ACHALASIA

Diagnosis and management,

surgical or endoscopic ?

Kunsemedi Setyadi

Digestive Surgery, Diponegoro Medical School

Semarang
 Definition
Achalasia is 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.
These abnormalities 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: Birds 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
Causeobliteration 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
Worlds 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
surgeons 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