CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2013;11:887– 897
PERSPECTIVES IN CLINICAL
GASTROENTEROLOGY AND HEPATOLOGY
Presentation, Diagnosis, and Management of Achalasia
JOHN E. PANDOLFINO and PETER J. KAHRILAS
Department of Medicine, Northwestern University, Feinberg School of Medicine, Chicago, Illinois
T he literal definition of achalasia is “failure of a ring muscle
to relax.” Despite this general definition, the term has
been adopted to specifically describe esophageal achalasia, an
entity also sometimes named cardiospasm or esophageal aperi-
stalsis. Achalasia is characterized by impaired lower esophageal
sphincter (LES) relaxation and aperistalsis in the distal esoph-
agus. However, it is important to note that aperistalsis, also
termed absent peristalsis, means that there is no progressively
sequenced esophageal contraction; it does not imply the com-
plete absence of intraluminal pressure. Consequently, absent
peristalsis does not exclude the occurrence of spastic contrac-
tions or panesophageal pressurization. In fact, understanding
these patterns of pressurization is the key to understanding the
current classification scheme for achalasia and management of
this disease.1
Pathophysiology
Achalasia is associated with functional loss of myenteric
plexus ganglion cells in the distal esophagus and LES.2 Al-
though the ultimate initiating factor for the neuronal degen-
eration is uncertain, it is an autoimmune process that is likely
triggered by an indolent viral infection (herpes, measles) in
conjunction with a genetically susceptible host.3 The inflamma-
tory reaction is associated with a T-cell lymphocyte infiltrate
that leads to a slow destruction of ganglion cells. The distribu-
tion and end result of this plexitis are variable and may be
modified by the host response or the etiologic stimulus. Data to
support a genetic basis come from studies in twins and siblings
as well as its association with genetic diseases such as Allgrove
syndrome, Down’s syndrome, and Parkinson disease.4 – 6 Alter-
natively, achalasia can be one of several manifestations of the
widespread myenteric plexus destruction found in Chagas dis-
ease, a late consequence of infection with the parasite Trypano-
soma cruzi.7
The consequences of the myenteric plexus inflammation
leading to achalasia are degeneration and dysfunction of inhib-
itory postganglionic neurons in the distal esophagus, including
the LES. These neurons use nitric oxide and vasoactive intesti-
nal peptide as neurotransmitters, and their dysfunction results
in an imbalance between excitatory and inhibitory control of
the sphincter and adjacent esophagus. Unopposed cholinergic
stimulation can result in impairment of LES relaxation, hyper-
contractility of the distal esophagus including the LES, and
rapidly propagated contractions in the distal esophagus. How-
ever, there is variable expression of these abnormalities among
individuals, with specific features dominating in some and not
manifest in others; only impaired deglutitive LES relaxation is
universally required as a defining feature of achalasia.
Many of the contractile abnormalities observed in achalasia
can be explained by the imbalance between the excitatory and
inhibitory innervation of the distal esophagus. Key functions of
the inhibitory ganglionic neurons are to facilitate LES relax-
ation and to sequence the peristaltic contraction in the distal
esophagus.8 Without them, the sphincter cannot negate its
myogenic tone and may actually contract after swallows be-
cause of stimulation by unopposed cholinergic neurons. This is
even the case during transient LES relaxations, elegantly dem-
onstrating the selective physiological defect in achalasia. Acha-
lasia can still trigger transient LES relaxations in response to
gastric distention and still exhibit the ensuing complex motor
pattern of crural diaphragm inhibition, esophageal shortening,
and an esophageal after-contraction, but the LES paroxysmally
contracts rather than relaxes.9
Adjacent to the LES, the distal esophagus has no myogenic
tone and is flaccid in the absence of neuronal stimulation.
However, inhibitory ganglionic cells are normally dominant in
this area, delaying the swallow-initiated peristaltic contraction
such that it occurs only after several seconds of LES relaxation.
In the absence of that inhibitory innervation, a pattern of
premature contraction occurs, not allowing sufficient time for
esophageal emptying and trapping the bolus in the distal
esophagus. On X-ray, the trapped bolus sometimes assumes a
“corkscrew” or “rosary bead” configuration, consistent with the
textbook description of distal esophageal spasm. One proposed
model of disease progression in achalasia is that the neuronal
degeneration at the ganglionic level then progresses to involve
the cholinergic neurons, eventually leading to the classic pat-
tern of absent peristalsis. However, it is also proposed that the
subsequent loss of contractility may result from progressive
esophageal dilatation that occurs as a consequence of chronic
functional obstruction. That model of disease progression hy-
pothesizes that panesophageal pressurization represents an
early stage of achalasia when the primary abnormality of out-
flow obstruction is associated with preserved esophageal short-
Abbreviations used in this paper: EGJ, esophagogastric junction;
EPT, esophageal pressure topography; HRM, high-resolution manom-
etry; IRP, integrated relaxation pressure; LES, lower esophageal
sphincter; POEM, per oral endoscopic myotomy.
© 2013 by the AGA Institute
1542-3565/$36.00
http://dx.doi.org/10.1016/j.cgh.2013.01.032
888 PANDOLFINO AND KAHRILAS
ening, upper esophageal sphincter contraction, and some cir-
cular muscle contraction.10,11 Absent peristalsis would then
represent a later stage of the disease that is attributable to more
widespread neuronal degeneration or long-term obstruction. By
either pathway, if left untreated, achalasia can progress to severe
esophageal dilatation, an undesirable and unnecessary outcome
potentially associated with severe morbidity.
