Acute esophageal necrosis

Last updated
Acute esophageal necrosis
Other namesGurvits syndrome, black esophagus, acute necrotizing esophagitis, [1] esophageal infarction
Illu esophagus.jpg
Areas represented in image (distals) are most common locations for acute esophageal necrosis to occur. AEN defines itself predominantly in the first of three distals of the esophagus. [2]
Specialty Gastroenterology
Risk factors Cancer, hypertension, chronic lung disease, kidney failure, malnutrition, alcohol use disorder, recreational drug use
Diagnostic method Upper endoscopy (EGD)
TreatmentVaries
DeathsMortality rate 30–50%

Acute esophageal necrosis (AEN), black esophagus, or Gurvits syndrome is a rare esophageal disorder. AEN defines itself with dark pigmentation of the esophagus, found during an upper gastrointestinal endoscopy. [2] Pigmentation is usually black friable mucosa.[ citation needed ] The disorder is extremely rare, as only 89 patients over a span of 40 years have received this diagnosis. [2] Specific study of the disorder's mortality rate is mentioned at 31.8%, [2] but new research suggests mortality rates vary from 30–50%. [3] [4] The exact triggering mechanism for this disorder is still unknown, but is likely multifactorial. [2]

Contents

Signs and symptoms

AEN has never been recorded as a one symptom disorder, but instead present by multiple symptoms. [2] 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. [5] Necrosis can be found mostly between the three distals of the esophagus, but stops abruptly at the gastroesophageal junction. [2] The basic and most common symptoms reported are blood in stool and blood in vomiting. Upper gastrointestinal bleeding then is reported, and is very commonly represented in elderly patients. [3] 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. [2]

Risk factors

Modifiable

Having cancer (current or previous) is currently one of the most prevalent out of all conditions among patients. High blood pressure, Chronic lung conditions, Alcohol use disorder, excessive alcohol use combined with other recreational drug use (e.g. cocaine), Kidney failure, and malnutrition are other major risk factors. [2] [6]

Nonmodifiable

Lesser or unknown of effect

Diagnosis

Acute esophageal necrosis can only be diagnosed by an upper gastrointestinal endoscopy. [2] [5] [7] It is usually preceded by haemodynamic stress, which can have a variety of etiologies. [8]

Treatment

Currently, there is no direct treatment for AEN. [6] 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 antagonists, and/or a proton pump inhibitor. [6] Sucralfate was used as an option. Parenteral nutrition greatly increased chance of recovery. An esophagectomy can be issued if the disorder is severe enough. [6]

Prognosis

The prognosis for acute esophageal necrosis is generally poor, as the condition is associated with a high risk of mortality (up to 32%). [9] Most mortality is attributed to the underlying cause; mortality specifically caused by AEN is about 6 percent. [9]

Society and culture

Acute esophageal necrosis made an appearance on an American medical drama show, Dr. G: Medical Examiner. 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 alcohol use disorder.

History

Acute esophageal necrosis was first described by Goldenberg et al. in 1990. [2] [6] Cases have emerged since 1960, but have never been described as the common names for AEN. Due to its rarity, only 88 cases have been pronounced AEN, but most likely there are many more. Gurvits et al. describes AEN as "poorly described in medical literature". [2] Abdullah et al. published the first-ever systemic review on acute esophageal necrosis and reviewed around 154 patients reported in the literature at the time of publishing. [10]

Related Research Articles

<span class="mw-page-title-main">Gastroenterology</span> Branch of medicine focused on the digestive system and its disorders

Gastroenterology is the branch of medicine focused on the digestive system and its disorders. The digestive system consists of the gastrointestinal tract, sometimes referred to as the GI tract, which includes the esophagus, stomach, small intestine and large intestine as well as the accessory organs of digestion which include the pancreas, gallbladder, and liver. The digestive system functions to move material through the GI tract via peristalsis, break down that material via digestion, absorb nutrients for use throughout the body, and remove waste from the body via defecation. Physicians who specialize in the medical specialty of gastroenterology are called gastroenterologists or sometimes GI doctors. Some of the most common conditions managed by gastroenterologists include gastroesophageal reflux disease, gastrointestinal bleeding, irritable bowel syndrome, inflammatory bowel disease (IBD) which includes Crohn's disease and ulcerative colitis, peptic ulcer disease, gallbladder and biliary tract disease, hepatitis, pancreatitis, colitis, colon polyps and cancer, nutritional problems, and many more.

