Achalasia presentation, oesophageal motility disorder

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)