Clinical Presentation
Achalasia occurs with an annual incidence of 1 in
100,000 and prevalence of 10 in 100,000.12 There is no racial or
gender preference, and although the onset of symptoms can
occur at any age, they most commonly manifest between the
ages of 25 and 60 years. The primary presenting symptom is
dysphagia for both solids and liquids that is constant rather
than intermittent. Patients learn to adapt to the condition and
often describe themselves as “slow eaters.” Ironically, weight
loss is unusual until the end-stage of the disease. Dysphagia in
achalasia differs from that attributable to mechanical causes,
because it is often accompanied by regurgitation of undigested
food and saliva minutes or even hours after the meal. The
regurgitation can occur at night, and patients sometimes com-
pensate by elevating their head or even sitting upright to sleep.
Regurgitation in achalasia can be distinguished from reflux-
related regurgitation by its bland taste, which is devoid of
gastric acid or bile. However, patients sometimes also experi-
ence chest pain or heartburn, making this distinction diffi-
cult.13 This observation has led to the recommendation that an
esophageal manometry study be obtained in the evaluation of
refractory reflux to rule out achalasia before contemplating
antireflux surgery or accepting a diagnosis of functional heart-
burn.14 It is also important to note that the etiology of chest
pain in achalasia is less clear than is that of dysphagia or
regurgitation, and its response to therapy is usually less satis-
factory.
Diagnosis
The diagnosis of achalasia is contingent on demonstrat-
ing impaired LES relaxation and absent peristalsis in the ab-
sence of partial esophageal obstruction near the LES by a
stricture, tumor, vascular structure, implanted device (eg, LAP-
BAND, Allergan, Inc., Irvine CA), or infiltrating process.14 Thus,
the minimal requisite evaluation should include manometry to
document the motor findings and appropriate imaging studies
to rule out obstruction. There are many nuances to both eval-
uations to keep in mind. Straightforward cases are straightfor-
ward, but the increasing sophistication of diagnostic methods
has led to increasing recognition of variations in the physiolog-
ical manifestations of achalasia and of alternative disease pro-
cesses that can mimic the disease.
With regard to esophageal manometry, a major technolo-
gical evolution has occurred during the last decade wherein
conventional water perfused or strain gauge systems with a
polygraph and line tracing output have been replaced by high-
resolution manometry (HRM) systems outputting pressure
data in esophageal pressure topography (EPT). Nowhere has
this evolution had more impact than in the diagnosis of acha-
lasia. Diagnostic criteria have been tightened,1 and relevant
physiological subtypes have been identified.10 Particularly in-
strumental in establishing uniform diagnostic criteria for acha-
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY Vol. 11, No. 8
lasia was the development of a new metric devised for EPT to
quantify esophagogastric junction (EGJ) relaxation, the inte-
grated relaxation pressure (IRP). Measurement of the IRP uses
an electronic sleeve sensor initially described by Clouse and
Staino15 and conceptually similar to a Dent sleeve that com-
pensates for potential LES movement by tracking the sphincter
within a specified zone. This avoids the artifact of pseudorelax-
ation (apparent sphincter relaxation caused by elevation of the
sphincter above the sensor, displacing it into the stomach),
which was a fatal flaw in the assessment of LES relaxation with
nonsleeve conventional systems. The IRP is calculated from the
electronic sleeve as the mean of 4 seconds of maximal EGJ
relaxation after the pharyngeal contraction. The time scored
can be continuous or noncontinuous, as when it is interrupted
by a crural diaphragm contraction. The IRP provides a robust
and accurate assessment of deglutitive EGJ relaxation and op-
timally discriminates defects of sphincter relaxation character-
istic of achalasia.16
Before the introduction of HRM and EPT, there were no
data substantiating the prognostic value of conventional mano-
metric measures in achalasia, although there were qualitative
descriptions of variants such as vigorous achalasia, achalasia
with preserved peristalsis, and cases with complete or partial
LES relaxation.17,18 There were no established conventions for
making these measurements. However, with the adoption of
HRM with EPT, 3 distinct subtypes of achalasia were quantita-
tively defined by using novel EPT metrics (Figure 1).10 Further-
more, there are now 5 publications supporting the prognostic
value of these achalasia subtypes that consistently observe that
(1) type II patients have the best prognosis with myotomy or
pneumatic dilation, (2) the treatment response of type I pa-
tients is less robust (and reduced further as the degree of
esophageal dilatation increases), and (3) type III patients have a
worse prognosis, likely because the associated spasm is less
likely to respond to therapies directed at the LES.10,19 –22 In
addition, patients with impaired EGJ relaxation but some pre-
served peristalsis (Figure 2) are now recognized as a distinct
entity that can be a variant phenotype of achalasia. However,
EGJ outflow obstruction can also be a manifestation of other
disease entities including eosinophilic esophagitis, LES hyper-
trophy, strictures, paraesophageal hernia, and pseudoachalasia
that is due to tumor infiltration. Consequently, this finding
always mandates carefully imaging (often with biopsies) the
EGJ. Table 1 contrasts the defining manometric measures of
achalasia in conventional and EPT terms.1,23 Highlighted in
Table 1 is the finding that threshold values for abnormal
deglutitive EGJ relaxation by using the IRP are altered by the
pressurization pattern in the esophageal body such that a value
above 10 mm Hg is abnormal in type I patients and that
panesophageal pressurization is diagnostic of type II achalasia,
independent of the IRP value.24
The other requisite evaluation to establish a diagnosis of
achalasia is of imaging studies to rule out obstruction in the
region of the EGJ. In most instances, endoscopy will suffice.
