforation through advanced endoscopic techniques, thereby avoiding the need for surgical intervention.

Safety and Feasibility of Paediatric Day Case Laparoscopic Upper Gastrointestinal Surgery: Significance of Nurse-led Discharge.

Safety and Feasibility of Paediatric Day Case Laparoscopic Upper Gastrointestinal Surgery: Significance of Nurse-led Discharge.

Mid-term outcomes of the patients of achalasia cardia undergoing laparoscopic Heller's myotomy with angle of his accentuation versus laparoscopic Heller's myotomy with Toupet's fundoplication.

The choice of anti-reflux procedure with Laparoscopic Heller's myotomy (LHM) is debatable. LHM with Dor's fundoplication is the most common procedure performed and has shown similar outcomes to LHM with Toupet's fundoplication and LHM with Angle of His accentuation (AOH). The present study describes the mid-term outcomes comparing LHM with AOH and LHM with Toupet's fundoplication. Eighty-six patients who underwent LHM-AOH (group A, n = 43), versus LHM-Toupet's fundoplication (group B, n = 43) for achalasia cardia were followed up for symptomatic improvement (Eckardt score, ES ≤ 3), improvement in Achalasia disease specific health related quality of life (ADsQoL) and clinical reflux (DeMeester's heartburn score ≥ 2, and esophagitis on endoscopy). At a median duration of 52.5 (IQR 34-67) months, mean ES in both groups decreased (7.42 to 1.2, p < 0.00001, group A and 7.37 to 1.23, p < 0.00001, group B) with no difference among the two groups. Mean ADsQoL scores decreased in both groups significantly (23.3 to 13.7, p < 0.00001, group A and 23.2 to 13.3, p < 0.00001, group B) with the mean ADsQoL scores at the last follow-up being statistically similar in both the groups (p = 0.93). Clinically significant heartburn was seen in 5 (11.6%) and 7 (16.3%) patients in group A and B, respectively (p = 0.76). Ninety-one and 95% patients in group A and B, respectively, had good to excellent satisfaction. Follow-up UGIE (median: 12months) could be performed in 33 (group A) and 28 patients (group B), with 9 (27%) patients in group A and 4 (14.3%) patients in group B showing LA-A or B esophagitis (p = 0.22). No patient had LA-C/D esophagitis. Frequent use of proton pump inhibitors was there by 5 patients in each group (11.6%). LHM-AOH and LHM-Toupet's fundoplication are comparable with respect to symptomatic relief, improvement in quality of life and clinical reflux rates.

Concurrent Diagnosis of Superficial Esophageal Cancer and Esophageal Achalasia: A Case Report and Literature Review.

We report a case of a 70-year-old woman with esophageal achalasia and concurrent superficial esophageal squamous cell carcinoma. Three adjacent superficial lesions were resected en bloc by endoscopic submucosal dissection (ESD), with no lymphovascular invasion. Given that the patient's dysphagia was effectively controlled with medication and dietary modifications, peroral endoscopic myotomy (POEM) was deferred following a careful assessment of the risk-benefit balance. As both ESD and POEM involve submucosal intervention, this case highlights the importance of individualized treatment based on symptom severity and lesion characteristics.

Computer-aided detection for esophageal achalasia (withvideo).

