2. Introduction:
• Synonyms: Cardiospasm.
• Definition:
• A hypertonic lower oesophageal sphincter,
which fails to relax in response to the
swallowing wave.
• Achalasia is a type of neuromuscular disorder
from esophageal dysphagia.
• Achalasia result from autoimmune process.
3. Epidemiology:
• Incidence 1:100,000 population.
• Prevalence greatly exceeds its incidence;
estimates range from 7.1–13.4/100,000.
• Male = Female, bur some evidence said that
the disease is more in females.
• Age – any age but typically occurs in Middle
age 25 - 60y.
4. Etiology:
1. Primary achalesia : Idiopathic.
2. Secondary achalasia: infections such as
• Trypanosoma cruziin Chagas’ disease.
• Human herpes virus 1 (HSV-1) with genetic
susceptibilty.
3. Tertiary achalasia-like disease: diabetic
autonomic neuropathy, infiltrative disorders
such as malignancy, amyloidosis, or sarcoidosis,
and lesions of dorsal motor nuclei, which may
be produced by polio or surgical ablation.
5. Pathophysiology:
• Degeneration and defective of ganglion cells within
the myenteric plexus leads to :
1. Loss of Excitatory (cholinergic) ganglionic neurons
that normally participate in propagation of peristalsis.
2. Partially Loss of inhibitory (nitric oxide) ganglionic
neurons that normally participate in LES relaxation.
3. Degenerative changes in the dorsal motor
nucleus of the vagus that supplies the myenteric
plexus.
• Antibodies against myenteric neurons have been
shown in serum of achalasia patients {HLA
DQA1*0103 and DQB1*0603 alleles antiHSV1
antibodies and HSV1 DNA}.
6. Excitatory (cholinergic)
ganglionic neurons
Acetylcholine excites {M = myenteric plexes} and
esophageal smooth muscle cell
Partially Loss of inhibitory
(nitric oxide) ganglionic neurons
Dorsal motor nucleus
of the vagus
Acetylcholine
Nitric oxide, VIP =
relaxation of LES
Non relaxing
Sphincter
Continuous excitatory
of LES due to “Acetylcholine”
Loss of myenteric plexus
leads :
1. Lost of muscle tone.
2. Lost of muscle
movement for propelling
the food
FOOD
8. Pathology:
• Early in the course of the disease there is:
• Destruction of ganglion cells in the
esophageal wall.
• Disintegration of the axoplasm and myelin
sheaths within the vagus nerve.
• Degenerative changes in the dorsal
motor nucleus of the vagus.
• Normal myosites of the esophagus.
9.
10. 1. Intermittent dysphagia of both liquid and solid.
2. (+/-) Heartburn may or may not (due to closed oesophageal
sphincter).
3. Episodes of retrosternal chest pain due to oesophageal
spasm.
4. Regurgitation.
5. Nocturnal pulmonary aspiration develops.
6. Halitosis; bad smelling from mouth due to “food
putrefaction”
7. Weight loss; due to energy intake and energy loss.
8. Rare, airway compromise and stridor as a result of the dilated
esophagus compressing the membranous trachea.
11.
12. Differentialdiagnosis:
Diffuse esophageal spasm.
Chagas disease.
Pseudoachalasia (Malignancy at the gastro-
oesophageal junction mimics achalasia cardia).
Familial adrenal insufficiency with alacrima;
inherited as an autosomal recessive trait that
manifests itself with the childhood onset of
autonomic nervous system dysfunction
including achalasia.
Parkinson’s disease.
13. Investigation:
1 = Plain X-ray Chest:
• It shows absence of gastric air bubble with
retrocardiac air fluid level.
Absence of gastric bubble
‘normal this area is black’
Presence of retrocardiac air
fluid level ‘normal this area is white’
air
fluid
14. 2 = Barium or (Gastrograffin) swallow:
• Shows tapered narrowing of the lower
oesophagus, the oesophageal body is dilated,
aperistaltic and food-filled this is called (Rat Tail
or Bird’s Beak-like appearance).
Bird’s Beak-like appearance
Rat Tail
15. 3 = Endoscopy:
• Is used to differentiate carcinoma of the cardiac
from achalesia (‘pseudo-achalasia’).
16. 4 = Manometry:
• Confirms the high-pressure, non-relaxing lower
oesophageal sphincter with poor contractility of
the oesophageal body.
17. • Manometry identifies early disease before
esophageal dilatation and food retention, it is the
most sensitive diagnostic test.
• Three subtypes of achalasia are differentiated based
on the pattern of pressurization in the nonperistaltic
esophagus:
1. Classic achalasia = minimal pressurization of the
esophageal body.
2. Achalasia with compression = substantial fluid
pressurization is observed in achalasia with
esophageal compression.
3. Spastic achalasia = spastic esophageal contractions.
o All are characterized by impaired lower esophageal
sphincter (LES) relaxation and absent peristalsis.
18.
19.
20. A) Pharmacological:
• Anticholinergic drugs (probanthine 15-30 mg
thrice a day 15 minutes before meals).
• Calcium channel blockers (nifedipine 20 mg
twice daily).
• Smooth muscle relaxant (nitroglycerine 10-30
mg).
• Sildanefide (50 mg blocks the ).
• Prokinetic agents (metoclopramide 10 mg three
times a day).
• Proton pump inhibitor (omeprazole 20 mg
three times a day).
21.
22. B) Endoscopy:
I) Forceful pneumatic (Balloon) dilatation:
• Using a 30–35-mm-diameter (disrupts the
oesophageal sphincter and improves symptoms
in 80%).
23. II) Endoscopically directed injection of botulinum
toxin:
• Into the lower oesophageal sphincter but relapse
is common.
24. C) Surgical:
• Myotomy (Heller’s operation):
• Performed either laparoscopically or as an open operation
is effective.
• Both pneumatic dilatation and myotomy may be
complicated by gastro-oesophageal reflux - severe
oesophagitis.
• Partial Fundoplication is an anti-reflux procedure and is
accompanied by Heller’s myotomy.
25. Complications and Prognosis:
• Complications:
Esophageal cancinoma.
Esophageal stricture from reflux esophagitis.
• Prognosis:
o Over 90% of patients with achalasia are improved
following pneumatic dilatation or surgical
myotomy.
o Repeat dilatation is frequently required.