Endoscopy may also be helpful in determining the degree of
esophageal dilatation, whether there is significant esophageal
retention of food and fluid, and whether there is coexistent
stasis or fungal esophagitis. A barium esophagogram may suf-
fice in this capacity in instances that there are equivocal mano-
metric findings or when manometry is not feasible because of
severe dilatation and an inability to intubate the stomach with
August 2013 MANAGEMENT OF ACHALASIA 889

Figure 1. Subtypes of achalasia. The 3 subtypes are based on esophageal body contractility and pressurization. Type I (left) is associated with
absent peristalsis and no discernible esophageal contractility in the context of an elevated IRP. The esophagus is flaccid with some degree of
dilatation as evidenced by the associated esophagogram. The IRP threshold value for impaired EGJ relaxation in type I (⬎10 mm Hg) is lower than
for type II or III because there is no potential for esophageal body pressurization. Type II (center) is associated with abnormal EGJ relaxation and
panesophageal pressurization in excess of 30 mm Hg. This pressurization is likely related to longitudinal muscle and (nonocclusive) circular
contraction compressing the esophageal body compartment. In this example, the proximal esophagus is filled by air, which is evident by the
associated esophagogram and the overlying impedance signal showing liquid (purple transparency) only in the distal esophagus. Type III achalasia
(right) is associated with premature (spastic) contractions and impaired EGJ relaxation. The diagnostic criteria stipulate that at least 2 swallows be
associated with a contraction with distal latency of less than 4.5 seconds; other swallows may have absent peristalsis or rapid contractions, and as
in this example, panesophageal pressurization can also be seen. As in this example, the associated esophagogram for type III achalasia is often
interpreted as esophageal spasm because this has an extreme corkscrew with a small diverticulum above the distal contraction. With permission
from the Esophageal Center at Northwestern.

the manometry catheter. The esophagogram can also quantify
the efficacy of esophageal emptying when done as a timed
barium esophagogram protocol. When suspicion of pseudo-
achalasia is high, endoscopic ultrasound and/or computed to-
mography may be necessary. Figure 3 illustrates such an exam-
ple in which endoscopic ultrasound implicated an aortic
aneurysm as the ultimate cause of a pseudoachalasia syndrome.
Management
There are a variety of treatment options for achalasia
and a number of factors to consider in selecting among them.
High on the list are the patient’s overall state of health, patient
preference, and available center expertise. Patients who are
deemed to be in otherwise good health should be counseled to
pursue a definitive treatment capable of alleviating EGJ outflow
obstruction such as myotomy (laparoscopic or endoscopic) or
pneumatic dilation. Eliminating the outflow obstruction is ben-
eficial, not only because of the associated symptom relief but
also because this may arrest disease progression as evident by
progressive esophageal dilatation. Our current concept is that
such dilatation is irreversible and largely responsible for late
complications of achalasia such as recurrent aspiration pneu-
monia, weight loss, intractable stasis esophagitis, and, rarely,
squamous cell cancer of the esophagus. Medical therapy with
smooth muscle relaxants and/or botulinum toxin should be
reserved for patients with substantial comorbidity making
890 PANDOLFINO AND KAHRILAS
them poor surgical candidates, special circumstances in which a
temporizing intervention is necessary, or rare instances that
there is persistent uncertainty in the diagnosis of achalasia.
Medical Treatment
Pharmacologic therapy with oral calcium channel
blockers or nitrates causes a prompt reduction in LES pressure
ranging from 0% to 50%. These drugs are associated with mild
benefit with respect to dysphagia but often have limiting side
effects of headache, orthostatic hypotension, or edema. Fur-
thermore, their use has not been shown to halt disease progres-
sion. Consequently, calcium channel blockers or nitrates are
poor long-term treatment options and should be reserved for
patients who are judged to be poor candidates for myotomy or
pneumatic dilation. Sublingual nifedipine in doses of 10 –30
mg given 30 – 45 minutes before meals or sublingual isosorbide
dinitrate in doses of 5–10 mg given 15 minutes before meals
may be useful as short-acting temporizing treatments.