<span class="mw-page-title-main">Esophageal achalasia</span> Rare, incurable, progressive motility disorder due to failure of esophogeal motor neurons

Esophageal achalasia, often referred to simply as achalasia, is a failure of smooth muscle fibers to relax, which can cause the lower esophageal sphincter to remain closed. Without a modifier, "achalasia" usually refers to achalasia of the esophagus. Achalasia can happen at various points along the gastrointestinal tract; achalasia of the rectum, for instance, may occur in Hirschsprung's disease. The lower esophageal sphincter is a muscle between the esophagus and stomach that opens when food comes in. It closes to avoid stomach acids from coming back up. A fully understood cause to the disease is unknown, as are factors that increase the risk of its appearance. Suggestions of a genetically transmittable form of achalasia exist, but this is neither fully understood, nor agreed upon.

Heartburn, also known as pyrosis, cardialgia or acid indigestion, is a burning sensation in the central chest or upper central abdomen. Heartburn is usually due to regurgitation of gastric acid into the esophagus. It is the major symptom of gastroesophageal reflux disease (GERD).

<span class="mw-page-title-main">Gastroesophageal reflux disease</span> Medical condition

Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is one of the upper gastrointestinal chronic diseases in which stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/or complications. Symptoms include dental corrosion, dysphagia, heartburn, odynophagia, regurgitation, non-cardiac chest pain, extraesophageal symptoms such as chronic cough, hoarseness, reflux-induced laryngitis, or asthma. In the long term, and when not treated, complications such as esophagitis, esophageal stricture, and Barrett's esophagus may arise.

<span class="mw-page-title-main">Barrett's esophagus</span> Medical condition

Barrett's esophagus is a condition in which there is an abnormal (metaplastic) change in the mucosal cells lining the lower portion of the esophagus, from stratified squamous epithelium to simple columnar epithelium with interspersed goblet cells that are normally present only in the small intestine and large intestine. This change is considered to be a premalignant condition because it is associated with a high incidence of further transition to esophageal adenocarcinoma, an often-deadly cancer.

Hematemesis is the vomiting of blood. It can be confused with hemoptysis or epistaxis (nosebleed), which are more common. The source is generally the upper gastrointestinal tract, typically above the suspensory muscle of duodenum. It may be caused by ulcers, tumors of the stomach or esophagus, varices, prolonged and vigorous retching, gastroenteritis, ingested blood, or certain drugs.

Coffee ground vomitus refers to a particular appearance of vomit. Within organic heme molecules of red blood cells is the element iron, which oxidizes following exposure to gastric acid. This reaction causes the vomitus to look like ground coffee.

<span class="mw-page-title-main">Nissen fundoplication</span> Surgical procedure to treat gastric reflux and hiatal hernia

A Nissen fundoplication, or laparoscopic Nissen fundoplication when performed via laparoscopic surgery, is a surgical procedure to treat gastroesophageal reflux disease (GERD) and hiatal hernia. In GERD, it is usually performed when medical therapy has failed; but, with a Type II (paraesophageal) hiatus hernia, it is the first-line procedure. The Nissen fundoplication is total (360°), but partial fundoplications known as Thal, Belsey, Dor, Lind, and Toupet fundoplications are alternative procedures with somewhat different indications and outcomes.

<span class="mw-page-title-main">Gastrointestinal bleeding</span> Medical condition

Gastrointestinal bleeding, also called gastrointestinal hemorrhage (GIB), is all forms of bleeding in the gastrointestinal tract, from the mouth to the rectum. When there is significant blood loss over a short time, symptoms may include vomiting red blood, vomiting black blood, bloody stool, or black stool. Small amounts of bleeding over a long time may cause iron-deficiency anemia resulting in feeling tired or heart-related chest pain. Other symptoms may include abdominal pain, shortness of breath, pale skin, or passing out. Sometimes in those with small amounts of bleeding no symptoms may be present.

