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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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The classic history of esophageal rupture is one of severe retching and vomiting followed by excruciating retrosternal chest and upper abdominal pain. Odynophagia, tachypnea, dyspnea, cyanosis, fever, and shock develop rapidly thereafter.
Physical examination is usually not helpful, particularly early in the course. Subcutaneous emphysema (crepitation) is an important diagnostic finding but is not very sensitive, being present in only 9 of 34 patients (27 percent) in one series. A pleural effusion may be detected.
Mackler's triad includes chest pain, vomiting, and subcutaneous emphysema, and while it is a classical presentation, it is only present in 14% of people.
Pain can occasionally radiate to the left shoulder, causing physicians to confuse an esophageal perforation with a myocardial infarction.
It may also be audibly recognized as Hamman's sign.
Common misdiagnoses include myocardial infarction, pancreatitis, lung abscess, pericarditis, and spontaneous pneumothorax. If esophageal perforation is suspected, even in the absence of physical findings,chest xray, water soluble contrast radiographic studies of the esophagus and a CT scan should be promptly obtained. In most cases, non-operative management is administered based on radiological evidence contained in mediastinal collection.
A varix (pl. varices) is an abnormally dilated vessel with a tortuous course. Varices usually occur in the venous system, but may also occur in arterial or lymphatic vessels.
Examples of varices include:
- Varicose veins, large tortuous veins usually found on legs
- Sublingual varices
- Esophageal varices, commonly stemming from cirrhosis of the liver, also known as oesophageal varicose
- Gastric varices, commonly stemming from cirrhosis of the liver
- Intestinal varices
- Scrotal varices
- Vulvar varices
- Pelvic varices
- Vesical varices, varicose veins associated with the urinary bladder
- Rectal varices, which can be similar to external haemorrhoids
The following are risk factors that can result in a hiatus hernia.
- Increased pressure within the abdomen caused by:
- Heavy lifting or bending over
- Frequent or hard coughing
- Hard sneezing
- Violent vomiting
- Straining
- Stress
Hiatal hernia has often been called the "great mimic" because its symptoms can resemble many disorders. Among them, a person with a hiatal hernia can experience dull pains in the chest, shortness of breath (caused by the hernia's effect on the diaphragm), heart palpitations (due to irritation of the vagus nerve), and swallowed food "balling up" and causing discomfort in the lower esophagus until it passes on to the stomach. In addition, hiatal hernias often result in heartburn but may also cause chest pain or pain with eating.
In most cases however, a hiatal hernia does not cause any symptoms. The pain and discomfort that a patient experiences is due to the reflux of gastric acid, air, or bile. While there are several causes of acid reflux, it occurs more frequently in the presence of hiatal hernia.
In newborns, the presence of Bochdalek hernia can be recognised from symptoms such as difficulty breathing fast respiration, increased heart rate.
Esophageal stricture, or narrowing of the esophagus, is usually a complication of acid reflux, most commonly due to gastroesophageal reflux (GERD). These patients are usually older and have had GERD for a long time. Esophageal stricture can also be due to other causes, such as acid reflux from Zollinger-Ellison syndrome, trauma from a nasogastric tube placement, and chronic acid exposure in patients with poor esophageal motility from scleroderma. Other non-acid related causes of peptic strictures include infectious esophagitis, ingestion of chemical irritant, pill irritation, and radiation. Peptic stricture is a progressive mechanical dysphagia, meaning patients will complain of initial intolerance to solids followed by inability to tolerate liquids. When the diameter of the stricture is less than 12 mm the patient will always have dysphagia, while dysphagia is not seen when the diameter of the stricture is above 30 mm. Symptoms relating to the underlying cause of the stricture usually will also be present.
Esophageal cancer also presents with progressive mechanical dysphagia. Patients usually come with
rapidly progressive dysphagia first with solids then with liquids, weight loss (> 10 kg), and anorexia (loss of appetite). Esophageal cancer usually affects the elderly. Esophageal cancers can be either squamous cell carcinoma or adenocarcinoma. Adenocarcinoma is the most prevalent in the US and is associated with patients with chronic GERD who have developed Barrett's esophagus (intestinal metaplasia of esophageal mucosa). Squamous cell carcinoma is more prevalent in Asia and is associated with tobacco smoking and alcohol use.
Esophageal rings and webs, are actual rings and webs of tissue that may occlude the esophageal lumen.
- "Rings" --- Also known as Schatzki rings from the discoverer, these rings are usually mucosal rings rather than muscular rings, and are located near the gastroesophageal junction at the squamo-columnar junction. Presence of multiple rings may suggest eosinophilic esophagitis. Rings cause intermittent mechanical dysphagia, meaning patients will usually present with transient discomfort and regurgitation while swallowing solids and then liquids, depending on the constriction of the ring.
