GASTROENTEROLOGY 1999;117:233254
AGA Technical Review on Treatment of Patients
With Dysphagia Caused by Benign Disorders
of the Distal Esophagus
This literature review and the recommendations therein were prepared for the American Gastroenterological Association Clinical
Practice and Practice Economics Committee. The paper was approved by the committee on September 27, 1998.
ysphagia is the perception that there is an impedi-
D ment to the normal passage of swallowed material.
Odynophagia is the sensation of pain on swallowing.
Dysphagia can be caused by a number of disorders,
benign and malignant, that involve either the oropharynx
or the esophagus. The purpose of this report is to develop
a rational approach to the treatment of adult patients who
have dysphagia caused by benign disorders of the distal
esophagus. The report provides a critical review of
pertinent literature on which to base this approach.
Patient care strategies that emerge from the review are
summarized in the accompanying American Gastroenter-
ological Association (AGA) Medical Position Statement.
For this report, the distal esophagus is defined, somewhat
arbitrarily, as the segment of esophagus that extends from
the level of the aortic arch to the gastric cardia. The
muscularis propria in this esophageal segment is com-
posed predominantly of smooth muscle.1 Thus the distal
esophagus is susceptible to three general categories of
disease processes that can cause dysphagia (Table 1): (1)
mucosal (intrinsic) diseases that narrow the lumen of the
esophagus through inflammation, fibrosis, or neoplasia;
(2) mediastinal (extrinsic) diseases that encase and ob-
struct the esophagus by direct invasion or through lymph
node enlargement; and (3) diseases affecting the esopha-
geal smooth muscle and its innervation that disrupt
peristalsis, interfere with lower esophageal sphincter
relaxation, or both. This review considers clinical reports
on these disorders that have been published in peer-
reviewed journals since 1966. The reports were identified
primarily by a MEDLINE search using the following
MeSH terms: deglutition disorders, esophageal dysphagia,
esophageal stenosis, esophageal motility disorders, and esopha-
geal achalasia. Clinical studies published only in abstract
form are not included. However, even the peer-reviewed
literature on the treatment of patients with dysphagia
due to benign esophageal disorders consists predomi-
nantly of retrospective, uncontrolled studies of small,
heterogeneous patient populations who were followed up
only briefly. The conclusions that can be drawn from
these reports often are limited, and the serious deficien-
cies in study design and execution often preclude mean-
ingful meta-analyses. This report highlights the strengths
and weaknesses of the most relevant studies.
Diagnosis
History
It has been estimated that the cause of dysphagia
can be determined with an accuracy of approximately
80% on the basis of a careful history alone.2 Some key
elements of the history for patients with dysphagia are
highlighted below.
Is the dysphagia for solid foods, liquids, or both?
Mucosal and mediastinal diseases that involve the distal
esophagus cause dysphagia by narrowing the esophageal
lumen. Such narrowings usually pose little barrier to the
passage of liquids, and consequently these diseases charac-
teristically cause dysphagia only for solid foods.3 In
contrast, diseases that disrupt peristalsis by affecting the
smooth muscle and its innervation may cause dysphagia
for both solids and liquids. In achalasia, persistent
contraction of the lower esophageal sphincter (LES)
causes complete mechanical obstruction of the esophagus
that persists until either the sphincter relaxes or the
hydrostatic pressure of the retained material exceeds the
pressure generated by the sphincter muscle. Even in the
absence of peristalsis, gravity often can empty the
esophagus of liquid material effectively if the LES is
relaxed. Therefore, patients who have disordered peristal-
sis and profoundly hypotensive LESs often experience no
dysphagia or dysphagia only for solid foods.4,5
Where does the patient perceive that ingested
material sticks? Patients with esophageal obstruction
often perceive that swallowed material sticks at a point
that is either above or at the level of the lesion causing the
obstruction.68 In a recent radiographic study of 12
patients with lower esophageal mucosal rings that im-
peded the passage of a marshmallow bolus, the perceived
level of the obstruction was localized to the neck in 7, to
the sternal angle in 2, to the midchest in 2, and to the
lower chest in 1.7 It is uncommon for patients to perceive
234 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
Table 1. Diseases of the Distal Esophagus That Cause
Dysphagia
Mucosal diseases
GERD (peptic stricture)
Esophageal rings
Esophageal tumors
Caustic injury (e.g., lye ingestion, pill esophagitis, sclerotherapy)
Radiation injury
Infectious esophagitis
Mediastinal diseases
Tumors (e.g., lung cancer, lymphoma)
Infections (e.g., tuberculosis, histoplasmosis)
Diseases affecting smooth muscle and its innervation
Achalasia
Scleroderma
Other motility disorders
that swallowed material sticks at a level substantially
below that of the obstructing lesion. In a recent endo-
scopic study of 139 patients with dysphagia caused by
esophageal strictures, the patients perception of the level
of obstruction agreed with the endoscopists localization
of the stricture with an accuracy of 4 cm in 74% of
cases.8 Fifteen percent of patients with strictures of the
distal esophagus localized obstruction to the proximal
esophagus, whereas only 5% of patients with proximal
esophageal strictures perceived obstruction in the distal
esophagus. Thus, the perception that a swallowed bolus
sticks above the suprasternal notch is of little value for
localization of the obstruction because this sensation
could be caused by a lesion located anywhere from the
pharynx to the most distal esophagus. However, if the
patient localizes the obstruction to a point below the
suprasternal notch, the chances are excellent that the
dysphagia is caused by a disorder that involves the distal
esophagus.
Are there symptoms of oropharyngeal dysfunc-
tion? Dysphagia caused by oropharyngeal dysfunction is
the subject of another AGA Technical Review9 and is not
discussed in detail in this report. Oropharyngeal dyspha-
gia often results from diseases that affect the striated
muscles of the oropharynx or their innervation (e.g.,
muscular dystrophies, cerebrovascular accidents). Pa-
tients with these neuromuscular diseases may experience
difficulty in initiating swallowing, and swallowing may
be accompanied by nasopharyngeal regurgitation, pulmo-
nary aspiration, and a sensation that residual material
remains in the pharynx. If any of these symptoms are
prominent, evaluation for oropharyngeal dysfunction
may precede tests for esophageal disorders.
Is the dysphagia intermittent or progressive?
Patients who have lower esophageal mucosal (Schatzki)
rings typically complain of dysphagia that is intermittent
and nonprogressive. These patients characteristically ex-
GASTROENTEROLOGY Vol. 117, No. 1
perience discrete, short-lived episodes of dysphagia for
solid foods, often during a meal in a restaurant (hence the
term steak-house syndrome) or at a social function.
Episodes may be separated by weeks, months, or years,
and the patient typically experiences no swallowing
difficulty between discrete episodes. In contrast, esopha-
geal strictures usually cause dysphagia that is progressive
in frequency and severity. With benign strictures, the
progression is typically slow and insidious (over a period
of months to years), and weight loss is minimal. Malig-
nant esophageal strictures usually cause dysphagia that
progresses rapidly (over a period of weeks to months), and
weight loss may be profound.
Is there a history of chronic heartburn? Heart-
burn is the cardinal symptom of gastroesophageal reflux
disease (GERD), and a history of chronic heartburn
supports the possibility that dysphagia may be caused by
a peptic esophageal stricture. However, the history of
pyrosis should be interpreted with caution because the
sensation of burning, substernal chest discomfort is not
specific for GERD. For example, patients with achalasia
frequently complain of a heartburn sensation that may be
caused by abnormal motor activity as well as by esopha-
geal acid exposure.10 Conversely, many patients who
develop peptic strictures as a result of GERD have no
antecedent history of heartburn. In one study of 154
patients with benign (mostly peptic) esophageal stric-
tures, for example, only 75% of the patients related a
history of chronic heartburn.11 Finally, approximately
two thirds of patients with dysphagia caused by adenocar-
cinoma in Barretts esophagus have a history of long-
standing heartburn.12 Although the history of heartburn
provides useful clinical information, conclusions regard-
ing the etiology of dysphagia in an individual patient
should not be based primarily on the presence or absence
of heartburn.
Has the patient taken medications likely to
cause pill esophagitis? A number of medications taken
in pill form are potentially caustic to the esophagus and
can cause deep ulceration with stricture formation if they
have prolonged contact with the esophageal mucosa.
Although the list of medications that can cause pill
esophagitis is long, most cases reported in the United
States have been caused by antibiotics (e.g., doxycycline),
potassium chloride preparations, nonsteroidal anti-
inflammatory drugs (NSAIDs), or quinidine.13 Recently,
a number of cases of pill esophagitis have been attributed
to alendronate sodium, an agent used in the treatment of
osteoporosis in postmenopausal women.14
Is there a history of collagen-vascular disease?
Collagen-vascular diseases such as scleroderma, rheuma-
July 1999
toid arthritis, and systemic lupus erythematosus can
affect the distal esophagus and cause disordered motil-
ity.15 Esophageal dysmotility in these disorders is often,
but not invariably, associated with Raynauds phenom-
enon.15,16 In scleroderma and related collagen-vascular
disorders, fibrosis and vascular obliteration in gut smooth
muscle cause poor esophageal contractility and weakness
of the LES that predisposes to severe GERD.17 In
addition, patients with collagen-vascular disease often are
treated with medications such as NSAIDs that can cause
pill esophagitis. Consequently, dysphagia associated with
collagen-vascular disease may be the result of disordered
esophageal motility, severe GERD, pill esophagitis, or
some combination of these abnormalities. Although it
seems logical to assume that pill esophagitis should occur
more frequently in patients with esophageal motor
disorders, there are few published data to support this
notion.
