The Esophagus

Historical Aspects

The earliest esophageal procedures were limited to the cervical region (removal of foreign bodies-1863)Modified ureteroscope used to diagnose carcinoma of the thoracic esophagus-1868Esophagoscopy with distal light source developed around 1900Flexible fiber-optic esophagoscopy-1964

AnatomyA hollow muscular tube approximately 25 cm in length divided into four segments

Pharyngoesophageal, Cervical, Thoracic and Abdominal

The cervical esophagus is a midline structure positioned posterior and slightly to the left of the tracheaThe thoracic esophagus passes into the posterior mediastinum continuing on the left side of the mainstem bronchus and eventually enters the abdomen through the crus in the diaphragmThe abdominal esophagus attaches to the cardia (or EG junction) of the stomach (is of variable length)

Anatomy (Continued)

The esophagus has three distinct areas of naturally occurring anatomic narrowing

Cervical constriction

Bronchoaortic constriction

Diaphragmatic constriction

Anatomy (Continued)A mucosal-lined muscular tube that lacks a serosaIt is surrounded by adventitaThe adventita surrounds a coat of longitudinal muscle that overlies a inner layer of circular muscleBetween the two muscular layers is a thin intramuscular layer of fine blood vessels and ganglion cellsThe upper (two-thirds) layer of muscle is striated and lower is notThe esophageal mucosa consists of squamous epithelium except for the distal 1-2 cm

Anatomy (Continued)

The esophagus has both sympathetic and parasympathetic innervation

The esophagus has an extensive lymphatic drainage that consists of two lymphatic plexuses

The esophagus has segmental blood supply and is nourished by a number of arteries

Physiology

Its basic function is to transport swallowed material from the pharynx into the stomach

Retrograde flow of gastric contents into the esophagus is prevented by the lower esophageal sphincter (LES)

Entry of air into the esophagus is prevented by the upper esophageal sphincter (UES)

Physiology (Continued)Esophageal contractions-three types:

Primary peristalsisSecondary peristalsisTertiary contractions

Esophageal peristaltic pressures range from 20-100 mm Hg with a duration of contraction between 2-4 secondsLES-no anatomic sphincter has ever been demonstrated (resting pressures are elevated in this area)

Disorders of Esophageal Motility

Are classified as functional disorders because they interfere with a normal act of swallowing or produce dysphagia without any associated organic obstruction or extrinsic compression

Information from esophageal manometry is extremely helpful

Some conditions are indistinguishable by x-rays (barium) but have specific manometric characteristics

Disorders of Esophageal Motility

As a basic rule the tests below constitute the basic evaluation of a patient with suspected disorders of esophageal motility:

Barium swallow

Esophagoscopy

Esophageal manometry

Esophageal pH reflux testing

Disorders of Esophageal Motility

Upper esophageal sphincter dysfunctionVarious (old) terms have been used:

• Achalasia

• Spasm

• Cricopharyngeal chalasia

The terms oropharyngeal dysphagia or cricopharyngeal dysfunction better described the symptoms that occur when there’s difficulty propelling liquid or solid food from the oropharynx into the upper esophagus

Causes of Oropharyngeal Dysphagia

Neurogenic

Myogenic

Structural causes

Mechanical causes

Iatrogenic causes

Gastroesophageal reflux

Clinical Presentation

The patient complains of cervical dysphagia which is localized between the thyroid cartilage and the suprasternal notch (the classical “lump in the throat”)

Expectoration of excessive saliva is common

Intermittent hoarseness can occur

Weight loss secondary to impaired caloric intake may occur

Diagnostic Tests and Treatment

Barium swallow may be normal especially in patients with intermittent symptoms

Esophageal function studies (manometric and acid reflux testing) should be performed whenever possible

In patients with severe symptoms and no reflux, surgical intervention may be necessary

Esophagomyotomy

Motor Disorders of the Body of the Esophagus

Esophageal motor disorders range from hypomotility (achalasia) to hypermotility (diffuse spasm)

Achalasia is defined as a failure or lack of relaxation

The name focuses on the distal sphincter however the condition involves the entire esophageal body

Diffused esophageal spasm is poorly understood and poorly treated

AchalasiaThe etiology is not knownThe characteristic clinical, radiographic and manometric findings have occurred following a variety of situations:

Severe emotional stressMajor physical traumaChagas’ disease

Various animal model suggests a central or peripheral vagal nerve dysfunction resulting in the development of achalasiaThe classic triad of presenting symptoms include dysphagia, regurgitation and weight loss

