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Acute esophageal necrosis is a life-threatening condition characterized by acute ischemia of the esophagus, typically caused by reduced blood flow or chemical injuries. This condition presents significant challenges in diagnosis and management. Symptoms may include severe chest pain, difficulty swallowing, and signs of systemic infection. The condition is associated with medically comorbid patients. Prompt diagnosis and aggressive supportive treatment are needed in the initial setting, with early consideration of surgical intervention to prevent poor outcomes. This review evaluates the etiology, clinical presentation, and diagnostic approaches to acute esophageal necrosis and the critical management steps required by the interprofessional team to treat this rare entity. Participants gain a thorough understanding of how to recognize early signs, interpret diagnostic findings, and implement effective treatment plans. The activity also highlights the role of an interprofessional healthcare team in enhancing patient outcomes through timely and informed interventions. Objectives: Identify the relevant clinical anatomy of the esophagus to understand the implications for diagnosing and treating esophageal necrosis. Assess the etiologic risks associated with the development of acute esophageal necrosis. Apply evidence-based treatment protocols tailored to the stage and cause of esophageal necrosis. Collaborate with a multidisciplinary team, including gastroenterologists, surgeons, and intensive care specialists, to improve clinical outcomes for patients affected by esophageal necrosis. Access free multiple choice questions on this topic.

Acute esophageal necrosis (AEN), also referred to as black esophagus, Gurvits syndrome, or acute necrotizing esophagitis, is a rare and life-threatening condition affecting the esophagus. This condition is characterized by either partial or total circumferential blackening of the esophagus that stops abruptly at the gastroesophageal junction, as observed on an upper gastrointestinal endoscopy. AEN was first documented in 1990 by Goldberg et al, and the exact cause remains unclear, but AEN tends to follow a pattern of acute ischemia associated with a topical insult to the esophagus.[1] AEN has a prevalence of up to 0.2% in the autopsy series and ranges from 0.01% to 0.28% in the endoscopy series. This condition occurs more frequently in males, with an incidence up to 4 times higher compared to that in females, and typically affects middle-aged individuals. AEN predominantly impacts the distal esophagus, affecting 97% of cases. AEN typically spares the proximal two-thirds of the esophagus and almost always stops at the gastroesophageal junction.[2] Management of AEN is primarily medical but can include surgical intervention in the event of complications such as perforation and mediastinitis. Overall mortality associated with AEN is approximately 30%; however, mortality specific to AEN is closer to 5%.

The etiopathogenesis of AEN is considered multifactorial, so a comprehensive approach is essential for accurate diagnosis. AEN is believed to result from impaired mucosal barrier function and chemical or ischemic insults to the esophagus. Risk factors associated with this syndrome include male sex, malignancies, diabetes mellitus, cardiovascular diseases, prothrombotic conditions, chronic kidney disease, malnutrition, alcohol abuse, and gastroesophageal reflux disease.[2] The main developing theory is the 2-hit hypothesis, which combines a hemodynamic compromise and increased metabolic requirement from local insult leading to necrosis.[3][4] Most AEN occurs in the distal esophagus, comprising 60% to 90% of cases.[5][6][7] The distal third of the esophagus, which receives blood from the left gastric or inferior phrenic arteries, has the poorest blood supply with the poorest collateral support compared to the proximal two-thirds.[8][9] This segment is closest to the stomach and most at risk from gastric acid reflux. Gastric acid increases esophageal mucosal blood flow and the oxygen requirement in normal physiological conditions (see Image. Esophageal Necrosis Endoscopy).[10] Significantly increased volumes of gastric acid, for example, as a result of gastric outlet obstruction, have been shown to cause AEN.[3][4] The cause appears to be the combined effect of preexisting vasculopathy, an acute low-flow state, and an increased oxygen demand. Contrary to this, there have been cases of AEN in the absence of other medical conditions or a low-flow state.[11][12] However, in all cases, at least 2 of the following are present—acute ischemia, chronic vascular disease, and significant topical injury.

AEN is a rare but potentially underdiagnosed condition. In a review of published cases, only 88 patients were documented with AEN over 40 years.[3] In this published case series, nearly 80% of the cases occurred in males (70 males out of 88 subjects), with only 20% in females. In an autopsy series, the prevalence of blackening of the esophagus, a symptom of AEN, was 2%.[13] However, this may have occurred postmortem as opposed to in vivo. In endoscopic studies, prevalence appears to be between 0.1% and 0.3%, with the average age of occurrence between 70 and 80. Overall, AEN is approximately 4 times more likely to occur in males.[13][14][15][16][17] In a more recent literature review, the average age of occurrence was 62, with 70% of patients being male.[7] In contrast to the above studies, a study from Japan found a prevalence of approximately 6% of patients on who an endoscopy was performed; the other most common findings were a hiatus hernia and nonsteroidal anti-inflammatory drug usage.[18] This revelation implies that AEN may be more common and present transiently in patients with upper gastrointestinal bleeding.[18] Risk factors for AEN include the following: Type 2 diabetes (approximately 40%) Hypertension (approximately 35%) Ischaemic heart disease (12%) Alcoholism (approximately 25%) Chronic kidney disease (approximately 15%) Chronic liver disease (approximately 15%) Solid organ malignancy (approximately 10%) Peripheral vascular disease (approximately 10%) Chronic obstructive pulmonary disease (approximately 5%) Poor nutritional state [3][4][5][6][7][15][17][19]