Is the patient immunosuppressed? Infectious
esophagitis occurs frequently in patients whose immune
system has been compromised severely by infection with
the human immunodeficiency virus, by advanced malig-
nancy, or by organ transplantation with the administra-
tion of potent immunosuppressive drugs. It has been esti-
mated that 30%40% of patients who have the acquired
immunodeficiency syndrome develop symptoms of esopha-
geal disease.18 Most esophageal infections are caused by
one or a combination of only three organisms: candida,
cytomegalovirus, and herpes simplex virus.19 Odynopha-
gia is usually the predominant symptom for patients with
infectious esophagitis, but most patients experience
dysphagia as well.19 Esophageal stricturing is an uncom-
mon late complication of infectious esophagitis.20
Physical Examination
Physical examination is important for assessment
of the patients nutritional status and ability to tolerate
the invasive procedures that may be necessary to manage
the esophageal disorder. However, the physical examina-
tion infrequently provides specific clues to the cause of
dysphagia. For patients with dysphagia caused by collagen-
vascular diseases, physical examination may show charac-
teristic features such as joint abnormalities, calcinosis,
telangiectasias, sclerodactyly, and rashes. A palpable left
supraclavicular (Virchows) lymph node suggests dyspha-
gia caused by a malignancy within the abdomen (e.g.,
adenocarcinoma of the esophagogastric junction). Diffuse
dental erosions may be a sign of GERD.21 Finally, the
physical examination may show evidence of neuromuscu-
lar disorders that can interfere with swallowing such as
Parkinsons disease, although most neuromuscular dis-
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 235
eases that cause dysphagia do so by involving the striated
muscle of the oropharynx and proximal esophagus (not
the smooth muscle of the distal esophagus).
Barium Swallow
For decades, physicians have debated whether
barium swallows should be performed early in the
evaluation of esophageal dysphagia, or whether it is more
cost-effective to bypass the radiographic examination
entirely and proceed directly to endoscopic evaluation.
Proponents of the latter approach argue that endoscopy is
almost always required in the evaluation of esophageal
dysphagia for both diagnostic and therapeutic purposes
and that barium swallows usually do not provide enough
additional information to justify the expense, inconve-
nience, and potential risk from radiation exposure. Those
who advocate the former approach contend that a well
done barium swallow provides valuable anatomic informa-
tion about the esophagus that may help to direct therapy
and prevent procedural complications. In the absence of
studies validating the cost-efficacy of either approach,
this debate will continue.
Despite the lack of data on cost-efficacy, a number of
observations suggest that the practice of early radio-
graphic evaluation for patients with esophageal dyspha-
gia is useful. Barium contrast examination appears to be
more sensitive than endoscopy for the detection of subtle
narrowings of the esophagus such as those caused by rings
and by peptic strictures that are 10 mm in diam-
eter.2224 In one study of 60 patients with lower esopha-
geal rings, for example, barium swallows showed the
rings in 95% of cases, whereas rings were demonstrated
by endoscopic examination in only 58% of the patients.22
However, this study is more than 12 years old, and few
data have been published that confirm these observations.
Having the patient perform a Valsalva maneuver or
swallow a solid bolus such as a marshmallow may increase
the sensitivity of the radiographic evaluation for detec-
tion of structural and functional lesions of the esopha-
gus.25,26 Furthermore, fluoroscopic examination can iden-
tify abnormalities in esophageal motility.2729 When the
patient swallows barium while in the supine or right
oblique position, the fluoroscopist can assess the efficacy
of esophageal peristalsis. Barium swallow may be espe-
cially helpful in suggesting the diagnoses of achalasia and
diffuse esophageal spasm, conditions that may be difficult
to identify endoscopically in early cases. In one study that
assessed the accuracy of esophageal radiography in pa-
tients with manometrically verified esophageal motility
disorders, for example, barium swallows identified achala-
sia in 18 of 19 cases (95%) and diffuse esophageal spasm
236 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
in 5 of 7 cases (71%).28 Early radiographic demonstration
of achalasia may prevent the situation in which endo-
scopic examination is performed initially for diagnostic
purposes and then repeated later for therapy because the
endoscopist either did not recognize the disorder or was
not prepared to perform a pneumatic dilation or botuli-
num toxin injection on the initial evaluation. Barium
swallow can identify lesions that may pose potential
hazards or create confusion for the endoscopist such as
large Zenkers or epiphrenic diverticula or large para-
esophageal hernias. For patients with esophageal stric-
tures, barium esophagram can provide information on the
length and tightness of the lesion that may be helpful in
choosing the type of dilator to be used for treatment and
in deciding whether dilation should be done with
fluoroscopic guidance (see below). Finally, initial barium
swallow provides an objective baseline record of the
esophagus that can be useful in assessing the response to
therapy or progression of disease. Despite all these
proposed advantages of early radiographic evaluation,
however, no study yet has verified the contention that
barium swallow performed before endoscopy decreases
complications or improves outcome.
Endoscopy
Unless contraindicated by serious comorbidity,
endoscopic evaluation is recommended for most patients
with dysphagia of esophageal origin to establish or
confirm a diagnosis, to seek evidence of esophagitis, to
exclude malignancy, and when appropriate to implement
therapy. Unlike the radiologist, the endoscopist can
obtain biopsy and brush cytology specimens of esopha-
geal lesions that may establish a diagnosis of neoplasms or
specific infections. Endoscopy is more sensitive than
radiology for identification of subtle mucosal lesions of
the esophagus (e.g., mild esophagitis caused by gastro-
esophageal reflux or infection).30 The precise sensitivity
of endoscopy for identification of mucosal lesions is not
entirely clear, however, because endoscopy often has been
used as the gold standard for establishment of the
presence of mucosal disease. Therefore, if a subtle mucosal
lesion is missed by endoscopic examination, it would
probably be missed or dismissed as a spurious finding on
an alternative diagnostic study such as barium swallow.
The acceptance of endoscopy as a gold standard test for
mucosal disease also results in bias in evaluation of the
sensitivity of other diagnostic modalities. For example,
reported estimates on the sensitivity of barium swallow
for identification of moderate esophagitis range between
79% and 93%.30 Because endoscopy was used as the gold
standard for mucosal disease in these studies, the sensitiv-
GASTROENTEROLOGY Vol. 117, No. 1
ity of radiology could not possibly exceed that of
endoscopy.
Esophageal Manometry
The clinical use of esophageal manometry is the
subject of a recent AGA technical review, and the reader
is referred to that report for details about the procedure.31
Esophageal manometry is the gold standard test for
esophageal motility disorders. Esophageal manometry
has been shown to be especially useful for establishment
of diagnoses of achalasia and diffuse esophageal spasm and
for detection of esophageal motor abnormalities associ-
ated with collagen-vascular diseases.31
For patients with dysphagia of esophageal origin,
history and results of barium swallow or endoscopy can be
used to decide whether esophageal manometry is neces-
sary. Esophageal motility study usually is not needed at
all for patients with mechanical causes of dysphagia such
as strictures or rings. These patients can be treated with
esophageal dilation and antireflux therapy if necessary,
and manometry can be considered for those whose
dysphagia persists despite adequate treatment of the
mechanical and inflammatory lesions. For patients thought
to have dysphagia caused by motility disorders other than
achalasia, it usually is not critical that manometry
precede endoscopy because there are no specific endo-
scopic therapies for these disorders. Therefore, these
patients generally will not require second endoscopy
solely for therapeutic purposes, as might occur if the
diagnosis of achalasia were not established before the
endoscopic evaluation. Endoscopy is performed in pa-
tients with motility disorders to assess the degree of
esophagitis and to seek mechanical lesions (e.g., esopha-
geal rings) that might be contributing to the dysphagia.
Any mechanical lesions noted at endoscopy can be treated
during or immediately after the procedure, irrespective of
the precise nature of the underlying motility problem.
For patients thought to have dysphagia as a result of
motility abnormalities associated with collagen-vascular
diseases, manometry need not be performed routinely if
dysphagia disappears with treatment of any associated
reflux esophagitis and esophageal stenoses. For patients
whose dysphagia persists despite such treatment, manom-
etry can establish the nature of the motility problem.31
However, it is not clear that the information provided by
esophageal manometry justifies the expense and inconve-
nience of the procedure, even in this setting. There are no
specific treatments for motility disorders other than
achalasia and its variants, so esophageal manometry often
does not alter patient treatment. One might argue that
the results of the motility study can be used to direct
therapy with prokinetic agents. According to this argu-
July 1999
ment, prokinetic agents that augment smooth muscle
contraction would be used only for patients with disor-
ders characterized by weak esophageal motility such as
scleroderma and would be avoided for patients with
spastic motility disorders such as diffuse esophageal
spasm. However, the esophageal effects of the few
available prokinetic agents often are only marginal for
patients with weak esophageal motility, and the effects
are not specific for any individual disorder.32 Although it
seems logical to assume that prokinetic agents would
have detrimental effects for patients with spastic motility
disorders, few published data support this notion. Many
patients with dysphagia caused by esophageal motility
disorders are treated empirically with prokinetic agents
without the benefit of a motility study, and it is not clear
that documentation of the disorder by manometric
examination has a substantial influence on patient man-
agement or outcome. One older study of 363 patients
referred for esophageal manometry concluded that the
procedure changed the course of treatment in only 4% of
cases.33 One recent report provides a more optimistic
assessment of the procedure, reporting that esophageal
manometric examination resulted in a change in patient
treatment in 49% of 268 patients referred to a motility
laboratory.34 However, this report provides no details on
precisely how the results of manometry changed treat-
ment. Furthermore, the investigators considered mano-
metric confirmation of certain clinical diagnoses to be a
change in treatment. Thus it is not clear that early
esophageal manometry for patients with conditions other
than achalasia is preferable to a course of empiric therapy
with prokinetic agents.
