Achalasia cardia

AchalasiaAchalasia CardiaCardia
Dr.B.SELVARAJ MS;Mch;FICS;
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Dr.B.SELVARAJ MS;Mch;FICS;
PEDIATRIC SURGEON
SVMCH&RC
PONDICHERRY-605102
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ObjectivesObjectives
Review the etiopathogenesis of achalasia
Discuss the clinical features of achlasia
Discuss the workup of achalasia
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Discuss the workup of achalasia
Discuss the current options for the management
of achalasia
Describe a new development in the treatment of
achalasia
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History ofHistory of achalasiaachalasia
In 1913 Ernest Heller performed the first surgical
intervention for achalasia and the procedure still
bears his name
It was not actually called achalasia until a 1927
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article by Arthur Hurst
◦ The treatment of achalasia of the cardia: so-called
‘cardiospasm’
◦ Achalasia is Greek for lack of relaxation
Ellis et al described the first transthoracic approach
in 1958
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History ofHistory of achalasiaachalasia
The first laparoscopic Heller myotomy by Sir
Alfred Cuschieri in 1991
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What isWhat is achalasiaachalasia??
Aperistalsis of the esophageal body
Hypertonic lower esophageal sphincter
Due to a degeneration of the neurons of the
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Due to a degeneration of the neurons of the
esophageal wall
Second most common benign disorder of the
esophagus requiring surgical intervention
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Histopathology ofHistopathology of achalasiaachalasia
Histologic examination shows a decrease in
the neurons of the myenteric plexuses
(Auerbach’s plexus)
There is a preferential decrease in the nitric
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There is a preferential decrease in the nitric
oxide producing cells
◦ These contribute to LES relaxation
There is a relative sparing of the cholinergic
neurons
◦ responsible for maintaining LES tone
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Histopathology ofHistopathology of achalasiaachalasia
The loss of these inhibitory neurons leads to
an increased resting tone in the LES
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an increased resting tone in the LES
It also leads to aperistalsis of the esophagus
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Etiology ofEtiology of achalasiaachalasia
While primary achalasia is considered
idiopathic, there are a few theories
HLA DQw1 has been shown to be associated
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HLA DQw1 has been shown to be associated
with achalasia and the presence of anti-
myenteric antibodies
◦ This has led some to propose that achalasia may be
an autoimmune disorder
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Some have shown an association with chronic
herpes zoster or measles
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T-cell evaluation of patients with achalasia
has shown a reactivity to HSV-1, which may
suggest that achalasia can be due to an HSV-1
infection
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Secondary achalasia can be due to Chagas
disease
Chagas disease occurs mainly in Central and
South America
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South America
It is due to an infection by the protozoan
parasite Trypanosoma cruzi which is carried
by Rhodnius prolixus
Infection results in the loss of ganglion cells
in Auerbach’s plexus
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When evaluating patients for achalasia, it is
important to rule out the possibility of
malignancy, which can mimic achalasia
Things that may suggest malignancy include:
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Things that may suggest malignancy include:
◦ Presence of symptoms for less than six months
◦ Onset after age 60
◦ Excessive weight loss
◦ Difficultly passing endoscope past GEJ
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AchalasiaAchalasia-- IncidenceIncidence
Has an annual incidence of 1.6 per 100,000
people
The relative infrequency of the disease has
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made it more difficult to study in comparison
to more common disease processes
Occurs equally in men and women
Usually occurs in individuals age 20-50
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Clinical manifestations ofClinical manifestations of achalasiaachalasia
Most common symptom of achalasia is
dysphagia
◦ Food > 90%
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◦ Food > 90%
◦ Liquids > 80%
Other dysmotility disorders of the esophagus
may also have dysphagia, but not with the
frequency of achalasia
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Clinical manifestations ofClinical manifestations of achalasiaachalasia
Mild weight loss (usually < 10 kg)
Regurgitation
Chest pain
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Chest pain
Heartburn
Patients may sense a lump in their throat
(globus)
Hiccups
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Diagnosis ofDiagnosis of achalasiaachalasia
Onset of symptoms is slow and gradual. The
average time between onset of symptoms and
diagnosis is over four years.
