Benign Esophageal Diseases

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Achalasia Achalasia means“failure to relax” an

uncommon motility disorder It is characterized by degeneration of

the myenteric neurons that innervate LES and esophageal bodyt

this degeneration results in hypertension of the LES and failure of the LES to relax on pharyngeal swallowing, as well as pressurization of the esophagus, esophageal dilation, and resultant loss of progressive peristalsis.

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pathogenesis

presumed to be idiopathic or infectious neurogenic degeneration.

Yet studies suggest that other factors might be involved

•Severe emotional stress,

•trauma,

•drastic weight reduction,

•and Chagas’ disease (infection with T.cruzi)

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Clinical features Achalasia is also known to be a premalignant condition

8% chance of developing carcinoma over a 20-year period

Most commonly squamous cell carcinoma

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Symptomsthe classic triad of presenting symptoms consists of

• dysphagia; begins with liquids and progresses to solids

• regurgitation; approximately 60% of patients

• weight loss; occurs In final disease

• However, heartburn, postprandial choking, and nocturnal coughing are commonly seen.

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Clinical features• retrosternal chest pain is experienced and can

be severe until the LES opens,

• with progressive disease, aspiration can become lifethreatening.with complications like

• Pneumonia

• lung abscess

• bronchiectasis

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Diagnosis Esophagogram:”Barium study” • The classic finding is a gradual tapering at

the end of the esophagus, similar to a bird's beak

Upper endoscopy • is performed to evaluate the mucosa for evidence of esophagitis or cancer.

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Diagnosis Esophageal manometry is the gold

standard:i. aperistalsis of the distal esophageal bodyii. Esophageal body with pressure above

baselineiii. Low amplitude waveformsiv. incomplete or absent LES relaxationv. hypertensive LES higher than 35mmhg

vigorous achalasia normal to high amplitude esophageal body

contractions in the presence of a nonrelaxing LES

may represent an early stage of achalasia

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Treatment All are palliative and do not

address the problem of decreased motility

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Medical Therapy Nitrates effective treatment of

achalasia headaches limit their tolerability by

patients Calcium channel antagonists have a

better side-effect profile when compared with nitrates

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Botulinum Toxin injected into the LES targets the

excitatory, acetylcholine-releasing neurons that generate LES basal muscle tone

is easy to administer and associated with relatively few side effects

It is apparent that, with repeated injections, the response rates reported are similar or lower to that achieved with the initial injection

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Bougie dilationMay offer months of relief but

requires repeated dilations

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Bougie dilation15

Pneumatic Dilation pneumatic dilation remains one of the

most effective first-line therapies Effective in 60% of cases Esophageal perforation 4%

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Surgical Therapy acid exposure is a known

complication of surgical intervention for achalasia

The current technique is a modification of Heller myotomy laparoscopic or open

Eliminates risk of cancer

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Surgical TherapyEsophagectomy is considered in any symptomatic patient with

•tortuous esophagus (megaesophagus),

•failure of more than one myotomy

•undilatable stricture.

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Diffuse Esophageal Spasm DES is a hypermotility disorder of

the esophagus The basic pathology is related to a

motor abnormality of the esophageal body that is most notable in the lower two thirds of the esophagus

Witch results in repetitive simultaneous and high amplitude contractions

Muscular hypertrophy and degeneration of the branches of the vagus nerve in the esophagus have been observed.

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Symptoms and Diagnosis The clinical presentation of DES is

typically that of chest pain and dysphagia

These symptoms may be related to eating or exertion and may mimic angina

Patients will complain of a squeezing pressure in the chest that may radiate to the jaw, arms, and upper back

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DiagnosisThe classic manometry findings in DES are

simultaneous multipeaked contractions of high amplitude (>120 mm Hg) or long duration (>2.5 seconds;

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Treatment the mainstay of treatment for DES

is nonsurgical, and pharmacologic or endoscopic intervention is preferred

Surgery is reserved for patients with recurrent incapacitating episodes of dysphagia and chest pain who do not respond to medical treatment

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Treatment Eliminating trigger foods or drinks

from diet Peppertmint may provide

temporary reliefe Nitrates, CCB and anticholinergic

drungs?

