Search

Methods: This expert review was commissioned and approved by the American Gastroenterological Association (AGA) Institute Clinical Practice Updates Committee (CPUC) and the AGA Governing Board to provide timely guidance on a topic of high clinical importance to the AGA membership, and underwent internal peer review by the CPUC and external peer review through standard procedures of Clinical Gastroenterology and Hepatology. These Best Practice Advice (BPA) statements were drawn from a review of the published literature and from expert opinion. Since systematic reviews were not performed, these BPA statements do not carry formal ratings regarding the quality of evidence or strength of the presented considerations., Description: Infectious and immune-mediated esophageal disorders are poorly understood and often under-diagnosed conditions that lead to esophageal dysfunction and health care costs due to repeated procedures and a lack of understanding of their etiology and pathogenesis. Without a high index of suspicion, these disorders may be overlooked. Esophageal dysfunction may arise from active, localized infection and immune-mediated disease (ie, candida, etc.) or from an organ-specific manifestation of a more diffuse immune-mediated disease or infection (ie, systemic sclerosis, connective tissue disease, neurologic disease). These conditions can sometimes lead to neuromuscular dysfunction and subsequent esophageal dysmotility. Awareness of local and systemic processes that lead to esophageal dysfunction will improve patient outcomes by focusing therapeutics and limiting unnecessary procedures. Therefore, the purpose of this AGA Clinical Practice Update Expert Review is to provide BPA on diagnostic considerations of immune-mediated disorders that should be considered when encountering patients with dysphagia, heartburn, and odynophagia. Best Practice Advice Statements: BEST PRACTICE ADVICE 1: Gastroenterologists should be aware of the esophageal manifestations of systemic immunologic and infectious diseases to reduce diagnostic delay. Clinicians should identify if there are risks for inflammatory or infectious possibilities for a patient's esophageal symptoms and investigate for these disorders as a potential cause of esophageal dysfunction. BEST PRACTICE ADVICE 2: Once esophageal infection is identified, clinicians should identify whether accompanying signs/symptoms suggest immunocompromise leading to a more systemic infection. Consultation with an infectious disease expert will aid in guiding appropriate treatment. BEST PRACTICE ADVICE 3: If symptoms do not improve after therapy for infectious esophagitis, evaluation for refractory infection or additional underlying sources of esophageal and immunologic dysfunction should be performed. BEST PRACTICE ADVICE 4: In individuals with eosinophilic esophagitis (EoE) who continue to experience symptoms of esophageal dysfunction despite histologic and endoscopic disease remission, clinicians should be aware that some patients with EoE may develop motility disorders. Further evaluation of esophageal motility may be warranted. BEST PRACTICE ADVICE 5: In individuals with histologic and endoscopic features of lymphocytic esophagitis, clinicians should consider treatment of lymphocytic-related inflammation with proton-pump inhibitor therapy or swallowed topical corticosteroids and as needed esophageal dilation. BEST PRACTICE ADVICE 6: In patients who present with esophageal symptoms in the setting of hypereosinophilia (absolute eosinophil count [AEC] >1500 cells/uL), consider further work-up of non-EoE eosinophilic gastrointestinal (GI) disease, hypereosinophilic syndrome, and eosinophilic granulomatosis with polyangiitis (EGPA). Consultation with allergy/immunology may help guide further diagnostic work-up and treatment. BEST PRACTICE ADVICE 7: In individuals with rheumatologic diseases of systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), or Sjogren's disease, clinicians should be aware that esophageal symptoms can occur due to involvement of the esophageal muscle layer, resulting in dysmotility and/or incompetence of the lower esophageal sphincter. The degree of dysfunction is often especially significant in those with SSc or MCTD. BEST PRACTICE ADVICE 8: In individuals with Crohn's disease, clinicians should be aware that a minority of individuals can develop esophageal involvement from inflammatory, stricturing, or fistulizing changes with granulomas seen histologically. Esophageal manifestations of Crohn's disease tend to occur in individuals with active intestinal disease. BEST PRACTICE ADVICE 9: In individuals with dermatologic diseases of lichen planus or bullous disorders, clinicians should be aware that dysphagia can occur due to endoscopically visible esophageal mucosal involvement. Esophageal lichen planus, in particular, can occur without skin involvement and can be difficult to define on esophageal histopathology. BEST PRACTICE ADVICE 10: Clinicians should consider infectious and inflammatory causes of secondary achalasia during initial evaluation. One should query for any history of recent COVID infections, risks for Chagas disease, and symptoms or signs of eosinophilic disease., (Copyright © 2024. Published by Elsevier Inc.)

Competing Interests: The authors declare that they have no conflict of interest.

Competing Interests: The authors declare that they have no conflict of interest.