- "Webs" --- Usually squamous mucosal protrusion into the esophageal lumen, especially anterior cervical esophagus behind the cricoid area. Patients are usually asymptomatic or have intermittent dysphagia. An important association of esophageal webs is to the Plummer-Vinson syndrome in iron deficiency, in which case patients will also have anemia, koilonychia, fatigue, and other symptoms of anemia.
Achalasia is an idiopathic motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation as well as loss of peristalsis in the distal esophagus, which is mostly smooth muscle. Both of these features impair the ability of the esophagus to empty contents into the stomach. Patients usually complain of dysphagia to both solids and liquids. Dysphagia to liquids, in particular, is a characteristic of achalasia. Other symptoms of achalasia include regurgitation, night coughing, chest pain, weight loss, and heartburn. The combination of achalasia, adrenal insufficiency, and alacrima (lack of tear production) in children is known as the triple A (Allgrove) syndrome. In most cases the cause is unknown (idiopathic), but in some regions of the world, achalasia can also be caused by Chagas disease due to infection by "Trypanosoma cruzi".
Scleroderma is a disease characterized by atrophy and sclerosis of the gut wall, most commonly of the distal esophagus (~90%). Consequently, the lower esophageal sphincter cannot close and this can lead to severe gastroesophageal reflux disease (GERD). Patients typically present with progressive dysphagia to both solids and liquids secondary to motility problems or peptic stricture from acid reflux.
Spastic motility disorders include diffuse esophageal spasm (DES), nutcracker esophagus, hypertensive lower esophageal sphincter, and nonspecific spastic esophageal motility disorders (NEMD).
- "DES" can be caused by many factors that affect muscular or neural functions, including acid reflux, stress, hot or cold food, or carbonated drinks. Patients present with intermittent dysphagia, chest pain, or heartburn.
Rare causes of esophageal dysphagia not mentioned above
- Diverticulum
- Aberrant subclavian artery, or (dysphagia lusoria)
- Cervical osteophytes
- Enlarged aorta
- Enlarged left atrium
- Mediastinal tumor
Esophageal varices (sometimes spelled oesophageal varices) are extremely dilated sub-mucosal veins in the lower third of the esophagus. They are most often a consequence of portal hypertension, commonly due to cirrhosis; patients with esophageal varices have a strong tendency to develop bleeding. Esophageal varices are typically diagnosed through an esophagogastroduodenoscopy.
Its main symptoms are pain and difficulty in swallowing (dysphagia).
Esophageal webs are thin (2-3mm) membranes of normal esophageal tissue consisting of mucosa and submucosa that can partially protrude/obstruct the esophagus. They can be congenital or acquired. Congenital webs commonly appear in the middle and inferior third of the esophagus, and they are more likely to be circumferential with a central or eccentric orifice. Acquired webs are much more common than congenital webs and typically appear in the cervical area (postcricoid).
Clinical symptoms of this condition are selective (solid more than liquids) dysphagia, thoracic pain, nasopharyngeal reflux, aspiration, perforation and food impaction (the last two are very rare).
Patients usually complain of dysphagia (the feeling of food getting stuck "several seconds" after swallowing), and will point to the suprasternal notch or behind the sternum as the site of obstruction.
Nutcracker esophagus is characterized as a motility disorder of the esophagus, meaning that it is caused by abnormal movement, or peristalsis of the esophagus. Patients with motility disorders present with two key symptoms: either with chest pain (typically reported as non-cardiac chest pain as it is esophageal in origin), which is usually found in disorders of spasm, or with dysphagia (difficulty with swallowing). Nutcracker esophagus can present with either of these, but chest pain is the more common presentation. The chest pain is very severe and intense, and mimics cardiac chest pain. It may spread into the arm and back. The symptoms of nutcracker esophagus are intermittent, and may occur with or without food. Rarely, patients can present with a sudden obstruction of the esophagus after eating food (termed a food bolus obstruction, or the 'steakhouse syndrome') requiring urgent treatment. The disorder does not progress to produce worsening symptoms or complications, unlike other motility disorders (such as achalasia) or anatomical abnormalities of the esophagus (such as peptic strictures or esophageal cancer). Many patients with nutcracker esophagus do not have any symptoms at all, as esophageal manometry studies done on patients without symptoms may show the same motility findings as nutcracker esophagus. Nutcracker esophagus may also be associated with metabolic syndrome. The incidence of nutcracker esophagus in all patients is uncertain.
