A rare cause of acute esophageal necrosis: A case report.

Acute esophageal necrosis (AEN) or black esophagus is a rare entity characterized by diffuse circumferential black pigmentation of the esophageal mucosa due to ischemic necrosis. It may be lethal, especially among elderly patients with multiple comorbidities and hemodynamic instability. Diagnosis is based on gastroscopy. Treatment consists of intravenous fluids, proton pump inhibitors, and additional therapies to treat the underlying illness. We report a rare case of a woman in her 50s with cervical cancer who presented with hematemesis and sepsis. Upper gastrointestinal endoscopy showed a black esophagus and an ulcerobudding duodenal process. Few days later, she developed abdominal distension with diffuse pain. Abdominal CT scan demonstrated perforation of gastroduodenal tumor. The treatment was based on resuscitation, proton pump inhibitors, antibiotics, and surgery of the perforated tumor. Unfortunately, the patient died 2 days later because of septic shock. The black esophagus is a fatal complication, thus diagnosis at an early stage and timely management may improve survival. This is the first case reported of AEN due to perforated duodenal tumor explained by septic shock leading to an ischemic esophageal injury.

Black Esophagus: A Rare Case of Acute Esophageal Necrosis.

Acute esophageal necrosis (AEN) also known as necrotizing esophagitis or black esophagus is an extremely rare cause of upper gastrointestinal (GI) bleeding. This condition is considerably rare, and the exact pathophysiology of the development of AEN is still unclear. There is consensus that it is caused by a combination of esophageal mucosal injury due to gastric acid and ischemic injury due to vascular compromise. The management of AEN includes correcting the multitude of underlying predisposing conditions as well as agile symptomatic management and close monitoring for signs of hemodynamic compromise. We here present an interesting case of a middle-aged male patient who presented with hematemesis and underwent emergent esophagogastroduodenoscopy (EGD), which revealed severe necrotic esophagus. We also discussed the risk factors, pathophysiology, and management of AEN.

Ketosis-prone Diabetes presenting with Acute Esophageal Necrosis or "Black Esophagus": An Intriguing New Clinical Association.

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.

Acute Esophageal Necrosis and Duodenal Disease in the Setting of Recently Initiated Chemotherapy.

Introduction: Acute esophageal necrosis (AEN), commonly referred to as "black esophagus" or Gurvits syndrome, is a rare condition characterized by diffuse black mucosa in the distal esophagus. Most often, the patient is an older male with multiple comorbidities, presenting with upper gastrointestinal bleeding. The exact pathogenesis is unclear, but it is often thought to be secondary to acute vascular hypo-perfusion or ischemia of the esophageal mucosa in critically ill patients with certain secondary comorbid conditions such as renal insufficiency, diabetes mellitus, dyslipidemia, coronary artery disease, malnourishment, alcohol abuse, or association with an underlying malignancy. Case Presentation: We present a case of AEN in a 78-year-old female following the recent start of a chemotherapy regimen with carboplatin and paclitaxel two weeks prior. The patient underwent EGD and was found to have AEN throughout the entirety of her esophagus with necrosis and eschars seen up to the second part of the duodenum. The patient initially improved after receiving blood transfusions, being made nil-per-os, and starting proton pump inhibitor (PPI) therapy, but she ultimately died given the severity of her clear cell uterine cancer and other comorbidities. Conclusion: Although it is rare that initiation of chemotherapy leads to AEN, it should be considered as a potential etiology.

"Black Esophagus" or Gurvits Syndrome: A Rare Cause of Upper Gastrointestinal Bleeding in Diabetic Ketoacidosis.