Background: Esophageal melanosis (EM) is a rare condition characterized by melanin pigmentation in the esophageal mucosa. It is not well understood and has been documented in less than 100 cases worldwide., Case Summary: We report two cases of African American patients who complained of significant weight loss (over 20 pounds in approximately six months) and abdominal pain during their first visit. The first case involves a 54-year female with a history of hepatic steatosis and polysubstance abuse, who also experiences nausea and vomiting. The second case is a 59-year-old male with hypertension and gastroesophageal reflux disease (GERD), who was diagnosed with esophageal squamous cell carcinoma. Both cases show benign melanocytes in the basal layer on the esophagus biopsy and are diagnosed as EM., Conclusion: It is important to note that EM has been associated with malignancies such as carcinoma and melanoma. Therefore, accurate diagnosis and appropriate management are crucial. Patients with EM, especially those with concurrent risk factors ( e.g. , GERD, smoking), should be carefully monitored for any signs of malignancy., Competing Interests: Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Stevens-Johnson syndrome (SJS) is a severe adverse drug reaction affecting the skin and mucosal membranes, characterised by lesions covering less than 10% of the body's surface. Although gastrointestinal (GI) complications are usually mild, severe GI involvement can be life-threatening. A woman in her 30s with end-stage renal disease developed sepsis from methicillin-sensitive Staphylococcus aureus After 15 days of hospitalisation and antibiotic treatment, she developed new skin lesions, followed by massive GI bleeding and haemodynamic instability. Oesophagogastroduodenoscopy revealed necrotic plaques and mucosal sloughing along the oesophagus. Skin biopsy results confirmed SJS. Despite intensive care and multiple blood transfusions, she required intravenous dexamethasone. The patient improved and was discharged after 6 weeks. SJS can lead to serious complications, including rare but potentially life-threatening GI involvement. Effective management involves drug withdrawal, intensive care, immunosuppression and symptomatic treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Aim: Patients with proton-pump-inhibitor (PPI)-unresponsive reflux symptoms, often caused by functional esophageal disorders (FED), are frequently encountered in clinical practice. We aimed to investigate the prevalence of FED and its associated clinical characteristics in patients with PPI-unresponsive reflux symptoms., Methods: We retrospectively identified patients who were evaluated for persistent typical reflux symptoms, despite ≥8 weeks of PPI treatment, at the National Taiwan University Hospital from 2014 to 2023. All patients underwent a comprehensive evaluation comprising validated gastroesophageal reflux disease (GERD) symptom questionnaires, 5-item Brief Symptom Rating Scale (BSRS-5), Pittsburgh Sleep Quality Index (PSQI), esophagogastroduodenoscopy, high-resolution impedance manometry, and 24-h impedance-pH monitoring off PPI therapy. Diagnosis of FED and non-erosive reflux disease (NERD) was based on the Rome IV criteria., Results: We analyzed 190 patients [46.8% male, median age 52 (interquartile range, 42-61) years], of whom 32 (16.8%) had NERD and 158 (83.2%) had FED (57.9% with functional heartburn and 25.3% with reflux hypersensitivity). Patients with FED had a lower body mass index than those with NERD and a higher prevalence of psychological comorbidities and poor sleep quality than healthy volunteers. The severity of reflux symptoms among FED patients was significantly associated with the severity of psychological comorbidities and sleep quality., Conclusions: A notably high prevalence (83.2%) of FED was observed among patients experiencing PPI-unresponsive reflux symptoms. Patients with FED had a higher level of psychological distress and diminished sleep quality, both of which were associated with reflux symptom severity., (© 2024 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Acute esophageal necrosis (AEN), known as black oesophagus or Gurvit's syndrome, is an extremely rare clinical syndrome. Patients usually present with life-threatening symptoms of upper gastrointestinal haemorrhage (70-90% of cases), as in this case report. Diagnosis of AEN is confirmed based on oesophagogastroduodenoscopy which reveals diffuse or patchy circumferential black necrotic oesophageal mucosa. The presented patient had some comorbidities, such as poorly controlled diabetes mellitus, secondary to chronic pancreatitis, hypertension, after cholecystectomy, addiction to alcohol and tobacco, and taking a small spoon of baking soda three times a day for a few months because of heartburn. Despite the poor prognosis of AEN, with mortality about 32-35%, most patients present with endoscopic improvement in short time - from 7 days to 1 month. Fortunately, the patient did not to have any complications in the course of AEN, and the treatment was effective.

Background: Spontaneous rupture of the oesophagus is a potentially fatal condition. Symptoms can vary and diagnosis can be challenging., Case Presentation: A woman in her seventies presented to the emergency department with sudden-onset epigastric pain after a meal. A computed tomography (CT) showed signs of oesophageal rupture. Upper gastrointestinal endoscopy revealed an oesophageal rupture, and a stent was placed. The patient developed fever, dyspnoea and hypotension after the procedure. Additional CT revealed increasing pleural effusion, pneumomediastinum and loculaments of air in the peritoneum, and a mediastinal abscess. Laparoscopy with lavage and debridement was performed. A catheter was placed in the abscess and a chest tube in her right hemithorax. The stent was removed after 27 days. Further investigation revealed eosinophil oesophagitis as the likely cause of her oesophageal rupture., Interpretation: This case highlights the importance of early diagnosis and proper treatment of spontaneous oesophageal rupture. Treatment depends on the cause of the rupture and severity of the patient's condition.

Competing Interests: Conflicts of interest The authors declare no conflicts of interest.

Background: Oesophageal disorders and chronic liver disease are common worldwide and significantly impact quality of life. The intricate link between these conditions, including how oesophageal disorders like GERD, Barrett's oesophagus and oesophageal cancer affect and are affected by chronic liver disease, remains poorly understood., Aims: To review the relationship between oesophageal disorders and chronic liver disease, evaluating epidemiology, pathophysiology and therapeutic factors., Methods: We reviewed the literature on the relationship between oesophageal disorders and chronic liver disease, including cirrhosis, using the PubMed database RESULTS: Oesophageal disorders such as gastroesophageal reflux disease, Barrett's oesophagus, oesophageal cancer, oesophageal motor disorders and oesophageal candidiasis are prevalent among individuals with cirrhosis, exacerbating the burden of liver disease. These diseases have a multifaceted symptomatology and pathogenic basis, posing a significant challenge in cirrhotic patients that necessitates careful diagnosis and management. Additionally, therapies frequently used for these diseases, such as proton pump inhibitors, require careful consideration in cirrhotic patients due to potential adverse effects and altered pharmacokinetics. Managing oesophageal disorders in cirrhotic patients requires a cautious approach due to possible interactions with medications and the risk of adverse effects. Furthermore, symptoms associated with these conditions are often exacerbated by common interventions in patients with cirrhosis, such as band ligation for oesophageal varices., Conclusions: Oesophageal disorders are common in cirrhosis and increase the disease burden. These conditions require careful management due to complex symptoms and treatment risks. Proton pump inhibitors and other therapies must be used cautiously, as cirrhosis interventions can worsen symptoms., (© 2024 The Author(s). Alimentary Pharmacology & Therapeutics published by John Wiley & Sons Ltd.)