Symptoms of esophageal strictures include heartburn, bitter or acid taste in the mouth, choking, coughing, shortness of breath, frequent burping or hiccups, pain or trouble swallowing, throwing up blood, or weight loss.
In simple words, when there is excessive pressure within the lower pharynx, the weakest portion of the pharyngeal wall balloons out, forming a diverticulum which may reach several centimetres in diameter.
More precisely, while traction and pulsion mechanisms have long been deemed the main factors promoting development of a Zenker's diverticulum, current consensus considers occlusive mechanisms to be most important: uncoordinated swallowing, impaired relaxation and spasm of the cricopharyngeus muscle lead to an increase in pressure within the distal pharynx, so that its wall herniates through the point of least resistance (known as Killian's triangle, located superior to the cricopharyngeus muscle and inferior to the Thyropharyngeus muscle. Thyropharyngeus and Laryngopharyngeus are the superior and inferior parts of inferior constrictor muscle of pharynx respectively). The result is an outpouching of the posterior pharyngeal wall, just above the esophagus.
While it may be asymptomatic, Zenker diverticulum can present with the following symptoms:
- Dysphagia (difficulty swallowing), and sense of a lump in the throat
- Food might get trapped in the outpouching, leading to:
- Regurgitation, reappearance of ingested food in the mouth
- Cough, due to food regurgitated into the airway
- Halitosis, smelly breath, as stagnant food is digested by microorganisms
- Infection
It rarely, if ever, causes any pain.
Cervical webs are seen associated in 50% of patients with this condition.
Rarer forms of cervical esophageal diverticula are the Killian's diverticulum and the Laimer's diverticulum. Killian's diverticulum is formed in the Killian-Jamiseon triangle (located inferior to the cricopharyngeus on both sides of this muscle's insertion into the cricoid cartilage). Laimer's diverticulum is formed in Laimer's triangle (located inferior to the cricopharyngeus in the posterior midline above the confluence of the longitudinal layer of esophageal muscle). Laimer's triangle is covered only by the circular layer of esophageal muscle.
It can be caused by or associated with gastroesophageal reflux disease, esophagitis, a dysfunctional lower esophageal sphincter, disordered motility, lye ingestion, or a hiatal hernia. Strictures can form after esophageal surgery and other treatments such as laser therapy or photodynamic therapy. While the area heals, a scar forms, causing the tissue to pull and tighten, leading to difficulty in swallowing.
Not all patients with Schatzki rings have symptoms; barium swallow tests of the esophagus sometimes show Schatzki rings in patients with no swallowing difficulties.
When Schatzki rings cause symptoms, they usually result in episodic difficulties with swallowing (dysphagia) solid foods, or a sensation that the food "sticks" while swallowing, especially if the food is not chewed thoroughly. Patients usually are able to regurgitate or force through the food material and resume eating. However, complete obstruction of the esophagus by a bolus of food (often called steakhouse syndrome) can occur. This can cause crushing chest pain and may need immediate treatment with endoscopy, which is the use of a specialized fibre-optic camera in order to remove the lodged food. After the obstruction is located, snares or forceps are inserted to pull the food out of the esophagus or to push it into the stomach. The latter is done with caution, usually when the anatomy of the structures around the obstruction is already known.
A Zenker's diverticulum, also pharyngoesophageal diverticulum, also pharyngeal pouch, also hypopharyngeal diverticulum, is a diverticulum of the mucosa of the pharynx, just above the cricopharyngeal muscle (i.e. above the upper sphincter of the esophagus). It is a pseudo diverticulum (not involving all layers of the esophageal wall).
It was named in 1877 by German pathologist Friedrich Albert von Zenker.
Signs and symptoms may include a sudden pain in the epigastrium to the right of the midline indicating the perforation of a duodenal ulcer. In a gastric ulcer perforation creates a history of burning pain in epigastrium, with flatulence and dyspepsia.
In intestinal perforation, pain starts from the site of perforation and spreads across the abdomen.
Gastrointestinal perforation results in severe abdominal pain intensified by movement, nausea, vomiting and hematemesis. Later symptoms include fever and or chills. In any case, the abdomen becomes rigid with tenderness and rebound tenderness. After some time the abdomen becomes silent and heart sounds can be heard all over. Patient stops passing flatus and motion, abdomen is distended.
The symptoms of esophageal rupture may include sudden onset of chest pain.
Esophageal spasm is rare. Often, symptoms that may suggest esophageal spasm are the result of another condition such as gastroesophageal reflux disease (GERD) or achalasia. The symptoms can also include dysphagia, regurgitation, noncardiac chest pain, heartburn, globus pharyngis (which is a feeling that something is stuck in the throat) or a dry cough.
