3. DEFINITION;
Achalasia is a motility
Disorder of the oesophagus
characterized by absence of
peristalsis and failure of relaxation of
lower esophageal sphincter.
This causes obstruction at level of
oesophago-gastric junction.
4. INCIDENCE
Common in adults (age 25-40 yrs).
Only 5% in children.
Also occurs in elderly in association
with malignancy.
5. STRUCTURE OF
ESOPHAGUS
OESOPHAGUS IS A MUSCULAR TUBE,
ABOUT 25cm LONG,BEGINS FROM THE
PHARYNX (C6) AND ENDS AT THE
CARDIAC ORIFICE OF STOMACH (T11).
Esophagus consists of four
layers.Theses are from inside
outwards;
1. Mucosa.
2. Submucosa.
3. Muscularis externa.
4. Serosa.
9. PERISTALSIS
definition:
it is a wave like contractions of
smooth muscles of the esophagus
that propel the food contents onward
by alternative contraction and
relaxation.
13. PATHOPHYSIOLOGY
ACHALASIA IS
CHARECTERIZED BY INCREASED
LOWER ESOPHAGEAL SPHINCTER
PRESSURE,(LES)
DECREASED or ABSENT
PERISTALSIS IN THE DISTAL
PORTION OF THE ESOPHAGUS,
AND LACK OF LES RELAXATION IN
RESPONSE TO SWALLOWING.
15. NEUROTRANSMITTERS IN
MYENTERIC PLEXUS
Myenteric plexus lies in b/w longitudinal and circular
layers.
Acetylcholine and substance p are
Excitatory neurotransmitters causing contractions
of the esophagus.
Nitric oxide (no) and vasoactive
Intestinal peptide (vip) are inhibitory
Neurotransmitters and responsible for
Relaxation of the l.E.S and coordinated esophageal
peristalsis.
16. MYENTERIC PLEXUS
Myenteric plexus provides motor
innervation to both muscular layers,
Secretomotor innervation to the
mucosa and
Controls esophageal
motility.
18. AETIOLOGY
Exact cause is not known. Degeneration of
myenteric plexus.
The loss of inhibitory neurons allows
unopposed stimulation by cholinergic
neurons,
Which leads to failure in l.E.S relaxation
and aperistalsis of distal esophagus.
19. SIGN AND
SYMPTOMS
Dysphagia.
.More for solids.
.Progressive and worsening.
.May lead to weight loss.
Chest pain.
.Induced by eating.
.Retrosternal.
20. INFECTED TEETH AND GUMS DUE TO
ACID REFLUX, FERMENTED FOOD AND
POOR ORAL HYGIENE.
21. SIGN AND SYMPTOMS.
Regurgitation of undigested food.
.Some patients learn to induce it to relieve
pain.
Heartburn.
Sensation of food sticking in lower
esophagus.
Nocturnal cough.
22. SIGN AND SYMPTOMS
Late features
Inhalation of refluxed
contents leading to
pneumonia.
Weight loss.
25. BARIUM SWALLOW IN
ACHALASIA
The Characteristic Features Are;
Dilated Oesophagus,
Incoordinated Esophageal
Contractions,
Obstruction At The
Oesophagogastric Junction And
Rat-tail Appearance.
28. ENDOSCOPY
It Shows Narrowing Or Obstruction Of
Distal Part Of The Esophagus.
Also To Exclude Organic Cause
Of Obstruction.
30. Esophageal Manometry
Esophageal Manometry Test
Measures The Motility And Function Of
The Esophagus And Esophageal
Sphicter.
A Tube Is Passed Through Nose Into The
Esophagus.
The Pressure Of The Sphincter And
Contractions Waves Are
Recorded.
This Procedure Has Risks Of
Aspiration Due To Increased
Salivation.
31. TREATMENT
There Are Four Treatment
Options;
1. Pharmacological.
2. Forceful Dilatation.
3. Botulinum Toxin.
4. Oesophageal Myotomy.
32. 1. Begin a calcium channel
blocker
2. Begin botulinum toxin
injections
3. Endoscopic balloon
dilation of the lower
esophageal sphincter
4. Upper endoscopy
5. Myotomy with
fundoplication
fIGURES:
A
33. PHARMACOLOGICAL TX
Calcium channel blockers like nifedipine
And nitrates like isosorbide dinitrate;
As calcium ions are responsible for activity of myofibrils and
tension generation.
C.C.B act by decreasing calcium entry and
Reducing the pressure in lower esophageal sphincter.
These bring only short term relief.
Side effects are low blood pressre and headache.
34. FORCEFUL DILATATION
It is the treatment of choice in adults.
Procedure
A Balloon Catheter Is Placed Into The
Esophagus And Forcefully Inflating It
Within The Lower Esophageal Sphincter.
Tear Is Produced In Les Which Become
Relax And Allow The Food To Pass Into
Stomach.
35. It Has Success Rate Of 60-90 %.
The Advantage Of Dilatation Is That It
Can Be Repeated If Symptoms
Return.
