Acute esophageal necrosis
Encyclopedia
Acute esophageal necrosis (AEN), also known as Gurvits Syndrome, Black Esophagus, acute necrotizing esophagitis, and esophageal infarction; is a rare esophageal
disorder. AEN defines itself with dark pigmentation of the esophagus
, found during an upper gastrointestinal endoscopy
. Pigmentation is usually black friable mucosa. The disorder is extremely rare, as only 88 patients over a span of 40 years have received this diagnosis. Specific study of the disorder's mortality rate is mentioned at 31.8%, but new research suggests mortality rates vary from 30-50%. The exact triggering mechanism for this disorder is still unknown, but is likely a multifactorial.
and blood in vomiting. Upper gastrointestinal bleeding
then is reported, and is very commonly represented in elderly patients. Black or bloody stools and hematemesis account for over three quarters of the case presentations. Abdominal pain, nausea, vomiting, and unstable vital signs are common. A cardiovascular event (such as a heart attack
) was reported in ten percent of the total known cases.
(current or previous) is currently one of the most prevalent out of all conditions among patients. High blood pressure
,Chronic lung conditions, Alcohol abuse
,Kidney failure
, Malnutrition
are another major risk factors. .
, and/or a proton pump inhibitor
. Sucralfate
was used as an option. Parenteral nutrition greatly increased chance of recovery. An esophagectomy
can be issued if the disorder is severe enough.
. Jan Garavaglia, the show's host, receives a female body, that at time of the autopsy had a severe case of acute esophageal necrosis due to chronic alcoholism
.
Esophagus
The esophagus is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. During swallowing, food passes from the mouth through the pharynx into the esophagus and travels via peristalsis to the stomach...
disorder. AEN defines itself with dark pigmentation of the esophagus
Esophagus
The esophagus is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. During swallowing, food passes from the mouth through the pharynx into the esophagus and travels via peristalsis to the stomach...
, found during an upper gastrointestinal endoscopy
Endoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...
. Pigmentation is usually black friable mucosa. The disorder is extremely rare, as only 88 patients over a span of 40 years have received this diagnosis. Specific study of the disorder's mortality rate is mentioned at 31.8%, but new research suggests mortality rates vary from 30-50%. The exact triggering mechanism for this disorder is still unknown, but is likely a multifactorial.
Signs and symptoms
AEN has never been recorded as a one symptom disorder, but instead present by multiple symptoms. The symptoms vary from the severity of the disorder. The most classic sign of AEN is the dark pigmentation of esophageal mucosa in an upper endoscopy, usually viewed as an ulcer or as an infectious disease. Necrosis can be found mostly between the three distals of the esophagus, but stops abruptly at the gastroesophageal junction. The basic and most common symptoms reported are blood in stoolBlood in stool
In medicine, when referring to human feces, blood in stool can refer to multiple conditions:* Melena, with more blackish appearance, originating in upper parts of the gastrointestinal tract...
and blood in vomiting. Upper gastrointestinal bleeding
Upper gastrointestinal bleeding
Upper gastrointestinal bleeding refers to hemorrhage in the upper gastrointestinal tract. The anatomic cut-off for upper GI bleeding is the ligament of Treitz, which connects the fourth portion of the duodenum to the diaphragm near the splenic flexure of the colon.Upper GI bleeds are considered...
then is reported, and is very commonly represented in elderly patients. Black or bloody stools and hematemesis account for over three quarters of the case presentations. Abdominal pain, nausea, vomiting, and unstable vital signs are common. A cardiovascular event (such as a heart attack
Myocardial infarction
Myocardial infarction or acute myocardial infarction , commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die...
) was reported in ten percent of the total known cases.
Modifiable
Having cancerCancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
(current or previous) is currently one of the most prevalent out of all conditions among patients. High blood pressure
Hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...
,Chronic lung conditions, Alcohol abuse
Alcoholism
Alcoholism is a broad term for problems with alcohol, and is generally used to mean compulsive and uncontrolled consumption of alcoholic beverages, usually to the detriment of the drinker's health, personal relationships, and social standing...
,Kidney failure
Renal failure
Renal failure or kidney failure describes a medical condition in which the kidneys fail to adequately filter toxins and waste products from the blood...
, Malnutrition
Malnutrition
Malnutrition is the condition that results from taking an unbalanced diet in which certain nutrients are lacking, in excess , or in the wrong proportions....
are another major risk factors. .
Nonmodifiable
- Advanced ageAgeingAgeing or aging is the accumulation of changes in a person over time. Ageing in humans refers to a multidimensional process of physical, psychological, and social change. Some dimensions of ageing grow and expand over time, while others decline...
(average patient aged 57 years old) - Male sexMaleMale refers to the biological sex of an organism, or part of an organism, which produces small mobile gametes, called spermatozoa. Each spermatozoon can fuse with a larger female gamete or ovum, in the process of fertilization...
(4 to 1 male dominance) - CirrhosisCirrhosisCirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrosis, scar tissue and regenerative nodules , leading to loss of liver function...
- AIDSAIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
- Herpes virusHerpes simplex virusHerpes simplex virus 1 and 2 , also known as Human herpes virus 1 and 2 , are two members of the herpes virus family, Herpesviridae, that infect humans. Both HSV-1 and HSV-2 are ubiquitous and contagious...
- Atrial fibrilation
- Diabetes (currently the most prevalent out of all conditions in patients)
Lesser or unknown of effect
- Aortic dissectionAortic dissectionAortic dissection occurs when a tear in the inner wall of the aorta causes blood to flow between the layers of the wall of the aorta and force the layers apart. The dissection typically extends anterograde, but can extend retrograde from the site of the intimal tear. Aortic dissection is a medical...
- Anti-cardiolipin antibodiesAnti-cardiolipin antibodiesAnti-cardiolipin antibodies are antibodies often directed against cardiolipin and found in several diseases including syphilis, antiphospholipid syndrome, livedoid vasculitis, vertebrobasilar insufficiency, Behçet's syndrome, idiopathic spontaneous abortion, and systemic lupus erythematosus. They...
- CMV infectionCytomegalovirusCytomegalovirus is a viral genus of the viral group known as Herpesviridae or herpesviruses. It is typically abbreviated as CMV: The species that infects humans is commonly known as human CMV or human herpesvirus-5 , and is the most studied of all cytomegaloviruses...
- Herpetic infection
- HyperglycemiaHyperglycemiaHyperglycemia or Hyperglycæmia, or high blood sugar, is a condition in which an excessive amount of glucose circulates in the blood plasma. This is generally a glucose level higher than 13.5mmol/l , but symptoms may not start to become noticeable until even higher values such as 15-20 mmol/l...
- HypersensitivityHypersensitivityHypersensitivity refers to undesirable reactions produced by the normal immune system, including allergies and autoimmunity. These reactions may be damaging, uncomfortable, or occasionally fatal. Hypersensitivity reactions require a pre-sensitized state of the host. The four-group classification...
to broad-spectrum antibiotics - HypothermiaHypothermiaHypothermia is a condition in which core temperature drops below the required temperature for normal metabolism and body functions which is defined as . Body temperature is usually maintained near a constant level of through biologic homeostasis or thermoregulation...
- IschemiaIschemiaIn medicine, ischemia is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. It may also be spelled ischaemia or ischæmia...
- Gastric volvulusGastric volvulusGastric volvulus or volvulus of stomach a twisting of all or part of the stomach by more than 180 degrees with obstruction of the flow of material through the stomach, variable loss of blood supply and possible tissue death. The twisting can occur around the long axis of the stomach, this is called...
- Posterior mediastinal haematoma
- Septic shockSeptic shockSeptic shock is a medical emergency caused by decreased tissue perfusion and oxygen delivery as a result of severe infection and sepsis, though the microbe may be systemic or localized to a particular site. It can cause multiple organ dysfunction syndrome and death...
- Steven Johnson syndrome
Diagnosis
Acute esophageal necrosis can only be diagnosed by an upper gastrointestinal endoscopy.Treatment
Currently, there is no direct treatment for AEN. Only treatment is for the underlying main diseases or conditions. Appropriate hydration is set. Antacids are also added for further recovery support. Common support drugs of antacids are either H2 receptor antagonistsReceptor antagonist
A receptor antagonist is a type of receptor ligand or drug that does not provoke a biological response itself upon binding to a receptor, but blocks or dampens agonist-mediated responses...
, and/or a proton pump inhibitor
Proton pump inhibitor
Proton-pump inhibitors are a group of drugs whose main action is a pronounced and long-lasting reduction of gastric acid production. They are the most potent inhibitors of acid secretion available today. The group followed and has largely superseded another group of pharmaceuticals with similar...
. Sucralfate
Sucralfate
Sucralfate is a cytoprotective agent, an oral gastrointestinal medication primarily indicated for the treatment of active duodenal ulcers. Brand names include Sucramal in Italy; Carafate in U.S.A.; Pepsigard, Sucral, Sucrafil, Hapifate in India; Sutra or Musin in parts of South-East Asia; Sulcrate...
was used as an option. Parenteral nutrition greatly increased chance of recovery. An esophagectomy
Esophagectomy
Esophagectomy or Oesophagectomy is the surgical removal of all or part of the esophagus .-Purpose:...
can be issued if the disorder is severe enough.
Society and culture
Acute esophageal necrosis made an appearance on an American medical drama show, Dr. G: Medical ExaminerDr. G: Medical Examiner
Dr. G: Medical Examiner is a reality television series shown on the Discovery Fit & Health. Dr. G, or Dr. Jan Garavaglia, is the Chief Medical Examiner with Florida's District Nine Medical Examiner's office in Orlando, Florida.This series features Dr...
. Jan Garavaglia, the show's host, receives a female body, that at time of the autopsy had a severe case of acute esophageal necrosis due to chronic alcoholism
Alcoholism
Alcoholism is a broad term for problems with alcohol, and is generally used to mean compulsive and uncontrolled consumption of alcoholic beverages, usually to the detriment of the drinker's health, personal relationships, and social standing...
.