ACHALASIA
DR(can). dr. Yusmaidi, SpB – KBD, FinaCS
Digestive Surgery, Lampung University
Definition
Achalasiais a primary esophageal motility disorder
characterized by the absence of esophageal peristalsis
and impaired relaxation of the lower esophageal
sphincter (LES) in response to swallowing.
The LES is hypertensive in about 50% of patients.
Theseabnormalities cause a functional obstruction at
the gastroesophageal junction (GEJ).
Signs and symptoms
Symptoms of achalasia include the following:
•Dysphagia (most common)
•Regurgitation with rumification
•Chest pain
•Heartburn
•Weight loss
Physical examination is noncontributory.
Diagnosis
Laboratory studies are noncontributory.
Studies that may be helpful include the following:
Barium swallow: Bird’s beak appearance, esophageal dilatation
Esophageal manometry (the criterion standard): Incomplete LES
relaxation in response to swallowing, high resting LES pressure,
absent esophageal peristalsis
Prolonged esophageal pH monitoring to rule out gastroesophageal
reflux disease and determine if abnormal reflux is being caused
by treatment
Esophagogastroduodenoscopy to rule out cancer of the GEJ or
fundus
Concomitant endoscopic ultrasonography if a tumor is suspected
Barium swallow
demonstrating the bird-
beak appearance of the
lower esophagus, dilatation
of the esophagus, and stasis
of barium in the esophagus
Esophageal manometry (see the
image) is the criterion standard in
helping to diagnose the classic
findings of achalasia
•Incomplete relaxation of the
LES in response to swallowing
•High resting LES pressure
•Absent esophageal peristalsis
Treatment
Palliation of dysphagia is the key relieve
functional obstruction of distal esophagus
Pharmacotherapy
Botulinum toxin
Esophageal dilation
Operative myotomy
Algorythm
Guideline The American College of
Gastroenterology
Treatment recommendations are as follows:
Initial therapy should be either graded pneumatic dilation (PD) or
laparoscopic surgical myotomy with a partial fundoplication in patients
fit to undergo surgery
Procedures should be performed in high-volume centers of excellence
Initial therapy choice should be based on patient age, sex, preference,
and local institutional expertise
Botulinum toxin therapy is recommended for patients not suited to PD
or surgery
Pharmacologic therapy can be used for patients not undergoing PD or
myotomy and who have failed botulinum toxin therapy (nitrates and
calcium channel blockers most common)
Pharmacotherapy
Poorly absorbed and short lived, best
reserved as adjunct to other therapies
Nitrates
Ca++ channel blockers
Anticholinergics
Opiods
Botulinum toxin therapy
Botox injection
Bind to cholinergic nerves and irreversibly
inhibit Acetyl Choline release
60 - 85% of patient get relief but 50% get
recurrent symptoms within 6 months.
Endoscopically injected
For patients who are not candidates for other
therapies
Botox injection
Advantages: safety, ease of administration,
minimal side effects
Disadvantages: expensive, need for multiple
injections, and efficacy decreased with repeated
injection
Cause obliteration of the dissection planes
between submucosa and muscular layer which
will make subsequent surgery more difficult and
increase risk of perforation.
Pneumatic dilator
Esophageal dilation (under fluroscopy)
-Standard nonoperative therapy
-Break the muscle fibers
-For pts with limited life expectancy
-Can have repeated dilatation
-60-80% success rate, 5yr recurrence rate 50%
-Efficacy is decreased after second dilatation
-Perforation rate ~ 2%
-PPI reduces the need for repeat dilatation
Dilatation vs Botox
Surgical treatment
Excellent results in 90-95%
1914 - Ernest Heller- double myotomy
Modified by Zaaijer- single myotomy
World’s largest experience
Brazil, Chagas’ disease-endemic
1 in 8 inhabitants, in which 5% develops achalasia
Traditionally trans-thoracic or trans-abdominal
Now minimally invasive Laparoscopic / Thoracoscopic
Robotic Heller myotomy
Indication
Younger than 40yrs old (group which PD is <50% effective)
High risk of perforation
- Esophageal diverticula
- Previous surgery of GE junction
- Tortuous or dilated distal esophagus
Recurrent symptoms despite Botox or PD therapy
Personal choice of therapy
- Lower risk of perforation
- Better long term outcome
- Decrease chance of re-intervention
Esophageal myotomy
Heller myotomy
extending 1.5 cm onto
the gastric wall.
Dor fundoplication, left
row of sutures (after
division of short gastric
vessels).
Completed Dor fundoplication
Surgical complication
Intra-op: Mucosa perforation
Post-op:
- Dysphagia- adhesion, inadequate myotomy
- GERD- long myotomy, nerve damage
- Delay perforation- inadequate myotomy
Controversies ?
Which esophageal technique should be
used?
Any role for anti-reflux procedure?
Trans-thorasic
-Excellent result
-Less GERD* compare to trans-abdominal
* Phreno-esophageal ligament is not disrupted and shorter myotomy
- No fundoplication is necessary
Trans-abdominal
Excellent result – comparable to trans-thoracic
More GERD*, less dysphagia
*Longer myotomy onto stomach (3cm)
Laparocopic
Excellent result
*Decrease hospital stay (average 42-48hrs post-op)
Improve GERD by antireflux procedure
Comparison
Currently, no prospective randomized trials
comparing the various approaches to myotomy
Excellent results
Technique used should depend on individual
surgeon’s comfort and experience
Anti-reflux should be performed with abdominal
approach
Dilation vs Surgery
POEM (Per Oral Endoscopic Myotomy)
POEM has been introduced relatively recently as a novel
approach to achalasia.
This procedure is performed under general anesthesia with
endotracheal intubation.
A 2-cm longitudinal mucosal incision is made on the mucosal
surface to create a mucosal entry to the submucosal space.
An anterior submucosal tunnel is created downwards, passing
the gastroesophageal junction and about 3 cm into the proximal
stomach
POEM
In a comparative study that evaluated the
symptomatic and objective outcomes of of
laparoscopic Heller myotomy with POEM for
achalasia, Bhayani et al reported a shorter
hospitalization in those who underwent POEM than
those who underwent myotomy, but both procedures
showed equivalent improvement in symptoms and
esophageal physiology as well as equivalent
postoperative esophageal acid exposure. Worrell et
al reported similar findings.
POEM
Gastroesophageal reflux is reported in up to 50%
of patients after POEM, replicating the results
obtained when a myotomy alone was performed
without an antireflux operation.
Surgical revision in patients with recurrent
dysphagia after POEM might be challenging. The
presence of adhesions between the submucosal
and longitudinal muscular layers after POEM might
make the dissection at this level very difficult.
Conclusion
Ballon dilation is the first choice
Laparoscopic Heller myotomy, preferably with anterior (Dor;
more common) or posterior (Toupet) partial fundoplication
Per Oral Endoscopic Myotomy (POEM)
Patients in whom surgery fails may be treated with an
endoscopic dilatation first. If this fails, a second operation
can be attempted once the cause of failure has been
identified with imaging studies. Esophagectomy is the last
resort
THANK YOU