Hiatus hernia (HH) is a prevalent endoscopic finding in clinical practice, frequently co-occurring with esophageal disorders, yet the prevalence and degree of association remain uncertain. We aim to investigate HH's frequency and its suspected association with esophageal disorders. We reviewed endoscopic reports of over 75,000 consecutive patients who underwent gastroscopy over 12 years in two referral centers. HH was endoscopically diagnosed. We derived data on clinical presentation and a comprehensive assessment of benign and malignant esophageal pathologies. We performed multiple regression models to identify esophageal sequela associated with HH. The overall frequency of HH was (16.8%); the majority (89.5%) had small HHs (<3 cm). Female predominance was documented in HH patients, who were significantly older than controls (61.1±16.5 vs. 52.7±20.0; P < 0.001). The outcome analysis of esophageal pathology revealed an independent association between HH, regardless of its size, and erosive reflux esophagitis (25.7% vs. 6.2%; OR = 3.8; P < 0.001) and Barrett's esophagus (3.8% vs. 0.7%; OR = 4.7, P < 0.001). Furthermore, following rigorous age and sex matching, in conjunction with additional multivariable analyses, large HHs were associated with higher rates of benign esophageal strictures (3.6% vs. 0.3%; P < 0.001), Mallory Weiss syndrome (3.6% vs. 2.1%; P = 0.01), and incidents of food impactions (0.9% vs. 0.2%; P = 0.014). In contrast, a lower rate of achalasia was noted among this cohort (0.55% vs. 0%; P = 0.046). Besides reflux-related esophageal disorders, we outlined an association with multiple benign esophageal disorders, particularly in patients with large HHs., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: Esophageal safety following radiofrequency (RF) left atrial (LA) linear ablation has not been established. To determine the esophageal safety profile of LA linear RF lesions, we performed systematic esophagogastroduodenoscopy in all patients with intraesophageal temperature rise (ITR) ≥ 38.5°C., Methods and Results: Between December 2021 and July 2023, a total of 200 consecutive patients with atrial tachyarrhythmia (ATA) underwent linear ablation with posterior dome (roof or floor) or posterior mitral isthmus line transection. Patients with ITR ≥ 38.5°C were scheduled for esophageal endoscopy ~3 weeks after ablation. Patient and ATA characteristics, procedural parameters, endoscopy findings and ablation lesion data were collected and analyzed. One hundred thirty-three out of 200 (67%) patients showed ITR ≥ 38.5°C during LA linear ablation. ITR (with maximal temperature of 45.7°C) was more frequently observed during floor line ablation (82% of cases). ITR was less observed during roof line ablation (34%) and posterior mitral isthmus ablation (4%). Endoscopy, performed in 115 patients after 24 ± 10 days, showed esophageal ulceration in four patients (two patients Kansas City classification [KCC] 2a and two patients KCC 2b). No patient showed esophageal perforation or fistula., Conclusion: Temperature rise during LA linear ablation is frequent and ulceration risk exists, particularly when floor line is performed. Safety measures are needed to avoid potential severe complications like esophageal perforation and fistula., (© 2024 Wiley Periodicals LLC.)

Background: Ketosis-prone diabetes (KPD) is an intermediate subtype of diabetes mellitus, usually affecting Afro-American adults, presenting with diabetic ketoacidosis (DKA), without the classic phenotype of autoimmune type 1 diabetes. Patients require insulin therapy at onset for the acute decompensation, then usually remain insulin-free for prolonged periods with diet alone or with other antidiabetic drugs. DKA can be rarely complicated by upper gastrointestinal bleeding and mucosal necrosis, a severe complication named acute esophageal necrosis (AEN) burdened by high mortality. The association of KPD presenting with DKA complicated by AEN is here reported for the first time, to the knowledge of the authors, in the medical literature., Case Presentation: Here we report an interesting case of middle-aged African woman, newly diagnosed with KPD, presenting with DKA hematemesis. The patient was first treated at Intensive Care Unit for the ketoacidosis with intravenous fluids combined with continuous insulin infusion, and then switched to subcutaneous regimen. At the same time, esophagogastroduodenoscopy (EGD) was performed to diagnose acute esophageal necrosis, which was promptly managed with proton pump inhibitors infusion, fasting, and parenteral nutrition. After the correct clinical evaluation, the patient was switched to oral antidiabetic and basal insulin at discharge and an EGD follow-up was scheduled., Conclusions: KPD remains an under-recognized and under-diagnosed type of diabetes which can present as DKA. Since DKA could be a possible trigger of AEN, a rare but potentially lifethreatening condition, that clinicians should be aware of, in patients presenting with upper gastrointestinal bleeding and ketoacidosis. The prompt management and classification of DKA, combined with the EGD execution for early AEN diagnosis and follow-up, is essential., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Acute esophageal necrosis may be a potential complication of Coronavirus Disease 2019 (COVID-19). COVID-19 has been associated with a variety of sequelae, including acute respiratory distress syndrome, myocarditis, and thromboembolic events. Here, we present a case of a 43-year-old male who was admitted for acute necrotizing pancreatitis and found to have COVID-19 pneumonia. He subsequently developed acute esophageal necrosis requiring a total esophagectomy. Currently, there are at least five other reported cases of esophageal necrosis with concomitant COVID-19 infection. This case is the first requiring esophagectomy. Future studies may establish esophageal necrosis as a known complication of COVID-19., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Acute esophageal necrosis (AEN) or black esophagus is a rare cause of mortality in patients with gastrointestinal bleeding. We present a case of a 54-year-old female who presented with diabetic ketoacidosis (DKA) and developed melena eventually attributed to AEN. The esophagogastroduodenoscopy (EGD) identified severe inflammation with black discoloration consistent with acute esophageal necrosis in the middle and lower esophagus. The patient was managed with intravenous pantoprazole and total parenteral nutrition (TPN) until she was able to tolerate an adequate diet. Black esophagus should be added to the differential diagnosis of patients with DKA who develop gastrointestinal bleeding. This need is stressed by the fact that early treatment is essential to reducing complications and mortality associated with the condition., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We report a case of extensive intramural hematoma of the esophagus (IHE) due to esophageal thermal injury, which is a rare disease with multiple causes. However, IHE caused by thermal injury is even rarer . A typical patient may present with a triad of chest pain, dysphagia, and vomiting of blood. The treatment of choice for IHE is conservative therapy.