Achalasia is an esophageal motility disorder that impairs quality of life and is often missed (20-50%) on endoscopy. A newly developed computer-aided detection (CAD) software has shown high accuracy for achalasia diagnosis in preclinical settings. However, its benefit in a clinical setting remains unclear. Between February and August 2023, 83 endoscopists from 27 centers assessed 50 randomized endoscopic videos (25 achalasia, 25 nonachalasia) without and with CAD. Endoscopists assessed videos without CAD, then with CAD after 2 months. The primary end-point was improvement in sensitivity for nonexperienced endoscopists (no endoscopic experience of achalasia). Sensitivity, specificity, and accuracy with and without CAD were compared using the McNemar test. Sensitivity for diagnosing achalasia increased significantly with CAD, rising from 74.2% (95% confidence interval [CI] 72.2-76.0%) to 91.2% (95% CI 89.9-92.4%) for all readers, showing a difference of 17.1% (95% CI 15.1-19.0%). Specifically, sensitivity improved from 66.9% (95% CI 63.6-70.0%) to 91.9% (95% CI 89.9-93.6%) among nonexperienced endoscopists, resulting in a difference of 25.0% (95% CI 21.7-28.4%), and from 79.5% (95% CI 77.1-81.8%) to 90.8% (95% CI 89.0-92.3%) among experienced endoscopists (endoscopic experience of at least one achalasia case), with a difference of 11.3% (95% CI 8.9-13.6%). Accuracy and specificity improved significantly with CAD assistance, regardless of reader's experience. CAD improves achalasia detection by 17%, confirming preclinical results. The benefit was higher for nonexperienced endoscopists. CAD assistance may lead to prompt and effective treatment, minimizing the risk of false-negative diagnosis in clinical practice. This study was registered in the University Hospital Medical Information Network Clinical TrialRegistry (https://www.umin.ac.jp/ctr/) number: UMIN000053047.

Enteric Neuromuscular Manifestations in Neurofibromatosis Type I.

Several genetic syndromes that affect nerve development and functioning may involve the enteric nervous system and present clinically as dysmotility syndromes, typically in childhood. To review the enteric neuromuscular manifestations, predominantly observed in adults, of neurofibromatosis type I at a tertiary referral center. We conducted a medical records review at the Mayo Clinic and documented clinical manifestations and findings on radiology, pathology, and specialized motility tests of the esophagus, stomach, colon, and rectal evacuatory functions. The tests included scintigraphic gastrointestinal and colonic transit measurements, intraluminal esophageal, gastrointestinal, colonic, and anorectal manometry, and balloon expulsion test. Among 2406 with documented NF1, the gastrointestinal manifestations were obstruction or dysmotility, seen in 2% of the cohort. Thirteen patients had small bowel or colonic obstructions: 4 gastrointestinal stromal tumors, 3 malignant peripheral nerve sheath tumors, 3 neurofibromas, 1 diffuse ganglioneuromatosis, 1 schwannoma, and 1 inflammatory fibroid polyp. In addition, 38 patients had abnormal gut motility, including esophageal achalasia or spasm, delayed gastric emptying, slow colonic transit, and dyssynergic defecation. Gastric, small bowel, and colonic manometry were characterized by normal amplitude incoordinated contractions suggestive of neuropathy. In the few resected specimens, myenteric plexus proliferation or diffuse ganglioneuromatosis was identified histologically. In addition to mechanical obstruction, typically due to benign tumors affecting smooth muscle or components of nerve (sheath or nerve fiber), patients with NF1 may present with dysmotility syndromes such as gastroparesis, slow colonic transit, or global dysmotility. Neuropathic dysmotility in NF1 can be identified by manometry and by histological evidence of myenteric plexus proliferation or diffuse ganglioneuromatosis.

Gastric-Shaped Esophagus in Achalasia.

Gastric-Shaped Esophagus in Achalasia.

Редкий генетический синдром Оллгрова в практике детского эндокринолога

Abstract Diagnostics of orphan diseases is often associated with certain difficulties. Allgrove syndrome (Alacrimia, Achalasia, Adrenalinsufficiency, AAAS), the "three A" syndrome, is a rare autosomal recessive multisystem disease characterized by chronic adrenal insufficiency, alacrimia and achalasia of the cardia. The prevalence of the pathology in question is less than 1 case per 1 000 000, no more than 100 cases have been described in the literature since its publication in 1978 [1]. The clinical picture of this syndrome is diverse, symptoms, their severity, and the sequence of manifestation vary with age, and therefore treatment tactics are selected individually when making a diagnosis. The article presents a clinical case of a patient who was diagnosed with Allgrove syndrome at the age of 10. The clinical picture is represented by adrenal insufficiency, delayed neuropsychic development, convulsive syndrome, alacrimia, achalasia of the esophagus with the phenomena of "nervous" esophagus, as well as a number of additional signs – vulgar ichthyosis, congenital heart defect: defect of the interventricular septum, patent oval window. Given the rarity and high phenotypic heterogeneity of this pathology, the diagnosis was delayed. A number of primary and concomitant conditions contributed to the involvement of specialists of different profiles in therapeutic tactics, and also determined the need for long-term dispensary observation.