Although the exact pathophysiology of AEN remains unclear, it is widely believed to result from a combination of esophageal mucosal injury caused by gastric acid and ischemic damage due to vascular compromise. The unique vascular supply of the esophagus, with the distal portion being relatively less vascularized than the proximal and middle sections, likely explains why AEN predominantly affects the distal esophagus. Typically, AEN presents with patchy, friable, blackish discoloration of the esophageal mucosa that is circumferential and primarily involves the distal esophagus. A mucosal biopsy is necessary to differentiate AEN from other conditions that cause similar dark discoloration and to identify any underlying acute infectious processes.[20][21]

In most case studies, AEN cases initially present symptoms of an upper gastrointestinal bleed, with haematemesis or melaena occurring in 70% to 85% of cases and hemodynamic instability.[3][7] Patients are typically aged 60 to 80, male, and have numerous comorbidities.[5] On examination, the patient may exhibit epigastric tenderness, pallor, and peripheral hypoperfusion. Vital signs often show low-grade pyrexia, hypotension, tachycardia, and, in more severe cases, hypoxia. As AEN often presents as a result of hemodynamic instability, the underlying cause of this instability can further complicate the clinical picture.[22][23][24] Cases of AEN have been observed as a result of sepsis, cardiogenic shock, hemorrhage from either planned operation or trauma, or other medical conditions, including diabetic ketoacidosis, diarrhea, and vomiting.[25][26][27][24]

Blood tests typically reveal a raised white blood cell count, anemia, and lactic acidosis.[8] In cases of gastrointestinal bleeding and upper endoscopy, the typical presentation of AEN is a diffuse circumferential black discoloration of the esophageal mucosa that stops abruptly at the gastroesophageal junction.[8] As stated, it is most common in the distal third of the esophagus. However, in a literature review, esophageal necrosis can extend proximally to involve the whole esophagus in 36% of cases.[6] Other pathologies may also be present, such as hiatus hernias, active bleeding, gastric ulceration, blood clots, or gastric outlet obstruction.[4] Although a biopsy is not always necessary to diagnose AEN, it is highly recommended. Biopsy findings typically include necrotic debris, the absence of squamous epithelium, and mucosal necrosis. In our case, biopsy samples were collected during the second esophagogastroduodenoscopy due to mucosal fragility observed in the first endoscopy. Histological analysis revealed ulcerated and partially necrotic mucosa, along with signs of granulation tissue, indicating the esophageal mucosa's attempt at self-repair.[2] Imaging is typically not performed except in cases of diagnostic uncertainty. In such cases, computed tomography (CT) scanning shows esophageal thickening and edema, or if there is concern about esophageal perforation, a chest CT may reveal air in the mediastinum (see Image. Esophageal Thickening, Chest CT).[5][18][2]

In isolation, AEN has a poor prognosis. When AEN is observed as one of the multiple symptoms (it should be viewed as a poor prognostic factor), initial efforts should be made to treat the underlying cause. Treatment for AEN typically involves fluid resuscitation with accompanying blood transfusion if there is significant anemia.[5][28] In addition, patients should be placed on a nil per os (NPO; "nothing through the mouth") or nothing-by-mouth order, and intravenous proton pump inhibitors should be given to reduce local insult to the vulnerable esophagus.[6][8][29] Sucralfate has also been used to further reduce local insult to the esophagus. Due to an often lengthy period of NPO, there has been some evidence that total parenteral nutrition improves outcomes.[4][30] Nasogastric tubes should not be inserted due to the risk of esophageal perforation.[29] Antibiotics should be given in the presence of sepsis or evidence of esophageal perforation but are otherwise not required.[4] Acute complications include upper gastrointestinal bleeding and esophageal perforation, which may be present at the time of presentation to clinicians or during the first few days in the hospital. Localized bleeding is controllable with an adrenaline injection during a gastroscopy or metallic stent insertion.[31] The incidence of esophageal perforation due to AEN appears to be approximately 5%.[6][29][32] If there is evidence of perforation, surgery is the primary treatment. Surgery was performed in approximately 4% of cases in an analysis.[6] The procedure involves an initial emergency esophagectomy followed by elective esophageal reconstruction after recovery from the initial event.[33] In some cases, stent insertion in the initial phase was effective but required subsequent intervention to correct stent migration.[34] Another approach involved a combination of video-assisted thoracoscopic surgery and drain placement, which was reportedly successful.[35]