Introduction: Acute esophageal necrosis (AEN) is a rare medical disorder, which is characterized by a diffuse black esophageal mucosal during upper gastrointestinal endoscopy which is a highly recommended diagnostic tool. Its high mortality rate requires to be quickly evocated and an early management., Case Report: We report a case of a 93-year-old patient with upper gastrointestinal bleeding. The upper endoscopy shows a grade D AEN according to the Los Angeles classification. Treatment consists of a parenteral nutritional support and an intravenous proton pump inhibitors treatment, which increase chances of a favorable outcome on endoscopic controls at 2 and 6 weeks., Conclusion: AEN has to be quickly evocated in a polyvascular and old patient with upper gastrointestinal bleeding. Our experience confirms that optimal and early management allow a esophageal complete healing at 6weeks., (Copyright © 2021 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Background: Cricopharyngeal bars (CPBs) are a unique etiology of oropharyngeal dysphagia. Symptomatic patients are managed with endoscopic dilation or surgical myotomy. Cricopharyngeal peroral endoscopic myotomy (CP-POEM) is an emerging technique for the management of dysphagia due to CPBs. This study evaluated technical success, clinical success, adverse events, and long-term recurrence following CP-POEM., Methods: Consecutive patients who underwent POEM for management of CPBs between May 2015 and December 2020 at four tertiary care centers were included. Primary outcome was clinical success (defined as improvement of dysphagia score to ≤ 1). Secondary outcomes were technical success, rate and severity of adverse events, procedure duration, and symptom recurrence., Results: 27 patients (mean age 69 years; 10 female) underwent CP-POEM during the study period. The most common presenting symptoms at the time of index procedure were dysphagia (26; 96.3 %) and regurgitation (20; 74.1 %). Clinical and technical success were achieved in all patients. Mild/moderate adverse events occurred in two patients (7.4 %). CP-POEM significantly reduced the median dysphagia score., Conclusions: CP-POEM was a safe and effective treatment for symptomatic CPBs. Although symptom recurrence was low, long-term outcome data are needed. CP-POEM should be considered as a management option for symptomatic CPBs at centers with POEM expertise., Competing Interests: Financial Disclosures: Mouen A. Khashab is a consultant for Boston Scientific, Olympus, Medtronic, GI Supply and Triton. Jose M. Nieto is a consultant for Boston Scientific and ERBE Saowanee Ngamruengphong is a consultant for Boston Scientific. All other others have nothing to disclose., (Thieme. All rights reserved.)

A 73-year-old woman was hospitalized with sudden chest pain and hematemesis. Chest computed tomography and upper gastrointestinal endoscopy revealed an idiopathic submucosal hematoma from the cervical esophagus to the esophagogastric mucosal junction. Idiopathic esophageal submucosal hematoma is often prone to a bleeding tendency of an underlying disorder. The patient had a history of essential thrombocythemia (ET) and was taking aspirin. She successfully recovered after aspirin discontinuation and conservative treatment; however, died of cardiopulmonary arrest in the ward on day 9 of hospitalization. The autopsy revealed that the cause of death was pulmonary thromboembolism. This is the first report of ET with submucosal hematoma of the esophagus. The possibility of an esophageal submucosal hematoma should be considered when patients with ET complain of chest pain since ET and treatment with aspirin are considered risk factors for bleeding. Additionally, close attention should be focused on the risk of developing thrombosis if a patient with myeloproliferative neoplasm is required to discontinue antithrombotic therapy due to a bleeding event.

Aim: Black esophagus, or acute esophageal necrosis, is a rare entity with multifactorial aetiology. Modern theories suggest a combination of ischemia, compromised mucosa defences and corrosive agent's injury., Material and Methods: We investigated black esophagus by means of a retrospective review of 26 cases in literature. A Medline overview is performed until May 2021 by considering the Italian results. The search terms were "black esophageal syndrome in Italy", "black esophagus in Italy", "black esophageal necrosis in Italy", and "Gurvits syndrome in Italy". To complete these case reports, we illustrate our first experience of the syndrome successfully treated with esophagectomy, cervical diversion and gastrostomy., Results: Black esophagus is common in adult males (M/F: 21/5) (Range: 47-89 years; Average: 70.6 year-old). The most common symptoms are hematemesis, epigastric pain and dysphagia. Endoscopically, diffuse involvement of acute esophageal necrosis is diagnosed in 42.3% of cases. The treatment consisted on red blood cell transfusions, sucralfate administration, proton pump-inhibition, enteral nutrition and antimicrobial agents. Overall mortality was 38.4% and only one case underwent surgery for acute bleeding., Conclusions: Black esophagus is often reversible both anatomically and functionally. Its treatment is based on supported therapies and hemodynamic resuscitation. This syndrome shows high mortality related to the coexisted medical conditions rather than acute esophageal necrosis. Only in selected cases, surgical treatment is indicated., Key Words: Acute necrotizing esophagitis, Black esophagus, Ischemia.

