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Eosinophilic oesophagitis, histologic features

Image: “Eosinophilic oesophagitis, histologic features” by Mattopaedia. License: CC BY-SA 3.0


Congenital Malformations of the Esophagus

Atresia of the esophagus with tracheoesophageal fistula

Esophageal atresia is a malformation of the esophagus. In the embryonic period, the esophagus develops from the foregut. At first, there is a connection between the airways and the gastrointestinal tract; however, this is sealed in the course of development by the tracheoesophageal septum, except for the proximal part.

Disorders in this development often lead to a blind-ended superior part of the esophagus and tracheoesophageal fistula in the inferior part.

Polyhydramnios is an intrauterine complication that can end in premature labor and premature birth. This condition occurs because the fetus swallows amniotic fluid that does not reach the gastrointestinal tract because of the malformation; therefore, the fluid cannot be absorbed, but rather it accumulates. Conspicuous symptoms in a newborn are frequent coughing attacks and salivation triggered by misleading food and rattling respiration with respiratory disorders, even including cyanosis.

The disease is often associated with other malformations, the so-called VACTERL association:

  • Vertebral malformations
  • Anal atresia
  • Cardiac malformations
  • TracheoEsophageal fistula
  • Renal malformations
  • Limb malformations

Functional Disorders of the Esophagus

Achalasia

With an incidence of 1 in 100,000 people, achalasia is a rather rare disease with an unknown cause. Even more rarely, it presents in the context of the autosomal recessive triple-A syndrome.

Achalasia is characterized by decreased peristalsis of the esophagus and insufficient relaxation of the inferior esophageal sphincter, which leads to the cardinal symptoms of dysphagia and retrosternal pain.

Especially when lying down, regurgitation of food, accompanied by bad breath, occurs. This can potentially lead to the complications of aspiration pneumonia and esophagitis. This inflammation of the esophagus, called retention esophagitis, increases the risk for carcinoma by 30 times as compared with the healthy population.

The cause of achalasia is the degeneration of the ganglion cells of the myenteric plexus in the inferior part of the esophagus; thus, it is neuromuscular. There is a primary form with an unclear genesis and a secondary form, which mostly develops in the context of a malignant disease via infiltration of the nerve structures.

Diagnosis of achalasia

  • Endoscopy with biopsy
  • Barium swallow typically shows so-called bird’s beak narrowing with prestenotic dilation (mega-esophagus).
  • Manometry (physical pressure measurement)
Megaesophagus with champagne-glass-shape

Image: Typical champagne glass shape during a contrast medium swallow test. By Farnoosh Farrokhi and Michael F. Vaezi. License: CC BY 2.0.

Treatment of achalasia

  • Balloon catheter dilation of the inferior esophageal sphincter
  • Reversible paresis of the inferior esophageal sphincter with an injection of botulinum toxin
  • Surgical cardiomyotomy with the possible complication of postsurgical reflux disease
Note: Because of an increased risk of carcinoma, regular endoscopic checks are necessary.

Esophageal spasm

Esophageal spasm is a rare, benign functional disorder of the esophagus. Retrosternal pain and difficulty swallowing occur in paroxysms and can lead to bolus impaction. In milder cases, glycerol trinitrate is administered. In severe cases, botulinum toxin is injected or surgical interventions are performed.

Hiatal Hernia

In a hiatal/diaphragmatic hernia, the area of the esophageal hiatus of the diaphragm is the hernial orifice through which parts of the stomach or the whole stomach enter the intrathoracic cavity in the peritoneal hernial sac.

Different forms of the hiatal hernia

Sliding (axial) hernias represent about 90% of the cases and significantly increase in frequency with age: 50% of all patients over 50 years old have a sliding hernia. The cardia and fundus of the stomach relocate into the thoracic space, manifesting clinically as reflux disease. In rare cases, bolus obstruction can occur if the superior edge of the hernia has narrowed to form a so-called Schatzki ring.