<span class="mw-page-title-main">Gastritis</span> Stomach disease that is an inflammation of the lining of the stomach

Gastritis is inflammation of the lining of the stomach. It may occur as a short episode or may be of a long duration. There may be no symptoms but, when symptoms are present, the most common is upper abdominal pain. Other possible symptoms include nausea and vomiting, bloating, loss of appetite and heartburn. Complications may include stomach bleeding, stomach ulcers, and stomach tumors. When due to autoimmune problems, low red blood cells due to not enough vitamin B12 may occur, a condition known as pernicious anemia.

<span class="mw-page-title-main">Plummer–Vinson syndrome</span> Medical condition

Plummer–Vinson syndrome is a rare disease characterized by difficulty swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome.

Indigestion, also known as dyspepsia or upset stomach, is a condition of impaired digestion. Symptoms may include upper abdominal fullness, heartburn, nausea, belching, or upper abdominal pain. People may also experience feeling full earlier than expected when eating. Indigestion is relatively common, affecting 20% of people at some point during their life, and is frequently caused by gastroesophageal reflux disease (GERD) or gastritis.

<span class="mw-page-title-main">Eosinophilic esophagitis</span> Allergic inflammatory condition of the esophagus

Eosinophilic esophagitis (EoE) is an allergic inflammatory condition of the esophagus that involves eosinophils, a type of white blood cell. In healthy individuals, the esophagus is typically devoid of eosinophils. In EoE, eosinophils migrate to the esophagus in large numbers. When a trigger food is eaten, the eosinophils contribute to tissue damage and inflammation. Symptoms include swallowing difficulty, food impaction, vomiting, and heartburn.

Stretta is a minimally invasive endoscopic procedure for the treatment of gastroesophageal reflux disease (GERD) that delivers radiofrequency energy in the form of electromagnetic waves through electrodes at the end of a catheter to the lower esophageal sphincter (LES) and the gastric cardia – the region of the stomach just below the LES. The energy heats the tissue, ultimately causing it to swell and stiffen; the way this works was not understood as of 2015, but it was thought that perhaps the heat causes local inflammation, collagen deposition and muscular thickening of the LES and that it may disrupt the nerves there.

<span class="mw-page-title-main">Nutcracker esophagus</span> Medical condition

Nutcracker esophagus, jackhammer esophagus, or hypercontractile peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or duration. Nutcracker esophagus is one of several motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. It causes difficulty swallowing, or dysphagia, with both solid and liquid foods, and can cause significant chest pain; it may also be asymptomatic. Nutcracker esophagus can affect people of any age but is more common in the sixth and seventh decades of life.

<span class="mw-page-title-main">Esophageal food bolus obstruction</span> Medical condition

An esophageal food bolus obstruction is a medical emergency caused by the obstruction of the esophagus by an ingested foreign body.

<span class="mw-page-title-main">Herpes esophagitis</span> Medical condition

Herpes esophagitis is a viral infection of the esophagus caused by Herpes simplex virus (HSV).

<span class="mw-page-title-main">Esophageal intramural pseudodiverticulosis</span> Medical condition

Esophageal intramucosal pseudodiverticulosis (EIPD) is a rare condition wherein the wall of the esophagus develops numerous small outpouchings (pseudodiverticulae). Individuals with the condition typically develop difficulty swallowing. The outpouchings represent the ducts of submucosal glands of the esophagus. It typically affects individuals in their sixth and seventh decades of life. While it is associated with certain chronic conditions, particularly alcoholism, diabetes and gastroesophageal reflux disease, the cause of the condition is unknown. Treatment involves medications to treat concomitant conditions such as reflux and esophageal spasm, and dilation of strictures in the esophagus.

