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DISORDERS OF THE OESOPHAGUS

Adult anatomy
The esophagus is a hollow muscular tube guarded by upper and lower sphincters
and extends from the lower border of the cricoid cartilage (C6 vertebra) to the
gastric cardia.
Its length is 25-30 cm and it has three parts: cervical, thoracic and abdominal.
When viewed endoscopically, the beginning of the esophagus is found at 15 cm
from the incisor teeth and the cardio-esophageal junction is encountered at 40 cm
in the male and 37 cm in the female.
Upper esophageal sphincter is made up of the cricopharyngeal muscle.
The esophagus descends in front of the lower cervical and thoracic vertebrae. It
deviates to the left in the neck and then to the right of the midline in the thorax,
except at the lower end when it again inclines to the left before passing through
the diaphragmatic hiatus in front of the aorta.
These deviations from the midline are important surgically in that the cervical
esophagus is best approached from the left side of the neck and the distal portion
through a left thoracotomy or left thoraco-abdominal approach.
The cardia denotes the junction between the esophagus and the stomach. The
term cardia is used to describe the junctional zone between the esophagus with
the epithelium of the stomach.
This squamo-columnar junction is situated within 1-4 cm.
The tubular esophagus meets the saccular stomach at the gastroesophageal
junction where the esophagus is anchored by the phrenoesophageal ligament.
The lower esophageal sphincter cannot be defined anatomically but it is a 3-5 cm
high-pressure area.
The layers of the esophageal wall:
1. An outer adventitial connective tissue layer
2. Outer longitudinal muscle layer
3. Inner circular muscle layer, the upper thirds consisting of striated muscle
and the lower part smooth muscle
4. The submucosa consists of mucous glands, lymphatics and Meissner’s
neural plexus
5. The mucosa consists of striated squamous epithelium, except for the distal
1-2 cm which are lined by columnar epithelium.

Physiology
1. The upper esophageal sphincter relaxes during swallowing.
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2. Peristalsis of the esophagus consists of wave-like movements that pass down

the body of the esophagus and become stronger towards the lower portion.
Esophageal peristaltic pressures range from 25-80 mm. Hg.

3. The lower esophageal sphincter is a high-pressure zone at the lower portion of

the esophagus. It functions to prevent gastro-esophageal reflux. The lower
esophageal sphincter pressure is influenced by a number of factors and
substances.
- LOS pressure is increased by a protein meal, alkalinization of the stomach and
cholinergic drugs.
- LOS pressure is decreased by nitroglycerin, glucagon, chocolate, fatty meals
and gastric acidification.

Assessment of Esophageal Disease

This includes a careful history, physical examination and appropriate
investigations to establish the nature of the underlying pathology.
Symptoms
The presentation of esophageal disease is often typical with one or more of the
well-known classic symptoms.
The typical symptoms of esophageal disease are: dysphagia, regurgitation,
odynophagia, chest pain, waterbrash.

Dysphagia
Difficulty in swallowing may be due to organic disease (benign stricture,
esophageal carcinoma) or result from an esophageal motility disorder (achalasia,
diffuse esophageal spasm).
The patient feels the food sticking and often points to a particular site on the
sternum although this does not correlate well with the exact anatomical location
of the disease.

Dysphagia for solids implies significant disease which may be organic or

functional, whereas dysphagia for liquids only is likely to be the result of an
esophageal motility disorder.
Persistent and progressive dysphagia indicates organic narrowing of the
esophageal lumen. This is usually associated with regurgitation and is not
relieved by sipping fluids or repeated swallowing.
Eventually with progression to total dysphagia, the patient is unable to swallow
his saliva and exhibits constant drooling (running saliva from the mouth).
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Causes of dysphagia