Black esophagus, also called Gurvits syndrome or acute esophageal necrosis (AEN), is a rare, life-threatening condition characterized by necrosis of the esophageal mucosa. We present a 36-year-old man who presented with hematemesis and was admitted for diabetic ketoacidosis (DKA) management. He then had a further episode of hematemesis with hemodynamic instability. The esophagogastroduodenoscopy (EGD) revealed ulcerative, necrotizing, circumferential esophagitis in the middle and distal third of the esophagus. The patient was treated with intravenous fluid resuscitation, proton pump inhibitors, empiric antibiotics, and antifungals. Hematemesis in DKA should raise suspicion for black esophagus. Prompt detection of AEN allows for early management and thus reduces mortality and associated complications such as perforations and strictures.

Comprehensive Review: Acute Esophageal Necrosis in the Setting of Gastric Volvulus.

BACKGROUND AND AIMS: Acute esophageal necrosis (AEN) in the setting of gastric volvulus is a rare condition with only a handful of cases reported. Volvulus may contribute to AEN by limiting tissue perfusion and promoting massive reflux of gastric contents on compromised esophageal mucosa. METHODS: We reviewed 225 original articles, literature reviews, case series, brief reports, case reports, and discuss six total cases of co-occurring esophageal necrosis and gastric volvulus. RESULTS AND CONCLUSIONS: We present the first comprehensive analysis of all reported cases in the literature to date and formulate management strategies for the co-occurrence of AEN and volvulus. Management of AEN should be directed at correcting underlying medical conditions, providing hemodynamic support, initiating nil-per-os restriction, and administering high-dose proton pump inhibitor therapy. Surgical intervention is typically reserved for cases of esophageal perforation with mediastinitis and abscess formation.

Acute Esophageal Necrosis in Acute Pancreatitis-Report of a Case and Endoscopic and Clinical Perspective.

Esophageal stroke, also known as acute esophageal necrosis or Gurvits syndrome, is an entity that has gained more and more recognition in the last two decades. It is also named "black esophagus" because of striking black discoloration of the esophageal mucosa, with an abrupt transition to normal mucosa at the gastroesophageal junction. Its most common clinical presentation is represented by upper gastrointestinal bleeding and esophagogastroduodenoscopy is the main diagnostic tool. Among the etiopathogenetic and multiple predisposing factors described are hypovolemia, shock state, ischemia, congestive heart failure, acute renal failure, infections, trauma, and diabetes mellitus. Current management of this condition consists of treating the underlying pathology, nil per os, and antacid administration in uncomplicated cases. Although most of the cases have favorable prognosis, complications such as pneumomediastinum or esophageal stricture may occur and fatal cases are a consequence of underlying comorbidities.

Thoracoscopic esophagectomy for stenosis of thoracic esophagus due to acute esophageal necrosis associated with alcoholic ketoacidosis.

Acute esophageal necrosis (AEN) is a rare disease characterized by the appearance of diffuse black mucosa on upper gastrointestinal endoscopy; the condition often progresses to esophageal stenosis in the chronic phase. A 70-year-old man was admitted to a neighborhood hospital with the diagnosis of alcoholic ketoacidosis and an upper gastrointestinal endoscopy performed to investigate the symptom of esophageal tightness revealed AEN. The patient developed esophageal stenosis with scarring in the chronic phase and was referred to our hospital for surgery 6 months after the diagnosis of AEN. We performed thoracoscopic esophagectomy with the patient in the prone position. Although the esophagus was thickened and strong adhesions were present around the esophagus due to inflammation, we were able to complete the surgical procedure thoracoscopically. In patients presenting with benign esophageal stenosis developing after AEN, thoracoscopic esophagectomy may be a useful treatment option, even in the presence of severe fibrosis.

Esófago negro asociado a estado de choque. A propósito de un caso / Black esophagus associated with shock. About a case

Resumen La necrosis esofágica aguda, también conocida como esófago negro, es una patología poco común que se diagnostica mediante endoscopia en la que se muestra una mucosa esofágica de aspecto negro. Su causa es desconocida, pero se le atribuye un origen multifactorial. Debe considerarse una lesión esofágica de origen isquémico. Se presenta el caso de un paciente de 56 años con síntomas respiratorios sugestivos de proceso infeccioso grave, con un cuadro clínico rápidamente progresivo y requerimiento de aseguramiento de la vía aérea, soporte vasopresor y orgánico multimodal, estudio endoscópico por clínica de hemorragias de vías digestivas altas con evidencia de lesiones compatibles con necrosis esofágica aguda. A pesar de manejo multimodal en unidad de cuidados intensivos el paciente fallece al día 34 desde su ingreso hospitalario.

