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Forth stage
Medicine
Lec-7
د.اسماعيل
18/10/2015
Motility disorders of oesophagus
Primary
Secondary
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 characterized 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.
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
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empties poorly and nocturnal pulmonary aspiration develops. Achalasia predisposes to
squamous 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
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.
Surgical
Surgical myotomy (Heller’s operation), performed eitherlaparoscopically 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 centers (perioral endoscopic myotomy, POEM
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
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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
Benign oesophageal stricture is usually a consequence of gastro-oesophageal reflux disease
and occurs most often in elderly patients who have poor oesophageal clearance. Rings, due
to submucosal fibrosis, are found at the oesophago-gastric junction (‘Schatzki ring’) and
cause intermittent dysphagia, often starting in middle age. 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 wireguided bougies or balloons are used to
disrupt the fibrous tissue of the stricture.
Other uncommon causes of oesophageal stricture
Eosinophilic oesophagitis
Extrinsic compression from bronchial carcinoma
Corrosive ingestion
Post-operative scarring following oesophageal resection
Post-radiotherapy
Following long-term nasogastric intubation
Bisphosphonates
Tumors of the oesophagus
Benign tumors
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 tumors in the upper oesophagus are squamous cancers. Adenocarcinomas
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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
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 tumour
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
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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
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.
A.L.Y