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Nutcracker esophagus The most common esophageal

hyper motility disorder Most painful of al esophageal

hypermotility disorders High amplitude peristaltic

contractions (hypertensive peristalsis)

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Diagnosis the gold standard of diagnosis is the

subjective complaint of chest pain

with simultaneous evidence of peristaltic esophageal contractions 2 standard deviations (SDs) above the normal values on manometric tracings. Amplitudes higher than 400 mm Hg are common

While the LES pressure and relaxation are normal

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Treatment Patients with nutcracker esophagus may have

triggers and are counseled to avoid

caffeine, cold, and hot foods.

Calcium channel blockers, nitrates,

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Esophageal Diverticula can occur in several places along

the esophagus The three most common sites of

occurrence are pharyngoesophageal (Zenker's), parabronchial (midesophageal), and epiphrenic

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Esophageal Diverticula false diverticula occur because of

elevated intraluminal pressures generated from abnormal motility disorders

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Esophageal Diverticula Zenker's diverticulum and an

epiphrenic diverticulum fall under the category of false diverticula.

Traction, or true, diverticula result from external inflammatory mediastinal lymph nodes adhering to the esophagus

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f

inflamed mediastinal lymph nodes from an infection with tuberculosis accounted for most cases Infections with histoplasmosis and resultant fibrosing mediastinitis have now become more common.

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Pharyngoesophageal (Zenker's) Diverticulum is the most common esophageal

diverticulum It usually presents in older patients in the 7th decade of life

found herniating into Killian's triangle, between the oblique fibers of the thyropharyngeus muscle and the horizontal fibers of the cricopharyngeus muscle

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Symptoms and Diagnosis Commonly, patients complain of a

sticking in the throat. cough, excessive salivation, and

intermittent dysphagia often are signs of progressive disease

As the sac increases in size, regurgitation of foul-smelling, undigested material is common

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Symptoms and Diagnosis

Halitosis, voice changes, retrosternal pain, and respiratory infections are especially common in the elderly population

The most serious complication from an untreated Zenker's diverticulum is aspiration pneumonia or lung abscess

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Symptoms and Diagnosis

Diagnosis is made by barium esophagram

Neither esophageal manometry nor endoscopy is needed to make a diagnosis of Zenker's diverticulum.

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Treatment Surgical or endoscopic repair of a

Zenker's diverticulum is the gold standard of treatment

Open repair involve : myotomy of the proximal and distal

thyropharyngeus and cricopharyngeus muscles

diverticulectomy or diverticulopexy are performed through an incision in the left neck

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Treatment An alternative to open surgical

repair is the endoscopic Dohlman procedure : division of the

common wall between the esophagus and the diverticulum using a laser or stapler has also been successful

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Barrett's Esophagus Barrett's esophagus is a condition

whereby an intestinal, columnar epithelium replaces the stratified squamous epithelium that normally lines the distal esophagus

Chronic gastroesophageal reflux is the factor that both injures the squamous epithelium and promotes repair through columnar metaplasia

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Barrett's Esophagus 10% of patients with GERD develop

Barrett's esophagus 40-fold increase in risk for

developing esophageal carcinoma in patients with Barrett's esophagus

Prospectively following 100 patients with Barrett’s esophagus for 1 year an incidence of 1%/year for developing adenocarcinoma, a similar risk to that of patients with a 20 pack-year smoking history developing lung cancer.

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Histological findings• Columnar epithelial gastric surface

cells

• intestinal goblet cells,

• intestinal absorptive cells with a rudimentary brush border

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Barrett's Esophagus With continued exposure to the

reflux disaese, metaplastic cells undergo cellular transformation to low- and high-grade dysplasia

these dysplastic cells may evolve to cancer

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Barrett's Esophagus 70% of patients are men aged 55 to

63 years Men have a 15-fold increased

incidence over women of adenocarcinoma of the esophagus

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!!!many investigators believe that once metaplasia is present, it is exposure to bile and other reflux related substances, not necessarily acid, that encourages the progression of dysplasia to cancer. **In vitro studies have demonstrated cellular and molecular changes in cells of all types when exposed to bile salts.

**it has been shown that patients with adenocarcinoma of the distal esophagus are three times more likely to have been taking acid suppression medications

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Pathophysiology An incompetent LES, with or without a hiatal

hernia, plays an important role in the development of GERD and Barrett’s esophagus.