Achalasia (Continued)Retrosternal pain on swallowing (odynophagia) is not characteristic Effortless regurgitation after eating especially upon bending forward is usually not associated with a sour taste of undigested food-in contrast to acid regurgitationOften results in recurrent respiratory symptoms due to aspiration pneumonitisIs a premalignant esophageal lesion with carcinoma developing as a late complication in patients who have this condition an average of 15-25 years

Radiographic Appearance of Achalasia

Varies with the extent of the diseaseMild dilatation and early stages progressing to massive dilatation and tortuosity and later stagesPeristalsis is disordered in early stages and lacking in later stagesThe radiographic hallmark is the distal bird beak taper of the (EG) junction

Testing

Manometric criteria of achalasia are failure of the LES to relax with swallowing and a lack of progressive peristalsis throughout the length of the esophagus

Esophagoscopy is indicated an achalasia to rule out severe retention esophagitis, carcinoma or tumor of the cardia (stomach) that mimics achalasia

Treatment

Incurable

Palliative measuresNonsurgical

Surgical

Both are directed toward relieving the obstruction caused by the nonrelaxing LES

Nonsurgical Treatment

Early stagesSublingual nitroglycerin

Long-acting nitrates

Calcium channel blockers

Passage of Mercury weighted bougies

Surgical Treatment

Forceful dilatation (balloon)

Esophagomyotomy

Diffuse Esophageal Spasm (DES)Is poorly understood hypermotility disorder

Results from repetitive high amplitude esophageal contractions

The etiology is unknownThese patients typically are anxious and complain of chest pain inconsistent to eating, exertion and positionThe character of pain may mimic that of anginaSymptoms are greatest during periods of emotional stressPatients may experience slow emptying of the esophagus and obstructive symptoms are uncommon

Radiographic Findings

Frustratingly variableClassic “corkscrew”

Beaklike taper

Increase in esophageal wall thickness

Testing

EsophagoscopyDistal esophageal obstructing lesions may produce proximal esophageal contractions that are confused with DES

Esophageal manometryDiagnostic when present

Classic criteria are:• Simultaneous, multiphasic, repetitive, high

amplitude contractions that occur after a swallow

Treatment

Due to the lack of understanding of this condition the treatment is less than satisfactory

Antispasmodics are occasionally helpful

Response to sublingual nitroglycerin is variable

SclerodermaEsophageal motor disturbances occur in several of the collagen vascular diseases

DermatomyositisPolymyositisLupus erythematosusScleroderma (extremely common)

Etiology is unknownCharacterized by induration of skin, fibrous replacement of smooth muscle of internal organs and progressive loss of visceral and cutaneous functionDisruption of esophageal peristalsis is common

Testing

Esophageal manometry and intraesophageal pH readings are the most sensitive means of detection

Treatment

Standard antireflux medicine includes H-2 blockers

Cimetidine

Ranitidine

In patients with intractable symptoms gastroesophageal reflux surgery should be considered

Diverticula of the Esophagus

Esophageal DiverticulaAlmost all are acquired and occur predominantly in adulthoodAre classified according to their:

Site of occurrence• Pharyngoesophageal• Parabronchial• Epiphrenic

Wall thickness• True• False

Mechanism of formation• Pulsion• Traction

Pharyngoesophageal Diverticula (Zenker)

The most common esophageal diverticulumOccurs between the ages of 30-50 (believed to be acquired)Arises within the inferior pharyngeal constrictor, between the oblique fibers of the thyropharyngeus muscle and the cricopharyngeus muscleIs a pulsion diverticulumComplaints are of cervical dysplasia, effortless regurgitation of food or pills sometimes consumed hours earlierSometimes a gurgling sensation in the neck after swallowing is felt

Diagnosis and Treatment

Barium swallow establishes the diagnosis

Surgery is indicated in symptomatic patients regardless of the size

It is the degree of cricopharyngeal muscle dysfunction and not the size of the diverticulum that determines the relative severity of cervical dysphagia

Midesophageal (Traction) Diverticula

Are typically associated with mediastinal granulomatous disease (TB, histoplasmosis)They are usually small with a blunt tapered tip that points upwardThese are usually an incidental finding on barium swallowThey rarely cause symptoms or require treatmentNeed to be differentiated from pulsion diverticula which can also occur in this location (associated with neuromotor esophageal dysfunction)

