1
Forth Stage
Medicine
Lec-8
د.اسماعيل
12/12/2015
Motility disoreders of Esophagus
A. Primary
.
1 Pharyngeal pouch
This occurs because of incoordination of swallowing within the pharynx, which leads
to herniation through the cricopharyngeus muscle and formation of a pouch.
It is rare, and it usually develops in middle life but can arise at any age. Many patients
have no symptoms, but regurgitation, halitosis and dysphagia can be present. Some
notice gurgling in the throat after swallowing.
The investigation of choice is a barium swallow, which demonstrates the pouch and
reveals incoordination of swallowing, often with pulmonary aspiration. Endoscopy
may be hazardous, since the instrument may enter and perforate the pouch.
Surgical myotomy (‘diverticulotomy’), with or without resection of the pouch, is
indicated in symptomatic patients.
2.Achalasia of the oesophagus
Achalasia is characterised by:
a hypertonic lower oesophageal sphincter, which fails to relax in response to the
swallowing wave
failure of propagated oesophageal contraction, leading to progressive dilatation of
the gullet.
The cause is unknown. Defective release of nitric oxide by inhibitory neurons in the lower
oesophageal sphincter has been reported, and there is degeneration of ganglion cells
within the sphincter and the body of the oesophagus. Loss of the dorsal vagal nuclei within
the brainstem can be demonstrated in later stages. Infection with Trypanosoma cruzi in
Chagas’ disease causes a syndrome that is clinically indistinguishable from achalasia.
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Clinical features
The presentation is with dysphagia. This develops slowly, is initially intermittent, and
is worse for solids and eased by drinking liquids, and by standing and moving around
after eating.
Heartburn does not occur because the closed oesophageal sphincter prevents
gastro-oesophageal reflux.
Some patients experience episodes of chest pain due to oesophageal spasm.
As the disease progresses, dysphagia worsens, the oesophagus empties poorly and
nocturnal pulmonary aspiration develops.
Achalasia predisposes to squamous cell carcinoma of the oesophagus.
Investigations:
Endoscopy should always be carried out because carcinoma of the cardia can mimic
the presentation and radiological and manometric features of achalasia (‘pseudo-
achalasia’)
. A barium swallow shows tapered narrowing of the lower oesophagus and, in late
disease, the oesophageal body is dilated, aperistaltic and food filled.
Manometry confirms the high pressure, non-relaxing lower oesophageal sphincter
with poor contractility of the oesophageal body
Management
1-Endoscopic
Forceful pneumatic dilatation using a 30–35-mm diameter fluoroscopically
positioned balloon disrupts the oesophageal sphincter and improves symptoms in
80% of patients. Some patients require more than one dilatation but those needing
frequent dilatation are best treated surgically.
Endoscopically directed injection of botulinum toxin into the lower oesophageal
sphincter induces clinical remission but relapse is common.
2-Surgical
Surgical myotomy (Heller’s operation), performed either laparoscopically or as an
open operation, is effective but is more invasive than endoscopic dilatation.
Both pneumatic dilatation and myotomy may be complicated by gastro-oesophageal
reflux, and this can lead to severe oesophagitis because oesophageal clearance is so
poor. For this reason, Heller’s myotomy is accompanied by a partial fundoplication
anti-reflux procedure.
PPI therapy is often necessary after surgery.
Recently, a complex endoscopic technique has been developed in specialist centres
(peroral endoscopic myotomy, POEM).
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3 .Other oesophageal motility disorders
Diffuse oesophageal spasm presents in late middle age with episodic chest pain that
may mimic angina, but is sometimes accompanied by transient dysphagia. Some
cases occur in response to gastro-oesophageal reflux. Treatment is based upon the
use of PPI drugs when gastro-oesophageal reflux is present. Oral or sublingual
nitrates or nifedipine may relieve attacks of pain. The results of drug therapy are
often disappointing, as are the alternatives: pneumatic dilatation and surgical
myotomy.
‘Nutcracker’ oesophagus is a condition in which extremely forceful peristaltic activity
leads to episodic chest pain and dysphagia. Treatment is with nitrates or nifedipine.
Some patients present with oesophageal motility disorders which do not fit into a
specific disease entity. The patients are usually elderly and present with dysphagia
and chest pain. Manometric abnormalities, ranging from poor peristalsis to spasm,
occur. Treatment is with dilatation and/or vasodilators for chest pain
B. Secondary causes of oesophageal dysmotility
In systemic sclerosis or CREST syndrome, the muscle of the oesophagus is replaced
by fibrous tissue, which causes failure of peristalsis leading to heartburn and
dysphagia. Oesophagitis is often severe, and benign fibrous strictures occur. These
patients require long-term therapy with PPIs.
Dermatomyositis, rheumatoid arthritis and myasthenia gravis may also cause
dysphagia.
Common causes of benign oesophageal stricture
1. Benign oesophageal stricture is usually a consequence of gastro-oesophageal reflux
disease and occurs most often in elderly patients who have poor oesophageal
clearance.
2. Rings, due to submucosal fibrosis, are found at the oesophago-gastric junction
(‘Schatzki ring’) and cause intermittent dysphagia, often starting in middle age.