In a paraesophageal hernia, a part of the stomach—in the most severe case, even the whole stomach (upside-down stomach)—slides through the hernial orifice beside the esophagus. Paraesophageal hernias can be asymptomatic or show nonspecific symptoms, such as belching and pressure in the cardiac area. However, they always carry the risk of incarceration, erosions, ulcers, and bleeding, which is why they may be operated on in the symptom-free stage.

Mixed hernia is a combination of a sliding hernia and a paraesophageal hernia. It is rather rare, occurring in only 5% of cases.

Diagnosis of hiatal hernia

  • Barium swallow
  • Endoscopy
Hiatus Hernia

Image: Hiatal hernia in the lateral chest X-ray. The arrow shows the air–liquid level. By Hellerhoff. License: CC BY-SA 3.0.

Treatment of hiatal hernia

In patients with pre-existing reflux disease, sliding hernias are treated symptomatically. Because of the risk of incarceration, paraesophageal hernias are an indication for surgery. During surgery, the stomach is repositioned and fixed to the anterior abdominal wall, which is called transabdominal gastropexy.

Esophageal Diverticulum

Esophageal diverticula are outpouchings in the walls of the esophagus. Small diverticula often have no symptoms; larger ones most often do cause symptoms. Dysphagia, globus sensation, and nocturnal regurgitation of undigested food with the risk of aspiration can occur.

The deposition of food residue in these protrusions leads to malodorous fetor ex ore (bad breath). Diverticula can inflame and form fistulas.

Pulsion diverticula as esophageal diverticula

Pulsion diverticula are considered pseudodiverticula because only the mucosa and submucosa are pushed outward through the muscular layers. The most common sites are the following weak spots in the esophageal muscles:

  • epiphrenic diverticula

    Image: Pulsion diverticula of the middle esophagus. By Hellerhoff. License: CC BY-SA 3.0.

    The area of the Laimer’s triangle (an area free of longitudinal muscles at the superior esophagus)

  • Above the diaphragm, as epiphrenic diverticula; because these are often asymptomatic, they are most likely to be additional findings on X-ray examinations.
Note: Zenker’s diverticula in the area of Killian’s triangle (a weak area between the pharyngeal and esophageal muscles) are also pseudodiverticula; however, they occur at the hypopharynx.

Traction diverticula as esophageal diverticula

Traction diverticula are true diverticula because all wall layers are affected by the protrusion (e.g., parabronchial diverticula). They form because of the pulling forces from the outside due to inflammation and processes in the areas surrounding the esophagus.

Diagnosis of traction diverticula

  • Barium swallow
  • Endoscopy

Treatment of traction diverticula

Clinically relevant cases are treated surgically by diverticula resection.

Gastroesophageal Reflux Disease

Slight temporary reflux from the stomach into the esophagus is physiologic; however, increased gastroesophageal reflux of stomach contents can lead to gastroesophageal reflux disease (GERD). Most patients with this disease have a severely impaired quality of life. Two forms of GERD can be distinguished:

  1. In nonerosive reflux disease (NERD), the patient often has symptoms of reflux but endoscopy does not reveal any signs of esophagitis.
  2. In erosive reflux disease (ERD), the patient has reflux disease with esophagitis.

Concurrent esophageal lesions are present in 40% of patients with GERD; 5% of these patients develop epithelial dysplasia of the esophagus, which is called Barrett’s esophagus.

Barretts esophagus

The esophageal epithelium reacts to the chronic reflux of gastric acid by developing epithelial dysplasia. In this disorder, the stratified, noncornified epithelium is replaced by columnar epithelium. Epithelial dysplasia is considered a precancer because it makes development of adenocarcinomas more likely.

Micrograph of Barrett’s esophagus

Image: Micrograph of Barrett’s esophagus. By Nephron. License: CC BY-SA 3.0.

Causes of GERD

The most frequent cause of GERD is an insufficiency of the inferior esophageal sphincter. Additional promoting factors include the following:

  • Progressed pregnancy
  • Abdominal obesity
  • Previous surgical treatment of achalasia
  • Stenosis of the gastric exit
  • Scleroderma
  • Sliding (gliding) hernia

Symptoms of GERD

The most prominent symptom of GERD is pyrosis. Also, meteorism and flatulence, as well as eructation, may be observed as rather nonspecific symptoms. The increased reflux of the acid at night can lead to hoarseness in the morning. Reflux-induced vagal irritation leads to a dry, irritating cough.