Abstract Acute esophageal necrosis, also known as the black esophagus, is a rare pathology diagnosed by endoscopy that shows a black-looking esophageal mucosa. Its cause is unknown, but a multifactorial origin is attributed to it. An esophageal lesion of ischemic origin should be considered. We present the case of a 56-year-old patient with respiratory symptoms suggestive of a severe infectious process, with a rapidly progressive clinical picture and requirement of airway security, multimodal vasopressor, and organic support, and an endoscopic study due to clinical symptoms of upper digestive tract bleeding with evidence of lesions compatible with acute esophageal necrosis. Despite multimodal management in the intensive care unit, the patient died 34 days after hospital admission.

Association of Candida esophagitis with acute esophageal necrosis.

Acute esophageal necrosis (AEN) is a clinical rarity and its pathogenesis is poorly understood. It usually presents as upper gastrointestinal bleeding and is associated with poor outcomes. We report a case of a 53-year-old man with diabetes and cirrhosis who presented with diabetic ketoacidosis and had an upper gastrointestinal bleed during the hospital stay from AEN associated with Candida esophagitis. In addition to the standard supportive therapy, such patients require antifungal therapy. This case underscores how a rare disease can present like a common disease and prove to be a management challenge if the differential is not kept broad.

Gurvits syndrome: a case of acute esophageal necrosis associated with diabetic ketoacidosis.

BACKGROUND: Acute esophageal necrosis (AEN), commonly referred to as Gurvits syndrome or "black esophagus", is a rare clinical disease. We present a case of AEN associated with diabetic ketoacidosis (DKA). CASE PRESENTATION: A 66-year-old male came to our hospital with coffee-ground emesis, dyspnea, and general malaise. He was treated for type 2 diabetes mellitus using insulin and had not been taking his medication, including insulin, for several days. Laboratory analysis revealed severe hyperglycemia (730 mg/dL), normocytic anemia (hemoglobin level, 7.7 g/dL; mean corpuscular volume, 100.4 fL), high serum potassium (7.6 mEq/L), and a high level of blood urea (98.7 mg/dL). Ketones and glucose were detected in the urine, and serum ß-hydroxybutyrate was elevated (2132 µmol/L). Arterial blood gas analysis confirmed metabolic acidosis (pH, 7.29; HCO3, 10.5 mmol/L). Collectively, the patient was diagnosed with DKA and upper gastrointestinal bleeding. The patient's condition improved with intravenous fluids, and he received intravenous insulin to treat DKA. According to these findings, the patient was diagnosed with DKA and upper gastrointestinal bleeding. The patient underwent esophagogastroduodenoscopy (EGD) which revealed a circumferential necrosis of the middle and distal esophagus, immediately proximal to the gastroesophageal junction. The patient was then treated with an intravenous proton pump inhibitor. The patient continued to improve with conservative treatment and was subsequently discharged in a stable condition. An EGD repeated 14 days after discharge showed complete healing of the necrotic-like mucosal change without stricture formation of the esophagus. CONCLUSIONS: AEN is rare but potentially life-threatening case of upper gastrointestinal bleeding. Therefore, a clinician should be aware of AEN as a potential cause of upper gastrointestinal bleeding in elderly patients with poorly controlled diabetes and significant comorbidities.

Acute Esophageal Necrosis: An In-depth Review of Pathogenesis, Diagnosis and Management.

