Prominent black esophagus, Wischnewsky spots, and black duodenum in a fatal hypothermia case with an underlying diabetic ketoacidosis.

Hypothermia can occur in patients with diabetic ketoacidosis (DKA), and these two conditions can exacerbate each other. Fatal hypothermia and DKA have overlapping features and findings such as Wischnewsky spots (WS), black esophagus, basal subnuclear vacuolization in the renal tubule, dehydration, and increased acetone levels. Therefore, it may be challenging to differentiate or clarify the context of these two conditions. Herein, we report a case of a 49-year-old man with type 1 diabetes who was found lying in his house in mid-winter. He experienced cardiopulmonary arrest 10 h after the initial discovery and died at the hospital. On autopsy, florid left cardiac blood was observed. Black discoloration of the distal part of the esophageal mucosa, widespread WS in the gastric mucosa, and black discoloration of the duodenal mucosa were observed. Histologically, neutrophil infiltration in the esophageal mucosa, neutrophil infiltration and bleeding in the gastric mucosa, basal subnuclear vacuolization and Armanni-Ebstein lesion in the renal tubule epithelium in the kidney, and hyalinization of the islets of Langerhans were observed in the pancreas. Blood acetone and ß-hydroxybutyrate levels were 538 µg/mL and 8947 µmol/L, respectively. Glycated hemoglobin A1c and glucose levels were 16.2% and 883 mg/dL, respectively, while C-reactive protein level was 3.64 mg/dL. In conclusion, obnubilation due to DKA was assumed to be the underlying cause of hypothermia, and the combination of these two conditions led to the outcome of death. The concurrent presence of these conditions likely contributed to the conspicuous mucosal findings in the upper gastrointestinal tract.

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 Secondary to Cyanotic Spells Associated with Unrepaired Tetralogy of Fallot.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease. Cyanotic spells occur more frequently after infancy in unrepaired cases. Acute esophageal necrosis (AEN) is a rare disease that causes circumferential mucosal necrosis in the distal esophagus. We report the case of a 26-year-old man who was admitted due to coffee-ground emesis, black stools, and decreased oxygen saturations. The patient had an unrepaired ToF and a congenital portosystemic venous shunt. An upper gastrointestinal endoscopy revealed AEN, which could be due to unstable hemodynamics of cyanotic spells. This is the first adult case presenting these 2 conditions occurring simultaneously.

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.

Comparison of Clinical Characteristics of Patients with Acute Esophageal Mucosal Lesion and those with Severe Reflux Esophagitis.

BACKGROUND: The term "acute esophageal mucosal lesion (AEML)" includes black esophagitis, and non-black esophagitis characterized by diffuse circumferential erosions without black-appearing mucosa. Black esophagitis is easily diagnosed, whereas non-black esophagitis is often misdiagnosed as severe reflux esophagitis (sRE). The aim of this study was to determine differences in clinical characteristics of patients with AEML and those with sRE. METHODS: Thirty-nine patients with sRE and 32 patients with AEML were diagnosed on the basis of endoscopic findings from 2009 to 2016. Characteristics assessed included age, sex, medication use, coexisting endoscopic finding, comorbidities, laboratory tests results, and chief complaints. RESULTS: In contrast with sRE, male sex, need for emergency endoscopy, presence of duodenal lesions, hypertension, and renal dysfunction were positively associated with AEML. Analysis of associations between laboratory data and AEML showed that high white blood cell count, blood urea nitrogen, and blood glucose were significantly associated with an increase OR for AEML. CONCLUSIONS: We showed that AEML differed from sRE regarding both endoscopic findings and clinicopathological features. AEML has not been widely recognized, but it should be defined as a distinct inflammatory disease of the esophagus consisting of both black and non-black esophagitis.

[Rare inflammatory diseases of the esophagus]. / Seltene Entzündungen des Ösophagus.

Because of its high incidence gastroesophageal reflux disease (GERD) is at the forefront of medical attention. On the other hand, vigilance for rare inflammatory diseases of the esophagus, such as herpes esophagitis, radiation-induced esophagitis, eosinophilic esophagitis, pseudodiverticulosis and Crohn's disease is low. Moreover, these entities are rarely distinguishable from GERD by clinical features alone. For all atypical and treatment refractory supposedly reflux diseases, the diagnosis should therefore be questioned and re-evaluated by endoscopy. When the macroscopic findings of esophagoscopy are ambiguous biopsies can be performed and histological examination then often leads to a definitive diagnosis. This is particularly important because the required treatment of rare forms of esophagitis often significantly differs from that of GERD.

Black esophagus: a case series and literature review of acute esophageal necrosis.

Black esophagus or acute esophageal necrosis (AEN) is a rare medical disorder which is characterized by a diffuse circumferential black esophageal mucosa. The majority of patients present with signs of upper gastrointestinal bleeding. Diagnosis is made based on esophagogastroduodenoscopy. Treatment consists of intravenous fluids, proton pomp inhibitors and additional therapies to treat the underlying illness. In this article we present five cases of patients with AEN and briefly review the literature of AEN.

Necrotizing Esophagitis and Bleeding Associated With Cefazolin.