As discussed above, an esophageal motility study
ideally should precede endoscopy for patients thought to
have achalasia. Ordinarily, the motility catheter is passed
blindly through the nose or mouth into the stomach. In
patients with achalasia whose esophagus is dilated and
tortuous, however, it may not be possible to advance the
motility catheter blindly into the stomach because the
flexible catheter curls in the capacious esophagus rather
than passing through the hypertensive sphincter. In this
situation, the information on LES function necessary to
establish a manometric diagnosis of achalasia is not
available. Few published data guide clinicians on how to
deal with this problem. The options are (1) to pass the
motility catheter using fluoroscopic guidance; (2) to pass
the motility catheter over a guidewire placed endoscopi-
cally in the stomach; (3) to drag the motility catheter into
the stomach using endoscopic techniques; and (4) to
dispense with the motility study altogether. The first
three options for catheter passage can be cumbersome and
uncomfortable, and the success rate is not well docu-
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 237
mented. Furthermore, endoscopic methods for catheter
placement often require the administration of sedatives
that can influence esophageal motility. When the clini-
cian is unable to position the motility catheter success-
fully in a patient with suspected achalasia, it seems
reasonable to review the case critically before using
invasive techniques for catheter passage. If the diagnosis
of achalasia appears clear-cut based on typical clinical and
radiographic features, the motility study usually can be
deferred and endoscopy can be performed for diagnostic
and therapeutic purposes. For such patients, the diagnos-
tic problem usually involves distinction among primary
(idiopathic) achalasia, secondary achalasia (e.g., caused by
malignancy), and hypocontraction disorders (e.g., sclero-
derma) with peptic stricture formation. Manometry
cannot reliably distinguish primary and secondary achala-
sia, and endoscopy usually can differentiate between
achalasia (in which the esophageal narrowing caused by
the sphincter muscle can be traversed easily with gentle
pressure on the endoscope) and peptic stricture (in which
the fibrotic, and typically inflamed, stricture poses
substantial resistance to passage of the endoscope). Inva-
sive techniques for positioning of the manometry catheter
can be considered for patients who have clinical or
radiographic features that are suggestive but not entirely
typical of achalasia.
Videofluoroscopy
Videofluoroscopy, in which a motion recording is
made of swallows involving barium suspensions and
barium-coated materials, is an excellent technique for
assessment of oropharyngeal function. However, this
technique is of little value for evaluation of esophageal
disorders.35
Esophageal Transit Scintigraphy
In esophageal transit scintigraphy, patients swal-
low a radiolabeled liquid (e.g., water mixed with techne-
tium-99m sulfur colloid), and radioactivity within the
esophagus is measured over time using a gamma cam-
era.36 Patients with esophageal motility disorders typi-
cally have delayed disappearance of the radiolabel from
the esophagus. The test provides a quantitative estimate
on the efficacy of emptying in different regions of the
esophagus and is well tolerated by patients because it
requires no intubation. Radionuclide transit studies are
less sensitive and specific than manometry for establish-
ment of the diagnosis of specific esophageal motility
disorders, but scintigraphy provides information on bolus
transit through the esophagus that can complement
manometric data.29,36,37 Esophageal transit scintigraphy
presently is used primarily for research, and routine
238 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
application of this test is not yet recommended for
clinical purposes.
Treatment of Specific Disorders
Benign Esophageal Strictures
In most cases, benign strictures of the esophagus
are presumed to develop as sequelae of deep esophageal
ulcerations that stimulate fibrous tissue production and
collagen deposition.38 However, the precise mechanisms
that lead to stricture formation are not known, and it is
conceivable that some strictures develop as a result of
chronic esophageal inflammation even without mucosal
ulceration. It has been estimated that 60%70% of all
benign esophageal strictures in the United States are
peptic in origin, i.e., a consequence of reflux esophagi-
tis.39 Stricture formation also may complicate the esopha-
geal ulceration that can occur with caustic ingestion, pill
esophagitis, and infectious esophagitis. It is not clear that
mucosal ulceration is a requisite factor in the develop-
ment of radiation strictures of the esophagus. Most
reported series on the treatment of benign esophageal
strictures are composed predominantly or exclusively of
patients with peptic strictures, and few reports have
focused specifically on the treatment of fibrotic strictures
that are not peptic in origin.40 Consequently, published
guidelines on the management of benign esophageal
strictures are based primarily on the results of studies of
patients with peptic lesions.
In the era before proton pump inhibitors, peptic
strictures were widely regarded as fixed, fibrotic lesions
that would respond only to a mechanical therapy aimed at
stretching or tearing the fibrous tissue. Antireflux therapy
was used to control the symptoms of esophagitis and to
prevent progression of the stricture, but there was little
expectation that elimination of reflux esophagitis would
widen the established stenosis or improve dysphagia.
Clinical trials comparing treatment with H2-receptor
antagonists with placebo for patients with peptic esopha-
geal stenoses supported this notion of stricture immuta-
bility.4143 These studies showed a significant decrease in
reflux esophagitis in the patients treated with H2-
blockers but no reduction in the need for stricture
dilation. Although a small surgical series published in
1975 had shown a significant improvement in dysphagia
and a substantial increase in stricture diameter for
patients treated with antireflux surgery alone (without
stricture dilation), this report received relatively little
attention from gastroenterologists.44 Recent studies of
patients with peptic strictures have shown that chronic,
aggressive acid suppression therapy with proton pump
inhibitors both improves dysphagia and decreases the
GASTROENTEROLOGY Vol. 117, No. 1
need for subsequent esophageal dilations.4547 For ex-
ample, in one study of 366 patients with peptic esopha-
geal strictures who were randomly assigned to receive
medical therapy with either omeprazole (20 mg daily) or
ranitidine (150 mg twice daily) for 1 year after baseline
stricture dilation, repeat dilation was required in only
30% of patients in the omeprazole group compared with
46% in the ranitidine group (P 0.01).47 In a study
correlating esophageal stricture diameter (measured radio-
graphically), grade of esophagitis (determined endoscopi-
cally), and severity of dysphagia (estimated using a
numerical scoring system), Dakkak et al.48 found that
stricture diameter alone could explain only 30% of the
variation in dysphagia score, whereas the combination of
stricture diameter and severity of esophagitis could
account for 66% of that variation. The authors concluded
that the degree of esophagitis is as important as stricture
diameter in causing dysphagia. In summary, these studies
show a reversible component of reflux esophagitis that
contributes to dysphagia in some patients with peptic
esophageal strictures.4548 This esophagitis can be con-
trolled with either proton pump inhibitors or antireflux
surgery. For patients who remain symptomatic despite
treatment with proton pump inhibitors or fundoplica-
tion, 24-hour esophageal pH monitoring can be used to
document the adequacy of therapy in controlling acid
reflux.
In addition to aggressive antireflux therapy, patients
with benign esophageal strictures usually are treated (at
least initially) with dilation. Esophageal dilation has been
practiced since the 16th century, when physicians used
tapered wax wands to dislodge material stuck in the
esophagus.49 Indeed, the word bougie is derived from
Boujiyah, the name of an Algerian city that was the center
of the medieval wax candle trade. Today, three major
types of esophageal dilating devices are used com-
monly50: (1) mercury-filled bougies that are passed
blindly through the mouth (e.g., tapered-tipped Maloney
dilators, blunt-tipped Hurst dilators); (2) polyvinyl bou-
gies that can be passed over a fine guidewire that is
positioned within the stricture using either fluoroscopic
or endoscopic guidance (e.g., Savary dilators); and (3)
balloon dilators that are passed either over a guidewire or
through the endoscope (so called through-the-scope, or
TTS, balloons). The first two types of dilators are pushed
through the stenotic segment and thus deliver axially
directed shearing forces as well as radially directed
dilating forces to the stricture. In contrast, balloon
dilators deliver only radially directed dilating forces. In
theory, therefore, balloon dilators should stretch the
stricture uniformly while eliminating complications asso-
ciated with the application of shearing stresses.51 Despite
July 1999
these proposed advantages for balloon dilators over
bougies, no study yet published has demonstrated convinc-
ingly that any commonly used dilator is superior to
another in efficacy or safety. Indeed, historical data
suggest that mercury-filled rubber bougies may have the
best safety record for any of the dilator types, although no
meaningful comparative study has been conducted to
prove this contention.52 There are relatively few pub-
lished reports of studies directly comparing the different
dilator types for patients with esophageal strictures.5358
Among five randomized trials that compared balloon
dilation to push dilation (Table 2), three found a modest
advantage for push dilation, one found no difference
between the two, and one described a modest advantage
for balloon dilation. None of these studies has established
the superiority of one dilator type over another. In
considering the cost of dilation therapy, however, the
fragility of the TTS balloons is a disadvantage because
unlike bougies, the balloons break easily with repeated
usage.
Although many physicians have abandoned mercury-
filled bougies in favor of the newer balloon dilators and
guided polyvinyl bougies, a strong case can be made for
mercury-filled bougies as the dilators of choice for
esophageal strictures with diameters larger than 1012
mm.52,59,60 First, these bougies have a record of excellent
efficacy and safety that has been established over a period
of more than 80 years. Next, mercury dilators are passed
without a guidewire, and fluoroscopic guidance usually is
not necessary for dilation of simple strictures. Thus the
added time, inconvenience, expense, and risk associated
with guidewire passage and fluoroscopy are obviated.
Finally, mercury-filled bougies often can be passed with
minimal or no sedation, a major advantage for patients
who require frequent bougienage. The flexibility of the
mercury dilators that undoubtedly contributes to their
safety becomes a disadvantage in dilation of strictures
Table 2. Results of Randomized Trials Comparing Balloon and Push Dilators
No. of
Study ( yr) patients Dilators compared
Kelly49 (1988) 71 Balloon vs. Celestin and
EderPuestow
Tytgat50 (1990) 60 Balloon vs. SavaryGilliard
McBride and Ergun51 (1992) 71 Balloon vs. EderPuestow
Tulman and Boyce52 (1994)a 93 Balloon vs. Celestin and
EderPuestow
Cox et al.53 (1995) 34 Balloon vs. Savary
a This series includes the patients described in the study by Kelly.49
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 239
complicated by tightness, length, and tortuosity. Mer-
cury dilators with diameters of less than 10 mm (30F) are
so floppy that they tend to curl in the esophagus rather
than to traverse such complicated strictures. Therefore,
guided dilation using polyvinyl bougies or balloons may
be necessary for stenoses that are exceptionally tight,
long, or tortuous. Also, guided dilation should be
considered for patients in whom strictures are associated
with esophageal diverticula (either true or pseudodiver-
ticula) whose thin walls might be perforated by the tip of
a blindly passed dilator. The choice between balloons and
polyvinyl bougies for patients with complicated stric-
tures should be based on the availability of the dilators in
a given institution and on the operators experience and
comfort in using them because published experience has
not convincingly established the superiority of one
dilator type over another. Once an adequate esophageal
luminal diameter has been achieved using a guided
dilation technique, it may be possible to perform subse-
quent dilations using mercury-filled bougies.