Heller Myotomy for Esophageal Achalasia: Outcomes in 1010 Patients with Longitudinal Follow-Up.

Heller Myotomy for Esophageal Achalasia: Outcomes in 1010 Patients with Longitudinal Follow-Up.

Esophageal Contrast Transesophageal Echocardiography for Mitral Valve Transcatheter Edge-to-Edge Repair in Severe Esophageal Achalasia.

Esophageal Contrast Transesophageal Echocardiography for Mitral Valve Transcatheter Edge-to-Edge Repair in Severe Esophageal Achalasia.

Expert opinion on the management of esophageal achalasia from the Society for Surgery of the Alimentary Tract (SSAT) Global Outreach Committee

Expert opinion on the management of esophageal achalasia from the Society for Surgery of the Alimentary Tract (SSAT) Global Outreach Committee

Robotic Surgery for the Treatment of Achalasia with Intraoperative Manometry: Case Report

Introduction: Achalasia, the Greek term meaning, is a disease of unknown cause in which there is a failure to relax the lower esophageal sphincter and loss of peristalsis in the distal esophagus. Achalasia was first described by Sir Thomas Willis in 1674. Clinical case: 63-year-old female with no significant history for the case. Upon admission, the patient presented dysphagia, reflux, aspiration, substernal pain, and weight loss. Endoscopy showed Schatzki ring; Type I hiatal hernia of 5 cm, grade D esophagitis (Los Angeles classification). Manometry showed an IRP -3.06; absent esophageal clearance, without evidence of hiatal hernia, with a diagnosis of probable achalasia. The SEGD presented an area of V stenosis and a Heller myotomy with Dor-type fundoplication was performed using robotic surgery. Discussion: In clinical practice, the combined use of esophagogastroduodenoscopy (EGD) and upper gastrointestinal series (SGD) helps to evaluate patients who present with dysphagia and/or regurgitation, which are the most common clinical findings in these patients. High-resolution esophageal manometry (HREM) has become an essential tool in the last decade to categorize types of esophageal achalasia with associated lack of relaxation of the lower esophageal sphincter. Current pharmacological, endoscopic and surgical treatment options aim to reduce the hypertonicity of the lower esophageal sphincter, but unfortunately, the absence of esophageal peristalsis does not restore it to normal propulsive waves. Pharmacological treatments, including calcium channel blockers or sequential injection of botulinum toxin, are usually limited to patients who are not amenable to surgical treatment and have limited effects.

2024 Update to SAGES guidelines for the use of peroral endoscopic myotomy (POEM) in the treatment of achalasia.

Peroral Endoscopic Myotomy (POEM) is a safe treatment for esophageal achalasia with comparable safety to Heller myotomy plus fundoplication and pneumatic dilatation (PD). More data comparing POEM to Heller Myotomy plus fundoplication and pneumatic dilation are available since the previous Society of American Gastrointestinal and Endoscopic Surgeons (SAGES) guideline published on this topic in 2021. This guideline provides an update to the evidence-based guideline from SAGES published in 2021. It is intended to support clinicians, surgeons, and patients in decisions about the use of POEM for the treatment of achalasia. The panel agreed on 3 recommendations for adults with achalasia. A conditional recommendation for the use of POEM over PD was issued. Conditional recommendations included the option of using either POEM with appropriate use of proton pump inhibitors (PPIs) or HM with fundoplication to treat achalasia. POEM was favored over HM with fundoplication for achalasia subtype III. No new literature was found for children, and the previous recommendation from 2021 should be referred to for this population. POEM is a safe and effective treatment for achalasia in adults. It demonstrates greater efficacy than PD, though there are specific situations where PD may be the more appropriate choice. POEM has previously been compared to Heller myotomy, but this comparison is less relevant to modern surgical practice. Shifting the paradigm, POEM plus appropriate use of PPIs should be compared to Heller plus fundoplication. This reflection of current practice allows for a conditional recommendation for either approach in the treatment of achalasia subtypes 1 and 2, and for a preference of POEM for subtype 3.