Factors that have been implicated in the pathophysiology of the LES

• age

• obesity

• stress

• caffeinated products

• alcohol

• tobacco

• spicy, fatty, and acidic

foods.

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Symptoms and Diagnosis Many patients are asymptomatic Most patients present with symptoms of

GERD. • Heartburn• regurgitation• acid or bitter taste in the mouth• excessive belching Recurrent respiratory infections, adult

asthma, and infections in the head and neck also are common complaints.

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Symptoms and Diagnosis The diagnosis of BE is made by

endoscopy and pathology The presence of any endoscopically

visible segment of columnar mucosa within the esophagus that on pathology identifies intestinal metaplasia defines BE

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Treatment Yearly surveillance endoscopy is

recommended in all patients with a diagnosis of Barrett's esophagus

For patients with low-grade dysplasia, surveillance endoscopy is performed at 6-month intervals for the first year and then yearly thereafter if there has been no change

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Treatment Patients undergoing surveillance

are placed on acid suppression medication and monitored for changes in their reflux symptoms.

Controversy surrounds the benefits of antireflux surgery in patients with Barrett's esophagus

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The controversy Surgeons: medical therapy and

endoscopic surveillance may treat the symptoms but fail to address the problem, the functional impairment of the LES

gastroenterologists: adequate surveillance for the development of cancer is impossible after a fundoplication

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TreatmentStudies have demonstrated regression of metaplasia to normal mucosa up to 57% of the time in patients who have undergone antireflux surgery

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Ablative therapy Photodynamic therapy (PDT) is the most

common ablative method used to treat BE• Complication:

Esophageal strictures 34% Persistent metaplasia 50%

Endoscopic mucosal resection (EMR) is gaining favor for the treatment of Barrett's esophagus with low-grade dysplasia. Increase in stricture rate with larger resictions

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Treatment Esophageal resection for Barrett's

esophagus is recommended only for patients in whom high-grade dysplasia is found

Pathologic data on surgical specimens demonstrate a 40% risk for adenocarcinoma within a focus of high-grade dysplasia

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Caustic Injury the best cure for this condition is

prevention

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Caustic Injury Alkali ingestion is more common

than acid ingestion because of its lack of immediate symptoms

Acids cause an immediate burning sensation in the mouth

alkali ingestion are much more devastating and almost always lead to significant destruction of the esophagus

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Caustic injury there are several sites that are prone to injury because of a relative delay in transit through the esophagus.

They correlate to the anatomic narrowings

i. the level of the UES,

ii. midesophagus where the aorta abuts the left mainstem bronchus,

iii. proximal to the LES.

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Alkali ingestion• Alkaline substances dissolve tissues by

liquefactive necrosis, that penetrates the tissue

• there are three phases of tissue injury from alkali ingestion

bands constrict the esophagus

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Acid ingestion Very difficult, immediate burning

pain Causes coagulate necrosis Formation of eschar limits the

tissue penetration! Rarely causes full thickness injury Within 48hrs the extent of the

injury is already determind

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Symptoms and Diagnosis During phase one, patients may complain of

oral and substernal pain hypersalivation odynophagia dysphagia Hematemesis vomiting

During stage two, these symptoms may disappear only to see dysphagia reappear as fibrosis and scarring begin to narrow the esophagus throughout stage three

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Symptoms and Diagnosis Pain in the back perforation of the

mediastinal esophagus abdominal pain abdominal visceral

perforation Hematemesis/respiratory distress severe

injury

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Physical exam evaluating the mouth, airway,

chest, and abdomen Careful inspection of the lips,

palate, pharynx, and larynx is done signs of perforation Auscultation to the lungs upper

airway involvement

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Endoscopy Early endoscopy is recommended

12 to 24 hours after ingestion identify the grade of the burn C.I

Hemodynamic instability Evidence of perforation

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Treatment acute phase limiting and identifying the extent

of the injury It begins with neutralization of the

ingested substance Alkalis are neutralized with half-

strength vinegar or citrus juice

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Treatment Acids are neutralized with milk, egg

whites, or antacids Emetics and sodium bicarbonate

need to be avoided because they can increase the chance of perforation

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Management of complicationsIf full-thickness perforation of the esophagus or stomach is found at any time

•emergent exploratory laparotomy is indicated.