Objectives: Recently, incidence of Mycobacterium abscessus (Mab) pulmonary disease (Mab-PD) is increasing worldwide. We aimed to identify factors associated with severity of Mycobacterium abscessus (Mab) pulmonary disease (Mab-PD)., Methods: All patients diagnosed as Mab-PD based on the official ATS/IDSA statement between 2017 January 1 and 2021 July 31 were included (n = 13). We reviewed medical records, bacteriological and laboratory data of the patients. Severity of lung lesions and esophageal diameters in chest CT were quantitatively evaluated. Gaffky score in the sputum was used as airway mycobacterial burden. We explored the factors associated with high CT score and high Gaffky score., Results: Maximum diameter of esophagus (MDE) in severe disease (CT score≧10) was greater than that in milder disease (CT score<10) (18.0±7.9mm, 9.3±3.1mm, respectively, p = 0.01), and MDE was well correlated with CT score (R = 0.69, p = 0.007). MDE in high mycobacterial burden group (Gaffky score ≧5) tended to be greater than that in low mycobacterial burden group (Gaffky score <5) (16.1±6.8mm, 10.1±5.5mm, respectively, p = 0.12), and MDE was well correlated with Gaffky score (R = 0.68, p = 0.009). Lung lesions were bilateral and predominant in middle or lower lobes., Conclusions: Esophageal dilatation was correlated with severity of Mab-PD and airway mycobacterial burden. Gastroesophageal reflux might be associated with Mab disease progression., Competing Interests: The authors have declared that no competing interests exist.

The association between eosinophilic esophagitis and celiac disease is still controversial and its prevalence is highly variable. We aimed to investigate the prevalence of esophageal eosinophilia and eosinophilic esophagitis in a large group of children with celiac disease, prospectively followed over 11 years., Methods: Prospective observational study performed between 2008 and 2019. Celiac disease diagnosis was based on ESPGHAN criteria. At least four esophageal biopsies were sampled in patients who underwent endoscopy. The presence of at least 15 eosinophils/HPF on esophageal biopsies was considered suggestive of esophageal eosinophilia; at the same time, eosinophilic esophagitis was diagnosed according to the International Consensus Diagnostic Criteria for Eosinophilic Esophagitis., Results: A total of 465 children (M 42% mean age 7.1 years (range: 1-16)) were diagnosed with celiac disease. Three hundred and seventy patients underwent endoscopy, and esophageal biopsies were available in 313. The prevalence of esophageal eosinophilia in children with celiac disease was 1.6% (95% CI: 0.54-2.9%). Only one child was diagnosed as eosinophilic esophagitis; we calculated a prevalence of 0.3% (95% CI: 0.2-0.5%). The odds ratio for an association between eosinophilic esophagitis and celiac disease was at least 6.5 times higher (95% CI: 0.89-47.7%; p = 0.06) than in the general population., Conclusion: The finding of an increased number of eosinophils (>15/HPF) in celiac patients does not have a clinical implication or warrant intervention, and therefore we do not recommend routine esophageal biopsies unless clinically indicated.

Esophageal injury is a rare but serious complication of atrial fibrillation (AF) ablation. To minimize esophageal injury, our persistent AF (PerAF) protocol involves complete left atrial posterior wall (LAPW) and pulmonary vein (PV) isolation (box isolation), with a centerline away from the esophagus. However, there has been a concern that extensive LA isolation might deteriorate LA function. There has been a paucity of data on LA remodeling after box isolation. Therefore, we compared LA size pre- and post-box isolation with an LAPW centerline in patients with PerAF.Patients who underwent catheter ablation (CA) for PerAF between November 2016 and December 2018 were retrospectively evaluated.The LAPW, including all PVs, was completely isolated in 105 consecutive patients (75 men; mean age: 68 ± 10 years) with PerAF, including 58 patients with long-standing PerAF. During a follow-up of 660 ± 332 days, 76 patients (72%) were arrhythmia-free. The LA dimension (38 ± 6 mm versus 42 ± 7 mm; P < 0.0001) and volume index (38 ± 13 mL/m 2 versus 47 ± 14 mL/m 2 ; P < 0.0001) at 6 months post-ablation were significantly decreased in patients who maintained sinus rhythm compared to pre-ablation. In patients with recurrent AF/atrial tachycardia (AT), these parameters were also significantly decreased (P < 0.001, respectively).Box isolation with a posterior centerline has no esophageal complications and a high clinical success rate in patients with PerAF. Reverse remodeling could be achieved even when using extensive isolation of the PV and LAPW in patients with PerAF.