OBJECTIVE: To report the case of a patient who presented with rare necrotizing esophagitis related to cefazolin-associated coagulopathy. A review of the literature is also provided. CASE SUMMARY: A 53-year-old male patient was admitted for management of septicemia and femur osteomyelitis. Long-term cefazolin treatment was initiated after cultures and sensitivity revealed methicillin-sensitive Staphylococcus aureus. The patient was given intravenous cefazolin 2 g every 24 hours. On day 15 of cefazolin treatment, the patient presented to the emergency department complaining of black coffee ground emesis. On upper-gastrointestinal endoscopy, the patient was determined to have necrotizing esophagitis. He was found to have an international normalized ratio (INR) of 8.11 and prothrombin time (PT) of 89.2 s. Intravenous vitamin K, fresh frozen plasma, and packed red blood cells were administered. The INR was rechecked 4 hours later and found to have decreased to 1.55 with a PT of 17 s. The patient did not have medical conditions or take medications that could have caused bleeding. DISCUSSION: Based on the Naranjo algorithm, it was determined that cefazolin had a "probable" relationship. Increased bleeding risk has been associated with other cephalosporins, although much less commonly with cefazolin. Possible mechanisms and implications are discussed. CONCLUSIONS: High-risk patients being treated with cefazolin therapy should be monitored for potentially severe adverse events, including bleeding and necrotizing esophagitis.

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.

A View in the Dark: Two Cases of Acute Esophageal Necrosis in the Setting of Diabetic Ketoacidosis.

Acute esophageal necrosis (AEN), also known as Gurvits syndrome, is a rare and potentially life-threatening condition characterized by necrosis of the esophageal mucosa. Acute esophageal necrosis is often associated with critical conditions, such as myocardial infarction, diabetic ketoacidosis (DKA), coronavirus disease 2019 (COVID-19) infection, or post-surgical complications. Patients typically present with nausea, hematemesis, acute dysphagia, and melena. Given its high mortality rate, prompt detection with upper endoscopy and early initiation of treatment are crucial. Most cases of Gurvits syndrome are managed conservatively using intravenous fluids, proton pump inhibitors, and antibiotics. Herein, we present a case series of AEN in the setting of DKA. Both patients received supportive care and were discharged in a stable condition.

A Case of Diabetic Ketoacidosis Complicated With Necrotizing Esophagitis.

Acute necrotizing esophagitis (ANE) is known as the "black esophagus." We present a case of ANE in a patient with slowly progressive type 1 diabetes mellitus. A 49-year-old man presented with vomiting, characterized by coffee residue-like emesis, and was diagnosed with diabetic ketoacidosis. Upper gastrointestinal endoscopy revealed black mucosa extending from the middle of the esophagus to the gastric junction, leading to a diagnosis of ANE. The patient was treated with proton pump inhibitors and showed marked improvement. The patient was discharged on the 20th day of illness.

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.

An Unusual Case of Black Esophagus.

Patients with acute esophageal necrosis often present with hematemesis and upper gastrointestinal bleeding. Our case report describes a patient's incidental discovery of black esophagus without recent hemodynamic instability, symptoms, or evidence of blood loss anemia. As illustrated in this case, it is important to recognize these findings in patients without classic signs and symptoms to act promptly and prevent tissue ischemia or perforation. Early recognition can also help reduce the risk of long-term complications such as stricture formation. Thus, a high index of suspicion is essential for the diagnosis of acute esophageal necrosis.

Acute Esophageal Necrosis or Black Esophagus in the Setting of Diabetic Ketoacidosis.

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.

"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.

Clinical features of acute esophageal mucosal lesions and reflux esophagitis Los Angeles classification grade D: A retrospective study.

BACKGROUND: Acute esophageal mucosal lesions (AEMLs) are an underrecognized and largely unexplored disease. Endoscopic findings are similar, and a higher percentage of AEML could be misdiagnosed as reflux esophagitis Los Angeles classification grade D (RE-D). These diseases could have different pathologies and require different treatments. AIM: To compare AEML and RE-D to confirm that the two diseases are different from each other and to clarify the clinical features of AEML. METHODS: We selected emergency endoscopic cases of upper gastrointestinal bleeding with circumferential esophageal mucosal injury and classified them into AEML and RE-D groups according to the mucosal injury's shape on the oral side. We examined patient background, blood sampling data, comorbidities at onset, endoscopic characteristics, and outcomes in each group. RESULTS: Among the emergency cases, the AEML and RE-D groups had 105 (3.1%) and 48 (1.4%) cases, respectively. Multiple variables exhibited significantly different results, indicating that these two diseases are distinct. The clinical features of AEML consisted of more comorbidities [risk ratio (RR): 3.10; 95% confidence interval (CI): 1.68-5.71; P < 0.001] and less endoscopic hemostasis compared with RE-D (RR: 0.25; 95%CI: 0.10-0.63; P < 0.001). Mortality during hospitalization was higher in the AEML group (RR: 3.43; 95%CI: 0.82-14.40; P = 0.094), and stenosis developed only in the AEML group. CONCLUSION: AEML and RE-D were clearly distinct diseases with different clinical features. AEML may be more common than assumed, and the potential for its presence should be taken into account in cases of upper gastrointestinal bleeding with comorbidities.

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.

Analysis of the pathogenesis of acute necrotizing esophagitis (black esophagus): A report of three autopsy cases.

Acute necrotizing esophagitis (ANE) is a rare condition characterized by black discoloration of the esophageal mucosa. We describe three autopsy cases of ANE, also known as black esophagus. The black discoloration was confined to the esophageal mucosa rather than to the gastric mucosa. The histological findings of brown pigmentation and acute inflammation led to an ANE diagnosis. The immediate cause of death was certified as ANE in all cases. In the three cases, one had hypertension, diabetes, and multiple cerebral infarctions, another had alcoholism, whereas the pre-existing condition was unknown in the remaining patient. Petechial hemorrhages were found on the gastric mucosa of all three patients as a finding of terminal hypothermia. In one case, frequent vomiting was observed prior to death. Blood alcohol was detected (the patient had been drinking immediately prior to death), and the onset of ANE was considered to have occurred several hours before death. The findings indicate that ANE occurs shortly before death in combination with frequent vomiting and terminal hypothermia in the setting of cerebrovascular disease or alcoholism.

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.