3. A post-cricoid web is a rare complication of iron deficiency anaemia (Paterson–Kelly
or Plummer– Vinson syndrome), and may be complicated by the development of
squamous carcinoma.
Benign strictures can be treated by endoscopic dilatation, in which wire-guided
bougies or balloons are used to disrupt the fibrous tissue of the stricture.
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Other uncommon causes of oesophageal stricture
1. Eosinophilic oesophagitis
2. Extrinsic compression from bronchial carcinoma
3. Post-operative scarring following oesophageal resection
4. Post-radiotherapy
5. Bisphosphonates
6. Corrosive ingestion
7. Following long-term nasogastric intubation
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Tumours of the oesophagus
Benign tumours
The most common is a leiomyoma. This is usually asymptomatic but may cause bleeding or
dysphagia.
Carcinoma of the oesophagus
Squamous oesophageal cancer is relatively rare in Caucasians but is more common in
Iran, parts of Africa and China.
Squamous cancer can occur in any part of the oesophagus, and almost all tumours in
the upper oesophagus are squamous cancers.
Adenocarcinomas typically arise in the lower third of the oesophagus from Barrett’s
oesophagus or from the cardia of the stomach. The incidence is increasing , this is
possibly because of the high prevalence of gastro-oesophageal reflux and Barrett’s
oesophagus in populations.
Despite modern treatment, the overall 5-year survival of patients presenting with
oesophageal cancer is only 13%.
Squamous carcinoma: aetiological factors (risk factors)
•
Smoking
•
Alcohol excess
•
Chewing betel nuts or tobacco
•
Achalasia of the oesophagus
•
Coeliac disease
•
Post-cricoid web
•
Post-caustic stricture
•
Tylosis (familial hyperkeratosis of palms and soles)
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Risk factors for adenocarcinoma (lower part) of oesophagus
1. Barrett’s oesophagus .
2. Smoking .
3. Obesity .
Clinical features
Most patients have a history of progressive, painless dysphagia for solid foods.
Others present acutely because of food bolus obstruction.
In late stages, weight loss is often extreme; chest pain or hoarseness suggests
mediastinal invasion.
Fistulation between the oesophagus and the trachea or bronchial tree leads to
coughing after swallowing, pneumonia and pleural effusion.
Physical signs may be absent but, even at initial presentation, cachexia, cervical
lymphadenopathy or other evidence of metastatic spread is common.
Investigations
The investigation of choice is upper gastrointestinal endoscopy with biopsy.
A barium swallow demonstrates the site and length of the stricture but adds little useful
information.
Once a diagnosis has been made, investigations should be performed to stage the tumor
and define operability. Thoracic and abdominal CT, often combined with positron emission
tomography (CT-PET), should be carried out to identify metastatic spread and local
invasion.
Invasion of the aorta, major airways or coeliac axis usually precludes surgery, but patients
with resectable disease on imaging should undergo EUS to determine the depth of
penetration of the tumour into the oesophageal wall and to detect locoregional lymph
node involvement.
These investigations will define the TNM stage of the disease.
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Management
The treatment of choice is surgery if the patient presents at a point at which resection is
possible.
Patients with tumours that have extended beyond the wall of the oesophagus (T3) or
which have lymph node involvement (N1) have a 5-year survival of around 10%. However,
this figure improves significantly if the tumour is confined to the oesophageal wall and
there is no spread to lymph nodes.
Overall survival following ‘potentially curative’ surgery (all macroscopic tumour removed)
is about 30% at 5 years, but recent studies have suggested that this can be improved by
neoadjuvant chemotherapy.
Although squamous carcinomas are radiosensitive, radiotherapy alone is associated with a
5-year survival of only 5%, but combined chemoradiotherapy for these tumours can
achieve 5-year survival rates of 25–30%.
Approximately 70% of patients have extensive disease at presentation; in these, treatment
is palliative and should focus on relief of dysphagia and pain. Endoscopic laser therapy or
self-expanding metallic stents can be used to improve swallowing. Palliative radiotherapy
may induce shrinkage of both squamous cancers and adenocarcinomas but symptomatic
response may be slow. Quality of life can be improved by nutritional support and
appropriate analgesia.
Perforation of the oesophagus
The most common cause is endoscopic perforation complicating dilatation or intubation.
Malignant, corrosive or post-radiotherapy strictures are more likely to be perforated than
peptic strictures.
A perforated peptic stricture is managed conservatively using broad-spectrum antibiotics
and parenteral nutrition; most cases heal within days.
Malignant, caustic and radiotherapy stricture perforations require resection or stenting.
Spontaneous oesophageal perforation (‘Boerhaave’s syndrome’) results from forceful
vomiting and retching. Severe chest pain and shock occur as oesophago-gastric contents
enter the mediastinum and thoracic cavity. Subcutaneous emphysema, pleural effusions
and pneumothorax develop. The diagnosis can be made using a water-soluble contrast
swallow but, in difficult cases, both CT and careful endoscopy (usually in an intubated
patient) may be required. Treatment is surgical. Delay in diagnosis is a key factor in the high
mortality associated with this condition.