Fluoroscopic guidance has been proposed as a means of
enhancing the safety of esophageal dilation, but the
published data to support this proposal are few and
unconvincing.52 Fluoroscopic guidance usually is not
necessary in dilation of esophageal strictures with mercury-
filled rubber bougies, but fluoroscopy can be helpful in
selected cases to ensure that the dilator has traversed the
stricture. In a study of 145 patients with peptic esopha-
geal strictures or Schatzkis rings treated with Maloney
dilators, fluoroscopy was found to alter the dilation
technique in 24%.61 Fluoroscopy was especially useful for
ensuring proper dilator passage in patients who had large
hiatal hernias. Fluoroscopic guidance is used commonly
for passing of bougies over a guidewire, but recent
retrospective studies have challenged the need for fluoros-
copy even in this setting.6264 Kadakia et al.62 used
endoscopy alone (without fluoroscopy) to pass a marked
Perforations Significant differences
0 Bougie modestly better than balloon for
reduction of dysphagia and maintenance
of stricture patency
0 Bougie modestly better than balloon for
reduction of dysphagia
None
1 Bougie, 1 balloon Bougie modestly better than balloon for
reduction of dysphagia and maintenance
of stricture patency
0 Balloon modestly better than bougie for pre-
vention of stricture recurrence; required
fewer treatment sessions, produced less
procedural discomfort
240 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
guidewire through esophageal strictures in 138 patients
and successfully performed bougienage over the guidewire
without fluoroscopic guidance and without complica-
tions in all of these cases. However, it is not clear whether
the relatively simple strictures in these patients could
have been dilated successfully even with no guidewire
using only mercury-filled rubber bougies. Kozarek et
al.64 found that fluoroscopic guidance was necessary in
only 8% of more than 300 patients who had esophageal
strictures dilated successfully with polyvinyl bougies.
Thus it appears that fluoroscopically guided dilation
should be necessary only in the minority of patients who
have complicated esophageal strictures or large hiatal
hernias.
Esophageal dilators are sized using the French gauge
system in which the dilators diameter can be estimated
using the following formula: Dilator Diameter (in mm)
French Gauge Size 3. For example, a 30F dilator has
a diameter of 10 mm. There is no clear consensus on the
optimal size to which a peptic stricture should be dilated.
For patients with Schatzki rings, dysphagia is the rule
when the ring diameter is less than 13 mm (39F) and
uncommon when the ring diameter exceeds 20 mm
(60F).65 In a number of published series of patients with
predominantly peptic esophageal strictures, progressive
dilation to a gauge size between 40 and 60F resulted in
good relief of dysphagia in approximately 85% of cases
with a very low rate of complications.11,6670 In theory,
successful dilation might decrease the mechanical barrier
to gastroesophageal reflux imposed by the stricture and
thereby result in an increase in heartburn and regurgita-
tion. One investigation on this issue did not find a
significant overall postdilatation increase in acid reflux
by protracted esophageal pH monitoring in a group of 10
patients with peptic strictures, although certain individu-
als in that group experienced a marked exacerbation of
acid reflux after the dilating procedure.71 With the
availability of highly effective antisecretory medications
such as proton pump inhibitors, concerns about exacerba-
tion of reflux disease should not be a major factor in
limiting the extent of esophageal dilation. Nevertheless,
it seems preferable to individualize dilation therapy
rather than to aim for the same arbitrary dilator size for
all patients. Although no study on benign strictures has
documented clearly that the risk of esophageal perfora-
tion increases with dilator sizes up to 60F, it seems logical
to assume that fibrotic strictures have a critical size
beyond which they cannot stretch without rupturing.
Furthermore, the extent of initial stricture dilation does
not seem to influence either stricture recurrence or the
requirement for subsequent dilation39; therefore, there is
little support for the concept that strictures should be
GASTROENTEROLOGY Vol. 117, No. 1
dilated aggressively to prevent recurrence. For a patient
who has experienced complete relief of dysphagia with
dilation to 48F, there is little to be gained by dilation to
larger diameters. Most patients experience good relief of
dysphagia when dilated to a bougie size between 40 and
54F. The extent of dilation in an individual patient
should be based on the symptomatic response to therapy
and on difficulties encountered during the dilation
procedure.
When dilating a stricture with bougies, the initial
choice of dilator size is based on an estimate of stricture
diameter provided by a barium swallow or endoscopic
examination. A more physiological approach to estima-
tion of stricture diameter involves having the patient
swallow barium spheres of known diameter,53 but this
technique is seldom used in clinical practice and has not
been shown to improve the results of dilation. It is
generally recommended that the first bougie passed have
a diameter approximately equal to that estimated for the
stricture.60 The rule of threes is a clinical maxim that
holds that no more than three bougies of progressively
increasing size should be passed at any one dilation
session to minimize the risks of esophageal perforation
and hemorrhage.52 Mercury-filled rubber bougies (for
which the rule of threes originally was formulated)
increase in size by 2F units, so the diameter of the third
bougie is only 1.3 mm larger than the first (e.g., dilation
of a stricture using 36, 38, and 40F bougies increases the
stricture diameter 1213.3 mm). Although the rule of
threes seems reasonable as a clinical guideline, no studies
verify that adherence to the rule improves dilation
efficacy or safety. Furthermore, with balloon dilation,
strictures are routinely dilated in one session to a
diameter far greater than that which could be achieved
with the passage of three bougies. Balloons are designed
to burst if a certain pressure is exceeded during dilation,
but it is not clear that the burst pressure is less than that
required to rupture the diseased esophagus. One report
described no perforations among 35 patients with a mean
stricture diameter of 7.6 mm who underwent dilation in
one session using a 60F balloon.53 This represents a mean
increase in stricture diameter of 12.4 mm. To achieve this
degree of dilation with mercury dilators of progressively
increasing diameter would require the passage of at least
18 bougies. No study has systematically evaluated the
safety of passing so many bougies in one sitting. If one
elects to dilate a stricture with mercury-filled bougies
rather than balloons, it seems a reasonable concession to
the unvalidated rule of threes to pass bougies of progres-
sively increasing diameter until resistance is first encoun-
tered, and to pass no more than two bougies after that in
the same session. This may not be a reasonable approach
July 1999
when using polyvinyl dilators passed over a guidewire,
because these dilators may not provide the operator with
a meaningful tactile impression of stricture resistance.50
With polyvinyl dilators, the resistance to passage per-
ceived by the operator may be more a function of friction
produced by the guidewire than of resistance provided by
the esophageal stenosis. Nevertheless, esophageal dilation
using polyvinyl dilators has an excellent safety record,
and it is not clear that adherence to the rule of threes
enhances safety. In one recent study, benign esophageal
strictures in more than 400 patients were dilated using
either a single, large polyvinyl dilator (45F) or multiple
polyvinyl dilators so that the stricture diameter increased
by 2 mm in one session.64 Despite this flagrant
violation of the rule of threes, only one perforation was
observed in 662 dilation sessions. If one elects to dilate a
stricture using balloon dilators, it seems prudent to limit
the initial dilation to no more than 45F. Although a
previously mentioned report found no complications in a
small series of patients dilated initially with 60F bal-
loons,53 it seems preferable to be more conservative until
results of future studies on the safety and efficacy of
balloon dilation are available.
The major complications of esophageal dilation are
perforation and bleeding. These two complications ap-
pear to occur with approximately equal frequency, al-
though there is substantial variation among the reported
series. For example, the 1974 American Society for
Gastrointestinal Endoscopy survey found rates of perfora-
tion and bleeding of 0.1% and 0.3%, respectively, among
13,139 esophageal dilations performed with mercury-
filled rubber bougies.72 In contrast, Patterson et al.11
observed 5 perforations and only 1 hemorrhage during
esophageal dilation in 154 patients, most of whom had
multiple sessions of bougienage performed over a period
of up to 87 months. In another American Society for
Gastrointestinal Endoscopy survey conducted in 1984,
10 hemorrhages and 2 perforations were reported among
456 patients treated with balloon dilation of esophageal
strictures for an overall complication rate of 2.5%.73 It is
difficult to provide precise estimates on the rate of
complications for esophageal dilation because of inconsis-
tencies in the available studies. The patient populations
in most reported series were heterogeneous, composed of
patients with strictures of varying complexity caused by a
number of different disease processes. It is likely that the
complication rate is highest for dilations performed for
strictures that are exceptionally tight, long, or tortuous,
but most reports do not supply specific information
regarding these stricture variables. If guided dilation
techniques are used preferentially for patients with
complex strictures (those most likely to be associated
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 241
with procedural complications), then guided dilation
may spuriously appear to be more dangerous than blind
bougienage. Many reports do not specify precisely the
criteria used for the choice of dilation technique, few
studies are randomized, and it is difficult to perform a
blinded trial of dilation therapy. With one or more of
these deficiencies present in virtually every reported
study on esophageal dilation, it is difficult to perform a
meaningful meta-analysis. Based on a review of the
flawed studies available, it appears that serious complica-
tions can be expected in approximately 0.5% of all
dilation procedures. With such a low rate of complica-
tions, a meaningful comparative study seeking to demon-
strate a significant safety benefit of one procedure over
another will require large numbers of patients, numbers
far greater than those included in any comparative study
reported to date (Table 2).