In patients with suspected achalasia, there are
three important tools in diagnosing achalasia
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three important tools in diagnosing achalasia
◦ Barium swallow
◦ Endoscopy
◦ Manometry
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Barium swallowBarium swallow
Barium swallow is an excellent tool in the
diagnosis of achalasia
Classic appearance shows a dilated esophagus
which tapers to a classic “bird’s beak”
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which tapers to a classic “bird’s beak”
appearance
The diagnostic accuracy of a barium swallow
was 95% in one study
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ManometryManometry –– Three classic findingsThree classic findings
Elevated resting LES
pressure (often above 45
mmHg)
Incomplete LES relaxation
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Incomplete LES relaxation
◦ The LES should drop to <8
mmHg
◦ In achalasia LES relaxation in
response to a swallow may be
incomplete or absent
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ManometryManometry –– Three classic findingsThree classic findings
Aperistalsis of the esophagus.
◦ A swallow may have no corresponding esophageal
contractions.
◦ Alternatively, there may be simultaneous
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contractions.
◦ While the contractions are classically low
amplitude, there is a subset of patients who have
high amplitude, simultaneous contractions. This
has been termed "vigorous" achalasia.
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Upper GI EndoscopyUpper GI Endoscopy
All patients with suspected achalasia should
undergo endoscopy to rule out malignancy
On entering the esophagus, it is usually large
and will potentially have retained food
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and will potentially have retained food
While the LES does not open spontaneously,
it can be passed with gentle pressure
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Upper GI EndoscopyUpper GI Endoscopy
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Diagnosis ofDiagnosis of achalasiaachalasia
Additional modalities such as CT scan or
endoscopic ultrasound (EUS) can be helpful
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endoscopic ultrasound (EUS) can be helpful
in the workup of a patient for achalasia if
another cause is suspected (such as
malignancy)
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Treatment optionsTreatment options
Medical therapy with calcium channel
blockers or nitrates
They are taken 10-30 minutes before meals
While they have been shown to have
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While they have been shown to have
moderate success, they require the patient to
take them perpetually
They are not recommended as first-line
therapy
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BOTOXBOTOX
Botulinum neuortoxin type A
Inhibits the release of acetylcholine
The idea for the use of BOTOX came from an
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The idea for the use of BOTOX came from an
understanding of the pathophysiology of achlasia
By blocking the release of Ach from the
presynaptic channels in the ganglia of
Auerbach’s plexus, the theory is that the balance
of neurotransmitters is restored
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BOTOXBOTOX
Injection is done in the area of the lower esophageal
sphincter (LES)
It is administered endoscopically
The standard technique is to inject 1 mL (20 to 25
units BT/mL) into each of four quadrants
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units BT/mL) into each of four quadrants
approximately 1 cm above the Z-line.
Complications include:
◦ Mediastinitis
◦ Esophageal mucosal ulceration
◦ Pneumothorax
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BOTOXBOTOX
BOTOX has the downside of not being as
effective as other interventions
While studies have reported symptomatic
relief as high as 90% after a few months, the
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relief as high as 90% after a few months, the
effects generally fall to 50% or lower at one
year and continue to diminish after that
The current consensus on BOTOX is that it
should only be used on patients who are not
fit for other interventions
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Pneumatic dilatationPneumatic dilatation
Considered the most effective nonsurgical
treatment of achalasia
Involves passing the pneumatic device to the
LES, using both endoscopy and fluoroscopy
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LES, using both endoscopy and fluoroscopy
to properly place the balloon
The balloon is inflated to a pressure between
7 to 15 psi
Patients are usually observed for six hours
and then discharged home
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The two best predictors of success:
◦ Post-dilation pressure (or some report the
difference between pre- and post-dilation
pressures)
◦ Older age
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◦ Older age
The biggest concern with pneumatic dilation is
esophageal perforation, which has been reported to
be as low as 1.6% while other studies have reported
an incidence of around 10% (one study reported
21% perforation rate)
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Heller’sHeller’s MyotomyMyotomy
First described by Ernest Heller in 1913 where he
used an abdominal approach to perform an anterior
and posterior esophagomyotomy
Surgical therapy now involves usually performing
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Surgical therapy now involves usually performing
only an anterior myotomy, via either abdominal or
thoracic approach
In addition to laparoscopic myotomy, thoracoscopic
myotomy has also been described
Thoracic approach does have certain drawbacks
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At this point in time, laparoscopic myotomy is
considered the standard operation
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considered the standard operation
When compared to open techniques, similar
rates of complications with much shorter
hospital stay and recovery times
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In performing Heller’s myotomy, there are a
few important questions to consider…
To do a fundoplication?