The 5=-phosphodiesterase inhibitors such as sildenafil have
also been used in achalasia and spastic disorders of the esoph-
agus.25 Sildenafil lowers EGJ pressure and attenuates distal
esophageal contractions by blocking the enzyme that degrades
cyclic guanosine monophosphate induced by nitric oxide.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY Vol. 11, No. 8
Figure 2. The criteria for EGJ
outflow obstruction are an abnor-
mal EGJ relaxation pressure asso-
ciated with some preserved weak
or normal peristalsis, thereby not
meeting the diagnostic criteria for
types I, II, or III achalasia. Ulti-
mately, this pattern may prove to
be a phenotype of achalasia as in
the case of (A), where the patient
also had a large epiphrenic diver-
ticulum. This patient was treated
with a laparoscopic myotomy and
diverticulectomy, with a good
symptomatic and functional re-
sponse. In contrast, EGJ outflow
obstruction can also be associ-
ated with mechanical obstruction
in the region of the EGJ, as proved
to be the case with the patient il-
lustrated in (B). The patient was
reported to have a patulous EGJ,
and a 9-mm endoscope passed
with no resistance noted at the
EGJ. However, the IRP was ab-
normal, and there was compart-
mentalized pressurization be-
tween the preserved peristaltic
contraction and the EGJ. The as-
sociated esophagogram revealed
a subtle stricture just proximal to
the EGJ where passage of a
12.5-mm barium tablet was de-
layed. The patient responded to
18-mm balloon dilation and pro-
ton pump inhibitor therapy. With
permission from the Esophageal
Center at Northwestern.
Hence, sildenafil is a viable alternative in patients not respond-
ing to or proving intolerant of calcium channel blockers or
nitrates. The typical dose for sildenafil is from 25 to 50 mg with
each meal. However, the current cost of sildenafil is much
greater than that of nifedipine or isosorbide dinitrate, and
patients are usually denied insurance coverage for the drug in
this off-label indication. Furthermore, minimal long-term treat-
ment data exist that are pertinent to using 5=-phosphodiester-
ase inhibitors in long-term achalasia treatment. Nonetheless, it
is reasonable to consider these agents for second-line medical
therapy.
Botulinum Toxin
Botulinum toxin (Botox, Allergan, Inc.) injection into
the muscle of the LES was proposed as an achalasia treatment
on the basis of its ability to block acetylcholine release from
nerve endings by cleaving the SNAP-25 protein, an essential
component of that process. The standard approach is to inject
100 units of Botox with a sclerotherapy needle during endos-
copy. Injections are done about 1 cm proximal to the squamo-
columnar junction in 4 radially dispersed aliquots; this corre-
sponds to the region of greatest tone within the LES. When
using this technique, Pasricha et al26 reported improved dys-
August 2013
Table 1. Comparison of Conventional Manometry and EPT Definitions of Achalasia
Technology Manometric subtypes
Conventional manometry with line Classic
tracing format23
Atypical disorders of LES relaxation
“vigorous achalasia”
HRM with EPT metrics Type I (classic)
Type II (with compression)
Type III (spastic)
EGJ outflow obstruction
aMay be mixed with absent, rapid, hypertensive contractions or pressurization.
phagia in 66% of achalasia patients for 6 months. No increase
in efficacy has been demonstrated with greater doses.27 Physi-
ologically, the effect of Botox is hard to demonstrate because it
blocks only the nonmyogenic component of LES pressure. This
effect is eventually reversed by axonal regeneration, and subse-
quent clinical series report minimal continued efficacy after 1
year.26 –29 On the positive side, Botox injection adds very little
time to endoscopy and is very safe, although it has been asso-
ciated with chest pain after the injection, rare instances of
mediastinitis, and allergic reactions to egg protein. On the
negative side, most patients relapse and require retreatment
within 12 months, and repeated treatments have been shown to
make subsequent Heller myotomy more challenging.30 Further-
more, Botox injection has not been shown to attenuate the
progressive esophageal dilatation, leaving these individuals
prone to the long-term complications of achalasia. Conse-
quently, Botox injection should rarely be used as a first-line
therapy for achalasia; instead, it should be reserved for poor
surgical candidates and special circumstances.
Pneumatic Dilation
An achalasia dilator is a noncompliant, cylindrical bal-
loon that is positioned across the LES and inflated with air by
using a hand-held manometer. The only design currently avail-
able in the United States, the Rigiflex dilator (Boston Scientific
Corporation, Natick, MA), is positioned fluoroscopically over a
guidewire and is available in 30-, 35-, and 40-mm diameters.
Bougie and standard through-the-scope balloon dilators (max-
imal diameter of 20 mm) have no sustained efficacy in achalasia
and should not be used. A cautious approach to dilation with
the Rigiflex dilators is to initially use the 30-mm dilator and
follow with a 35-mm dilator 2– 4 weeks later if the initial
dilation was insufficient. The reported efficacy of pneumatic
dilation ranges from 32% to 98%.31 Patients with a poor result
or rapid recurrence of dysphagia are unlikely to respond to
MANAGEMENT OF ACHALASIA 891
Manometric criteria
● Impaired LES relaxation (no consistent criteria)
● Absent peristalsis (no apparent contractions or
simultaneous contractions ⬍40 mm Hg)
● Elevated LES pressure (variably present)
● Impaired LES relaxation (no consistent criteria)
● Some preserved peristalsis
● Simultaneous contractions (⬎8 cm/s) of
⬎30 mm Hg amplitude
● Impaired EGJ relaxation (IRP ⬎10 mm Hg)
● Absent peristalsis
● No significant esophageal pressurization
● Impaired EGJ relaxation (IRP ⬎15 mm Hg)
● Absent peristalsis
● ⱖ20% swallows with panesophageal
pressurization to ⬎30 mm Hg
● Impaired EGJ relaxation (IRP ⬎17 mm Hg)
● Absent peristalsis
● ⱖ20% swallows with premature contractions
(distal latency ⬍4.5 s)
● Impaired EGJ relaxation (IRP ⬎15 mm Hg)
● Some preserved weak or normal peristalsisa
additional dilations, but subsequent response to myotomy is
not influenced. The major complication of pneumatic dilation
is esophageal perforation. Although the reported incidence of
perforation from pneumatic dilation ranges from 0% to 16%, a
recent systematic review on the topic concluded that by using
modern technique, the risk was less than 1%, comparable to the
risk of unrecognized perforation during Heller myotomy.32 Fur-
thermore, most perforations are clinically obvious, “limited”
perforations can be managed conservatively, and major perfo-
rations recognized and surgically repaired within 6 – 8 hours
have outcomes comparable to patients undergoing elective
Heller myotomy.