Bacteremia complicates esophageal dilation more often
than any other procedure performed by gastroenterolo-
gists.51 A number of reports suggest that bacteremia
accompanies esophageal dilation in 20%45% of
cases.7476 Despite the high frequency of bacteremia,
clinically recognizable infectious complications of esopha-
geal dilation such as endocarditis and brain abscesses have
rarely been reported.7780,81 Although antibiotic prophy-
laxis for esophageal dilation generally is recommended
routinely only for patients at high risk for endocarditis
according to the American Heart Associations guide-
lines,82,83 some authorities recently have suggested that
such prophylaxis should be given routinely even to
patients with intermediate risk lesions such as mitral
valve prolapse with insufficiency.75
After initial dilation, stricture recurrence is a frequent
phenomenon. Before proton pump inhibitors became
available, a number of investigations suggested that only
approximately 40% of patients would experience pro-
tracted relief of dysphagia after a single dilation session,
and approximately 60% would require multiple dila-
tions.11,69,84,85 With proton pump inhibitor therapy, as
few as 30% of patients may require repeat dilations
within 1 year.47 For patients with peptic strictures,
factors associated with the need for multiple dilations
include a history of weight loss and absence of heartburn
at the time of initial dilation.86 Neither the severity of the
initial stenosis nor the type and size of dilator used
appears to a have major influence on the likelihood of
stricture recurrence.11,69,86 Therefore, for individual pa-
tients there is no reliable method to predict the need for
repeated dilation. Patients require close follow-up after
initial dilation, and the procedure should be repeated if
dysphagia returns.
The role of surgical treatment for benign esophageal
242 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
strictures remains disputed. There are two major ap-
proaches to the surgical treatment of esophageal stric-
tures: (1) antireflux surgery with intraoperative stricture
dilation for patients with peptic strictures caused by
GERD (for patients whose peptic strictures are associated
with substantial esophageal shortening, a lengthening
procedure such as a Collis gastroplasty may be necessary
for successful fundoplication), and (2) esophageal recon-
struction for patients with strictures of any etiology that
are not responsive to dilation. Esophageal reconstruction
may rarely involve a procedure that widens the stricture
without a resection (e.g., Thal fundic patch) or, more
commonly, the stenotic esophagus may be resected and
reconstructed either by use of a gastric pull-up procedure
or by interposition of a loop of bowel (colon or jejunum)
between the remaining esophagus and the stomach. For
patients with peptic strictures treated by antireflux
surgery and intraoperative dilation, the success rate for
relief of dysphagia is similar to that reported for nonsurgi-
cal dilation therapy.8792 The major advantage of this
approach is that successful antireflux surgery obviates
lifelong medical therapy with its attendant expense and
inconvenience. However, there is no clear benefit for
surgical treatment of esophageal strictures over medical
therapy for relieving dysphagia per se, and there is a small
operative mortality rate (usually 1%) associated with
fundoplication. The requirement for repeated dilation
after antireflux surgery for strictures ranges between 1%
and 31% and appears to be somewhat smaller than that
described for medical treatment of esophageal steno-
ses.8792 One might argue that the small risk of operative
mortality might be offset by the reduced need for
repeated esophageal dilation with its attendant mortality
rate. However, there has been no meaningful comparative
study of medical and surgical treatments for peptic
esophageal strictures, so it is not clear that surgery is
truly superior to medical therapy in prevention of
stricture recurrence. Physicians should be especially
cautious in recommending antireflux surgery for patients
with peptic esophageal strictures caused by esophageal
motility disorders such as scleroderma. The combination
of abnormal esophageal motor function and mechanical
obstruction imposed by fundoplication can result in
severe postoperative dysphagia. However, scleroderma is
not an absolute contraindication to antireflux surgery,
and some reports have described excellent outcomes for
fundoplication in small series of selected patients with
peptic esophagitis and strictures caused by scleroderma.93
Finally, in rare cases, intractable esophageal strictures will
require surgical resection and reconstruction. Operative
morbidity and mortality are substantially higher for
GASTROENTEROLOGY Vol. 117, No. 1
esophageal resection and reconstruction procedures than
for antireflux surgery.
There are several reports on the use of endoscopic
steroid injection for the treatment of patients with
refractory esophageal strictures.9496 All of these studies
involve small numbers of patients with benign strictures
of diverse etiology that had not responded to at least one
attempt at esophageal dilation. Some patients appeared to
respond dramatically to steroid injection by exhibiting
substantial improvement in dysphagia and decreases in
their requirement for repeated dilations. These small
studies were not randomized or controlled, and the
conclusions that can be drawn regarding the efficacy of
steroid injection are very limited. Also, the mechanisms
by which steroid injection might improve esophageal
stenoses are not clear. Nevertheless, steroid injection
appears to be a relatively safe procedure, and a trial of this
unproved therapy seems reasonable for those rare patients
with benign esophageal strictures who derive no or
short-lived relief of dysphagia despite repeated attempts
at stricture dilation and aggressive control of reflux
esophagitis. Finally, the technique of self-bougienage can
be taught to patients who require very frequent esopha-
geal dilation despite intensive medical therapy and
steroid injection and for whom surgery is either contrain-
dicated or unacceptable. The very limited published data
available on self-bougienage suggest that the technique
can be both safe and effective.97
Lower Esophageal (Schatzki) Rings
In 1953, two independent groups of investigators
published descriptions of patients who had dysphagia
associated with ringlike constrictions of the distal esopha-
gus.98,99 The investigators had differing opinions about
the nature of these lower esophageal rings. Ingelfinger
and Kramer98 proposed that the ringlike narrowings were
caused by contraction of an overactive band of esophageal
muscle, whereas Schatzki and Gary99 believed that esopha-
geal rings were fixed structures that were not contractile.
Although it is now clear that lower esophageal rings are
quite common, controversy persists regarding the precise
nature and pathogenesis of these structures.100 Based on
an extensive review of the literature and on an autopsy
study of the distal esophagus, Goyal et al.101,102 con-
cluded that some of the controversy had arisen because
several different disorders had been included under the
rubric lower esophageal ring (e.g., muscular rings,
mucosal rings, and ringlike peptic strictures). Muscular
rings (also called A rings) are caused by a thickened band
of esophageal muscle fibers. The location of these rings
corresponds with an annular thickening in the muscularis
propria of the distal esophagus that anatomists have
July 1999
called the inferior esophageal sphincter (a structure that
should not be confused with the LES described function-
ally by physiologists).101 Muscular rings are located
approximately 2 cm above the esophagogastric junction
and rarely cause dysphagia. Most of the discussion that
follows pertains to lower esophageal mucosal rings (also
called Schatzki rings or B rings), which are by far the
most common type of lower esophageal ring found in
patients with dysphagia.101
The lower esophageal mucosal ring is a thin, dia-
phragm-like, circumferential fold of mucosa that pro-
trudes into the lumen of the distal esophagus, thereby
posing a physical barrier to the passage of solid material.
Mucosal rings usually are located at the squamocolumnar
junction with squamous epithelium lining the upper
surface and columnar epithelium lining the lower aspect
of the ring. Fibrous tissue often can be found in the
lamina propria. Some authorities have challenged the
contention that mucosal rings occur primarily at the
squamocolumnar junction, but the contrary evidence
presented is unconvincing.103 Schatzki rings are best
appreciated on barium swallow, where they are usually, if
not invariably, associated with hiatal hernias. With
careful radiological techniques aimed at distending the
distal esophagus, a lower esophageal ring can be found in
approximately 15% of all patients who have barium
swallows.104 However, few of these rings cause dysphagia.
The pathogenesis of the lower esophageal mucosal ring
is not clear, but a number of hypotheses have been
proposed.101,105 One hypothesis holds that the ring is
merely a pleat of redundant mucosa that forms when the
esophagus shortens either transiently (during contraction
of the longitudinal muscle) or permanently (from un-
known cause). Another hypothesis suggests that rings are
congenital in origin. Few data either strongly support or
clearly refute these two hypotheses. A third hypothesis
suggests that lower esophageal mucosal rings are thin
peptic strictures that develop as a consequence of GERD.
If this hypothesis is correct, treatment of patients with
rings might be directed at controlling reflux esophagitis.
However, data on the association of GERD and Schatzki
rings are inconclusive and contradictory. Goyal et al.102
found no evidence of reflux esophagitis associated with
any of 9 esophageal mucosal rings identified at postmor-
tem examination. Jamieson et al.105 found that GERD
symptoms and abnormal acid reflux (identified by esopha-
geal pH monitoring) were less frequent among 32
patients with Schatzki rings than among 32 control
patients who had hiatal hernias without rings. In con-
trast, Marshall et al.106 found evidence of GERD (abnor-
mal acid reflux by 24-hour pH monitoring or endoscopic
signs of reflux esophagitis) in 13 of 20 patients with
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 243
symptomatic Schatzki rings. Also supporting a potential
role for GERD in the pathogenesis of lower esophageal
mucosal rings are the observations that demonstrable
rings are virtually always associated with hiatal hernias
and that serial radiographic examinations in some pa-
tients with rings have shown progression in the esopha-
geal stenoses over time to the point that they resembled
peptic strictures more than rings.107 Finally, a role for pill
esophagitis has been suggested in the pathogenesis of
Schatzki rings. In one study, 62% of patients with rings
who had no signs or symptoms of GERD had a history of
ingestion of medications known to cause pill esophagi-
tis.105
The hypotheses on the pathogenesis of lower esopha-
geal mucosal rings need not be mutually exclusive. For
example, a congenital ring might be narrowed further by
scarring from reflux or pill esophagitis. It is also conceiv-
able that subtle rings do not cause symptoms unless there
is a supervening disorder that further interferes with
esophageal clearance such as esophagitis or dysmotility.
This could explain an apparent association between rings
and GERD, even if GERD plays no role in the pathogen-
esis of the rings per se.