When diagnosing esophageal necrosis, it is essential to consider a range of differential diagnoses due to the overlap of symptoms with other esophageal conditions. Accurate differentiation is critical for appropriate management and treatment. Differential diagnoses of AEN include the following: Malignant melanoma [36] Acanthosis nigricans [37] Coal dust deposition [38] Pseudomelanosis [39] Melanosis of the esophagus [40] Black dye ingestion [41] Direct caustic injury [1] Most of the above conditions follow a longer-term, more insidious course and are often found during elective gastroscopy rather than in an emergency. Exceptions include dye ingestion or direct caustic injury, where a detailed history is crucial for identifying the underlying cause of the black esophagus. If there is doubt over the pathogenesis of a black esophagus, then histology can be performed following an upper gastrointestinal endoscopy. If the condition is due to AEN, it shows mucosal and submucosal necrosis, necrotic debris, and a localized inflammatory response.[15][42][43]

AEN itself is a poor prognostic factor that results from other conditions. The most recent literature review reports a mortality rate of just under 30%.[7] This is similar to earlier reviews, which estimated mortality at 32%.[3][6][7][6] Because AEN is associated with various conditions, it can be challenging to determine which specific condition is responsible for the patient's death. Considering this, AEN-specific mortality has been noted to be as low as 5%.[3]

Acute complications of AEN include localized bleeding and esophageal perforation. Upper gastrointestinal bleeding is commonly present in approximately 85% of cases.[7] The perforation rate is believed to be 5% to 7%.[3][6] Mid- to long-term complications include esophageal strictures and tracheoesophageal fistulas. According to a literature review, the complication rate is approximately 12%, with 70% of these patients developing strictures and 30% developing fistulas.[7] These complications do not develop until at least 2 weeks after the initial event. The literature does not document stricture management well, but iterative balloon dilatation is an effective treatment.[44] Most patients recover without complication; however, a follow-up endoscopy is recommended at approximately 1 month to rule out other sequelae.[8]

Patients with esophageal necrosis often require consultations with a multidisciplinary team to ensure comprehensive care. Gastroenterologists are essential for initial diagnosis and endoscopic evaluation, whereas surgeons may be needed for cases involving perforation or other complications requiring surgical intervention. Dietitians are crucial in managing nutritional support, especially if long-term parenteral nutrition is necessary. Infectious disease specialists might be consulted to manage potential infections, particularly in cases of sepsis. In addition, critical care specialists are often involved due to the high morbidity associated with esophageal necrosis. Coordinated care among these professionals is vital to optimize treatment outcomes and ensure holistic patient management.

Deterrence and patient education are crucial in managing esophageal necrosis, with a focus on prevention and early intervention. Educating patients about the risk factors, such as alcohol abuse, uncontrolled diabetes, and vascular diseases, can help reduce the incidence of this condition. Clinicians should emphasize the importance of managing underlying health conditions and avoiding behaviors that increase the risk of esophageal injury. Recommended lifestyle factors include moderation of alcohol consumption, smoking cessation, regular exercise, and maintaining a healthy weight. Medical prevention for AEN has not been documented in the literature. In addition, informing patients about the symptoms of esophageal necrosis, such as severe chest pain and difficulty swallowing, can lead to earlier medical consultation and prompt treatment. Through comprehensive patient education and regular follow-up, healthcare professionals can significantly improve the prognosis and quality of life for individuals at risk of esophageal necrosis.

Effective management of esophageal necrosis requires a comprehensive approach involving various skills and strategies from an interprofessional team. Each member of the team brings specialized skills to the table. Physicians and advanced practitioners need diagnostic acumen and procedural expertise to accurately identify and treat esophageal necrosis. Nurses require strong clinical skills to monitor patients, manage symptoms, and provide supportive care. Pharmacists offer critical knowledge on medication management, ensuring that drug therapies are both safe and effective. AEN is an uncommon condition that can present in several different ways, including upper gastrointestinal bleeding, signs of sepsis, or peripheral hypoperfusion. Medically comorbid patients presenting with epigastric pain and hemodynamic compromise should raise concerns for urgent investigation and treatment. Early recognition and subsequent resuscitation form the cornerstone of management for these patients, regardless of diagnostic uncertainty. Upper gastrointestinal endoscopy is crucial for diagnosis and should be performed immediately. If the patient is not stable enough, it may be necessary to collaborate closely with the endoscopy department or the operating room. Evidence of perforation requires surgical intervention, and long-term parenteral nutrition should be initiated early with guidance from dietitians. Coordinated care involves seamless transitions between different stages of treatment and various healthcare team members, requiring meticulous planning and organization to prevent gaps in care, particularly in cases of prolonged rehabilitation. Care coordinators or case managers can play a pivotal role in this process, ensuring that all aspects of a patient's care are integrated and cohesive. This interprofessional approach not only enhances patient-centered care and outcomes but also ensures patient safety and optimizes team performance.