Introduction: Acute esophageal necrosis (AEN) is an uncommon but fatal cause of upper gastrointestinal bleeding. The complex pathophysiology of the disorder provides multiple points for intervention. Therefore, it is important to discuss the many multifaceted aspects of the disease. Methods: A scoping review was performed using PubMed, Google Scholar, and ClinicalTrials.gov. We reviewed literature from 1990 to 2021. The keywords used were 'acute esophageal necrosis', 'upper GI bleed', 'pathogenesis', 'EGD', 'prognosis'. Results and conclusions: The review summarized findings of 46 studies. AEN usually targets older males who have underlying cardiovascular disease. The middle part of the esophagus is commonly involved. The pathogenesis of AEN depends on conditions that increase risk of mucosal damage such as ischemia, lack of mucosal protection and excessive gastric reflux. Some medications are also responsible for the disease. Esophagogastroduodenoscopy is usually the gold standard for diagnosis. Findings suggestive of AEN include darkened, sharply demarcated circumferential areas. Supportive measures, including bowel rest, fluid supplementation and proton pump inhibitors are the cornerstone of therapy. A high index of suspicion should be maintained in patients with chronic health problems presenting with signs and symptoms of upper gastrointestinal bleeding because AEN can carry an unfavorable prognosis in these patients.

Acute Esophageal Necrosis Syndrome (Black Esophagus): A Case Report of Rare Presentation.

Acute esophageal necrosis (AEN) is a rare clinical finding due to multifocal factors consisting of an ischemic insult to the esophagus, corrosive injury from gastric content, and diminished mucosal defense. It is also referred to as "black esophagus" or acute necrotizing esophagitis. The clinical presentation mainly consists of upper gastrointestinal bleed and abdominal pain. Associated symptoms include nausea, vomiting, and dysphagia. AEN can be diagnosed by esophagogastroduodenoscopy (EGD) with findings of diffuse circumferential black pigmentation in the distal esophagus that classically extends to the gastroesophageal junction. A diagnostic biopsy is not required but recommended. Treatment of AEN is conservative management to maintain hemodynamic stability and treat coexisting medical conditions. Herein, we present the case of a 78-year-old male who initially presented with hematemesis and abdominal discomfort of five-day duration and was subsequently found to have AEN.

Case Report of Acute Esophageal Necrosis (Gurvits Syndrome) in Vaccinated, COVID-19-Infected Patient.

Acute esophageal necrosis (AEN) is a rare endoscopic finding associated with ischemic compromise of the distal esophagus. This finding can be seen in critically ill patients with COVID-19 infection. We present a case of a COVID-19-vaccinated elderly male with multiple comorbidities and active COVID-19 pneumonia admitted to the intensive care unit with septic shock and acute hypoxemic respiratory failure. The patient developed melena, and esophagogastroduodenoscopy (EGD) was performed, which showed necrosis of the lower esophagus suggestive of AEN. AEN has been associated with high mortality and should be considered when evaluating upper gastrointestinal bleed in a critically ill patient. This case describes the first report of isolated AEN in a patient fully vaccinated against COVID-19 presenting with a severe complicated COVID-19 infection.

Risk factors and clinical outcomes of acute esophageal necrosis: retrospective case series of a rare disease with "black" prognosis.

Acute esophageal necrosis is a rare syndrome classically characterized by black distal esophagus with a complex pathophysiology that usually involves a combination of esophageal ischemia, gastroesophageal reflux and impaired mucosal reparative mechanisms. We retrospectively analyzed the main risk factors, clinical characteristics and outcome in all patients diagnosed with acute esophageal necrosis between January 2015 and December 2020 at our center. Ten patients were identified in a total of 26854 upper digestive endoscopies (0.04%). Most patients were male (8/10) and the mean age of presentation was 71.1 years. The most common presenting symptoms were melena and hematemesis and half the patients required red blood cell transfusion. The most common risk factors were hypertension, diabetes mellitus, dyslipidemia, chronic kidney disease, peripheral artery disease, coronary artery disease, cerebrovascular disease, heart failure and malignancy. Compromised hemodynamic state was the most common precipitating event in four patients. Other recognized precipitating events included surgical interventions, decompensated heart failure, gastrointestinal bleeding from gastric malignancy and methotrexate. Endoscopic findings revealed diffuse and circumferential black distal esophagus with abrupt transition at gastroesophageal junction and variable proximal extension at presentation. The 1-month mortality rate was 30%, mostly from severe underlying illness. In conclusion, acute esophageal necrosis is a rare cause of upper gastrointestinal bleeding that should be suspected in older patients with multiple comorbidities. Although associated with a high mortality rate, appropriate treatment may result in favorable outcome in most patients.

