This document discusses achalasia, a motility disorder of the lower esophageal sphincter causing difficulty swallowing. It causes the sphincter to not relax properly during swallowing. The cause is unknown but may involve viral or autoimmune destruction of inhibitory neurons. Patients experience dysphagia, chest pain, and regurgitation. Diagnosis involves imaging tests and manometry. Treatment aims to reduce sphincter pressure and includes medications, botox injections, or surgical division of the sphincter. Complications include aspiration pneumonia and stricture formation if untreated.

1. ARANSI RILWAN A.

2.  Introduction
 Epidemiology
 Aetiology
 Pathogenesis
 Clinical Features
 Investigation
 Treatment
 Complications
 Differential diagnosis

3. A-chalasia (Gr.Word) meaning failure of
relaxation.
It is a primary oesophageal motility
disorder that occurs due to the failure of
the normally tonically contracted lower
oesophageal sphincter to relax.

4.  It may spread upwards to involve portions of or the whole
oesophagus.
 It is characterised by;
 1. inability if the cardiac sphincter to relax fully in response
to swallowing.
 2.hypertrophy and dilatation of the rest of the oesophagus.
 3. Absence or diminution of peristalsis in the oesophagus.

5. Achalasia may occur at any age, however,
incidence peaks in individuals in the 3rd
and 5th decade of life.

No sex predilection.

6.  Idiopahic
 Proposed causes include;
 1.Neuronal degeneration
 2.viral infection
 3.Chagas disease
 4.gastroesophageal junction obstruction
 5.genetic inheritance
 6.Autoimune disease.

7. Insults to the oesophagus perhaps a viral
infection or some other external factors
results in myenteric plexus inflammation.
Inflammation leads to autoimmune
response in a susceptible population who
may be genetically predisposed.

8.  Subsequent chronic inflammation leads to
destruction of the inhibitory nitrigenic myenteric
neurons resulting in inability of the L.O .S to
relax in response to swallowing.
 The muscle, especially the circular muscle, of
the rest of the oesophagus overworks to propel
food through the L. O. S and thus undergoes
hypertrophy.

9.  The disease process spreads upwards,
propulsive peristalsis ceases
 Food and saliva now accumulate in the distal
oesophagus and only trickle through the
L.O.S when the hydrostatic pressure is
high enough to overcome the intracardiac
pressure.

10. Intermittent dysphagia.
Retrostemal pain which may radiate to
the neck and interscapular and subcostal
regions.
Halithosis

11.  Regurgitation of food.
 Some may present with pulmonary
symptoms such as dyspnoea, pneumonitis
and chronic cough and purulent
expectoration indicative of lung abscess
due to aspiration of accumulated food.
 Weight loss.

12. Aim:
Confirm diagnosis
Complications
Optimize for treatment.

13.  Gold standard for diagnosis is oesophageal
manometry, however, these investigations
can be done in suspected cases;
 Chest radiograph:
retrocardiac dilation of the esophagus
retrocardiac air-fluid level
minimal or abscent gastric bubble
signs of aspiration.

14.  Barium Swallow
o Dilated, tortuous, oesophagus that smoothly
tapers down at the OGJ giving the "Bird's
beak" appearance.
o absence of gas in the fundus of the
stomach.
o weak, irregular, uncoordinated or absent
peristalsis on fluoroscopy.

17. Oesophagoscopy:
o The oesophagus contains food debris
and is dilated.
o Possibility of associated tumour (seen in
5-10%)

18.  Oesophageal manometry may show a high
resting pressure in the cardiac sphincter.
 Normal Manometric Findings
 LOS pressure 15-25mmHg with normal relaxation
on swallowing.
 Mean amplitude distal oesophageal peristaltic
w.ave is 30-100mmHg

20.  Resting/ excercise ECG: to rule out cardiac
cause of pain.
 FBC.
 Serum electrolyte, urea and creatinine.
 Urinalysis.

21.  Aim is to reduce the pressure of the L.O.S. so as to allow
food to pass into the stomach unimpeded.
 Achieved by
 i) pharmacological manipulation
 ii) dilatation or stretching and disruption of the circu1ar
muscle of the L.O.S to render it incompetent and
 iii) surgical division of the circu1ar muscle of the L.O.S
(cardiomyotomy).

22.  Calcium channel blockers (e.g. nifedipine) and
nitrates (e.g. glyceryl trinitrate), which relax the
smooth muscle of the L.O.S have been used.
 Taken 10-30minutes before meals .
 These are reserved primarily for patients who
refuse or are not good candidates for more
effective and invasive forms of therapy.

23.  Botulinum toxin injection:
 Botulinum toxin (Botox) is a potent inhibitor
of the release of acetylcholine from nerve
endings.
 It is injected endoscopically into the L.O.S
and poisons the excitatory (acetylcholline
releasing) neurons that increase the L.O.S
tone.

24.  Cardiomyotomy
Surgical intervention is indicated after
failure of repeated dilatation,
in mega-oesophagus,
when associated carcinoma is suspected
or as first line treatment.

25.  1. Shock.
 2. Perforation of the oesophagus
 3. Bleeding.
 4. Mediastinitis.
 5. Pneumonia.
 6. Septicaemia.
 7. Oesophageal Stricture.
 8. Gastric outlet obstruction.
 9. Malignant change may occur in a strictured
oesophagus of more than 16 years duration.

26. Carcinoma of lower end of oesophagus
Stricture of lower end of oesophagus
Hiatus hernia
Scleroderma

29. Principles and Practice of Surgical
Practice( Badoe)
The American Journal of
Gastroenterology(2005)