Treatment of patients with dysphagia caused by lower
esophageal mucosal rings begins with reassurance that
the condition is benign and with advice that food be
chewed slowly and carefully.100 However, there are no
data to show that this advice is beneficial, and dilation
therapy is recommended for most patients. Traditionally,
initial dilation therapy for Schatzki rings involves the
passage of a single large bougie or balloon (4560F)
aimed at fracturing (rather than merely stretching) the
mucosal fold.108 This approach differs from that discussed
above for peptic strictures, which are treated by gradual
stretching for fear of rupturing the fibrotic esophagus
with a single, abrupt dilation. The safety of abrupt
dilation for esophageal rings has been well established,
and most reported series limited to patients with Schatzki
rings describe no complications of the procedure. How-
ever, the contention that abrupt dilation is more effective
than gradual dilation for relief of dysphagia has not been
verified. Furthermore, the notion that ring fracture by
abrupt dilation should result in a low rate of recurrent
dysphagia has not been substantiated by published
experience. Indeed, a number of reports suggest that
recurrence is the rule rather than the exception after
dilation of symptomatic Schatzki rings.105,109,110 For
example, one recent report describes 33 patients with
symptomatic Schatzki rings who were treated by abrupt
dilation with the passage of a single, large bougie
(4658F) and were followed up for a mean duration of 2
years.109 Initial results were excellent, with all patients
244 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
reporting complete relief of dysphagia at a 4-week
follow-up examination. However, actuarial life-table analy-
sis showed that only 68% would remain free of dysphagia
after 1 year, whereas only 11% of patients were estimated
to be symptom-free by year 5. In another study of 61
patients with symptomatic Schatzki rings who were
followed up for a mean duration of 75 months, 63%
developed recurrent dysphagia after initially successful
dilation.110
No clinical features have been identified that are
consistently useful for predicting which patients will
need repeated dilations for Schatzki rings. The initial
diameter of the ring has not been found to correlate
significantly with recurrence.109,110 Some investigators
have suggested that recurrence is more likely in patients
who have GERD associated with their Schatzki rings,105
whereas others have found no correlation between the
presence of GERD and the need for repeated ring
dilations.109 For patients who have both Schatzki rings
and GERD, antireflux therapy aimed at eliminating the
signs and symptoms of reflux esophagitis clearly is
appropriate despite the lack of proof that such treatment
reduces the frequency of recurrent dysphagia. All patients
with rings who are treated with dilation should be
advised that recurrence is likely and that dilation may
need to be repeated if dysphagia returns.
A number of different treatments have been used for
patients with defiant rings that do not respond to smooth muscle.119,120 In some patients, degenerative
abrupt dilation using standard balloons and bougies, or
that recur quickly after initial relief of dysphagia. There
are anecdotal reports on the successful use of pneumatic
dilation with large balloon dilators such as those usually
reserved for the treatment of achalasia.111 Some investiga-
tors have performed endoscopic electrosurgical incision of
the rings with good results.112,113 Others have used
surgery either to rupture or excise the rings, and to repair
the associated hiatal hernias.114,115 However, one report of
such surgical therapy describes the recurrence of symp-
toms in 14 of 36 patients (39%).114 A concomitant
motility disorder could explain the frequent recurrence of
symptoms in some patients with defiant Schatzki rings,
but few studies have systematically searched for esopha-
geal dysmotility in these patients. The possibility of a
motility disorder should be explored with a manometric
examination before one of the potentially hazardous
therapies mentioned above is used for patients with
defiant lower esophageal mucosal rings.
Physicians occasionally encounter patients who have
intermittent dysphagia for solid foods suggestive of a
Schatzki ring but have no demonstrable abnormality on
barium swallow or endoscopic examination of the esopha-
gus. Few reports have addressed the treatment of such
GASTROENTEROLOGY Vol. 117, No. 1
patients. One study explored the role of empiric esopha-
geal dilation in patients who had esophageal dysphagia
and a normal esophagoscopy, barium swallow, or both.116
Among 20 such patients who had dysphagia for solid
foods only, empiric bougienage to 54F resulted in
immediate and complete resolution of dysphagia in 19
cases (95%). During a median follow-up period of 20
months, furthermore, 13 of those 19 patients experienced
no recurrence of dysphagia. In contrast, complete resolu-
tion of dysphagia was seen after empiric dilation in only 2
of 17 patients (12%) who had dysphagia for both solids
and liquids. It seems likely that empiric dilation was
effective in the former patients because they had subtle
rings, webs, or strictures that were missed by the
diagnostic studies, whereas the latter patients probably
had motility disorders. Although the design of this study
was far from ideal, the report suggests that a trial of
empiric bougienage is reasonable for patients who com-
plain of dysphagia for solid food and who have normal
findings on endoscopic examinations.
Achalasia
Primary achalasia is an esophageal disease of
unknown cause in which there is degeneration of neurons
in the wall of the esophagus.117,118 This degenerative
process preferentially involves the nitric oxideproducing
inhibitory neurons that effect the relaxation of esophageal
changes are found in brainstem ganglion cells in the
dorsal motor nucleus of the vagus, and Wallerian degen-
eration has been observed in vagal fibers that supply the
esophagus.121 However, the disordered motility that
characterizes achalasia appears to result primarily from
the degeneration of inhibitory neurons within the esopha-
gus itself. The smooth muscle of the LES is tonically
contracted at rest and relaxes when intramural neurons
release their inhibitory neurotransmitters. Loss of inhibi-
tory innervation in the LES causes basal sphincter
pressures to increase and renders the sphincter muscle
incapable of normal relaxation. Unlike the LES, the
smooth muscle of the esophageal body does not exhibit
resting tone; therefore, the loss of inhibitory neurons has
little effect on resting pressure in the body of the
esophagus. However, inhibitory influences are necessary
for normal peristalsis, and the loss of inhibitory neurons
results in aperistalsis.
Although the etiology of primary achalasia is not
known, certain recognized diseases can cause esophageal
motor abnormalities similar or identical to those of
primary achalasia. This condition is called secondary
achalasia or pseudoachalasia. In Chagas disease, seen in
Central and South America, for example, esophageal
July 1999
infection with the protozoan parasite Trypanosoma cruzi
can result in a loss of intramural ganglion cells that causes
aperistalsis and incomplete LES relaxation.122 Malignan-
cies rarely can cause pseudoachalasia either through direct
invasion of esophageal neural plexuses (e.g., adenocarci-
noma of the esophagogastric junction) or through release
of uncharacterized humoral factors that disrupt esopha-
geal function as part of a paraneoplastic syndrome.123 It is
important to exclude the diagnosis of pseudoachalasia
caused by malignancy before invasive therapies such as
pneumatic dilation or surgical myotomy (see below) are
implemented. It seems likely that these procedures would
be especially hazardous for patients with infiltrating
neoplasms of the distal esophagus, although few pub-
lished data are available to support this contention. In
most cases, a careful history and endoscopic examination
are sufficient to exclude the diagnosis of pseudoachalasia.
However, if the clinical history is strongly suggestive of
achalasia caused by malignancy (e.g., onset in old age,
rapid progression of symptoms, profound weight loss),
additional tests such as computed tomography or endo-
sonography might be necessary to exclude an infiltrating
neoplasm.
Presently, no therapy can reverse or even halt the
degeneration of enteric neurons that occurs in primary
achalasia. Therefore, the treatment of this disorder is
functional, aimed at decreasing resting pressure in the
LES (by pharmacological or mechanical means) to the
point that the sphincter no longer poses a substantial
barrier to the passage of ingested material. This therapeu-
tic attack on the LES does not reliably restore function in
the body of the esophagus, although the return of
peristaltic activity has been observed in some patients
after the administration of various therapies designed
solely to decrease LES pressure.124126 Nitrates and
calcium channel blockers have been shown to relax the
smooth muscle of the LES both in normal individuals and
in patients with achalasia, and these agents have been
used to treat the disorder with variable success.127135
Sublingual isosorbide dinitrate causes a substantial de-
crease in LES pressure within minutes, and the effect
often lasts for more than 1 hour.127 Limited studies
suggest that more than 75% of patients with achalasia
experience substantial improvement in dysphagia when
they take isosorbide in a dose of 510 mg sublingually 10
minutes before meals.127,128 One comparative trial in 15
patients with achalasia found that nitrates were superior
to nifedipine both for decreasing LES pressure (64%
decrease from baseline for nitrates vs. 47% decrease for
nifedipine) and for relieving dysphagia (swallowing im-
proved in 87% of patients on nitrates vs. 53% of patients
on nifedipine).128 However, nitrate therapy often must be
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 245
discontinued because many patients experience intoler-
able side effects (predominantly headache) and because
some patients become refractory to the nitrates after an
initial good response.127,128 Like nitrates, calcium chan-
nel blockers administered sublingually also result in
substantial decreases in LES pressure for more than 1
hour.135 However, the results of studies on the effects of
calcium channel blockers on the symptoms of achalasia
are contradictory; some investigators report good,133,135
marginal,130 and no134 relief of dysphagia in small groups
of patients treated with these agents. Although verapamil
and diltiazem have been used, most published experience
has involved nifedipine given in a dose of 1020 mg
sublingually 30 minutes before meals. Adequate plasma
levels have been observed when calcium channel blockers
are administered orally (rather than sublingually) to
patients with achalasia,134 but the absorption of orally
administered drugs may be erratic for patients with
advanced disease in whom the pills can linger for hours in
the flaccid esophagus. In summary, pharmacotherapy for
achalasia is inconvenient, often ineffective, and frequently
associated with side effects and tachyphylaxis. It appears
that pharmacotherapy is best reserved for patients who
are unwilling or unable to tolerate the more effective
invasive forms of therapy discussed below.
At one time, authorities recommended esophageal
dilation using large, mercury-filled rubber bougies (50
60F) as the initial therapy for achalasia.136 As techniques
for forceful dilation of the LES in achalasia became
popular, bougienage was dismissed as a procedure that, at
best, provided only transient and incomplete relief of
dysphagia.137 In 1982, Mandelstam et al.136 reported that
bougienage to 58F resulted in relief of dysphagia for
months to years in 4 of their 5 patients with achalasia and
called for a reappraisal of the role of bougienage in the
treatment of this disorder. More recently, McJunkin et
al.138 reviewed their experience with achalasia and re-
ported that bougienage was successful when used as
initial therapy in 10 of 20 patients. Despite these reports,
most modern authors continue to dismiss bougienage as a
procedure that provides only temporary and incomplete
relief for patients with achalasia. Consequently, few
modern gastroenterologists have any experience with
bougienage in the treatment of achalasia. In the absence
of well-designed, prospective studies, it is difficult to
draw firm conclusions regarding the efficacy of bougie-
nage for achalasia. Presently, it appears that bougienage,
like pharmacotherapy, is best reserved as an alternative
treatment for patients who are unwilling or unable to
tolerate the more invasive forms of therapy discussed
below.