Epiphrenic (Supradiaphragmatic) Diverticula

Generally occur within the distal 10cm of the thoracic esophagusThese are pulsion diverticula that arise due to esophageal motor dysfunction or mechanical distal obstructionMany patients are asymptomatic when diagnosedWhen symptomatic their symptoms are difficult to differentiate from: hiatal hernia, DES, achalasia, reflux esophagitis and carcinomaDysphagia and regurgitation are common symptoms

Diagnosis and Treatment

Diagnosis is easily made with barium swallowEsophageal function studies should also be performed to rule out any motor disturbancesLesions < 3 cm often require no treatmentExtreme symptomatic patients sometimes require surgical repair

Miscellaneous Condition of the Esophagus

Mallory-Weiss syndromeDuring the act of forceful emesis against a closed glottis increased intra-abdominal pressure can cause a tear in the mucosa (Mallory-Weiss tear) of the esophagus at the esophagogastric junction

A transmural esophageal tear is called Boerhaave’s syndrome

A history of emesis followed by melena or hematemesis is suggestive for a Mallory-Weiss tear

Esophagoscopy

Indications and ContraindicationsIndications include:

DysphagiaRefluxHematemesisAtypical chest painMany other conditions

Contraindications:To assess reflux symptoms that respond to medical managementA uncomplicated sliding hiatal hernia

General Considerations

The esophagoscopy should be performed after barium swallow

Bacteremia during upper GI endoscopy has been well documented therefore prophylactic antibiotic treatment should be administered

Patient should be in NPO for 6-8 hours

ComplicationsThe minor ones:

Lacerations of the lips or tongueDislodgment or fracture of teeth and possible aspiration

Major complicationEsophageal perforation

• Cervical esophagus (40%)• Mid esophagus (25%)• Distal esophagus (35%)

Morbidity and mortality from perforation is directly related to the time interval between the occurrence of injury, diagnosis and repair

Tumors of the Esophagus

Benign Esophageal Tumors and Cysts

Benign tumors are rare (< 1 %)Classified in two groups

MucosalExtramucosal (intramural)

More useful classification:60% of benign neoplasms are leiomyomas20% are cysts5% are polypsOthers (< 2 percent)

LeiomyomasMost common benign tumor of the esophagusIntramuralOccur between 20-50 years of age with no gender preponderance80% occur in the middle and lower third of the esophagus, they are rare in the cervical regionObstruction and regurgitation may occur in large lesionsBleeding is a more common symptom of the malignant form of the tumor: leiomyosarcoma

Esophageal CystsArise as diverticula of the embryonic foregut¾ of this cyst present in childhoodOver 60% are located along the right side of the esophagusAre often associated with vertebral anomalies (ex: spina bifida)60% present in the first year of life with either respiratory or esophageal symptomsCyst found in the upper third of the esophagus present in infancy while lower third lesions present later in childhood

Pedunculated Intraluminal Tumors (Polyps)

Benign polyps are rare

Usually occur in older men and may cause intermittent dysphagia

Are sometimes easily missed with barium swallow and esophagoscopy

Malignant Tumors of the Esophagus

Usually are in advanced stages at the time of diagnosis (involving the muscular wall and extending into adjacent tissues)Alcohol consumption and cigarette smoking seem to be the most consistent risk factorsEsophageal squamous cell carcinoma (95% of all esophageal cancers) is a disease of men (5: 1) Squamous cell esophageal cancer occurs least frequently in the cervical esophagus and Squamous cell esophageal cancer occurs most often in the upper and midthoracic segments

Malignant Tumors of the Esophagus

Adenocarcinoma constitute approximate 8% of primary esophageal cancersThe frequency of adenocarcinoma is increasing dramatically in the U.S. at a rate surpassing any other cancerMost often occur in the distal third of the esophagus in the 6th decade of life. Male to female ratio is 3:1Patients with Barretts metaplasia are 40 times more likely to develop adenocarcinomaThese tumors are aggressive as well

Clinical Presentation

Dysphagia is the presenting complaint in 80-90% of patients with esophageal carcinoma

Early symptoms are sometimes nonspecific retrosternal discomfort or indigestion

As the tumor enlarges, dysphagia becomes more progressive.