Intraluminal Intramural Extrinsic

Upper esophagus foreign body corrosives goiter

carcinoma pharyngeal
pouch

myasthenia gravis
bulbar palsy
sideropenic web

Body of esophagus foreign body corrosives lymph nodes

esophagitis aortic aneurysm
carcinoma

Lower esophagus foreign body corrosives paraesophageal

hernia
esophagitis
carcinoma
diffuse esoph.spasm
scleroderma
achalasia

Regurgitation
Regurgitation is effortless return of the gastric content in the mouth and is often
precipitated by change of posture.
When occurs predominantly in the supine position especially at night, the
regurgitated material often stains the pillow.
Postural regurgitation which is a very common symptom of reflux disease, is
precipitated by meals and activities associated with a rise in the intra-abdominal
pressure (bending and straining).
Regurgitation may also occur as an overflow phenomenon due to the
accumulation of food in the esophagus proximal to a stenosing lesion.
This spills back into the pharynx and mouth at night and may lead to aspiration
pneumonitis.
In esophageal motility disorders, both overflow and postural regurgitation may
occur, although the former is more commonly encountered in these conditions.
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Odynophagia
This complaint consists of localised pain, usually in the lower sternal region,
immediately the patients swallow certain foods or liquids. It always indicates
organic disease, most commonly esophagitis.
Hot drinks, coffee and heavily spiced foods are the frequent dietary items which
induce odynophagia.

Pain
Esophageal pain is of two sorts: heartburn and angina-like tightening pain which
is often interpreted as evidence of coronary heart disease.
Heartburn is due to reflux of gastric juice which is injurious to the esophageal
mucosa and induces esophagitis.

The chemical injury is accentuated by a defective clearing of the refluxate by the

esophagus consequent on an impaired motility.
This increases the contact time with the esophageal mucosa.
Esophageal anterior chest pain described as a tightening or gripping, simulates
closely angina pectoris.
This type of pain is commonly found in patients with reflux esophagitis or
esophageal motility disorders.

Waterbrash
This symptom in uncommon and restricted to patients with reflux disease.
It is due to excessive salivation, the mouth becoming full of fluid which has a
salty taste.

Atypical Presentation of Esophageal Disease

Patients with esophageal disease may present with anemia due to chronic blood
loss and less commonly with acute upper gastrointestinal bleeding.
Chronic blood loss is usually due to an erosive esophagitis and active bleeding
results from the Mallory-Weiss syndrome or peptic ulceration in a hiatus hernia
or spontaneous perforation of the esophagus (Boerhaave syndrome).

There is a frequent encountered difficulty in distinguishing esophageal from

cardiac pain. Often patients are treated for angina for a while until aggravation
of symptoms indicates the need for coronary angiography.
20-40% of patients with chest pain and normal coronary angiograms are
subsequently found to have esophageal disease.

Presentation with pulmonary symptoms is common.

These include attacks of coughing, chocking and repeated chest infections due
to aspiration pneumonitis in patients with postural regurgitation.
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The chest-X ray then shows areas of consolidation/ abscess formation/pleural

effusion. Effective treatment of the reflux disease is often followed by a
considerable improvement of these patients.

Physical signs

Although the esophagus is inaccessible to physical examination, the following

signs are important and their presence should be checked for during the
examination of patients with esophageal disease:
- evidence of weight loss
- pallor due to anemia
- neck swelling: pharyngeal pouch, enlarged lymph nodes
- chest signs on ascultation and percussion of the lung fields
- hepatomegaly with or without clinical jaundice

Blood tests
A full blood count may exclude anemia. Serum urea and electrolytes may show
dehydration secondary to dysphagia. Liver function tests might show low plasma
proteins, abnormal clotting and elevated enzymes in the presence of metastatic
disease, and portal hypertension.

Investigations for Esophageal Disease

Category Test Indications
Radiology CXR Aspiration pneumonitis
Esophageal perforation

Barium swallow Dysphagia, perforation

Motility disorders

CT- scanning Staging of malignant disease

Ultrasound scanning External Staging of malignant disease

Endoscopic “-“

Radioisotope Labelled bolus Oesophageal transit

studies Reflux disease

Endoscopy Biopsy, cytology All patients with esophageal

symptoms

Physiological Manometry Motility disorders, reflux disease

Ph 24- hours monitoring Reflux disease.

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In addition to these investigations, tests to exclude cardiac disease, ECG at rest

and after exercise and coronary angiography may be necessary in patients who
present with episodes of anterior chest pain.

Radiology
Chest radiography
This investigation is necessary in all patients who have esophageal syndrome to
exclude aspiration pneumonitis, detect mediastinal widening which may suggest
nodal involvement in patients with esophageal malignancy and outline any soft
tissue shadow and fluid/gas level (intrathoracic stomach, achalasia).

In patients with suspected esophageal perforations or suture line dehiscence after

an esophagectomy, a chest radiograph to detect mediastinal emphysema and
pleural effusion is an essential investigation which is performed before contrast
studies.