Endoscopic Treatment of Black Esophagus With Fully Covered Metallic Stent.

Acute esophageal necrosis (AEN) is a condition characterized by black appearance and inflammation of the esophagus. Our patient developed AEN presenting in the setting of alcohol intoxication and extensive inferior vena cava thrombosis. Esophagogastroduodenoscopy showed blood and black discoloration of the middle and lower esophagus with oozing. To control bleeding, a self-expandable fully covered metallic esophageal stent was placed. Multiple studies and reviews agree that the initial management for AEN is supportive care, resuscitation, and proton pump inhibitors to protect the esophageal mucosa from injury from acid reflux. Our case highlights the benefits of esophageal stent placement to manage AEN with active bleeding.

Endoscopic balloon dilation for the prevention of severe strictures caused by acute esophageal necrosis.

A 58-year-old man who had the history of alcohol dependence was referred to our emergency center due to severe nausea, vomiting, and subsequent onset of chest and back pain. Esophagogastroduodenoscopy (EGD) showed black-appearing esophagus mucosa extending from the cervical esophagus to the esophagogastric junction with clear margins, a condition typically referred to as a black esophagus. Alcohol abuse was considered an important factor associated with acute esophageal necrosis in this patient. After admission, he received fluid resuscitation and proton-pump inhibitors, with restriction of oral intake and treatment of alcohol dependence. Follow-up EGDs and endoscopic balloon dilation were performed for the management of esophageal narrowing before the development of severe strictures. Strictures were successfully treated endoscopically without complications such as perforation.

Acute esophageal necrosis complicating diabetic ketoacidosis in a patient with type II diabetes mellitus and excessive cola consumption: a case report.

Acute esophageal necrosis (AEN) implicates poor tissue perfusion, functionally defective mucosal barrier, and corrosive injury of the esophageal mucosa, typically characterized by diffuse, circumferential greyish or black discoloration of the esophagus in esophagogastroduodenoscopy. Low-volume states, as diabetic ketoacidosis (DKA), predispose to AEN. Cola drinks diminish the esophageal pH by decreasing the lower esophageal sphincter pressure. We report a 47-year-old male shepherd with chest pain, nausea, odynophagia and gradual decline in level of consciousness, who reported consumption of 6-7 L of cola beverages per day, and was diagnosed with DKA. The patient had a record of type 2 diabetes mellitus and coronary heart disease, and he was administered empagliflozin 25 mg q24 hours, vildagliptin 50 mg bid, metformin 1000 mg bid, and insulin glargine 24 IU q 24. Esophagogastroduodenoscopy was indicative of a diffuse, edematous, and eroded mucosa of grey colour from the upper to the lower esophageal sphincter. CT scan supported the diagnosis, revealing diffuse thickening and edematous imaging of the esophageal wall with an abnormal edge of the mucosa in the lower half of the esophagus. Seven days after rigorous treatment with fluid resuscitation, insulin restoration, esomeprazole, fluconazole, cefoxitine, and metronidazole, the patient was ameliorated. A second endoscopy revealed obvious improvement. Pathophysiology, diagnosis, and treatment of DKA/AEN intertwining are thoroughly discussed. In conclusion, clinicians should not disregard AEN in the differential diagnosis of patients with DKA and clinical symptoms of esophagitis. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13340-021-00537-y.