For decades, pneumatic dilation of the LES using large
246 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
balloons has been a popular form of treatment for
achalasia.139155 This therapy was designed to weaken the
LES by tearing its muscle fibers, although experimental
evidence that the procedure indeed works by inducing
muscular tears is scant.148 Many different balloon dilators
have been used over the years for treatment of achalasia
(e.g., Mosher bag, Sippy dilators, BrownMcHardy dila-
tor, RiderMoeller dilator), but most are no longer being
manufactured. Presently, the two most popular pneu-
matic dilators in the United States are the Rigiflex dilator
(similar in design to the Gruntzig angioplasty catheter),
which is passed over a guidewire and positioned fluoro-
scopically,150 and the Witzel dilator, which is mounted on
an endoscope and inflated under direct vision.144
There is no clear consensus on the optimal method for
performing pneumatic dilation, and reported protocols
have varied widely with regard to the types of medica-
tions used for sedation (which theoretically could affect
the outcome if the sedatives cause LES relaxation, and
which have ranged from no sedatives whatsoever to
general anesthesia), the types of dilators used (e.g.,
Mosher, Sippy, BrownMcHardy, RiderMoeller, Rigi-
flex, Witzel), the maximum diameter of the balloon
(reported range, 2.45.0 cm), the pressure to which the
balloon is inflated (reported range 105 to 1000 mm
Hg), the rate of balloon inflation (rapid vs. gradual), the
duration of balloon inflation (reported range, several
seconds to more than 5 minutes), and the number of
balloon inflations per dilating session (reported range,
15).155 Most investigators have used a single pneumatic
dilator at each treatment session, with the need for
subsequent dilations determined empirically by the
symptomatic response to the initial dilation.154 Others
have used the method of progressive pneumatic dilation
advocated by Vantrappen and Janssens148 in which a
series of balloons of progressively increasing diameter are
inflated until there is manometric and radiographic
evidence of adequate LES disruption. With so many
possible permutations and combinations of the various
components of pneumatic dilation, and with so few
reports of prospective studies, it is difficult to perform a
meaningful meta-analysis on the outcome of the proce-
dure.
Despite the many variations in technique described
above, most studies (primarily retrospective reviews of an
institutions experience) describe good to excellent short-
term results in 60%85% of patients with achalasia
treated with a single session of pneumatic dilation. The
duration of follow-up in most of these retrospective
reports is relatively brief; consequently, few data are
available on the long-term outcome of pneumatic dila-
tion. One large retrospective study found that only 65%
GASTROENTEROLOGY Vol. 117, No. 1
of 313 patients with achalasia treated with one or more
pneumatic dilations still reported good to excellent
results after a median follow-up of 11 years.140 In one of
very few prospective studies on patients with achalasia
treated initially with pneumatic dilation, 28 of 54
subjects (52%) were found to require repeat dilations
during a median follow-up of 4.1 years.156 The estimated
probability of remaining in remission after a single
dilation was 59% at 1 year and 26% at 5 years. In a
retrospective investigation of 123 patients treated ini-
tially with pneumatic dilation, Parkman et al.157 found
that 42% required further treatment during a mean
follow-up of 4.7 years. These investigators also noted that
repeat pneumatic dilations became progressively less
effective than the initial procedure. For example, 58% of
patients who had a second pneumatic dilation required
further treatment, whereas 73% required additional
therapy after a third dilation. In a recent, more optimistic
report of a retrospective study, Katz et al.158 found that
pneumatic dilation was successful in relieving the symp-
toms of achalasia in 85% of 72 patients who were
followed up for a mean of 6.5 years, and only 4 patients
required more than one dilation procedure. Overall, these
studies suggest that approximately 50% of patients with
achalasia treated initially with a single pneumatic dila-
tion will require further therapy within 5 years and that
subsequent pneumatic dilations are progressively less
likely to result in a sustained remission. Some authors
have recommended that other forms of therapy be
considered after two or three unsuccessful pneumatic
dilations.117
A number of studies have sought to identify clinical
and technical factors that might help clinicians predict
responses to pneumatic dilation.156161 Young age consis-
tently has been shown to be associated with a poor
response.156159 In one prospective study, for example, the
2-year sustained remission rate for patients under age 40
was only 29%, compared with 67% for those age 40 years
and older.156 Perhaps young muscle is less susceptible to
damage by forceful dilation than old muscle. Among the
technical factors, LES pressure after dilation appears to be
the best predictor of outcome. In the aforementioned
prospective study, the 2-year remission rate was 100% for
patients whose postdilation LES pressure was 10 mm
Hg, 71% for those whose postdilation LES pressure was
1020 mm Hg, and 23% for those with postdilation LES
pressures of 20 mm Hg.156 The size of the dilating
balloon also appears to influence the outcome (i.e., the
long-term remission rate may be higher when larger
diameter balloons are used), although few studies have
specifically explored this issue.151,154,156 Factors that do
not appear to have a substantial influence on the response
July 1999
to pneumatic dilation include gender, duration of esopha-
geal symptoms before treatment, diameter of the esopha-
gus, pretreatment LES pressure, duration of balloon
inflation,161 number of balloon inflations per dilating
session, maximum inflation pressure, and chest pain
present during the procedure. Finally, although many
clinicians have been taught to look for blood on the
dilating balloon as proof that the procedure has been
consummated, this finding has not been found to be a
useful predictor of outcome.157,160
Despite the wide variations in equipment and tech-
niques used for pneumatic dilation, reported complica-
tion rates are remarkably similar. Esophageal perforation
is the most common serious complication of the proce-
dure, and most large series describe rates of perforation in
the range of 2%6%.117,155 Mortality from pneumatic
dilation is rare and has been estimated at approximately
0.2%.117 The new Rigiflex dilators do not appear to have
any safety advantage over the older balloons, and some
investigators even suggest that perforation rates are
higher with the newer instruments.162 There are very few
reports of studies that have prospectively compared the
different dilator types for safety and efficacy, and those
that have been published are primarily of historical
interest because some of the dilators compared are no
longer available.163 Achalasia is an uncommon disease,
and the perforation rate for pneumatic dilation is rela-
tively low. Consequently, it is difficult to conduct a
meaningful study on factors that might predispose to
esophageal perforation. A number of potential predispos-
ing factors have been suggested, including malnutrition,
weight loss, low LES pressure, high-amplitude contrac-
tions in the distal esophagus, previous pneumatic dila-
tions, administration of anesthesia, large balloon size, and
high inflation pressures.164166 However, none of these
factors has been shown consistently to influence the safety
of pneumatic dilation. In addition, the presence of a large
epiphrenic diverticulum has been regarded as a contrain-
dication to pneumatic dilation because of anecdotal
reports of perforations in this setting.147 However, the
risk of perforation imposed by these diverticula has not
been well established. Although it once was standard
clinical practice to hospitalize patients for pneumatic
dilation, it appears that performing the procedure in an
outpatient setting for otherwise healthy individuals does
not substantially increase the risk of complications.167,168
A number of authorities recommend that esophagraphy
be performed shortly after pneumatic dilation (usually as
soon as the sedation has worn off) to seek evidence of
perforation.117 Although water-soluble contrast (e.g.,
Gastrografin) is commonly recommended for this study,
such hypertonic agents can cause a chemical pneumonitis
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 247
if they are aspirated into the lungs. Consequently, these
agents should not be used in sedated patients or in
neurologically impaired individuals who are at risk for
aspiration. Indeed, it may be preferable to use dilute
barium rather than water-soluble contrast agents rou-
tinely for these postdilation esophagrams, but this conten-
tion has not been validated by formal investigation.
Finally, it has been estimated that approximately 2% of
patients treated with pneumatic dilation will develop
reflux esophagitis because of the resulting LES hypoten-
sion.117 Indeed, aggressive pneumatic dilation essentially
changes the manometric features of achalasia to those of
scleroderma, a disorder often associated with severe
GERD. One recent study describes abnormal acid reflux
by 24-hour esophageal pH monitoring in 6 of 17 patients
who had pneumatic dilation.169 With the availability of
highly effective antireflux therapies such as proton pump
inhibitors, concerns about the induction of GERD by
pneumatic dilation need not be overriding.
Surgical myotomy, in which the surgeon weakens the
LES by cutting its muscle fibers, traditionally has been
viewed as the primary alternative to pneumatic dilation
for achalasia. Cardiomyotomy was first performed in
1913 by Ernst Heller, and modern surgeons now use
modifications of Hellers original procedure.117 The stan-
dard open myotomy can be performed using either an
abdominal or, more commonly, a thoracic approach.170
184 Recently, a number of centers have reported their
preliminary results with minimally invasive surgery for
achalasia in which laparoscopic or thoracoscopic tech-
niques are used to perform the myotomy.185190 Other
important differences in technique among centers include
variations in the length and depth of the myotomy
incision and whether or not the myotomy is combined
with an antireflux procedure. Surgical myotomy results
in good to excellent relief of symptoms in 70%90% of
patients, with few serious complications and a mortality
rate similar to that reported for pneumatic dilation
(approximately 0.3%).117 Reflux esophagitis (which may
be complicated by esophageal ulceration, stricture, and
Barretts esophagus) has been found to develop in approxi-
mately 11% of patients treated by surgical myotomy,117
and surgeons continue to debate the need for the addition
of an antireflux procedure. The addition of a fundoplica-
tion appears to reduce the rate of symptomatic, postmy-
otomy GERD to approximately 4% but may increase the
frequency of postoperative dysphagia by imposing a valve
between the aperistaltic esophagus and the stomach.191 In
the absence of well-designed prospective studies, the
debate over the need for fundoplication in this setting
remains unresolved. However, the recent availability of
highly effective medical antireflux therapies such as
248 AMERICAN GASTROENTEROLOGICAL ASSOCIATION
proton pump inhibitors should simplify the management
of postoperative GERD considerably and perhaps obviate
routine fundoplication.