Contrast radiology
The standard contrast investigation for elective cases is the barium swallow
which is particularly useful in the following:
- patients with dysphagia due to esophageal motility disorders, especially
achalasia and diffuse oesophageal spasm where it is often diagnostic.
- esophageal carcinoma and benign strictures: the differentiation between the two
is usually possible on radiological grounds although it requires confirmation with
endoscopy.
- free reflux of barium into the esophagus may be observed in gastro-esophageal
reflux associated or not with hiatus hernia.
- in patients suspected of esophageal perforation or leaking esophageal
anastomosis.

CT- scanning
It is used in the preoperative assessment of esophageal malignancy: extent of
mural invasion, involvement of adjacent structures and mediastinal node
enlargement although differentiation between nodal deposits and reactive
lymphadenopathy is not possible.

Ultrasound scanning
Diaphragmatic respiratory movements can be seen on USS. Hypomotility due to
phrenic nerve paralysis indicates advanced inoperable intramediastinal
malignancy. Abdominal USS is a simple method for detecting liver metastases.
USS in the neck can identify metastatic lymphadenopathy from esophageal
tumours.
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Endoscopic ultrasonography can clearly visualize the layers of the esophagus

with excellent assessment of any tumour.
Radioisotope studies
These are used to assess gastro-esophageal incompetence in patients with reflux
symptoms and evaluate the esophageal transit of liquid and solid boluses in
individuals with motility disorders.
The radioisotope test for reflux entails the instillation of 300 ml. of saline
labelled with technetium 99 inserted into the stomach via a nasogastric tube
which is then removed.
External scintiscanning of the lower esophagus is used to detect reflux during a
stepwise increase in the intra-abdominal pressure achieved by means of external
compression of the abdomen with an inflatable binder.
Isotope studies are more useful in the measurement of the esophageal transit
time. When a labelled liquid bolus is used, the patient is placed in the supine
position and swallows on demand the labelled liquid previously held in his
mouth.
Normal individuals clear 90% of the liquid from the esophagus into the stomach
in 4-15 sec.
Prolonged transit times are encountered in all esophageal motility disorders with
no virtually transit in achalasia.
Endoscopy
Fibreoptic endoscopy is essential in all patients with dysphagia. It gives direct
visual information on the presence of esophagitis and its severity.
It is a crucial test for the detection of esophageal neoplasms.
Both biopsy and brush cytology are used in the diagnosis of esophageal
malignancy and their combined accuracy rate is 96% which is better than the
accuracy of either test alone.
Cytology appears to be more reliable in stenosing lesions whereas endoscopic
biopsy carries a higher positive yield in exophytic tumours.
Endoscopic biopsies are also necessary in the diagnosis and histological grading
of reflux esophagitis and in the detection of Barret’s epithelium in patients with
longstanding reflux disease.
Endoscopy can be complicated by perforation of the esophagus, which occurs
most frequently during stricture dilatation.

Physiological tests
These include manometry, 24-h pH monitoring.
Esophageal manometry
The techniques available for pressure recordings of the gastro-esophageal
junction use either catheters connected to external transducers or catheter-
mounted pressure transducers.
The pressure profile of the stomach, cardio-esophageal junction and proximal
esophagus is obtained, being extremely valuable in the diagnosis of the various
esophageal motility disorders.
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Esophageal manometry is also useful in the assessment of the results of anti-

reflux surgical procedures.
Oesophageal manometry indices
Normal Abnormal

HPZ =10-26 mm. Hg Less than 10, more than 26 mmHg.

Relaxes when reached by No relaxation with swallowing
primary wave. Absent, increased/decreased amplitude

Mean amplitude of primary Increased duration, abnormal wave forms.

peristaltic wave=50-110mmHg.

24-h. Ph monitoring
A Ph probe is inserted and positioned 5 cm. above the HPZ, monitoring is
continued for 24 hours after which the probe is removed and the data are
transferred into a microcomputer for analysis.
The reflux event is considered when Ph. falls below 4. This test gives the number
of reflux events, hour, mean duration throughout the 24-h period.
Finally, a graph of the Ph. against time is obtained which also depicts the time of
occurrence of the special events (pain, meals).
It is thus possible to determine whether a painful episode was associated with a
reflux event.
Prolonged acid reflux episodes which occur predominantly in the supine position
at night are associated with defective esophageal clearance motility.
There is a good correlation between the results of the prolonged ambulatory pH
monitoring and severe of esophagitis at endoscopy.
In addition, the results of the two investigations are complementary.