Incidence Of Perforation Varies
From 1 To 5 Percent.
39. BOTULINUM TOXIN
Botulinum toxin is injected into the
Lower esophageal sphincter using endoscope and
causes local relaxation.
It is effective in 90% but effect wears off after
several months.
50% of patients will require another tx within a year.
Side effect is chest pain after injection.
40. SURGICAL PROCEDURE
Myotomy
This involves a controlled incision into
the muscle of les to cause it to relax.
It has a success rate of upto 90%.
41. HELLER MYOTOMY
It was first performed by ernest
heller in 1913.
It can be performed either by open
procedure, through the chest
(thoracotomy) or through the abdomen
(laparotomy),or by laparoscopic
techniques.
44. A 37-year-old man presents to general medical clinic
with dysphagia. He notes that his symptoms began
several weeks ago and have worsened over time.
He now has trouble swallowing solids and liquids,
though liquids have always given him the most
trouble. He denies any other symptoms. He has no
significant past medical history. Travel history
reveals a recent trip to South America but no other
travel outside the United States. Vital signs are
stable.
Physical examination is within normal limits. He has
no
palpable masses. What is the next step in
management?
1.Upper endoscopy
2.Barium esophagram
3.Esophageal manometry
4.CT of the chest
45. DISCUSSION: This patient presents with signs and symptoms
concerning
for achalasia, possibly due to Chagas disease. A barium
esophagram is the next step in management and should
precede endoscopy in patients with dysphagia and a broad
differential diagnosis.
Recall that achalasia is a motor disorder of the distal
esophagus resulting from degeneration of Auerbach's plexus
where lower esophageal sphincter fails to relax during
swallowing. As a consequence, natural peristalsis is disrupted
and the patient experiences dysphagia to solids and liquids,
with liquids often being most problematic. It is the most
common motility disorder and is often found in patients under
50. The condition has been associated with Chagas disease,
where the parasitic amastigotes destroy ganglion cells.
A barium esophogram is helpful in making the diagnosis and
should reveal the classic bird's beak tapering at the
esophageal sphincter (see Illustration A). Diagnosis is
eventually confirmed with esophageal manometry.
46. Incorrect Answer:
Answer 1: Upper endoscopy would be more costly than
barium esophagram and is not the preferred next step
in management in dysphagia.
Answer 3: Esophageal manometry may be used to
confirm a
diagnosis of achalasia but should not be the next step in
management.
Answer 4: CT of the chest is not needed in the diagnosis
of achalasia but could be warranted if malignancy were
the cause of this patient's dysphagia.
Answer 5: Nifurtimox is successful in treating Chagas
disease which is caused by Trypanosoma cruzi and
transmitted by the Reduviid bug. However, diagnosis
should be made by blood smear before treating this
patient.
47. A 66-year-old woman presents to your outpatient clinic
for her regular checkup. During the visit, she tells you
that she feels "in great health," with the exception of
some recent trouble swallowing. Further questioning
reveals that she has difficulty swallowing solids and
liquids. These symptoms have been worsening slowly
for the past 5 months. Vital signs are within normal
limits, but her weight has decreased by 12 pounds
since her last visit 6 months ago. Barium swallow
reveals smooth tapering of the distal esophagus
(Figure A). Which of these choices is the most
appropriate next step in management?
FIGURES: A
1.Nifedipine
2.High-calorie nutritional supplementation
3.Botulinum toxin injection
4.Surgical myotomy
48. 5
DISCUSSION: This patient presents with the classic
signs and symptoms of achalasia. Upper GI endoscopy
to rule out malignancy is indicated prior to treatment
in cases of suspected achalasia.
Achalasia is a disorder of esophageal motility in which
esophageal peristalsis is absent and lower esophageal
sphincter relaxation after swallowing is impaired.
Patients report difficulty swallowing both solids and
liquids, and barium swallow shows the classic "bird's
beak" appearance. Besides dysphagia, patients frequently
report heartburn, chest pain, weight loss, and
regurgitation. Esophageal manometry and pH
monitoring are also used in the diagnosis of this
condition.
49. Incorrect Answers:
Answer 1: Calcium channel blocker administration may
help decrease lower esophageal sphincter pressure
and ease the symptoms of achalasia; however,
malignancy must be ruled out first through endoscopy.
Answer 2: High-calorie nutritional supplementation is
inappropriate in this case, as her weight loss is most
likely caused by a GI condition such as achalasia or
malignancy.
Answer 3: Botulinum toxin administration may help
decrease lower esophageal sphincter pressure and
ease the symptoms of achalasia; however, malignancy
must be ruled out first through endoscopy.
Answer 4: Surgical myotomy is indicated for treatment
of achalasia in many patients; however, malignancy
must first be ruled out through endoscopy
50. A 73-year-old female is being seen at
the emergency department after
having recurrent coughing spells and
regurgitation following meals. Her
breath is nearly unbearable upon
arrival to the ED. She is also noted to
have a palpable, fluctuant neck mass
on physical examination.