<span class="mw-page-title-main">Lymphocytic esophagitis</span> Medical condition

Lymphocytic esophagitis is a rare and poorly understood medical disorder involving inflammation in the esophagus. The disease is named from the primary inflammatory process, wherein lymphocytes are seen within the esophageal mucosa. Symptoms of the condition include difficulty swallowing, heartburn and food bolus obstruction. The condition was first described in 2006 by Rubio and colleagues. Initial reports questioned whether this was a true medical disorder, or whether the inflammation was secondary to another condition, such as gastroesophageal reflux disease.

Functional Lumen Imaging Probe (FLIP) is a test used to evaluate the function of the esophagus, by measuring the dimensions of the esophageal lumen using impedance planimetry. Typically performed with sedation during upper endoscopy, FLIP is used to evaluate for esophageal motility disorders, such as achalasia, diffuse esophageal spasm, etc.

References

  1. Julián Gómez L, Barrio J, Atienza R, et al. (November 2008). "[Acute esophageal necrosis. An underdiagnosed disease]". Rev Esp Enferm Dig (in Spanish). 100 (11): 701–5. doi: 10.4321/s1130-01082008001100006 . PMID   19159174.
  2. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Gurvits GE, Shapsis A, Lau N, Gualtieri N, Robilotti JG (January 2007). "Acute esophageal necrosis: a rare syndrome". J. Gastroenterol. 42 (1): 29–38. doi:10.1007/s00535-006-1974-z. PMID   17322991. S2CID   32862916.
  3. 1 2 3 4 5 6 7 8 9 10 Khan AM, Hundal R, Ramaswamy V, Korsten M, Dhuper S (August 2004). "Acute esophageal necrosis and liver pathology, a rare combination". World J. Gastroenterol. 10 (16): 2457–8. doi: 10.3748/wjg.v10.i16.2457 . PMC   4576312 . PMID   15285044.
  4. Watermeyer G, Shaw J, Krige J (2007). "Gastroentestinal: Acute necrotizing esophagitis". Journal of Gastroenterology and Hepatology. 22 (7): 1162. doi:10.1111/j.1440-1746.2007.05013.x. PMID   17608863. S2CID   71186463.
  5. 1 2 3 4 5 6 7 8 9 Trappe R, Pohl H, Forberger A, Schindler R, Reinke P (2007). "Acute esophageal necrosis (black esophagus) in the renal transplant recipient: manifestation of primary cytomegalovirus infection". Transplant Infectious Disease. 9 (1): 42–5. doi:10.1111/j.1399-3062.2006.00158.x. PMID   17313471. S2CID   1857761.
  6. 1 2 3 4 5 6 7 Carneiro M, Lescano M, Romanello L, et al. (2005). "Acute Esophageal Necrosis". Digestive Endoscopy. 17 (1): 89–92. doi:10.1111/j.1443-1661.2005.00464.x. S2CID   71488115.
  7. Grudell A, Mueller P, Viggiano T (2007). "Black esophagus: report of six cases and review of the literature, 1963–2003". Journal of Gastroenterology. 19 (2): 105–110. doi: 10.1111/j.1442-2050.2006.00549.x . PMID   16643179.
  8. Panesar, Divyansh; Schauer, Cameron; Raj, Ashok; Wilkins, James; Wagener, Jenny (July 2020). "Acute oesophageal necrosis following septic cellulitis". ANZ Journal of Surgery. 90 (7–8): 1491–1492. doi:10.1111/ans.15551. ISSN   1445-1433.
  9. 1 2 Dias, E; Santos-Antunes, J; Macedo, G (November 2019). "Diagnosis and management of acute esophageal necrosis". Annals of Gastroenterology. 32 (6): 529–540. doi: 10.20524/aog.2019.0418 . PMC   6826069 . PMID   31700229.
  10. Abdullah, Hafez Mohammad; Ullah, Waqas; Abdallah, Mohamed; Khan, Uzma; Hurairah, Abu; Atiq, Muslim (4 May 2019). "Clinical presentations, management, and outcomes of acute esophageal necrosis: a systemic review". Expert Review of Gastroenterology & Hepatology. 13 (5): 507–514. doi:10.1080/17474124.2019.1601555. PMID   30933549. S2CID   195661368.