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- Normal swallowing.
The muscular coat of the oesophagus has two layers. The inner layer
is circular and the outer layer is longitudinal. When a person swallows a
peristaltic event is triggered. It starts with the longitudinal muscles of
the oesophagus contracting in sequence to shorten the oesophagus. A ring
like contraction of the circular muscles then sweeps along the oesophagus
to propel the bolus of food into the stomach.
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- Disorders of oesophageal motor function.
Oesophageal motility disorders comprise any condition
whose symptoms, especially difficulty in swallowing (dysphagia) and chest
pain, are suspected of being oesophageal in origin. These disorders are
classically diagnosed by oesophageal manometry studies, which assess
- the pressure in the lower oesophageal sphincter and the degree of
relaxation of the lower oesophageal sphincter.
- the presence of peristalsis (a co-ordinated propulsion of food or
fluid) in the oesophageal body and the characteristics of the contraction
waves including high or low amplitude, duration, repetitive nature, and
the presence of either non transmitted or partly transmitted waves.
Most assessments concentrate on the distal (lower) two
thirds of the oesophagus. Normal values on manometry studies have been
calculated through the study of large healthy populations 1.
Other tests which can be useful in making the diagnosis
include barium swallow and gastroscopy.
Abnormalities in the motility of the muscle of the
oesophagus can lead to symptoms of chest pain and dysphagia (difficulty
swallowing). The classification for most disorders of oesophageal motor
function is imprecise. Achalasia has a defined and identifiable pathological
series of changes associated with it but other motor disorders do not. Chest
pain can be associated with any disease of motility of the oesophagus.
Patients may initially present to a doctor with what is thought to be angina
of cardiac origin.
In addition, a significant proportion of patients with
gastro-oesophageal reflux disease may suffer with ‘atypical’ chest pain
which presents as angina. It is well recognised that these patients often
restrict their lifestyle believing they have heart disease 2.
These patients often respond to proton pump inhibitor drugs which profoundly
suppress production of acid in the stomach.
A problematic feature of patients with oesophageal
dysmotility syndromes is that there is an inconsistent association between
oesophageal symptoms (such as chest pain), degree of dysmotility and
psychological symptoms. In a study of 113 patients with various types of
oesophageal dysmotility disorders, those with achalasia and diffuse
oesophageal spasm had normal psychometric profiles. In contrast, those with
other dysmotility disorders had increased psychological abnormalities
including depression. Interestingly, in the entire cohort of patients, the
presence of chest pain was closely associated with the presence of
psychometric abnormalities 3.
4.
Achalasia.
Achalasia (Latin a= absence, chalus = relaxation) is a
disease of unknown cause, which is characterised by an absence of
peristalsis in the smooth muscle oesophagus and failed or incomplete
relaxation of the lower oesophageal sphincter. A variant of achalasia,
called vigorous achalasia, shows the same abnormalities in the lower
oesophageal sphincter but vigorous contractions within the oesophageal body
4. Achalasia is a particular type of oesophageal dysmotility.
Achalasia is uncommon but not rare and has a prevalence
of 10 cases per 100,000 population 5. It becomes increasingly
common with increasing age 6. It is due to degeneration of nerve
cells within the myenteric plexus (the nerves within the wall of the
oesophagus) 7.
The clinical manifestations of achalasia are various.
Symptoms are often present for several years before the patient comes to
medical attention 8. The major symptoms include dysphagia to both
solids and liquids, regurgitation, heartburn, weight loss and foul breath.
Chest pain is often a major feature in the complex of symptoms. It is often
precipitated by eating, can waken the patient at night and may be so severe
as to cause decreased food intake and weight loss. If chest pain persists,
the presence of the variant vigorous achalasia may be more likely 9.
In an eighteen-year prospective study of 101 consecutive patients with the
diagnosis of new achalasia, 64 reported chest pain 10. Neither
manometric nor radiological findings predicted the occurrence of this
retrosternal pain. Patients with chest pain however were significantly
younger than those without and they had a shorter duration of symptoms.
Treatment with dilatation or surgical myotomy effectively diminished
dysphagia but had little effect on the occurrence of retrosternal chest pain
10. Over a course of a several years however chest pain
diminished in most patients and disappeared in the minority of them. This
has been interpreted by some people as being due to a decrease in visceral
sensitivity (sensitivity to internal stimuli) with increasing age 11.
Weight loss is common in achalasia, but with better
diagnostic tests over the past few years leading to earlier diagnosis,
patients may have a completely normal weight. Treatment may reverse the
weight loss or prevent it entirely.