Background & Aims: Malnutrition is common in patients with esophageal cancer, which affects their prognosis. The global leadership initiative on malnutrition (GLIM) criteria was recently proposed as the world's first diagnostic criteria for malnutrition. However, the association between esophageal cancer patients and the GLIM criteria is unclear. The purpose of this study was to evaluate the percentage of patients diagnosed with malnutrition preoperatively using the GLIM criteria, assess the impact of disease-specific symptoms on the severity of malnutrition, and assess the prognostic relevance of GLIM defined malnutrition in patients with esophageal cancer., Methods: This was a retrospective single-center cohort study. Preoperative nutritional status of patients with esophageal cancer hospitalized between June 2009 and July 2011 was evaluated according to the GLIM criteria. Factors related to severe malnutrition as per the GLIM criteria were analyzed using multivariable logistic regression analysis. The association between the severity of malnutrition based on the GLIM criteria and 5-year survival was assessed using a multivariable Cox proportional hazard model., Results: Overall, 117 esophageal cancer patients were nutritionally assessed. The percentage of moderate malnutrition and severe malnutrition was 21% and 23%, respectively. Subjective dysphagia [odds ratio (OR): 7.39, 95% confidence interval (CI): 1.46-37.52] and subjective esophageal obstruction (OR: 10.49, 95% CI: 3.47-31.70) were independent risk factors for severe malnutrition. The hazard ratio (HR) for 5-year mortality tended to be higher for moderate malnutrition (HR: 2.12, 95% CI: 0.91-4.95); however, it was not significantly associated with either moderate malnutrition or severe malnutrition (HR: 1.30, 95% CI: 0.52-3.27). Cases that were censored during the follow-up period probably affected the survival results., Conclusion: Subjective feelings of dysphagia and esophageal obstruction might be related to malnutrition severity in esophageal cancer patients. Malnutrition assessed by the GLIM criteria was not significantly associated with 5-year survival., Competing Interests: Conflict of interest The authors declare no conflicts of interest associated with this manuscript., (Copyright © 2021 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Background and Study Aims: Esophageal ulcers are a rare cause of upper gastrointestinal morbidity and may be due to different etiologies. We sought to systematically evaluate patients with esophageal ulcers and describe their presentations, endoscopic findings, etiologies, treatments, and outcomes., Patients and Methods: Patients diagnosed with esophageal ulcers over an 11-year period were retrospectively identified from our institution's electronic medical records., Results: We identified 100 patients with esophageal ulcers (0.49% of patients undergoing upper endoscopy). Half of them presented due to gastrointestinal bleeding and three-quarters were admitted to the hospital. The majority were in the lower esophagus. Twenty-two unique etiologies, including multiple iatrogenic causes, were diagnosed in 91 of the cases. The most common etiology was gastroesophageal reflux disease (57%), followed by non-steroidal anti-inflammatory drug use (7%), malignancies (3%), vomiting (3%), caustic ingestion (2%), pill esophagitis (2%) and radiation (2%). Many etiologies showed a predilection for specific segments of the esophagus. Nine ulcers required endoscopic intervention and all were treated successfully. Repeat endoscopies were performed 5 times for diagnostic or "second look" reasons, none of which changed the patients' diagnosis or treatment. No patients required surgery or stricture dilation. One patient's ulcer was complicated by perforation and he subsequently died. Four other patients died from non-ulcer related causes., Conclusions: While the majority of ulcers were due to gastroesophageal reflux disease, 22 different etiologies were identified. Many were due to medication or iatrogenic causes. Repeat endoscopy did not appear to be helpful. While the incidence was low, they were frequently associated with significant morbidity., (© Acta Gastro-Enterologica Belgica.)

Background: The jejunal interposition is our preferred esophageal replacement route when the native esophagus cannot be reconstructed. We report the evolution of our approach and outcomes., Methods: The study was a single-center retrospective review of children undergoing jejunal interposition for esophageal replacement. Outcomes were compared between historical (2010-2015) and contemporary cohorts (2016-2019)., Results: Fifty-five patients, 58% male, median age 4 years (interquartile range 2.4-8.3), with history of esophageal atresia (87%), caustic (9%) or peptic (4%) injury, underwent a jejunal interposition (historical cohort n = 14; contemporary cohort n = 41). Duration of intubation (11 vs 6 days; P = .01), intensive care unit (22 vs 13 days; P = .03), and hospital stay (50 vs 27 days; P = .004) were shorter in the contemporary cohort. Anastomotic leaks (7% vs 5%; P = .78), anastomotic stricture resection (7% vs 10%; P = .74), and need for reoperation (57% vs 46%; P = .48) were similar between cohorts. Most reoperations were elective conduit revisions. Microvascular augmentation, used in 70% of cases, was associated with 0% anastomotic leaks vs 18% without augmentation; P = .007. With median follow-up of 1.9 years (interquartile range 1.1, 3.8), 78% of patients are predominantly orally fed. Those with preoperative oral intake were more likely to achieve consistent postoperative oral intake (87.5% vs 64%; P = .04)., Conclusion: We have made continuous improvements in our management of patients undergoing a jejunal interposition. Of these, microvascular augmentation was associated with no anastomotic leaks. Despite its complexity and potential need for conduit revision, the jejunal interposition remains our preferred esophageal replacement, given its excellent long-term functional outcomes in these complex children who have often undergone multiple procedures before the jejunal interposition., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Approximately 10% of patients receiving anti-reflux procedures present with shortened esophagus. Collis gastroplasty (CG) is the current gold standard for esophageal lengthening, but mediastinal esophageal mobilization without gastroplasty may be an alternative approach. This study assesses preoperative and intraoperative hernia characteristics and mediastinal dissection impact in patients with large hiatal hernia repair (HHR)., Methods: A single-institution, prospectively collected database was reviewed for adults who underwent laparoscopic HHR with mesh and anti-reflux surgery between 2005 and 2016, hernia ≥ 5 cm. Preoperative hernia and follow-up were assessed using upper endoscopy and barium swallow. Intraoperative hernia characteristics were collected from the operative note. Esophageal symptom scores were collected pre- and postoperatively. Analyses were conducted using SPSS v26.0., Results: Among 662 patients who had anti-reflux surgery in this period, a total of 205 patients who underwent HHR with mesh met the inclusion criteria and were included in study. Mean age was 61.7 ± 13.6 years, and majority of patients were female and Caucasian. Mean BMI was 29.9 ± 6.0 kg/m 2 . Median hernia size was 6.5 cm [5.0-12.0 cm], and intra-thoracic stomach had a prevalence of 21.9%. Analysis of preoperative barium swallow revealed an average of elevated gastroesophageal junction above the diaphragm of 4.10 ± 1.67 cm. Radiographically, average hernia size was 6.34 ± 1.93 cm and 6.38 ± 1.92 cm in the anterior-posterior and obliquus view, respectively. Median follow-up time was 2.7 years [1-9 years]. Esophageal symptoms improved in all patients (p < 0.05). 45% of patients had radiographic recurrence, but only four presented symptomatic or were on PPI., Conclusions: CG has been the standard for ensuring adequate esophageal length prior to anti-reflux surgery. Our results support that CG is unnecessary in the majority of cases, and extensive mediastinal dissection was successfully used instead of CG with durable, long-term outcomes. Extended mediastinal dissection may mitigate CG risks in patients requiring additional intra-abdominal esophagus.