Late recurrence of dysphagia after surgical myotomy or
pneumatic dilation may be caused by a return of tone in
the damaged LES muscle, by GERD with peptic stricture
formation, or very rarely by squamous cell carcinoma of
the esophagus that develops with increased frequency in
patients with achalasia.192 Endoscopic examination should
readily differentiate these disorders. Late recurrence of
dysphagia appears to be less common after surgical
myotomy than after pneumatic dilation, but postopera-
tive recurrence of dysphagia is not unusual after surgical
treatment. For example, Jara et al.176 describe a sustained
remission rate of 81% after 10 years for patients treated
with esophagomyotomy. Ellis et al. performed a Kaplan
Meier analysis on the results of esophagomyotomy in 81
patients and estimated sustained remission rates of 86%
at 15 years and 67% at 20 years.193 Malthaner et al.194
also found a deterioration of surgical results over time
with good to excellent results reported by 21 of 22
patients (95%) at 1 year, 17 of 22 (77%) at 5 years, 15 of
22 (68%) at 10 years, 11 of 16 (69%) at 15 years, and 6 of
9 (67%) at 20 years or more.
Relatively few studies have directly compared the
results of myotomy and pneumatic dilation. In one
retrospective study, Okike et al.177 reported good to
excellent relief of dysphagia in 85% of 468 patients
treated with myotomy compared with only 65% of 431
patients who underwent pneumatic dilation for achalasia.
In another retrospective review, Anselmino et al.195
concluded that surgery was safer and more effective than
pneumatic dilation, although the outcome of pneumatic
dilation in this study was far worse than that reported by
most investigators (i.e., 15% rate of esophageal rupture,
39% rate of dysphagia relief after a mean follow-up of 55
months). In contrast, Abid et al.196 reviewed their
experience with pneumatic dilation (36 patients) and
surgical myotomy (9 patients) in patients followed up for
a mean interval of approximately 2 years and concluded
that the procedures are equally effective (success rate for
both procedures, approximately 90%) if both are per-
formed by skilled operators. In one of the two prospec-
tive, randomized trials of myotomy and pneumatic
dilation reported to date, Csendes et al.197 describe
excellent results after a median follow-up of approxi-
mately 5 years in 40 of 42 (95%) patients in the surgical
group, compared with 24 of 37 (65%) patients who
underwent pneumatic dilation. This study has been
criticized for using a pneumatic dilation protocol that
may have been suboptimal (i.e., use of atropine as
premedication, low balloon inflation pressures, short
GASTROENTEROLOGY Vol. 117, No. 1
duration of inflation),198 but as noted above, these
technical factors have not been shown to affect the
outcome of pneumatic dilation. A recent, smaller random-
ized trial of balloon dilation and myotomy found the two
procedures equally effective in relieving dysphagia for up
to 3 years.199 Despite the limitations of the available
studies, it appears that surgery generally is superior to
pneumatic dilation for both short-term and long-term
relief of dysphagia. Furthermore, the rate of serious
complications such as esophageal perforation appears to
be less for surgically treated patients, and the mortality
rates for the two procedures are approximately equal. The
major disadvantages for surgery are the high initial cost,
protracted recovery period, and frequent development of
GERD postoperatively. A recent cost analysis comparing
open surgery and pneumatic dilation concluded that
initial pneumatic dilation (with surgery reserved for
dilation failures) was the most cost-effective approach.157
However, this analysis did not consider myotomy per-
formed by minimally invasive techniques. The short-
term relief of dysphagia by minimally invasive surgery
appears to be at least as good as that described for the
open procedure, but long-term results are not yet avail-
able because the procedures are so new. Prospective,
comparative studies are sorely needed in this area. If
laparoscopic or thoracoscopic techniques enable surgeons
to perform myotomy at reduced cost and with shorter
recovery periods, if the operation is shown to be durable,
and if the incidence of severe GERD is acceptably low,
then myotomy by minimally invasive techniques may
well be more cost-effective than pneumatic dilation.
Pending the results of long-term studies on minimally
invasive surgery, however, the decision between pneu-
matic dilation and myotomy as initial therapy for
achalasia should be based on a consideration of the
patients preferences and on the availability of personnel
experienced in the two techniques.
Recently, an interesting new approach to the treatment
of achalasia has been introduced by Pasricha et al.200203
These investigators noted that botulinum toxin, a potent
inhibitor of the release of acetylcholine from nerve
endings, has been used successfully for decades to treat
certain spastic disorders of skeletal muscle such as
blepharospasm. They hypothesized that the local, peren-
doscopic injection of botulinum toxin into the LES of
patients with achalasia would poison the excitatory
(acetylcholine-releasing) neurons that contribute to LES
smooth muscle tone, thereby effecting a therapeutic
decrease in LES pressure. The investigators tested their
hypothesis in an animal model and verified that local
botulinum toxin injection caused a significant decrease in
LES pressure in piglets.200 Later, a pilot study in 10
July 1999
patients with achalasia suggested a beneficial therapeutic
effect for botulinum toxin injection of the LES,201 an
effect that was confirmed in a double-blind, placebo-
controlled trial in 21 patients.202 Most recently, this
group has described the results of botulinum toxin
injection in 31 patients with achalasia.203 Twenty-eight
of the 31 patients (90%) showed immediate symptomatic
improvement, but this effect was short-lived in many
cases. Despite repeated injections of the toxin when
symptoms returned, only 20 of the 31 patients (65%)
remained in remission 6 months after the initial treat-
ment. In these 20 responders, botulinum toxin injection
caused resting LES pressure to decrease by 45% compared
with baseline values. During a median follow-up of 2.4
years, however, 19 of the 20 responders experienced a
relapse of symptoms that required further therapy. Among
15 of these patients who were treated with another
botulinum toxin injection, only 9 had sustained remis-
sions. KaplanMeier analysis suggested that only 68% of
patients in remission at 6 months would remain in
remission at the end of 1 year. The median duration of
remission after initial treatment was 16 months, and the
longest remission observed was 28 months. Other groups
have confirmed the short-term efficacy of botulinum
toxin injection.204,205 Pasricha et al.200203 note that like
pneumatic dilation, botulinum toxin injection was more
effective in older patients (82% response rate for patients
age 50 years vs. 43% in younger patients). They also
note that the toxin was 100% effective for patients with
vigorous achalasia (defined by the presence of pressure
waves in the esophageal body with amplitudes 40 mm
Hg), whereas only 52% of patients with classic achalasia
showed a clinical response at 6 months. However, another
group of investigators did not find a difference in
response to botulinum toxin injection between patients
with vigorous and classic achalasia.204 Botulinum toxin
injection appears to be remarkably safe. Approximately
25% of patients experience transient, mild chest pain
immediately after the procedure, and fewer than 5% of
patients develop symptomatic GERD. The most serious
complication reported to date is a case report of a patient
who developed severe, ulcerative esophagitis (probably
caused by acid reflux) after toxin injection.206 This
patient also was found to have adhesions and periesopha-
geal inflammation when he subsequently underwent
surgical treatment for achalasia. These studies suggest
that botulinum toxin injection of the LES is a remarkably
safe procedure that can induce a clinical remission for at
least 6 months in approximately two thirds of patients
with achalasia. However, most patients need repeated
injections to maintain the remission, and only approxi-
mately two thirds of those patients in remission at 6
AMERICAN GASTROENTEROLOGICAL ASSOCIATION 249
months will remain in remission at 1 year despite
repeated injections. Vaezi et al.207 recently reported the
results of a prospective study in which 42 patients with
achalasia were randomly assigned to receive either botuli-
num toxin injection or pneumatic dilation. At 12
months, only 32% of patients treated with botulinum
injection were in symptomatic remission, compared with
70% of patients in the pneumatic dilation group.
Castell and Katzka208 point out that botulinum toxin
therapy is expensive (approximately $300 just for the
botulinum toxin used at each treatment session), the
failure rate is substantial, patients frequently need re-
peated injections, and the long-term efficacy of the
procedure is unknown. These concerns, combined with
the very poor response rate found in patients under the
age of 50, suggest that botulinum toxin injection may
not be the ideal choice of therapy for young patients with
achalasia. Further studies are needed before the procedure
can be recommended for clinical application outside of
research protocols. Presently, botulinum toxin injection
can be considered for the treatment of patients who have
serious comorbidity and for whom pneumatic dilation or
surgical myotomy poses inordinate risks.
Other experimental therapies have been proposed for
the treatment of achalasia, including endoscopic my-
otomy of the LES using a needle-knife209 and perendo-
scopic injection of ethanolamine into the LES.210 Al-
though preliminary reports are interesting, far more
investigation is needed before these potentially hazardous
treatments can be recommended for clinical use. In very
rare patients in whom all reasonable therapies have failed,
esophageal resection and reconstruction may be consid-
ered as a last resort.191 Finally, a feeding gastrostomy can
be considered for patients in whom all reasonable primary
therapies for achalasia have failed or for very elderly and
infirm patients for whom other therapies may be hazard-
ous. Few published data are available on the use of
gastrostomy for patients with achalasia, but many neuro-
logically intact patients undoubtedly will find this
therapy unacceptable.
STUART JON SPECHLER, M.D.
Dallas Department of Veterans Affairs Medical Center
and University of Texas Southwestern Medical Center
Dallas, Texas
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Address requests for reprints to: Chair, Clinical Practice and
Practice Economics Committee, AGA National Office, c/o Member-
ship Department, 7910 Woodmont Avenue, 7th Floor, Bethesda,
Maryland 20814. Fax: (301) 654-5920.
The Clinical Practice and Practice Economics committee acknowl-
edges the following individuals, whose critiques of the review paper
provided valuable guidance to the author: David Fleischer, M.D.,
Richard Kozarek, M.D., Robert Fisher, M.D., and Ann Ouyang, M.D.
r 1999 by the American Gastroenterological Association
0016-5085/99/$10.00