•esophagus and stomach and all affected surrounding organs and tissues are resected,

•end-cervical esophagostomy is performed, and a feeding jejunostomy is placed Postoperatively, the patient is monitored in the ICU and managed aggressively.

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Late complications Esophageal squamous cell

carcinoma The risk for development of

esophageal squamous cell carcinoma is 1000-fold higher in victims of alkali ingestion as compared to the general population

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Esophageal Perforation Perforation of the esophagus is a

surgical emergency Early detection and surgical repair

within the first 24 hours results in 80% to 90% survival

after 24 hours, survival decreases to less than 50%

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Esophageal Perforation Most esophageal perforations occur after

endoscopic instrumentation for a diagnostic or therapeutic procedure,

Perforation from forceful vomiting (Boerhaave's syndrome), foreign body ingestion, or trauma accounts for 15%, 14%, and 10% of cases, respectively

Other iatrogenics: endothracheal tube minitracheostomy and injury during dissections

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Boerhaave’s Syndrome

Recurrent emesis disrupts the normal vomiting reflex that enables sphinchter relaxation, resulting in an increase in intrathoracic esophageal pressure and perforation.

A tear in the esophageal mucosa, known as a Mallory-Weiss tear, also occurs after persistent retching, but is not associated with perforation.

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History • trauma• advanced esophageal cancer• violent wretching • swallowing of a foreign body• recent instrumentation

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Symptoms and DiagnosisSymptoms of neck, substernal,

or epigastric pain are consistently associated with esophageal perforation

Vomiting, hematemesis, or dysphagia also may accompany them

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Symptoms and Diagnosis Cervical perforations may present

with neck ache and stiffness due to contamination of the prevertebral space

Thoracic perforations present with shortness of breath and retrosternal chest pain lateralizing to the side of perforation

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Symptoms and Diagnosis Abdominal perforations present

with epigastric pain that radiates to the back if the perforation is posterior

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Examination On examination , patient may

present with tachypnea, tachycardia, and a low-grade fever but have no other overt signs of perforation

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Examination With increased mediastinal and pleural contamination, patients progress toward hemodynamic instability

On exam, subcutaneous air in the neck or chest, shallow decreased breath sounds, or a tender abdomen are all suggestive of perforation

Laboratory values of significance are an elevated white blood cell count and an elevated salivary amylase in the blood or pleural fluid.

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Laboratory

• elevated white blood cell count

• elevated salivary amylase level in the blood or pleural fluid

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Diagnosis Diagnosis of an esophageal

perforation may be made radiographically

A chest roentgenogram may demonstrate a hydropneumothorax

A contrast esophagram is done using barium for a suspected thoracic perforation and Gastrografin for an abdominal perforation.

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Diagnosis Most perforations are found above

the GEJ on the left lateral wall of the esophagus which results in a 10% false-negative rate in the contrast esophagram if the patient is not placed in the lateral decubitus position

Chest CT shows mediastinal air and fluid at the site of perforation

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Diagnosis A surgical endoscopy needs to be

performed if the esophagram is negative or if operative intervention is planned.

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Treatment Patients with an esophageal

perforation can progress rapidly to hemodynamic instability and shock

perforation is suspected, appropriate resuscitation

1.placement of large-bore peripheral IV catheters

2.urinary catheter3.secured airway

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Treatment• IV fluids • broad-spectrum antibiotics • patient is monitored in an ICU

The patient is kept NPO, and nutritional access needs are assessed

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Treatment Surgery is not indicated for every

patient with a perforation of the esophagus

management is dependent on several variables: stability of the patient, extent of contamination, degree of inflammation, underlying esophageal disease, and location of perforation

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Treatment The most critical variable that

determines the surgical management of an esophageal perforation is the degree of inflammation surrounding the perforation.

When patients present within 24 hours of perforation, inflammation is generally minimal, and primary surgical repair is recommended

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Clinically stable/unstable with contained perforation conservative therapy

• NPO and enteral access• Endolumenal stent endoscopically

placed

Partial resuloution continue conservative therapy

Persistense/ progression surgery

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Treatment With time, inflammation

progresses, and tissues become friable and may not be amenable to primary repair the golden period is within the first 24 hrs.

