What is Esophageal Necrosis?
Acute esophageal necrosis (AEN), sometimes known as black esophagus, Gurvits syndrome, or acute necrotizing esophagitis, is a rare and serious condition affecting the esophagus, which is the tube that links your mouth to your stomach. This condition results in the esophagus turning black either partially or entirely, stopping sharply at the junction where the stomach starts. This change can be seen during an upper gastrointestinal endoscopy, which is a procedure where a small camera is used to view your digestive system.
This condition was first recorded by Goldberg and his team in 1990, and we’re still not sure what exactly causes it. However, it generally appears to be connected to acute ischemia, which means a sudden reduction in blood supply, combined with direct damage to the esophagus.
AEN is not a common condition, occurring in up to 0.2% of autopsies and ranging from 0.01% to 0.28% in endoscopy procedures. It tends to occur more in males, who have a risk up to 4 times higher than females, and usually affects individuals in middle age. The lower part of the esophagus is affected in 97% of cases, whereas the upper two-thirds are usually unaffected. The blackening almost always stops at the junction where the esophagus meets the stomach.
The main way to manage AEN is through medical treatment, though surgery may be needed if the condition causes complications such as a torn esophagus or mediastinitis, which is an infection in the space between the lungs. About 30% of people with AEN die, but only about 5% of these deaths are directly linked to AEN itself.
What Causes Esophageal Necrosis?
Acute esophageal necrosis (AEN) is thought to develop due to a range of factors, so a thorough evaluation is needed for an accurate diagnosis. AEN is thought to occur from damage to the protective lining of the esophagus, along with chemical or blood supply-related injuries to the esophagus. Men, people with cancer, diabetes, heart diseases, conditions that cause blood clots, chronic kidney disease, malnutrition, alcohol abuse, and gastroesophageal reflux disease (heartburn) are more at risk of getting AEN.
A leading theory, known as the 2-hit hypothesis, suggests that AEN happens due to a combination of a decrease in blood supply and an increase in metabolic demand from local injury, leading to cell death. Most AEN cases occur in the lower part of the esophagus, which comprises 60% to 90% of cases. This area of the esophagus has the weakest blood supply and is most vulnerable to stomach acid reflux. Stomach acid naturally increases blood flow in the lining of the esophagus and its oxygen need in normal conditions.
There may be a significant increase in stomach acid volumes, for example, as a result of a blockage at the stomach outlet, which can cause AEN. The cause seems to be a combination of pre-existing vascular disease (abnormalities in the blood vessels), an acute state of low blood flow, and increased oxygen need. However, there have been cases of AEN without other medical conditions or a state of low blood flow. Even in these situations, at least two of the following are present – acute ischemia (insufficient blood supply), chronic vascular disease, and significant injury to the esophagus lining.
Risk Factors and Frequency for Esophageal Necrosis
AEN, also known as black esophagus, is a rare condition that may be overlooked in diagnosis. Over a 40-year period, there were only 88 recorded cases. Of these, nearly 80% were in males. It’s important to note that this condition might occur more often than records indicate, with some studies finding evidence of it in 2% of autopsies and between 0.1% and 0.3% of endoscope exams. Usually, people between the ages of 70 to 80 are affected, and it’s four times more likely in males.
A newer review indicated that the average age of occurrence was 62, and 70% of patients were male. Interestingly, a study from Japan suggested a prevalence of about 6% among patients who underwent an endoscopy, suggesting that AEN might be more common than previously thought, especially in cases of upper gastrointestinal bleeding.
Also, it’s worth noting some risk factors for AEN:
- Type 2 diabetes – (around 40% of cases)
- Hypertension – (around 35% of cases)
- Ischemic heart disease – (12% of cases)
- Alcoholism – (roughly 25% of cases)
- Chronic kidney disease – (about 15% of cases)
- Chronic liver disease – (around 15% of cases)
- Solid organ malignancy – (approximately 10% of cases)
- Peripheral vascular disease – (around 10% of cases)
- Chronic obstructive pulmonary disease – (about 5% of cases)
- Poor nutritional state
Signs and Symptoms of Esophageal Necrosis
Acute esophageal necrosis (AEN), sometimes also known as “black esophagus,” is often identified when a person experiences symptoms of an upper stomach bleed. This can include vomiting blood or passing dark, tarry stools, which are noticed in about 70% to 85% of cases. It can also lead to a drop in body stability. Typically, AEN is more likely to affect men aged 60 to 80, and those who have existing health conditions.