Although there is no standardized approach to the tech-
nique of pneumatic dilation, there are some basic principles
that should be followed (Table 2). Pneumatic dilation should be
performed by experienced physicians who are comfortable with
the technique, and surgical backup is requisite. The patient
should have appropriate dietary instructions before the proce-
dure so that there is minimal residual food in the esophagus
during the procedure. The balloon dilator is completely de-
flated before both passage and withdrawal by using a T-piece
and large syringe to minimize trauma to the oropharynx. Pneu-
matic dilation requires concomitant endoscopy and fluoros-
copy to place and visualize the guidewire and to verify appro-
priate balloon position. Our practice has been to use stiff
spring-tip Savary guidewires (Wilson-Cook Medical, Winston-
Salem, NC) rather than the flimsy wires provided by the man-
ufacturer. The balloon size is chosen by using a graded ap-
proach, starting with a 30-mm balloon and increasing to the
35-mm size if patients do not respond. Neither author recom-
mends using the 40-mm balloon because of data suggesting
unacceptable perforation rates; they prefer to refer patients
failing 35-mm dilation for myotomy. Accurate placement of the
balloon is crucial to the effectiveness of the procedure, and this
must be verified fluoroscopically during the initial stages of
892 PANDOLFINO AND KAHRILAS CLINICAL GASTROENTEROLOGY AND HEPATOLOGY Vol. 11, No. 8

Figure 3. Pseudoachalasia. The patient was referred for HRM on the basis of a presumed diagnosis of achalasia from the timed barium
esophagogram noting a dilated esophagus with esophageal retention at 5 minutes and a bird beak deformity at the EGJ. Although the patient
met criteria for type I achalasia, there was a strong vascular signal noted on the EPT plot that raised suspicion for vascular compression.
Endoscopic ultrasound revealed a large thoracic aneurysm compressing the distal esophagus. The patient underwent repair of the aneurysm,
and her esophageal function subsequently improved with no esophageal retention on esophagogram. With permission from the Esophageal
Center at Northwestern.

balloon inflation. The inflation pressure of the balloon is not
stipulated; full effacement of the sphincter on fluoroscopy is
the end point of interest, which is usually associated with
distention pressures of 8 –15 psi. Patients should be observed in
recovery for at least 2 hours with careful assessment for post-
procedure pain. A Gastrografin (Bracco Diagnostics Inc., Prince-
ton, NJ)/barium swallow study should be obtained if there is
any worry of perforation; one author (J.E.P.) prefers to obtain
studies in all patients. Patients should be explicitly advised to
seek care emergently if they develop fever, shortness of breath,
severe pain (especially if pleuritic), or subcutaneous emphy-
sema.
Studies that use pneumatic dilation as the initial treatment
of achalasia have reported excellent long-term symptom con-
trol. However, a third of patients will relapse in 4 – 6 years and
may require repeat dilation. Response to therapy may be related
to preprocedural clinical parameters such as age (favorable if
age ⬎45), gender (female ⬎ male),33 esophageal diameter (in-
versely related to response), and achalasia type (type II better
than I and III).10,21 Although surgical myotomy has a greater
response rate than a single pneumatic dilation, it appears that
a strategy that uses a series of dilations with the potential for
repeat is comparable to surgery and a reasonable alternative to
surgery. A recent randomized controlled trial compared this
type of graded strategy with surgical myotomy and found it to
be noninferior in efficacy.34
Myotomy
The standard surgical approach to achalasia is a Heller
myotomy, the essence of which is to divide the circular muscle
fibers of the LES. This can be achieved by laparotomy, thora-
cotomy, thoracoscopy, or laparoscopy. Laparoscopy is currently
the preferred approach on account of its lower morbidity and
comparable long-term outcome to that achieved with thoracot-
omy.35 Laparoscopic Heller myotomy is superior to a single
pneumatic dilation in terms of efficacy and durability, with
reported efficacy rates in the 90%–95% range.31 However, the
superiority of surgical myotomy over pneumatic dilation is less
evident when compared with a graded approach to pneumatic
dilation by using repeat dilations as mandated by the clinical
response.34,36
Similar to the case with pneumatic dilation, there is no
standardized approach to Heller myotomy in terms of the
specifics of the myotomy or the associated antireflux procedure.