Diagnosis of GERD

  • Endoscopy with biopsy for the assessment of the inflammatory stages
  • 24-hour pH meter via a nasal tube
  • Capsule-based pH meter

Treatment of GERD

Frequent symptoms and esophagitis make medication necessary, with proton-pump inhibitors (PPIs) such as omeprazole or pantoprazole being the first-line treatment because the mucosa can regenerate when acid is suppressed.

H2-receptor antagonists and antacids are recommended only in cases with mild symptoms and no inflammatory signs. Generally, any treatment of reflux disease should be enhanced by the adhering to the following measures:

  • Avoiding consumption of acidic and alcoholic beverages such as coffee, juice, and wine
  • Abstaining from nicotine use
  • Not eating late meals
  • Avoiding fatty and very sugary food
  • Sleeping with the head elevated
  • Avoiding stress

Laparoscopic Nissen fundoplication, in which a cuff is positioned around the inferior esophageal sphincter, is indicated only if conservative measures have not been successful.

Esophagitis

Symptoms of esophagitis

Symptoms include dysphagia, odynophagia, and retrosternal pain.

Infectious esophagitis

The severe general disease can be accompanied by esophagitis. For example, infection with Candida albicans can lead to thrush esophagitis, which presents the typical endoscopic picture of numerous white plaques that cannot be wiped away. In immunosuppressed patients, acquired immunodeficiency syndrome (AIDS), and patients with tumors, herpesviruses (herpes simplex virus (HSV) and cytomegalovirus (CMV)) can also trigger esophagitis.

Eosinophilic esophagitis

As part of allergic diseases, this form of esophagitis can be found frequently in children. Endoscopically, eosinophilic infiltration of the esophagus can be seen as whitish papules. Treatment consists of corticosteroid administration because in most cases, eosinophilic esophagitis does not respond to PPIs.

Eosinophilic oesophagitis, histologic features

Image: Eosinophilic esophagitis, histologic features. By Mattopaedia. License: CC BY-SA 3.0.

AIDS and esophageal diseases

In the context of fully developed AIDS, numerous esophageal diseases can develop, such as:

  • Thrush esophagitis
  • Ulcer formation in cases of infections with HSV or CMV
  • Stenosis of the esophagus with bleeding and perforations caused by Kaposi’s carcinoma

Other causes of esophagitis

  • Chemical causes: chemical burns, reflux of gastric acid (reflux esophagitis), alcohol abuse, medication
  • Physical causes: consequences of radiation therapy, gastric tube
  • Stenosis: retention esophagitis in cases of achalasia, tumorous processes

Treatment of esophagitis

Depending on the underlying disease, the triggering factors will be treated. Therefore, PPIs are used to treat reflux esophagitis, antimycotic agents are given for thrush esophagitis, and antiviral medications are prescribed for HSV- and CMV-associated inflammation.

Tumors of the Esophagus

Benign esophageal tumors

Benign tumors of the esophagus are rather rare and are frequently free from symptoms. They can grow intramurally or intraluminally. They are diagnosed by using a barium swallow examination, endoscopy, or endosonography. Smaller intraluminal tumors can usually be eliminated endoscopically with the diathermic sling; larger ones are surgically excised.

Esophageal carcinoma

The malignant neoplasms of the esophagus are squamous epithelial carcinomas or adenocarcinomas originating from the epithelium. Esophageal carcinoma is relatively rare in Western industrialized countries, making up only 2% of all cancers. Men are more frequently affected than women, and it is most frequently diagnosed in the sixth and seventh decades of life. Adenocarcinoma of the esophagus develops from Barrett’s esophagus after chronic esophagitis.

Triggers of squamous epithelial carcinomas include nitrosamines, aflatoxins, long-term consumption of highly concentrated alcohol and very hot food and beverages, and smoking. Furthermore, chemical burn scars, achalasia, and Plummer–Vinson syndrome can promote the formation of esophageal carcinoma.