During a medical check-up, the individual might show signs of tender upper abdomen, look unusually pale, and exhibit poor circulation in their arms and legs. Often, their vital signs may indicate mild fever, low blood pressure, unnecessarily fast heart rate, and in severe situations, inadequate oxygen levels.
As AEN is generally a result of instability in the body, its underlying cause can add to the complexity of the case. It has been seen as a consequence of severe infection, heart-induced shock, bleeding from a surgery or injury, or other medical conditions like uncontrolled diabetes, diarrhea, and vomiting.
Testing for Esophageal Necrosis
Blood tests usually show an increase in white blood cells, a decrease in red blood cells (anemia), and a high level of lactic acid. Acute esophageal necrosis (AEN), a condition where the esophagus cells die, is usually observed as a sudden darkening of the esophagus tissue found through an upper endoscopy, a procedure where a long, flexible tube is used to view the digestive tract. This change in color typically stops at the point where the esophagus meets the stomach. AEN usually occurs in the lower third of the esophagus. However, it can also affect the whole esophagus in about 36% of cases, according to a review of several studies. It is also common to find other conditions like hiatus hernias, active bleeding, stomach ulcers, blood clots, or blockages in the stomach’s exit.
A biopsy, a procedure where a small piece of tissue is taken for examination, can help diagnose AEN. It’s not always required, but it is highly recommended. The biopsy usually shows dead tissue, lack of squamous epithelium (flattened cells that line the esophagus), and tissue damage in the mucosa, the inner lining of the esophagus. In this case, biopsy samples were taken during the second endoscopy due to delicate mucosa observed in the first endoscopy. The examination of the tissue showed signs of ulcers, partial tissue death, and granulation tissue, the body’s attempt to heal the esophagus lining.
Images of the esophagus, typically through a computed tomography (CT) scan, are not always necessary unless the diagnosis is uncertain. In such cases, a CT scan might show a thickened esophagus and swelling, or if there is a risk of esophagus rupture, a chest CT scan may reveal air in the mediastinum, the space within the chest that contains the heart, lungs, and other organs.
Treatment Options for Esophageal Necrosis
When acute esophageal necrosis (AEN) is identified as a symptom, it often indicates a poor outcome. The first step in treating AEN is to address the underlying cause that led to its development. Patients with AEN usually need fluids, which are delivered via an IV, and a blood transfusion may be necessary if the patient is severely anemic.
Patients with AEN are typically instructed not to eat or drink anything, a measure doctors refer to as “NPO” or “nothing by mouth”. This is to help give the esophagus, the tube that carries food and liquid from the mouth to the stomach, a chance to heal. In the meantime, patients are given proton pump inhibitors, medicines that help decrease the production of stomach acid, to minimize further damage to the esophagus.
Other treatments might include the use of sucralfate, a medication that helps protect the esophagus. Since patients might not be able to eat for a long period, intravenous (IV) nutrition might be necessary to ensure the patient’s body gets the nutrients it needs. The use of a nasogastric tube, a tube inserted through the nose into the stomach, is generally avoided due to the risk of damaging the esophagus.
Antibiotics may be administered if the patient is showing signs of a severe infection (sepsis) or if there’s evidence of a perforation, or hole in the esophagus. Otherwise, antibiotics aren’t typically needed.
Possible immediate complications of AEN include upper gastrointestinal bleeding and esophageal perforation. If present, these complications may become apparent when the patient first enters medical care or within the first few days of hospitalization. Localized bleeding can generally be stopped through an adrenaline injection during a procedure where a camera is used to visualize the gastrointestinal tract, or by inserting a metallic stent.
About 5% of AEN patients experience esophageal perforation. If it occurs, surgery is the main treatment. Only a small number of AEN cases, around 4%, require surgery. This usually involves removing the damaged part of the esophagus and, after recovery, rebuilding the esophagus. In some instances, a stent may be inserted initially but may require later correction due to its movement. Another possible course of action combines minimally invasive surgery with inserting a drainage tube, which has been reported as successful.