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If so, what kind of fundoplication?
What to do with the sigmoid esophagus?
Length of myotomy?
Any benefit to the robot?
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ToTo fundoplicatefundoplicate, or not to, or not to
fundoplicatefundoplicate that is the questionthat is the question……
30% of pts complained of significant heartburn
24 hr pH probe or endoscopy demonstrated that
60% of pts had significant reflux
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“Objective analysis reveals an unacceptable rate
of gastroesophageal reflux in laparoscopic
Heller’s myotomy without an antireflux
procedure. We therefore recommend performing
a concurrent antireflux procedure.”
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DorDor vsvs ToupetToupet fundoplicationfundoplication
Dor fundoplication is an anterior 180
degree wrap
Toupet fundoplication is a posterior 270
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Toupet fundoplication is a posterior 270
degree wrap
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DorDor fundoplicationfundoplication
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ToupetToupet fundoplicationfundoplication
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DorDor vsvs ToupetToupet fundoplicationfundoplication
They showed no significant difference in
outcome
◦ Looked at dysphagia
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◦ Looked at dysphagia
◦ Looked at GER and use of PPIs
To date, there has been no randomized
controlled trial comparing the two procedures
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AchalasicAchalasic sigmoid esophagussigmoid esophagus
Markedly dilated
esophagus with
tortuous, angulated
shape
Previously believed
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Previously believed
that this would require
esophagectomy or at
the very least preclude
fundoplication.
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Length ofLength of myotomymyotomy
Often quoted as needing 5 cm of esophageal
myotomy with 1 cm of myotomy onto the
cardia
Long-term outcomes confirm the superior
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Long-term outcomes confirm the superior
efficacy of extended Heller’s myotomy with
Toupet fundoplication for achalasia
◦ 2007 article from Surgical Endoscopy
◦ By Wright et al from Unversity of Washington
◦ Retrospective review
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What about the robot?What about the robot?
Laparoscopic Heller myotomy for achalasia
facilitated by robotic assistance
◦ Galvani et al from University of Illinois, Chicago
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◦ Galvani et al from University of Illinois, Chicago
◦ 2006 article from Surgical Endoscopy
Showed it to be safe an effective
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A new approach toA new approach to achalasiaachalasia
Submucosal endoscopic esophageal myotomy:
a novel experimental approach for the
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◦ Published in Endoscopy, 2007
It has also been referred to as POEM: Peroral
endoscopic myotomy
It is considered a form of NOTES
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The leading expert in this technique is Dr.
Haruhiro Inoue, from Showa University
Northern Yokohama Hospital in Japan.
He has performed over 100 procedures
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He has performed over 100 procedures
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Start by entering the submucosal space
approximately 15 cm above the GE junction
Uses an endoscope with a special transparent
cap
Using a solution of saline with indigo dye, a
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Using a solution of saline with indigo dye, a
tunneled dissection is carried distally to about
2 cm past the GE junction
Then, myotomy is begun starting 10 cm
proximal to GE junction
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Myotomy is carried distally down to 2 cm
past the GE junction
Myotomy only takes the inner circular fibers
while leaving the outer longitudinal fibers
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while leaving the outer longitudinal fibers
intact
At the end of the procedure, the scope is
removed from the submucosal tunnel and the
entry site is closed with endoscopic clips
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POEMPOEM
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AchalasiaAchalasia-- AlgorithmAlgorithm
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SummarySummary
Achalasia is a process that affect the
myenteric plexus of the esophagus leading to
high resting LES pressures and esophageal
aperistalsis
Medical therapy is ineffective
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Medical therapy is ineffective
BOTOX should be reserved for patients who
are not able to undergo other interventions
Pneumatic dilation is effective, but has the
risk of perforation
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SummarySummary
Laparoscopic Heller’s myotomy has excellent
results
Should be accompanied by either Dor or
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Should be accompanied by either Dor or
Toupet fundoplication (not a Nissen)
The myotomy should be at least 5 cm on the
esophagus to 2 cm on the stomach, and
possibly longer
The robot may have a role in the future
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SummarySummary
Submucosal endoscopic myotomy(POEM)
definitely shows promise, but we lack long-
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term results and comparative studies to make
definitive statements
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Achalasia cardia

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Achalasia cardia