Fibrosis in the Hiatus of Esophagus in Patients With Primary Esophageal Motor Disorders: Radiomic Analysis

ABSTRACTIntroductionIn normal subjects, the esophagus and crus of the diaphragm slide relative to each other during esophageal peristalsis, which is not the case in patients with achalasia esophagus.ObjectiveTo examine the tissue characteristics of the esophageal hiatus in patients with achalasia esophagus, high amplitude esophageal contractions (HAEC), esophagogastric junction outflow obstruction (EGJOO), and normal subjects using radiomics analysis of the CT images.MethodsA large number of medical records (1221 controls, 409 achalasia, 562 EGJOO, and 349 HAEC) were screened to identify subjects with a CT scan of the abdomen/chest in their record that met the pre‐determined criteria. Using multiplanar rotation of CT images, the esophageal hiatus was visualized in a 2D plane. The grayscale intensity distributions (GSID) of the three regions of interest, i.e., (1) subcutaneous adipose tissue, (2) a posterior spine muscle (marker of fibro‐connective tissue), and (3) esophageal hiatus were determined.ResultsNo differences in the mean GSID of subcutaneous adipose tissue and muscle were found among the four groups. On the other hand, the esophageal hiatus mean GSID was significantly higher in the three patient groups compared to controls. The GSIDs were higher in achalasia patients than in HAEC and EGJOO patients. The mean GSI of the hiatus was closer to that of the muscle with greater overlap between the hiatus and muscle in the three patient groups; the overlap was greater in achalasia as compared to HAEC and EGJOO patients.ConclusionA higher GSI of the hiatus with greater overlap between the hiatus and muscle suggests an increase in fibro‐connective tissue in the esophageal hiatus of patients with primary esophageal motility disorders (EMD). We propose that fibrosis in the esophageal hiatus restricts axial separation between the LES and crural diaphragm, which may be important in the pathogenesis of esophageal motor disorders.

METABOLIC CHARACTERISTICS OF GASTRIC CONTENTS UNDER CONDITIONS OF NEUROMUSCULAR DYSFUNCTION OF THE ESOPHAGOGASTRIC JUNCTION

Neuromuscular dysregulation at the esophagogastric junction, characteristic of achalasia cardia, leads to alterations in the metabolic composition of gastric contents, particularly in acidity, pepsin concentration, bile acids, and protective mucosal factors. Investigating these changes is essential for understanding the pathophysiological mechanisms underlying motor-secretory dysfunction and the development of esophageal and gastric disorders. The relevance of such research is underscored by the limited available data on the biochemical composition of gastric juice in the context of esophageal dysfunction, especially achalasia cardia, thereby highlighting the need for further investigations in this area. Aim. The work was purposed to determine the biochemical characteristics of gastric contents in neuromuscular dysfunction of the esophagogastric junction. Materials and Methods. In total of 69 patients with achalasia of the cardia and 26 control subjects were examined. The following parameters were assessed: pH, pepsin, cholic acid, NOx, calcium, glycoproteins, fucose, glycosaminoglycans, and sialic acid. Results. Patients demonstrated increased gastric content volume (2.9 times), cholic acid (1.8 times), and sialic acid (2.4 times), as well as elevated NOx (68.2%) and calcium (31.6%) levels. Glycoproteins (85.7%), fucose (73.0%), and glycosaminoglycans (35.5%) were significantly decreased. Conclusions. Neuromuscular dysfunction is associated with alterations in the composition of gastric juice, weakening of protective factors, and accumulation of aggressive metabolites, indicating local homeostatic disruption.

Curriculum for training in peroral endoscopic myotomy (POEM) in Europe (Part I): European Society of Gastrointestinal Endoscopy (ESGE) Position Statement.

