Achalasia

Tova Rainis
Gastroenterology Unit
Bnai-Zion Medical Center
Achalasia ("does not relax") - loss of
peristalsis in the distal esophagus
and a failure of LES relaxation.
PATHOPHYSIOLOGY

PATHOPHYSIOLOGY
 ETIOLOGY
• The etiology of achalasia is not
known

• Autoimmune disorder - associated

with HLA-DQw1 - antibodies to
enteric neurons

• Chronic infections with herpes zoster

or measles viruses
• Chagas disease
(Trypanosoma
cruzi) can result in
a loss of intramural
ganglion cells
leading to
aperistalsis and
incomplete LES
relaxation
Malignancy - most
common cause of
pseudoachalasia
• Invasion of the
esophageal neural
plexuses directly, or
part of a
paraneoplastic
syndrome.
• Other tumors -
esophagus, lung,
lymphoma and
pancreatic carcinoma
CLINICAL MANIFESTATIONS
• Annual incidence of approximately 1
case per 100,000.

• Men and women are affected with

equal frequency.

• Usually diagnosed between the ages

of 25 and 60 years.
 DIAGNOSIS
• Patients typically experience symptoms for
years before seeking medical attention.

– In one series of 87 patients with newly

diagnosed achalasia, the mean duration of
symptoms was 4.7 years.

• Patients who have a clinical history

suggestive of achalasia require
radiographic, manometric, and endoscopic
evaluation to confirm the diagnosis.
• A barium swallow -
diagnostic accuracy is
approximately 95%

• Absence of peristalsis

• In some patients,
spastic contractions in
the esophageal body
("vigorous" achalasia)
 Manometry
 Elevated resting
LES pressure

 Incomplete LES
relaxation

 Peristalsis — or
simultaneous
contractions
 Endoscopy
• Exclude malignancies
• Dilated esophagus,
residual material.
• Inflammation and
ulceration
• Stasis - candida
infection.
• The LES does not
open spontaneously,
traversed easily with
gentle pressure
Overview of the
treatment of achalasia
 MEDICAL THERAPY
• Nitrates and calcium channel
blockers relax the smooth muscle of
the LES.

• Pharmacotherapy is often ineffective,

and associated with side effects.

• Used primarily for patients who are

unwilling or unable to tolerate more
effective invasive forms of therapy.
 BALLOON DILATATION
Weaken the LES by tearing its muscle fibers.
Short to medium-term results
(<10 years)
• 899 patients - 65% success rate, mean follow-up
of 6.5 years

• Single PD - effective in 85% of 144 patients,

followed for an average of 6.5 years.

• A systematic review of the literature published in

1998:
– 2418 patients
– 2/3 of patients had good to excellent improvement after
one or more dilations during a mean follow-up of 4.6
years
Long-term results (>10 years)
• Few studies

• One study – 50% developed recurrent

symptoms after 10 years

• In a prospective follow-up of 54 patients

(Single pneumatic dilation)
– 5 year remission rate of 40%
– 10 year remission rate of 36%
– Repeated dilations only mildly improved the
clinical response.
 Predictors of outcome
• A decrease in LES pressure to
approximately 10 mmHg

• Young age (<40 years) predicts a poor

response to pneumatic dilatation

• Sex
– Retrospective study including 49 male pts and
16 female pts, young men required repeat
treatment > young women.
 Complications
• Esophageal perforation – 3% - 5%
• Intramural hematomas
• Esophageal mucosal tears
• Fever (resolves spontaneously)
• Severe postprocedural chest pain –
15%
• Gastroesophageal reflux disease -
incidence of 2%
 BOTULINUM TOXIN

BTX reduce the LES pressure by

selectively blocking the release of
acetylcholine from presynaptic
cholinergic nerve terminals in the
myenteric plexus
BEFORE

AFTER
 Short-term response (<5
years)
• 21 patients were randomly assigned to
BTX injection or placebo

– One week after treatment, the mean decrease

in LES pressure was significantly greater in
patients who received BTX (33% versus 12%),

• Similar results in a number of other series

- overall efficacy has ranged from 65-90%
after one injection, lasting from 3 months
to more than 1 year
 Predictors of outcome
31 pts who were treated with BTX were
followed prospectively for a median
of 2.4 years

A response beyond three months was

significantly more likely in patients older
than age 50 (82 versus 43 percent) and
in patients with vigorous compared to
classic achalasia (100 versus 52
percent)
 Complications

• Post-procedural transient chest pain –

25%
• Heartburn – 5%
• Esophageal wall injury and
paraesophageal tissue inflammation are
rare
• “Botulism” - the low dose of BTX has
virtually no risk of causing generalized
neuromuscular blockade
Modified Heller approach
• Results in good to excellent relief of
symptoms - 70-90% with few serious
complications
• The mortality rate - approximately 0.3%
• Reflux esophagitis - 10% (fundoplication)
• The few long-term studies available
suggest that surgical myotomy results in:
– sustained remission rates of approximately
85% at 10 years, and 65% at 20 years.
 RECOMMENDATION
• About 50% of pts with PD will require further treatment at 5
years

• Almost all pts treated with BTX will relapse after a single
injection, usually in the first year or so.

• PD - attractive alternative for pts who are not not ready or fit
for surgery.

• On the other hand, BTX is a relatively safe procedure, and

thus may be preferred in centers in which an endoscopist
experienced in PD is unavailable.

• It can also be considered for the treatment of patients who

have serious comorbidity and for whom pneumatic dilation or
surgical myotomy has unacceptably high risk.
• Surgical myotomy - may provide a more
permanent solution

• For the high-risk patient, a trial of BTX is a

reasonable approach

• For most younger patients, a laparoscopic

myotomy offers the best chance for a
single permanent procedure
 DEVELOPMENT OF
ESOPHAGEAL CANCER
• Increased risk for developing esophageal cancer
(squamous cell type)

• A population-based study in Sweden:

– Risk increased 16-fold compared with controls.
– Cancer diagnosed an average of 14 years after
diagnosis.
– A similar increase in risk has been noted in a report from
the United States
– Some series note no increase in risk, particularly with
early treatment of achalasia

• No need for regular endoscopic surveillance.

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