Disorders of the Oesophageal Motility

1. Cricopharyngeal dysfunction
Cricopharyngeal dysfunction is caused by a failure of the upper esophageal
sphincter to relax properly.
The problem may be an incoordination between relaxation in the upper
esophageal sphincter and simultaneous contraction of the pharynx, which may
result in a pharyngoesophageal diverticulum (Zenker’s diverticulum).
This is a false diverticulum composed only of mucosa that herniates posteriorly
between the fibers of the cricopharyngeal muscle.
Cricopharyngeal dysfunction is frequently associated with hiatal hernia and
gastroesophageal reflux.
Symptoms include dysphagia, reflux of undigested food and if a large Zenker’s
diverticulum has developed a mass in the neck, usually on the left side which
occasionally causes tracheal compression.
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Diagnosis
The history and physical examination are usually adequate to diagnose
cricopharyngeal dysfunction.
X-rays, which include a barium swallow are helpful in delineating a
diverticulum.
Endoscopy is indicated to rule out other esophageal disorders, including
gastroesophageal reflux or neoplasm.
Treatment
- cricopharyngeal myotomy is the treatment of choice for cricopharyngeal
dysfunction.
- excision of the diverticulum may be combined with the myotomy.

2. Achalasia
Achalasia is an esophageal disease of unknown etiology, although it may be
secondary to ganglionic dysfunction ( neurological defect involving Auerbach’s
myenteric, parasympathetic plexus). Normal peristalsis is lost in the body of the
oesophagus, which causes:
- high resting lower esophageal sphincter pressure
- failure of the lower esophageal sphincter to relax during swallowing.

The body of the esophagus becomes dilated and the muscle hypertrophies in an
attempt to force material through the dysfunctional lower esophageal sphincter.
Carcinoma of the esophagus is 10 times commoner in patients with achalasia
than in the general population.
The condition presents in two main groups, young adults and the elderly. In the
latter, the cause may be a central rather than a local neurological defect.

Symptoms
Difficulty in swallowing fluids is the usual presenting symptom.
Solids tend to sink to the lower end of the dilated esophagus, whereas fluids spill
over into the trachea causing spluttering dysphagia (choking dysphagia).
Respiratory symptoms are present due to aspiration of the esophageal secretions
into the respiratory tree.
Vomiting, retrosternal pain may occur in more severe cases.
Dysphagia induces weight loss.
Diagnosis
Chest X-ray may show a widened mediastinal shadow of dilated esophagus and
possibly a fluid level in the esophagus behind the heart.
Barium swallow signs of achalasia are: smooth tapering narrowing of lower end
of esophagus (bird’s beak, rat’s tail) which fails to relax, dilated tortuous lower
esophagus, no gastric air bubble, uncoordinated or absent peristalsis under
fluoroscopic screening.
The constriction barely allows the passage of contrast into the stomach.
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Esophageal manometry, reveals the high resting esophageal sphincter pressure,

failure of relaxation during swallowing and lower than normal pressure in the
body of the esophagus.
Esophagoscopy is required to rule out neoplasia and to document the extent of
esophagitis.

Treatment for achalasia is palliative since lower esophageal function can never
be restored to normal. The condition is by its nature, incurable and treatment is
directed at relief of the distal obstruction.

Non surgical treatment consists of forceful pneumatic dilatation of the contracted

lower esophageal sphincter, which is just above the gastro-esophageal junction.
Surgical treatment is esophagomyotomy (Heller’s procedure). Care is taken not
to disturb the vagus nerve. The myotomy is confined to the lower portion of the
esophagus, usually 7-10 cm. in length.
The standard operation is via the abdomen and involves a longitudinal incision
of the lower esophageal and upper gastric muscle wall until the mucosa bulges
through (Heller’s cardiomyotomy).
Surgical results with the Heller’s procedure are generally better than with
pneumatic dilatation for relief of dysphagia.
Esophagomyotomy can be combined with an antireflux procedure.
Patients with achalasia should be followed up and periodically endoscoped to
exclude carcinoma of the esophagus.