The most common recommendation is for a single anterior
myotomy extending 2 cm onto the gastric cardia and about
7 cm in overall length. With respect to the antireflux repair, this
can range from none to an anterior 180° fundoplasty (Dor) to
August 2013 MANAGEMENT OF ACHALASIA 893

Table 2. Recommended Technique for Rigiflex Achalasia Dilation Procedure

Standard recommendation Suggested by some experts

Preprocedure preparation ● Fasting for at least 12 h ● Clear liquids for 24 h (or longer in instances of extreme
esophageal dilatation)
Sedation ● Same as for diagnostic EGD ● MAC anesthesia
Dilator size selection ● 30 mm unless this was previously unsuccessful, ● Initially use 35-mm balloon in young male patients
either within the past month or in prior treatment
series
Dilator positioning ● Over a stiff guidewire by using fluoroscopy to
localize the EGJ
Balloon inflation ● Inflate slowly, making sure to capture the ● Inflate to at least 8 psi
“waist” of the LES on the dilating surface
● Deflate and reposition if the waist is not visible
or is seen to migrate off the top of the balloon
● Maintain tension on the dilator during inflation to
resist the “pull” of the esophagus
Duration of inflation ● One inflation, slowly increasing balloon pressure ● Inflate for 15– 60 s
until the “waist” of the LES is seen to fully ● Repeat the dilation twice
efface on fluoroscopy; then fully deflate, aspirate
empty with a large syringe connected by a T-
piece, position the patient on their side, and
remove wire and dilator in unison
Postprocedure ● Observe in recovery for at least 2 h ● Routine Renografin swallow
● Renografin (Bracco Diagnostics Inc.) swallow ● Short course of as needed pain medications
before discharge if pain or other clinical ● Short course of proton pump inhibitors
parameters are concerning
Follow-up ● Assess efficacy at 2– 4 wk 6 mo, and 12 mo ● Repeat dilation at shorter intervals (2– 4 wk)
● Repeat dilation with 35-mm dilator if treatment
failure within 6 mo

Standard recommendations should be universally applied, while there is no consensus among experts on suggested recommendations and no
relevant controlled trial data exist.
EGD, esophagogastroduodenoscopy; MAC, monitored anesthesia care.

a 270° partial fundoplication (Toupet). There is general agree-
ment that a full 360° Nissen fundoplication is contraindicated.
A recent randomized controlled trial comparing the Dor pro-
cedure with Toupet fundoplication along with myotomy found
no significant difference in abnormal pH testing between the 2
groups and similar dysphagia rates.37 The current recommen-
dation from the Society of American Gastrointestinal and En-
doscopic Surgeons is that patients who undergo myotomy
should have a fundoplication to prevent reflux, without speci-
fying which procedure.38
Per Oral Endoscopic Myotomy
Although the current treatments for achalasia are ex-
tremely effective, both pneumatic dilation and laparoscopic
myotomy are associated with a perforation risk of about 1%,
and laparoscopic Heller myotomy still requires laparoscopy and
surgical dissection of the EGJ. Consequently, there has been
interest in developing a hybrid technique incorporating an
endoscopic approach, but applying principles of natural orifice
transluminal endoscopic surgery to perform a myotomy. This
technique termed per oral endoscopic myotomy, or POEM, was
initially described by Pashricha et al39 and subsequently devel-
oped by Inoue et al40 in Japan. The procedure requires making
a transverse mucosal incision in the mid-esophagus, entering it,
and creating a submucosal tunnel all the way to the gastric
cardia by using a forward viewing endoscope with a transparent
distal cap and a triangular endoscopic submucosal dissection
knife. Once the tunnel is complete, the endoscope is removed,
and its adequacy is assessed by luminal inspection of the EGJ
and proximal stomach (dye is instilled into the tunnel so that
its path is visible intraluminally). The tunnel is then reentered,
and selective myotomy of the circular muscle is accomplished
with electrocautery tools for a minimum length of 6 cm up the
esophagus and 2 cm distal to the squamocolumnar junction
onto the gastric cardia. The endoscope is then withdrawn,
collapsing the tunnel, and endoclips are used to seal the entry
incision. Initial reports of success rates of the POEM procedure
in prospective cohorts of achalasia patients have been greater
than 90%, comparable to those of laparoscopic Heller myot-
omy.41– 43 At this time, there have been no randomized prospec-
tive comparison trials of POEM with either laparoscopic myot-
omy or pneumatic dilation. Hence, although POEM is clearly a
very promising technique, its relative efficacy compared with
the well-studied alternatives of pneumatic dilation or laparo-
scopic Heller myotomy in terms of long-term dysphagia con-
trol, progression of esophageal dilatation, and postprocedure
reflux remain to be established. However, our preliminary re-
sults comparing more than 30 POEM cases with laparoscopic
Heller myotomy suggest comparable perioperative outcomes.44
Esophagogastric Junction Stents
Another logical extension of endoscopic techniques for
achalasia treatment would be developing a stent that could be
placed across the EGJ to maintain opening while limiting re-
flux. A randomized study of a custom, partially covered, remov-
able metallic stent vs pneumatic dilation was recently reported.
894 PANDOLFINO AND KAHRILAS
Although the dilation protocol used was much less aggressive
than standard technique used in the United States and Europe,
the 30-mm stent had an 83% success rate at 10 years compared
with success rates of 0% for pneumatic dilation.45 Although
these results seem promising, this treatment cannot currently
be recommended because the data are limited, and the device is
not available in the United States.