What else can Esophageal Necrosis be?
When a doctor is trying to diagnose esophageal necrosis, or dead tissue in the esophagus, they have to think about a few other conditions that have similar symptoms. It’s important to know exactly what’s causing the problem to make sure the patient gets the right treatment.
Here are some conditions that might be confused with esophageal necrosis:
- Malignant melanoma (a type of skin cancer)
- Acanthosis nigricans (skin condition causing dark, thick skin)
- Coal dust deposition (from inhaling coal dust)
- Pseudomelanosis (discoloration of internal organs)
- Melanosis of the esophagus (abnormal brown-black spots in esophagus)
- Black dye ingestion (from eating or drinking something with black dye)
- Direct caustic injury (damage from a burn, cut, or other injury)
Most of these conditions develop slowly and are usually found during regular check-ups instead of in an emergency. The exceptions are when someone has swallowed dye or harmed the esophagus in some way. In these cases, it’s really important for the doctor to have a thorough understanding of the patient’s history. If the doctor isn’t sure why the esophagus is black, they can do a biopsy during an upper GI endoscopy. This allows them to take a small sample of tissue to study under a microscope. If the problem is esophageal necrosis, they’ll see dead tissue, debris, and signs of inflammation.
What to expect with Esophageal Necrosis
Acute esophageal necrosis (AEN), a medical condition affecting the esophagus, is often a sign of serious health problems and is associated with a high risk of mortality. Recent studies show a mortality rate of nearly 30%, which aligns with earlier findings that cited a mortality rate of 32%.
Since AEN can be linked to various other health conditions, it can be difficult to pinpoint the exact cause of death. However, when looking at deaths specifically caused by AEN, the mortality rate is significantly lower at around 5%.
Possible Complications When Diagnosed with Esophageal Necrosis
Immediate complications of AEN, or Acute Esophageal Necrosis, often involve localized bleeding and tearing in the esophagus. It’s reported that nearly 85% of cases may include upper gastrointestinal bleeding. The risk of an actual tear or perforation in the esophagus is estimated to be between 5% to 7%.
If we look over a longer period, complications might arise such as the narrowing of the esophagus, known as strictures, and the creation of abnormal connections between the esophagus and windpipe – these are called tracheoesophageal fistulas. According to a reviewed set of data, the complication rate stands at about 12 %, and of these cases, 70% result in strictures and 30% in the formation of fistulas.
It’s important to note that these complications usually don’t happen until at least 2 weeks after the initial event. Though there isn’t much detailed information on how strictures are managed, one effective approach is the repeated expansion of the esophagus through a balloon dilation procedure. Luckily, most patients recover without any complications. Still, a follow-up endoscopy is suggested approximately one month later to make sure there are no lingering effects.
Typical complications:
- Localized bleeding
- Tearing in the esophagus (esophageal perforation)
- Upper gastrointestinal bleeding
- Narrowing of the esophagus (strictures)
- Abnormal connections between the esophagus and windpipe (tracheoesophageal fistulas)
Preventing Esophageal Necrosis
Helping a patient to avoid or manage esophageal necrosis, a condition which damages the esophagus, requires both prevention and prompt action when symptoms appear. Patients need to be aware of the factors that can increase their risk of this condition, including excessive alcohol consumption, unchecked diabetes, and vascular diseases, which are diseases related to blood vessels. Doctors should stress the importance of dealing with these underlying health issues and avoiding behaviors that could harm the esophagus.
Recommended lifestyle changes that could help are moderating alcohol intake, stopping smoking, exercising regularly, and keeping a healthy weight. However, there is no medical prevention specifically for what is also known as AEN given in medical literature.
Besides this, patients also need to know the symptoms of esophageal necrosis, such as extreme pain in the chest area and trouble swallowing. Recognising these symptoms can lead to quicker medical help and treatment. With thorough education about the condition and regular check-ups, those in the healthcare field can significantly improve the outcome and life quality of patients at risk of esophageal necrosis.