Symptoms of esophageal carcinoma

Esophageal tumors are often found rather late because they manifest with relatively nonspecific symptoms, such as:

  • Retrosternal pain, back pain
  • Dysphagia
  • Regurgitation
  • Singultus (hiccups) in cases of infiltration of the vagus nerve
  • Hoarseness in cases of infiltration of the recurrent laryngeal nerve
  • Irritating cough and other pulmonary symptoms
  • Hematemesis

Accompanying symptoms are weight loss, night sweats, and decreased stress resistance.

Pathology of esophageal carcinoma

Esophageal carcinomas tend to form in the three physiologically narrow areas of the esophagus. Both squamous cell carcinomas and adenocarcinomas extend into the lumen, infiltrate surrounding structures, and metastasize early.

Endoscopic image of patient with esophageal adenocarcinoma seen at gastro-esophageal junction

Image: Endoscopic image of a patient with an esophageal adenocarcinoma at the gastroesophageal junction. By Samir. License: CC BY-SA 3.0.

Staging of esophageal carcinoma

Tumor stages are classified according to the tumor–node–metastasis (TNM) classification. At the time of diagnosis, these tumors are mostly already in stage 3 or 4 because they remain symptom-free for a long time.

Diagnosis of esophageal carcinoma

  • Endoscopic and histologic examination of the biopsy material
  • Endosonography for the assessment of the T and N stages
  • Sonography of the abdomen
  • Chest X-ray
  • Bronchoscopy
  • Computed tomography (CT)

Treatment of esophageal carcinoma

The choice of treatment depends on the stage of the disease. The following are common treatments:

  • Endoscopic abrasion of early adenocarcinomas with a high healing rate
  • Esophageal resection with stomach elevation and radiotherapy and/or chemotherapy
  • Radiotherapy and/or chemotherapy are used if surgery is no longer feasible.

Palliative therapy for the maintenance of food passage includes the implantation of a metal stent for opening the esophageal lumen and laser coagulation.

Overall, the prognosis is rather bad because the first noticeable symptom of the disease, dysphagia, is already a late symptom. At the time of diagnosis, for 95% of the tumors, palliative treatment is the only therapeutic option left.

Emergencies

Rupture of the esophageal varices

Portal hypertension with different types of genesis causes the formation of portacaval anastomoses and can thus lead to the development of esophageal varices. These varices can rupture, leading very quickly to a life-threatening state due to the fulminant bleeding. Therapeutically, the following interventions are available:

  • Stabilize circulation (peripheral access, erythrocyte concentrations).
  • Possibly place a tracheal tube.
  • Administer terlipressin, a vasopressin analog.
  • Administer octreotide, a somatostatin analog.
  • Use antibiotic prophylaxis.
  • Place elastic ligatures or use sclerotherapy or obliteration of the varices.

In 10% of the cases, the bleeding persists and makes further measures necessary. Also, the risk for recurrences is relatively high, at 80%.

Chemical burns of the esophagus with acids and bases

Contact of the mucosa with acids and bases leads to deep necroses, from which inflammation can develop reactively; this can possibly cause shock in the patient. Thus, sufficient volume must be substituted. Also, the esophagus can perforate, which carries the risk of mediastinitis. Alkaline and acidic chemical burns can be differentiated with the help of litmus strips.

Contraindication: Vomiting must not be induced.

Chemical burns are classified according to the degree of severity. Third-degree burns make resection of parts of the esophagus necessary because if all wall layers are destroyed, there is a risk of perforation, with all of its sequelae.

The severity of chemical burns in classified into four stages:

  • Degree 1: reddening and formation of edema
  • Degree 2: mucous ulcers with fibrin plaques
  • Degree 3: deep necroses
  • Degree 4: perforation

Boerhaave’s syndrome

Boerhaave’s syndrome refers to the spontaneous rupture of the esophagus after an acute strain because of pressure (severe vomiting or choking). It involves the possible complication of a mediastinitis with generalized sepsis. Treatment consists of endoscopic fibrin sealing or temporary endoprosthetic treatment of the defect, abrosia, and the administration of antibiotics.

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