Acute Esophageal Necrosis as a Rare Complication of Metabolic Acidosis in a Diabetic Patient: A Case Report.

BACKGROUND Acute esophageal necrosis, or Gurvits syndrome, is a rare clinical process often secondary to a systemic low-flow state. It can be caused by several medical conditions, and it is thought to arise from a combination of impaired mucosal barrier and chemical and ischemic insults to the esophagus. Acute esophageal necrosis usually presents with severe complications due to delayed diagnosis and only rarely has surgical indications. We present a case of Gurvits syndrome, presumably triggered by metabolic acidosis in a diabetic patient. CASE REPORT A 61-year-old man with history of hypertension and type 2 diabetes mellitus treated with metformin, canagliflozin, glimepiride, and pioglitazone came to our attention with persistent vomiting, odynophagia, chest pain after each meal, and progressive weight loss. Arterial blood analysis showed mild metabolic acidosis, while the first esophagogastroduodenoscopy performed revealed a circumferential black appearance of the esophageal mucosa, as in concentric necrosis of the distal esophagus with possible fungal superinfection. Brushing cytology confirmed the infection by Candida spp. and the patient was treated with intravenous fluconazole. The second esophagogastroduodenoscopy, performed after 2 weeks, showed almost complete healing of the esophageal mucosa; in this case, biopsy confirmed mucosal ischemia and necrosis, without showing deep impairment of the mucosa by fungal agents. CONCLUSIONS Due to its high lethality, often caused by the underlying medical diseases, acute esophageal disease should be considered in the differential diagnosis of digestive symptoms, even without upper gastrointestinal bleeding. Prompt diagnosis and treatment of contextual collateral conditions can help clinicians to avoid the worst outcomes of the disease. Among the causative factors of metabolic acidosis leading to esophageal necrosis we recognized metformin and dapagliflozin.

COVID-19-induced Esophageal Necrosis Requiring Emergent Total Esophagectomy in a Vaccinated Patient.

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.

[Idiopathic esophageal submucosal hematoma during antithrombotic therapy for essential thrombocythemia].

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.

Esophageal stricture as an initial manifestation of Crohn's disease.

Crohn's disease located in the esophagus is rare, being exceptional as the initial manifestation of the disease. Erosive ulcerative esophagitis, stricture and fistula are forms of presentation, as in other esophageal pathologies, so the differential diagnosis is broad. The histologic features of esophageal Crohn's disease can be nonspecific and increase the diagnostic challenge. Esophageal Crohn's disease should be included in the differential diagnosis of esophageal strictures and may require esophagectomy if medical-endoscopic treatment is not effective.

Peroral endoscopic myotomy for management of cricopharyngeal bars (CP-POEM): a retrospective evaluation.

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.

Acute esophageal necrosis syndrome. The 2021 update from an Italian survey and personal experience.

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.

Esophageal Eosinophilia and Eosinophilic Esophagitis in Celiac Children: A Ten Year Prospective Observational Study.

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.

Real world management of esophageal ulcers: analysis of their presentation, etiology, and outcomes.

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.

Relationship between GLIM criteria and disease-specific symptoms and its impact on 5-year survival of esophageal cancer patients.

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.

Evolution, lessons learned, and contemporary outcomes of esophageal replacement with jejunum for children.

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.

Benign Tracheoenteric Fistula to a Zenker's Diverticulum With Complete Esophageal Obstruction.

A fistula between a Zenker's diverticulum and the trachea has only been reported once, in 1983. Here, we report a case of a fistula between a large Zenker's diverticulum and the trachea with complete occlusion of the esophagus. The fistula was repaired, first by an esophageal myotomy, followed by proximal resection of the diverticulum, completion of the esophageal myotomy, transection of the fistula, and repair of the trachea. The surgical repair provided complete resolution of symptoms without complications.

How high is too high? Extensive mediastinal dissection in patients with hiatal hernia repair.

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/m2. 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.

Long-Term Self-Expandable Metal Stent (SEMS) for Esophageal Variceal Bleeding: A Picture of the Natural History.

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.

Gastric metastasis presenting as an overt upper gastrointestinal bleeding treated with chemoembolisation in a patient diagnosed with papillary thyroid carcinoma.

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.

Black esophagus: a syndrome of acute esophageal necrosis associated with active alcohol drinking.

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.

IgG4 sclerosing disease of the esophagus: a case-based review.

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.

Applying the Functional Luminal Imaging Probe to Esophageal Disorders.

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.

Atypical dysphagia with end-stage oesophageal disease 30 years post Angelchik device placement in a 72-year-old man.

Here we describe an atypical presentation of progressive dysphagia in a 72-year-old man leading to frequent regurgitations over the course of 30 years. Investigations revealed a foreign body ring surrounding the proximal stomach and dilation of the oesophagus proximal to the gastro-oesophageal junction. An Angelchik device was extracted; however, the patient's rapid deterioration prior to surgery, in addition to his severely dysfunctional oesophagus, required placement of a jejunostomy feeding tube. Device removal was complicated by prior abdominal surgery, necessitating a thoracic approach. This case offers guidance on the management of patients with Angelchik prostheses who develop similar complications, while drawing attention to the importance and difficulties of early, definitive diagnosis in oesophageal pathology such as achalasia and gastro-oesophageal reflux disease.