Esophageal diseases can derive from congenital conditions, or they can be acquired later in life.
Many people experience a burning sensation in their chest occasionally, caused by stomach acids refluxing into the esophagus, normally called heartburn. Extended exposure to heartburn may erode the lining of the esophagus, leading potentially to Barrett's esophagus which is associated with an increased risk of adenocarcinoma most commonly found in the distal one-third of the esophagus.
Some people also experience a sensation known as globus esophagus, where it feels as if a ball is lodged in the lower part of the esophagus.
The following are additional diseases and conditions that affect the esophagus:
- Achalasia
- Acute esophageal necrosis
- Barrett's esophagus
- Boerhaave syndrome
- Caustic injury to the esophagus
- Chagas disease
- Diffuse esophageal spasm
- Esophageal atresia and Tracheoesophageal fistula
- Esophageal cancer
- Esophageal dysphagia
- Esophageal varices
- Esophageal web
- Esophagitis
- GERD
- Hiatus hernia
- Jackhammer esophagus (hypercontractile peristalsis)
- Killian–Jamieson diverticulum
- Mallory-Weiss syndrome
- Neurogenic dysphagia
- Nutcracker esophagus
- Schatzki's ring
- Zenker's Diverticulum
Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with choking, coughing, vomiting, and cyanosis coincident with the onset of feeding. Esophageal atresia and the subsequent inability to swallow typically cause polyhydramnios in utero. Rarely it may present in an adult.
A Schatzki ring or Schatzki–Gary ring is a narrowing of the lower esophagus that can cause difficulty swallowing (dysphagia). The narrowing is caused by a ring of mucosal tissue (which lines the esophagus) or muscular tissue. A Schatzki ring is a specific type of "esophageal ring", and Schatzki rings are further subdivided into those above the esophagus/stomach junction (A rings), and those found at the squamocolumnar junction in the lower esophagus (B rings).
Patients with Schatzki rings can develop intermittent difficulty swallowing or, more seriously, a completely blocked esophagus. The ring is named after the German-American physician Richard Schatzki.
Dilated submucosal veins are the most prominent histologic feature of esophageal varices. The expansion of the submucosa leads to elevation of the mucosa above the surrounding tissue, which is apparent during endoscopy and is a key diagnostic feature. Evidence of recent variceal hemorrhage includes necrosis and ulceration of the mucosa. Evidence of past variceal hemorrhage includes inflammation and venous thrombosis.
Many foods can lodge themselves in the esophagus, but the most common are meats such as steak, poultry, or pork leading to the colourful description of the phenomenon as steakhouse syndrome. People with food bolus obstruction typically display acute dysphagia (difficulty swallowing), often to the point that they cannot even swallow their saliva, leading to drooling. They may also suffer from chest pain, neck pain, regurgitation of food, or painful swallowing (odynophagia).
Patients with esophageal food boluses are also at risk of complications, such as perforation of the esophagus, and aspiration into the lungs. As a result, urgent treatment of patients with high-risk features, or a lengthy duration of symptoms, is recommended.
The main symptoms of achalasia are dysphagia (difficulty in swallowing), regurgitation of undigested food, chest pain behind the sternum, and weight loss. Dysphagia tends to become progressively worse over time and to involve both fluids and solids. Some people may also experience coughing when lying in a horizontal position.
The chest pain experienced, also known as cardiospasm and non-cardiac chest pain can often be mistaken for a heart attack. It can be extremely painful in some sufferers. Food and liquid, including saliva, are retained in the esophagus and may be inhaled into the lungs (aspiration).
Surgical repair can sometimes result in complications, including:
- Stricture, due to gastric acid erosion of the shortened esophagus
- Leak of contents at the point of anastomosis
- Recurrence of fistula
- Gastro-esophageal reflux disease
- Dysphagia
- Asthma-like symptoms, such as persistent coughing/wheezing
- Recurrent chest infections
- Tracheomalacia
A Killian–Jamieson diverticulum is an outpouching of the esophagus just below the upper esophageal sphincter.
The physicians that first discovered the diverticulum were Gustav Killian and James Jamieson. Diverticula are seldom larger than 1.5 cm, and are less frequent than the similar Zenker's diverticula. As opposed to a Zenker's, which is typically a posterior and inferior outpouching from the esophagus, a Killian–Jamieson diverticulum is typically an anterolateral outpouching at the level of the C5-C6 vertebral bodies, due to a congenital weakness in the cervical esophagus just below the cricopharyngeal muscle. It is usually smaller in size than a Zenker's diverticulum, and typically asymptomatic. Although congenital, it is more commonly seen in elderly patients.