3. Diffuse Esophageal Spasm

Diffuse oesophageal spasm is a disorder of esophageal motility that consists of
strong nonperistaltic contractions.
Unlike achalasia, this condition has normal sphincteric relaxation and may be
associated with gastro-esophageal reflux.
Symptoms consist of chest pain, which can radiate to the back, neck, ears, jaw
or arms and may be confused with typical angina pectoris.
The pain usually occurs spontaneously and many patients are considered to have
psychoneurosis.
Diagnosis
Manometry reveals high-amplitude repetitive contractions with a normal
sphincteric response to swallowing.
X-rays are normal in half of cases but may reveal diverticula, segmental spasm,
and a corkscrew-appearance of the esophagus.
Treatment
- surgery is moderately effective with good results obtained in over two-thirds of
the patients. The best results are obtained in emotionally stable patients with
severe disease and without associated lower gastro-intestinal problems.
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- surgery consists of a long esophagomyotomy that extends from the arch of the
aorta to just above the lower esophageal sphincter.
- care is taken to preserve lower esophageal sphincter function, which is usually
normal in these patients.
- if significant gastroesophageal reflux is present, an antireflux procedure is
performed.
- medical treatment consists of calcium channel blockers and smooth muscle
relaxants such as nitrates may ameliorate symptoms.

Esophageal reflux
Etiology
Esophageal reflux is secondary to dysfunction of the lower esophageal sphincter
which results in recurrent reflux of the gastric contents into the lower esophagus.
Lower esophageal sphincter dysfunction may be related to:
- decreased endogenous gastrin production
- operations on or near the esophageal hiatus (vagotomy, gastrectomy).
- a sliding-type esophageal hiatal hernia. However, many patients with this type
of hiatal hernia have no evidence of reflux and many patients with normal lower
esophageal anatomy suffer from esophageal reflux.
- scleroderma, a systemic cause of lower esophageal sphincter dysfunction
through weakening of the esophageal smooth muscle.
- exogenous causative agents, including tabacco and alcohol.
Symptoms of esophageal reflux are substernal pain, heartburn and regurgitation
all of which may worsen with bending and lying down.

Diagnosis is made by:

- manometry, which reveals decreased lower esophageal sphincter pressure
- esophagoscopy, which reveals varying degrees of esophagitis
- 24-hour pH monitoring in the lower esophageal area, which demonstrate
increased acidity.
- cineradiography which correlates the amount of reflux via motion pictures.

Treatment
Most patients can be managed conservatively; surgery is reserved for intractable
cases. The first line on medical treatment is to ask the patient to: lose weight,
avoid meals at night, sleeping on more pillows, to stop smoking.
Drug therapy:
- antiacids
- metoclopramide, which increases both lower esophageal sphincter pressure and
gastric motility thus increasing the rate of gastric emptying.
- histamine 2 receptor antagonists to reduce acidity
Reflux can be reduced considerably by taking smaller, more frequent and drier
meals. Smoking induces sphincter relaxation and quitting often reduces reflux
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dramatically. Weight reduction, however, is the most effective long-term

measure.

Surgical treatment
Indication for surgery include:
- symptoms refractory to medical treatment
- severe esophagitis, stricture formation, Barret’s esophagus (replacement of the
normal epithelial lining with columnar epithelium in the lower esophagus
secondary to esophagitis).

Antireflux operations are designed to increase lower esophageal sphincter tone.

All of the operations involve wrapping the lower esophagus with gastric fundus
and restoring the distal esophagus to its original intra-abdominal position with
the gastro-esophageal junction below the diaphragm.
The three most commonly used operations are:
- Belsey Mark IV operation, which is a 270 degrees wrap performed through a
left thoracotomy
- Nissen fundoplication, which is a 360 degrees wrap of the stomach around the
esophagus performed through the abdomen
- Hill repair, or posterior gastropexy, which uses the arcuate ligament to
reestablish the intra-abdominal position of the distal esophagus.

Study questions

1. What are the surgical approach modalities for cervical esophagus,

midthoracic esophagus and lower esophagus ?
2. What are atypical presentations of an esophageal disease ?
3. A 42 years old obese patient presents a 3 months history of heartburning
sensation interfering with sleeping. She becomes dyspneic on physical
effort and when she is tired, headache is troublesome. She smokes and
drinks alcohol on regular basis. Her BP is 180/90 and PR 90/min. regular.
How would you manage this patient?
4. What is the treatment for cure in achalasia ?