Post-treatment Follow-up
Patients should have a postprocedure evaluation of
effectiveness within the first 3 months after the intervention to
assess adequacy of functional and symptom response. The im-
portance of combining subjective findings of symptom reduc-
tion and objective findings evaluating esophageal retention and
continued EGJ outflow obstruction was highlighted in work
published by Vaezi et al46 that assessed long-term outcome in
patients after pneumatic dilation. They reported that patients
with concordance of symptom improvement and minimal bo-
lus retention on timed barium esophagogram had good long-
term improvement, whereas patients with discordance of im-
proved symptoms but poor bolus emptying on timed barium
esophagogram had a worse long-term prognosis and were more
prone to return with symptoms.
Timed Barium Esophagogram
A major objective of treating achalasia is to improve
esophageal emptying, thereby reducing regurgitation, aspira-
tion risk, and progressive esophageal dilatation. Thus, it is
logical that a timed barium esophagogram be incorporated into
the post-treatment assessment. A timed barium esophagogram
is done by having the patient drink 200 mL thin barium and
obtaining single images to assess bolus retention at 1, 2, and 5
minutes.47 If the barium has completely emptied from the
esophagus at 1 or 2 minutes, there is no need to continue
imaging the esophagus. Studies have shown that the result of a
postintervention timed barium esophagogram predicts treat-
ment success and the requirement for future intervention. Vaezi
et al47 first reported a significant association between the result
of the timed barium esophagogram and symptom resolution.
More importantly, they identified a group of patients with poor
esophageal emptying in the context of almost complete symp-
tom resolution. That study was followed by a report that timed
barium esophagogram was predictive of treatment failure at 1
year regardless of symptom outcome.46 Although these data do
not mandate an intervention that is based only on the outcome
of the timed barium esophagogram, they do support more
aggressive follow-up of patients with barium retention on
timed barium esophagogram, irrespective of reported symp-
toms.
Manometry
Because the demonstration of abnormal EGJ relaxation
is the cornerstone of the diagnosis of achalasia, it is logical to
incorporate an assessment of EGJ function in the post-treat-
ment follow-up. Supportive of this, a prospective study assess-
ing 54 patients found that patients with a post-treatment basal
EGJ pressure of less than 10 mm Hg were much more likely to
be in remission (100% vs 23%) at 10 years.48 Recent data ob-
tained by using HRM and EPT that used the IRP metric also
support this concept. A recent study by Nicodème et al49 of
achalasia treatment response after pneumatic dilation or myot-
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY Vol. 11, No. 8
omy reported that the post-treatment IRP ⬍15 mm Hg was
associated with lower Eckardt scores and less esophageal reten-
tion on timed barium esophagogram, demonstrating that this
metric is reflective of the degree of LES disruption achieved
with treatment. Another interesting observation in that study
was that the manometric finding of weak peristalsis after inter-
vention was also predictive of a successful outcome.
Post-treatment Symptoms
Patients with post-treatment symptoms should be eval-
uated on the basis of the specific symptom, their preinterven-
tion anatomy, and achalasia subtype. Patients with continued
dysphagia, chest pain, and regurgitation after treatment should
be evaluated to determine the effectiveness of the intervention
in relieving EGJ outflow obstruction and improving esophageal
emptying. Patients with significant bolus retention on timed
barium esophagogram or continued EGJ outflow obstruction
defined by an IRP ⬎15 mm Hg on high-resolution manometry
should be considered treatment failures.
Patients who have failed pneumatic dilation may be referred
for a repeat dilation with a larger balloon or myotomy. Patients
who have undergone myotomy with an antireflux procedure
should be evaluated for pseudoachalasia related to the fundo-
plication before repeat therapy is attempted. This can be as-
sessed by performing manometry with amyl nitrite challenge to
distinguish between an incomplete myotomy (EGJ relaxes with
amyl nitrite) or a fixed obstruction related to the wrap (EGJ
pressure does not change with amyl nitrite).50 Patients with
pseudoachalasia related to the fundoplication should also un-
dergo endoscopy to determine whether the nature of the ob-
struction mandates surgical intervention (slipped fundoplica-
tion, paraesophageal hernia) vs an attempt at dilation (20 mm
or pneumatic).
Patients with type III achalasia deserve special mention be-
cause these patients can be very difficult to manage. As cur-
rently defined, the key criterion for type III achalasia is 2 or
more swallows with a premature contraction (distal latency
⬍4.5 seconds).51 On the basis of the authors’ experience, it is
highly likely that the spastic contractions (and chest pain) will
persist after pneumatic dilation or myotomy. Thus, these pa-
tients should be counseled accordingly, and treatment with
antispasmodics should be attempted.
Patients may also develop esophagitis or new reflux symp-
toms after having a definitive treatment for achalasia, and
initiating proton pump inhibitor therapy in patients reporting
heartburn is reasonable. Endoscopy may also be helpful in
detecting esophagitis as a potential cause of poor treatment
response, especially in those patients who do not respond to
proton pump inhibitors. Routine reflux testing is not indicated
unless patients are refractory to proton pump inhibitor therapy,
have no relevant endoscopic findings, and have effective acha-
lasia treatment defined by manometry and/or timed barium
esophagogram.