5 Treatment of Achalasia
The degeneration of the nerves causing achalasia cannot
be corrected. Treatment is therefore directed at palliating symptoms and
preventing complications. This is mainly accomplished by reducing the lower
oesophageal sphincter pressure because peristalsis rarely returns with
therapy. This can be done with drugs, forceful dilatation and surgical
myotomy.
No drugs give long term improvement. Those tried include
nitrates, theophylline, calcium channel blockers, particularly nifedipine
and botulinum toxin.
Forceful dilatation to a diameter of 30 mm is needed to
tear the muscle in the lower oesophageal sphincter and achieve long lasting
reduction of sphincter pressure in these patients. Studies suggest
resolution of dysphagia in 32-98% of patients with younger patients and
those with a shorter duration of symptoms doing less well than older
patients 12-15. If initial success is achieved, only small
numbers will need repeat dilatation at a later stage.
Surgical myotomy is associated with good functional
improvement in 65-92% of patients 16;17. Gastroesophageal reflux
may occur after surgery in anything up to 52% of patients 18.
You might find the following web sites
useful
www.digestive-disorders.co.uk
6 REFERENCES
- Clouse RE,.Staiano.A. Manometric patterns using esophageal body and
lower sphincter characteristics: findings in 1013 patients. Dig Dis Sci
1992;37:289-96.
- Shrestha S,.Pasricha PJ. Update on noncardiac chest pain. Dig Dis
2000;18:138-46.
3. Song CW, Lee SJ, Jeen YT, Chun HJ, Um SH, Kim CD et al.
Inconsistent association of esophageal symptoms, psychometric
abnormalities and dysmotility. Am J Gastroenterol 2001;96:2312-6.
4. Textbook of Gastroenterology. Michigan USA: Lipincott Williams and
Wilkins, 1999.
5. Mayberry, J. F. and Atkinson, M. Variations in the prevalence of
achalasia in Great Briain and Ireland: An epidemiological study based on
hospital admissions. Q J Med 237, 67-74. 1987.
6. Mayberry, J. F. and Atkinson, M. Studies of incidence and
prevalence of achalasia in the Nottingham area. Q J Med 56, 451-456.
1985.
7. Cassella, R. R., Brown Jr, A. L., Sayre, G. P., and Ellis Jr, F.
Achalasia of the esophagus: pathologic and etiologic considerations. Ann
Surg 1964; 160: 474-484.
8. Kahrilas PJ. Esophageal motility disorders: current concepts of
pathogenesis and treatment. Can J Gastroenterol 2000;3:221-31.
9. Bondi, J. L., Godwin, D. H., and Garrett, J. M. "Vigorous"
achalasia. Its clinical interpretation and significance. Am J
Gastroenterol 1972; 58, 145-155.
10. Eckardt VF, Stauf B, Bernhard G. Chest pain in achalasia: patient
characteristics and clinical course. Gastroenterology 1999;116:1300-4.
11. Nylander DL, Aithal GP, Tanner AR, Dellipiani AW, Dwarakanath DA.
Chest pain in achalasia is an age-dependent phenomenon.
Gastroenterology 1999;117:1259-.
12. Vantrappen, C. and Janssens, J. To dilate or to operate? That is
the question. Gut 1983; 24, 1013-1019.
13. Sanderson, D. R., Ellis Jr, F., and Olsen, A. M. Achalasia of the
esophagus: results of therapy by dilation, 1950-1967. Chest 1970; 58:
116-121.
14. Olsen, A. M., Harrington, S. W., Moersch, H. J., and Anderson, H.
A. The treatment of cardiospasm: analysis of a twelve year experience. J
Thorac Cardiovasc Surg 1951; 22: 164-173.
15. Fellow, I. W., Ogilvie, A. L., and Atkinson, M. Pneumatic
diilatation in achalasia. Gut 1983; 24 1020-1027.
16. Okike, N., Paynes, W. S., Neufeld, N. T., Bernatz, P. E.,
Pairolero, P. C., and Sanderson, D. R. Esophagomyotomy versus forceful
dilation for achalasia of the esophagus: results in 899 patients. Ann
Thorac Surg 1979; 28: 119-125.
17. Csendes, A., Braghetto, I., Mascaro, J., and Henriquez, A. Late
subjective and objective evaluation of the results of esophagomyotomy in
100 patients with achalasia of the esophagus. Surgery 1988; 104:
469-475.
18. Jara, F. M., Toledo-Pereya, L. H., Lewis, J. H.,
and Muligan, D. J. Long-term results of esophagomyotomy for achalasia of
esophagus. Arch Surg 1979; 114: 935-936..