Introduction: We aim to discuss the utility of self-expandable esophageal metal stent (SEMS) in variceal bleeding in challenging cases., Methods: Case description, discussion on patient management and decision-making process in an uncommon situation, from a multidisciplinary point of view., Results: We report a case of a cirrhotic patient with refractory variceal bleeding who underwent a SEMS placement, which remained in situ for 9 months. This decision was based on the initial poor status and short life expectancy, limiting the consideration of other options for lowering portal hypertension, along with an underlying prothrombotic predisposition. However, the patient's general and hepatic improvement and the development of dysphagia led to the SEMS removal, exposing a large esophageal-tracheal fistula., Discussion: Early patient evaluation, risks of long-term SEMS, and life expectancy should be taken in consideration before SEMS placement.

Neonatal autoimmune subepidermal blistering disease is rare. Mucosal involvement is more common in neonatal linear immunoglobulin A (IgA) bullous dermatosis. We describe a neonate with subepidermal cutaneous blistering disease with severe laryngeal and esophageal involvement leading to acute respiratory distress. Histopathology demonstrated a subepidermal blister with neutrophils and eosinophils at the dermal base. Collagen IV was detected at the dermal floor, and direct immunofluorescence showed linear IgG, IgA, and C3 deposits at the basement membrane zone. The patient demonstrated markedly increased serum levels of anti-BP180 NC16A and anti-BP230 IgG antibodies (Abs) but failed to show anti-LAD-1 IgA Abs. His healthy mother showed serum anti-LAD-1 IgA Abs but did not show anti-BP180 and anti-BP230 Abs. The neonate responded promptly to systemic corticosteroid therapy. A review of the literature detected 11 cases of neonatal subepidermal blistering disease with linear IgA deposits. Nine of these cases demonstrated coexisting linear IgG deposits, often with C3. Respiratory compromise was present in most of the cases. Neutrophils and eosinophils were commonly present in the inflammatory cell infiltrates. Besides our case, 2 cases of neonatal IgG/IgA subepidermal blistering disease with esophageal involvement were previously described. IgA Abs were present in the sera of both cases. Anti-LAD-1 IgA Abs were detected in the mother's serum of our case alone, but IgA Abs do not cross the placenta. Our case was consistent with neonatal IgG/IgA pemphigoid. Neonatal IgG/IgA subepidermal blistering disease may be associated with severe laryngeal and esophageal involvement leading to respiratory compromise. Expedited diagnosis and prompt treatment are warranted.

IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing disease which causes tumor-like swelling of organs and commonly mimics symptoms of malignancy. It has been increasing in prevalence in the last decade, but esophageal involvement remains rare. IgG4-RD was first known to involve certain organs, such as the pancreas. It has, since, been described as a systemic disease process. IgG4-RD should be considered in patients presenting with dysphagia. Initiation of appropriate treatment with corticosteroids can avoid unnecessary procedures and improve outcomes. The aim of this review is to discuss 17 cases of IgG4-RD of the esophagus. Literature review was conducted using NCBI database (PMC and PubMed filters) using the keywords "IgG4 disease," "sclerosing," "esophagus" and "gastrointestinal." The search was narrowed to include cases describing IgG4 disease of the esophagus using the same filters. Literature review identified 16 documented cases of IgG4-RD involving the esophagus. Upon literature review, it remains clear that it is extremely rare for IgG4-RD to affect the esophagus. Sixteen cases have been reported. We present a 17th case and discuss the implications of IgG4-RD. It is important to keep a broad differential diagnosis that includes IgG4-RD for patients presenting with dysphagia, especially when symptoms are refractory.