Later symptoms include weight loss, odynophagia, chest pain and hematemesis

Diagnosis

Esophageal biopsy

Brushings for cytologic evaluation

Barium swallow

Lugol’s solution

Staging of Tumors

Endoscopic ultrasound-to define the depth of invasion and presence of paraesophageal lymph nodesChest x-ray ± abnormal findingsCT scan (most widely used and now standard radiographic means of staging)Bronchoscopy for tumors which are proximal to the trachea

TMN Classification for StagingThe esophagus is first divided into four segments

CervicalUpper thoracicMiddle thoracicLower

“T” defines the depth of invasion“N” defines regional lymph node involvement“M” defines the presence or absence of distant metastasisThe TNM categories are grouped into stages which have been shown to reflect the prognosis of tumors

Perforation of the Esophagus

Causes of Perforation

IatrogenicEndoscopy

Dilators

Esophageal intubation

Variceal sclerosis

IntraopoerativeMediastinoscopy

Thyroid surgery

SpontaneousPostemetic

Radiation therapy

TraumaticBlunt and penetrating

Caustic

Carcinomas

Clinical PresentationSymptoms and signs vary with the cause and location of the perforationPain is the most consistent symptom (70-90%)Blood tainted emesis is present and 30% of these patientsThe pain pattern is often misdiagnose as a dissecting aortic aneurysm, spontaneous pneumothorax or myocardial infarctionTachycardia and tachypnea is commonHypotension and shock can occur

Diagnosis

Chest x-ray (plain film)When obtained early may appear normal

Mediastinal emphysema may appear in one hour

Pleural effusions may take several hours

Definitive diagnosis-contrast studies

CT scan’s for atypical presentations

Esophagoscopy is rarely used for diagnosis of perforation

Treatment

Three factors affect management of esophageal perforation

Etiology

Location

The delay between rupture and treatment

Surgical treatment remains the mainstay of management in esophageal perforations

Hiatal Hernia and Gastroesophageal Reflux

Factors Affecting RefluxGastric juices

Gastric acid and bile

Gastric emptyingAbnormal emptying patterns (prolonged fundal distention)

Previous gastroesophageal operationsSocial habits and medication

Fatty foods, chocolate and peppermint reduces LES toneSmoking causes a significant decrease in LES resting pressuresAll medication affecting smooth muscle contraction have been shown to affect LES pressures

Signs and Symptoms of Gastroesophageal Reflux

DiagnosisEsophagoscopy

To note mucosal changes

Esophageal biopsiesTo note changes at the cellular level

Motilitiy studiesLow LES pressures are associated with reflux

pH monitoringThe most precise measure for the presence of acid in the esophageal lumen (24 hour monitoring)

Final Staging

The results from the four studies above are scored and patients are put into one of four categories

The treatment regimen depends on the stage of the disease

Medical Treatment

Surgical TreatmentIndications for surgical treatment are somewhat controversialStage 0 and Stage 1 disease should never be an indication for surgeryStage 2 disease should always undergo a well supervised period of medical management for at least six months to a yearStage 3 disease should also undergo medical therapy firstIn stage2 and in Stage 3 disease surgical options should be entertained after failed medical management

Surgical Treatment

Nissen fundoplicationTotal or partial

Their aim is to:Restore normal anatomy (intra-abdominal segment of esophagus)Re-creating an appropriate high-pressure sound at the esophagogastric junctionMaintaining this repair in the normal anatomic position

Corrosive Strictures of the Esophagus

EtiolgyThe most common chemicals implicated in corrosive burns of the esophagus include:

Alkaline caustics• Household drain cleaners• Dishwashing detergent• Washing soda• Ammonia• Disk shaped alkaline batteries

Acid or acid like corrosives• Automobile battery acids• A variety of commercial cleaners

Household bleach

Important Elements in Successful Management of a Corrosive BurnImmediate verification of the corrosive agent Accurate assessment of the depth and extent of injury (esophagoscopy)

Superficial injuries• Erythema• Edema or blistering

Deep injuries• ulceration

Subsequent treatment is individualized on the basis of these findingsIn the presence of injury the esophageal status should be assessed at repeated intervals of 3 weeks, 3 months and between 6 months to a year

Treatment Options

MechanicalIntraluminal Silastic stents

PharmacologicalCorticosteroids to modify the inflammatory response

Antibiotics to control secondary infection

Strictures

Most frequent complication of caustic burns

Usually develops between three and eight weeks after initial injury

Multiple areas of stricture can occur

Treatment Options for Strictures

Esophageal dilatation by the passage of bougies

Surgical reconstruction

Special Note

There is an increased incidence in patients who have previously suffered corrosive esophageal burns to develop esophageal carcinoma later in life (1000 fold increase)

The End