Achalasia - a detailed overview
7
Glossary of Technical Terms
(words may appear here that are not in the body of the text)
Achalasia |
A condition characterised by lack of normal relaxation of the valve at
the lower end of the gullet, which should occur during swallowing
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Angina |
Chest pain caused by lack of blood to the heart. This
caharacterisically occurs during exertion
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Barium Swallow |
An Xray examination using barium to assess the swallowing function
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Cardiac |
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Diffuse oesophageal
spasm |
Abnormal contraction of the muscular wall of the esophagus causing pain
and dysphagia, often in response to regurgitation of acid gastric
contents.
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Diltiazem |
A drug used for treating angina, high blood pressure and oesophageal
dysmotility syndromes that works by interfering with calcium flow in and
out of cells
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Dysphagia |
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Endoscopic
Ultrasound Scan |
Use of specially designed endoscope which allows ultrasound images to
be generated from within the gastrointestinal tract. This test gives very
high resolution pictures of the anatomy of the gut
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Endsoscopy |
Visual examination of interior sections of the body by introduction of
an instrument (an endoscope) through the mouth; examples include
esophagoscopy, gastroscopy, bronchoscopy. When used unualified, it is
often taken to mean gastroscopy
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Gastric |
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Gastro oesophageal
junction |
The junction between the oesophagus and stomach
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Gastroesophageal
reflux disease |
A syndrome due to structural or functional incompetence of the lower
oesophageal sphincter, which permits retrograde flow of acidic gastric
juice into the oesophagus.
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Gastroscopy |
Visual examination of oesophagus, stomach and duodenum by introduction
of an instrument (an endoscope) through the mouth
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Glyceryl trinitrate |
A drug used for angina and sometimes for oesopahgeal dysmotility
syndromes
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Ischaemic |
Deficient blood supply to any part of the body
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Ischaemic heart
disease |
Deficient blood supply to the muscles of the heart which can be
associated with pain and death of heart muscle (commonly called a heart
attack)
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Isosorbide
mononitrate |
A longer acting drug in the same class as glyceryl trinitrate
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Lower oesophageal
sphincter |
Musculature of the gastroesophageal junction that is continuously (tonically)
active except during swallowing.
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Manometry |
Measurement of the pressure of gases or fluids by means of a manometer.
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Myeneteric plexus |
A plexus of nerve fibers and autonomic cell bodies lying in the
muscular coat of the esophagus, stomach, and intestines
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Myotomy |
Surgical division of a muscle
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Nitrates |
Group of drugs including isosorbide and glyceryl trinitrate
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Odynophagia |
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Oesophageal
dysmotility |
Abnormal function of the oesophagus in which the normal muscle
contractions are altered
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Oesophagus/
Oesophageal |
Relating to the gullet - swalloing tube that passes between the throat
and stomach
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Omeprazole |
A drug that suppresses acid secretion in the stomach, of the proton
pump inhibitor class
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Parasympathetic |
Pertaining to a division of the autonomic (independent - self
governing) nervous system. These nerves cannot be controlled at will
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Peristalsis |
The movement of the intestine or other tubular structure, characterised
by waves of alternate circular contraction and relaxation of the tube by
which the contents are propelled onward
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Proton Pump
Inhibitor |
Potent drugs for suppressing acid secretion in the stomach. They
interact with the proton pump mechanism that creates acid in the wall of
the stomach
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Pseudoachalasia |
A condition which has the manometric features of achalasia but is due
to another cause such as a tumour
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Psychometry |
The science of mental testing
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Radiological |
The study of diagnosis of disease using X-rays and other allied imaging
techniques
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Retrosternal pain |
Pain behind the sternum (breast bone)
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Retrosternally |
Behind the sternum (breast bone)
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Sub-sternal |
Pain beneath the sternum (breast bone)
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Sympathetic nervous
system |
Pertaining to a division of the autonomic (independent - self
governing) nervous system. These nerves cannot be controlled at will
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Thoracic spine |
The middle part of the spine, behind the chest
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Ultrasound scan |
Production of a visible image from the use of high frequency sound
waves. Echoes of reflected sound are used to buyild up an electgronic
image of the various structures of the body
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Vagus nerve |
The 10th cranial nerve, which exits from the base of the skull and
supplies nerve fibres widely througout the thorasx (chest) and abdomen
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Vigorous Achalasia |
A variant of achalasia in which vigorous contractions of the
oesopahgeal body are present
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Visceral sensitivity |
Sensitivity to stimuli inside the organs of the body
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