Long-term Follow-up
Achalasia is not cured by current treatments, and pa-
tients should be monitored for progression of esophageal dil-
atation and development of megaesophagus or sigmoid esoph-
agus, often referred to as end-stage achalasia. The criteria for
end-stage achalasia, a condition potentially leading to esopha-
gectomy, are not standardized and somewhat controversial.52
August 2013 MANAGEMENT OF ACHALASIA 895

Severe achalasia is variably defined by a diameter greater than 6
cm or by distal angulation and sigmoid deformity on esopha-
gogram. These findings can be associated with an inability to
empty the esophagus because of a “sink trap” effect, wherein
the dependent portion of the esophagus resides inferior to the
LES and will not empty by gravity. These features of end-stage
achalasia increase the odds of failure for surgical myotomy.53
Surgical series report that an estimated 10%–15% of patients
who have undergone treatment for achalasia eventually fulfill
criteria for end-stage achalasia54 and that up to 5% of patients
may require esophagectomy.55 However, these are historical
data and likely not reflective of current management practices.
Nonetheless, even though it seems logical that successful treat-
ment of achalasia reduces the risk of developing end-stage
achalasia, there are no relevant substantiating data. Similarly,
there are no data supporting that routine of follow-up studies
(endoscopy, barium studies, manometry, esophageal scintigra-
phy) predict or prevent progression to end-stage achalasia.
Endoscopic Surveillance for Cancer
The risk of esophageal squamous cell carcinoma is
increased in achalasia, which is likely related to poor esophageal
emptying and chronic stasis esophagitis leading to dysplasia
and carcinoma. The hazard ratio for developing cancer is esti-
mated to be approximately 28, and this is associated with an
incidence of 1 cancer per 300 patient-years.56 Fortunately, the
overall number of cancers is low; more than 400 surveillance
endoscopies would be required to detect 1 cancer in male sub-
jects, the higher-risk gender.57 In addition, survival of these
patients is poor once the diagnosis is made,56 and surveillance
endoscopy in the setting of esophageal retention and stasis
esophagitis is very difficult. Consequently, the latest American
Society of Gastrointestinal Endoscopy guidelines do not advo-
cate endoscopic surveillance for achalasia patients; however,
they state that if surveillance was considered, it would be rea-
sonable to begin after 15 years of the onset of symptoms.58 In
our practice, there are divergent opinions regarding the role of
surveillance, with one author (J.E.P.) performing endoscopic
surveillance at 3-year intervals, and the other author (P.J.K.)
reserving endoscopy for patients who develop symptoms during
follow-up.
Conclusion
Although achalasia is the best-defined esophageal mo-
tility disorder, the presentation can be heterogeneous in terms
of presenting symptoms and esophageal contractile patterns,
which can result in a delayed or missed diagnosis. The com-
monality among all phenotypes is of EGJ outflow obstruction
attributable to impaired LES relaxation, but the associated
esophageal contractility can be of a completely flaccid esopha-
gus, spasm, panesophageal pressurization, weak or even normal

Figure 4. Algorithm for the diagnosis of achalasia. Suspicion for achalasia should be high in patients presenting with dysphagia, especially when
accompanied by chest pain or regurgitation in the context of a normal upper endoscopy that has ruled out structural or mucosal (eosinophilic
esophagitis) abnormalities. In addition, findings on endoscopy, such as esophageal dilatation, retained food, or resistance at the EGJ, should raise
suspicion for achalasia, and a careful retroflexed view of the EGJ is mandatory to evaluate for a subtle tumor. Once the suspicion of achalasia is
raised, HRM is indicated to assess EGJ relaxation peristalsis. Six EPT diagnoses are potentially consistent with achalasia, with the caveats indicated
on the flow chart. CT, computed tomography; EGD, esophagogastroduodenoscopy; EUS, endoscopic ultrasound; GERD, gastroesophageal reflux
disease.
896 PANDOLFINO AND KAHRILAS
peristalsis. Thus, the detection of achalasia requires vigilance
and an understanding of the varied EPT phenotypes to confirm
the diagnosis. This is clinically important because achalasia is
unique among esophageal motility disorders in having specific
effective therapies, disruption of the LES by either pneumatic
dilation or surgical myotomy. Once a diagnosis of achalasia is
made, definitive therapy aimed at relieving EGJ outflow ob-
struction should be offered, assuming the patient is a good
surgical candidate. Rendering a definitive therapy earlier rather
than later potentially halts the progressive esophageal dilata-
tion that ultimately leads to end-stage achalasia, a condition
with substantial morbidity and relatively poor therapeutic op-
tions. Medical therapies (Botox injection, calcium channel
blockers, nitrates, 5=-phosphodiesterase inhibitors) should be
used only for poor surgical candidates or patients with equiv-
ocal diagnoses even after exhaustive evaluation. Figure 4 details
the diagnostic considerations most relevant to each potential
achalasia phenotype. Short-term follow-up after pneumatic di-
lation or myotomy should assess for therapeutic efficacy in
alleviating EGJ outflow obstruction and for potential treatment
complications. Long-term follow-up after treatment is contro-
versial but is advocated by some authorities to assess for pro-
gressive esophageal dilatation and to control for the increased
risk of esophageal squamous cell cancer.
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Reprint requests
Address requests for reprints to: Dr John E. Pandolfino, Northwest-
ern University, Feinberg School of Medicine, Department of Medicine,
676 St Clair Street, Suite 1400, Chicago, Illinois 60611-2951. e-mail:
j-pandolfino@northwestern.edu; fax: (312) 695-3999.
Conflicts of interest
The authors disclose no conflicts.