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Surgery • If primary repair or the muscle flap fails or if

patient renders unstable, resection or exclusion of the esophagus with a cervical esophagostomy, gastrostomy, feeding jejunostomy, and delayed reconstruction is recommended

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Surgery there are four underlying conditions of the esophagus that affect the

treatment

1. resectable carcinoma,

2. megaesophagus from end-stage achalasia,

3. severe peptic strictures, or a

4. history of caustic ingestion.

If any of these is a factor, primary repair, even in the presence of a healthy tissue bed, is not recommended.

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Leiomyoma Leiomyomas constitute 60% of all

benign esophageal tumors They are found in men slightly more

often than women and tend to present in the 4th and 5th decades

They are found in the distal two thirds of the esophagus more than 80% of the time

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Leiomyoma They are usually solitary and remain

intramural, causing symptoms as they enlarge.

Recently, they have been classified as a gastrointestinal stromal tumor (GIST)

GIST tumors are the most common mesenchymal tumors of the gastrointestinal tract and can be benign or malignant

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Symptoms and Diagnosis Many leiomyomas are asymptomatic Dysphagia and pain are the most

common symptoms and can result from even the smallest tumors

A chest radiograph is not usually helpful to diagnose a leiomyoma, but on barium esophagram, a leiomyoma has a characteristic appearance.

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Leiomyoma During endoscopy, extrinsic

compression is seen, and the overlying mucosa is noted to be intact

Diagnosis also can be made by an endoscopic ultrasound (EUS), which will demonstrate a hypoechoic mass in the submucosa or muscularis propria

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GASTROESOPHAGEAL REFLUX DISEASE LES has the primary role of

preventing reflux of the gastric contents into the esophagus

GERD may occur when the pressure of the high-pressure zone in the distal esophagus is too low to prevent gastric contents from entering the esophagus

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GASTROESOPHAGEAL REFLUX DISEASEGERD is often associated with a hiatal

hernia

the most common is the type I hernia, also called a sliding hiatal hernia

Type II and III hiatal hernias are often referred to as paraesophageal hernias and they may be associated with GERD

Type IV when there is other organ herniated into the chest (Spleen ,Colon)

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GASTROESOPHAGEAL REFLUX DISEASE Defintion :

Symptoms OR mucosal damage produced by the abnormal reflux of gastric contents into the esophagus

Often chronic and relapsing May see complications of GERD in patients

who lack typical symptoms

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GASTROESOPHAGEAL REFLUX DISEASE Epidemiology :

About 44% of the US adult population have heartburn at least once a month

14% of Americans have symptoms weekly

7% have symptoms daily

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Clinical Presentations of GERD Classic GERD

Extraesophageal/Atypical GERD

Complicated GERD

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Clinical Presentations of GERD Classic GERD :

Substernal burning and or regurgitation

Postprandial

Aggravated by change of position

Prompt relief by antacid

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Extraesophageal Manifestations of GERDPulmonary

AsthmaAspiration pneumoniaChronic bronchitisPulmonary fibrosis

Other Chest pain Dental erosion

ENTHoarsenessLaryngitisPharyngitisChronic coughGlobus sensationDysphoniaSinusitisSubglottic stenosisLaryngeal cancer

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Clinical Presentations of GERD Symptoms of Complicated GERD :

Dysphagia Difficulty swallowing: food sticks or hangs up

Odynophagia Retrosternal pain with swallowing

Bleeding

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Diagnostic Tests for GERD Barium swallow

Endoscopy

Ambulatory pH monitoring

Esophageal manometry

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Treatment Lifestyle Modifications

Acid Suppression Therapy

Anti-Reflux Surgery

Endoscopic GERD Therapy

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Treatment Lifestyle Modifications

Elevate head of bed 4-6 inches Avoid eating within 2-3 hours of bedtime Lose weight if overweight Stop smoking Modify diet

Eat more frequent but smaller mealsAvoid fatty/fried food, peppermint, chocolate,

alcohol, carbonated beverages, coffee and tea

OTC medications prn

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Anti-Reflux Surgery Indication for Surgery : have failed medical management opt for surgery despite successful medical

management (due to life style considerations including age, time or expense of medications, etc)

have complications of GERD (e.g. Barrett's esophagus; grade III or IV esophagitis)

have medical complications attributable to a large hiatal hernia. (e.g. bleeding, dysphagia)

have "atypical" symptoms (asthma, hoarseness, cough, chest pain, aspiration) and reflux documented on 24 hour pH monitoring

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