Peroral endoscopic myotomy (POEM) is an advanced endoscopic procedure that has become a first-line treatment for esophageal achalasia and other esophageal spastic disorders. Structured training is essential to optimize the outcomes of this technique. The European Society of Gastrointestinal Endoscopy (ESGE) has recognized the need to formalize and enhance training in POEM. This Position Statement presents the results of a systematic review of the literature and a formal Delphi process, providing recommendations for an optimal training program in POEM that aims to produce endoscopists competent in this procedure. In a separate document (POEM curriculum Part II), we provide technical guidance on how to perform the POEM procedure based on the best available evidence. 1: POEM trainees should acquire a comprehensive theoretical knowledge of achalasia and other esophageal motility disorders that encompasses pathophysiology, diagnostic tool proficiency, clinical outcome assessment, potential adverse events, and periprocedural management. 2: Experience in advanced endoscopic procedures (endoscopic mucosal resection and/or endoscopic submucosal dissection [ESD]) is encouraged as a beneficial prerequisite for POEM training. 3: ESGE suggests that POEM trainees without ESD experience should perform an indicative minimum number of 20 cases on ex vivo or animal models before advancing to human POEM cases with an experienced trainer. 4: ESGE recommends that the trainee should observe an indicative minimum number of 20 live cases at expert centers before starting to perform POEM in humans. 5: The trainee should undertake an indicative minimum number of 10 cases under expert supervision for the initial human POEM procedures, ensuring that trainees can complete all POEM steps independently. 6: ESGE recommends avoiding complex POEM cases during the early training phase. 7: POEM competence should reflect the technical success rate, both the short- and long-term clinical success rates, and the rate of true adverse events. 8: A POEM center should maintain a prospective registry of all procedures performed, including patient work-up and outcomes, procedural techniques, and adverse events.

Factors predicting insufflation-related events in peroral endoscopic myotomy procedures.

Factors predicting insufflation-related events in peroral endoscopic myotomy procedures.

Endoscopic management of achalasia cardia: a regional approach for per oral endoscopic myotomy (POEM) in the Northern Region – lessons learnt in developing a service

IntroductionPer oral endoscopic myotomy (POEM) for achalasia is available in many but not all areas of the UK; this article maps lessons learnt in bringing POEM to the Northern Region...

Endoscopic gastrostomy as a method of enteral nutrition providing

The aim of the study - evaluation of the effectiveness and possibility of using endoscopic gastrostomy to ensure long enteral nutrition. Materials and methods. For the period from 2004 to 2023, 125 patients aged 16 to 89 years (average 65.23 years) underwent endoscopic percutaneous gastrostomy in the clinic (68 men, 57 women). The majority of patients (105-84%) were patients with brain pathology, impaired consciousness and swallowing. Absolute indications for the intervention were impaired swallowing function, severe patient condition requiring long-term enteral nutrition including for chemotherapy or radiation therapy (10-16% of patients), preoperative preparation (2 patients with stage IV esophageal achalasia for up to 8 months and 2 patients with Zenker’s diverticulum for 2 months). Results. Endoscopic gastrostomy was inserted in all patients. No complications were observed. Preliminary dilatation of the lumen of the esophagus and (or) pharynx by bougienage along the guidewire to No. 36-40 Fr was performed in 10 (8%) patients with tumor stenosis. In the early postoperative period 6 (4,8%) patients had complications: postoperative wound suppuration in 4, abdominal phlegmon in 2, in periods longer than 14 days in 9 (7.2%): bumper syndrome in 7, gastrostomy tube prolapse in 2 patients. Conclusions. Endoscopic gastrostomy is an effective and relatively safe method of providing enteral nutrition due to its low trauma. Indications for the intervention may be diseases and traumatic brain injuries with swallowing disorders, neurological disorders (apallic syndrome), head and neck tumors for the purpose of special treatment. This technique can be used for preoperative preparation in patients with neuromuscular diseases of the esophagus. Gastrostomy tube can be removed at the end of the treatment course and/or resumption of swallowing. It is necessary to follow the rules for gastrostomy care after the patient is discharged from the hospital to prevent complications associated with long-term presence of the gastrostomy.

POEM-F for sigmoid esophagus in patients of achalasia cardia: Technical challenges and short-term outcomes

POEM-F for sigmoid esophagus in patients of achalasia cardia: Technical challenges and short-term outcomes