We present a case of a patient with known papillary thyroid carcinoma presenting with overt upper gastrointestinal bleeding from a metastatic gastric mass. The gastric mass was diagnosed through transnasal endoscopy with tissue biopsies, revealing papillary thyroid carcinoma cells on histopathological studies. Bleeding was persistent despite standard medical therapy. Chemoembolisation of the major feeding vessels of such tumour provided resolution of bleeding., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Black esophagus, also known as acute esophageal necrosis (AEN) syndrome, is a rare entity characterized by patchy or diffuse circumferential black pigmentation of the esophageal mucosa from ischemic necrosis. It may present with life-threatening upper gastrointestinal hemorrhage resulting in high mortality in immunocompromised patients. Advanced age with multiple comorbidities compounded with compromised hemodynamic states are poor prognostic factors. Findings on laboratory work-up and radiological imaging are non-specific. After initial resuscitation, endoscopic evaluation and histological examination of esophageal biopsy are diagnostic. Early recognition and aggressive resuscitation are the fundamental principles for the management of AEN and better outcome of the disease. We report a case of a 56-year-old woman with diabetes mellitus, gastro-esophageal reflux disease, and active alcohol binging who presented with hematemesis and acute epigastric pain due to AEN. This case illustrates a rare etiology of AEN due to active alcohol drinking, which may be overlooked. Physician awareness about this etiology is important as early recognition and timely management may improve survival., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Aims: Crohn's disease [CD] can involve any part of the gastrointestinal tract. We aimed to characterize the clinical, endoscopic and histological features and treatment outcomes of CD patients with oesophageal involvement., Methods: We collected cases through a retrospective multicentre European Crohn's and Colitis Organisation CONFER [COllaborative Network For Exceptionally Rare case reports] project. Clinical data were recorded in a standardized case report form., Results: A total of 40 patients were reported (22 males, mean [±SD, range] age at oesophageal CD diagnosis: 25 [±13.3, 10-71] years and mean time of follow-up: 67 [±68.1, 3-240] months). Oesophageal involvement was established at CD diagnosis in 26 patients [65%] and during follow-up in 14. CD was exclusively located in the oesophagus in two patients. Thirteen patients [32.2%] were asymptomatic at oesophageal disease diagnosis. Oesophageal strictures were present in five patients and fistulizing oesophageal disease in one. Eight patients exhibited granulomas on biopsies. Proton-pump inhibitors [PPIs] were administered in 37 patients [92.5%]. Three patients underwent endoscopic dilatation for symptomatic strictures but none underwent oesophageal-related surgery. Diagnosis in pre-established CD resulted in treatment modifications in 9/14 patients. Clinical remission of oesophageal disease was seen in 33/40 patients [82.5%] after a mean time of 7 [±5.6, 1-18] months. Follow-up endoscopy was performed in 29/40 patients and 26/29 [89.7%] achieved mucosal healing., Conclusion: In this case series the endoscopic and histological characteristics of isolated oesophageal CD were similar to those reported in other sites of involvement. Treatment was primarily conservative, with PPIs administered in the majority of patients and modifications in pre-existing inflammatory bowel disease-related therapy occurring in two-thirds of them. Clinical and endoscopic remission was achieved in more than 80% of the patients., (Copyright © 2019 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Pemphigus vulgaris (PV) is a rare autoimmune blistering disease involving the skin and mucous membranes. The prevalence of esophageal involvement remains uncertain. The aim of our study was to determine the frequency of esophageal involvement in patients with PV. This is a single-center electronic database retrospective review of patients with a diagnosis of PV. Data abstracted included demographics, disease characteristics (biopsy results, symptoms, areas affected, treatments), and esophagogastroduodenoscopy (EGD) reports. Of the 111 patients that met eligibility criteria, only 22 (19.8%) underwent EGD. Demographic data were similar except those who underwent EGD were more likely to be female (77.3% vs. 51.7%, p = 0.05) and have hypertension (50.0% vs. 24.7%, p = 0.04). Esophageal symptoms were common in both groups; however, those experiencing dysphagia were more likely to undergo EGD (50.0% vs. 20.2%, p = 0.007). Those who underwent EGD had more refractory disease (≥ 3 treatment modalities: 100% vs. 58.4%, p < 0.001), but did not differ in areas affected. Of those who underwent EGD, only 4 (18.2%) had esophageal abnormalities either prior to PV diagnosis (1) or during a disease flare (3). Those having a flare were more likely to experience odynophagia (69.2%) or weight loss (61.5%), p = 0.02 and p = 0.05, respectively. While esophageal symptoms were common in our cohort of PV patients, a minority of patients underwent EGD, and the vast majority of those were unremarkable. This suggests that while esophageal symptoms are common in PV, permanent esophageal injury is more rare.

Objective: This study investigated the effects of transcranial direct current stimulation (tDCS) combined with conventional swallowing training on the swallowing function in brainstem stroke patients with cricopharyngeal muscle dysfunction (CPD)., Methods: Twenty-eight brainstem stroke patients with CPD were assigned randomly to an anodal tDCS group or a sham tDCS group. The patients received anodal tDCS or sham tDCS over the bilateral oesophageal cortical area combined with simultaneous catheter balloon dilatation and conventional swallowing therapy for 20 days. Swallowing function was assessed using the functional oral intake scale (FOIS) and the functional dysphagia scale (FDS) and by measuring the pharyngoesophageal Segment Opening (PESO) before and immediately after the intervention., Results: Both groups showed a significant improvement in the FDS, FOIS and PESO scores immediately after the intervention (all p < .005). However, compared with the sham stimulation group, the anodal tDCS group showed greater improvements in the FDS, FOIS and PESO scores immediately after the intervention (all p < .005)., Conclusion: The bihemispheric anodal tDCS combined with simultaneous catheter balloon dilatation and conventional swallowing therapy effectively improves the swallowing function in patients with CPD caused by a brainstem stroke. tDCS may be an effective adjuvant therapy in CPD rehabilitation.

Purpose of Review: The functional luminal imaging probe (FLIP) uses high-resolution planimetry to provide a three-dimensional image of the esophageal lumen by measuring diameter, volume, and pressure changes. The goal of this review is to summarize the most recent advances in applying the (FLIP) to esophageal disorders., Recent Findings: The FLIP has been studied in esophageal disease states including gastroesophageal reflux disease (GERD), achalasia, and eosinophilic esophagitis. It has also been used in the investigation of dysphagia. The FLIP is a valuable tool for the diagnosis of esophageal diseases as well as guiding treatments and predicting treatment response. As further research is done, the FLIP may become the initial test for the patient with undifferentiated dysphagia at the time of their index endoscopy.