13 
Esophageal Motility Disorders and Diagnosis
Neil R. Floch
ESOPHAGEAL MOTILITY DISORDERS
The connection between unexplained chest pain and esophageal spasm
was first discovered by William Osler in 1892. Since then, multiple
esophageal motility disorders have been encountered in clinical practice,
with a wide range of symptoms, manometric findings, and responses
(Fig. 13.1). These disorders vary from minimal changes to extensive
radiologic and manometric abnormalities. The etiology of motility
disorders has yet to be clearly defined.
Esophageal pressure topography (EPT) analysis of high-resolution
manometry (HRM) and analysis by the Chicago Classification (CC)
has clarified the diagnosis of esophageal motility disorders by first
determining the lower esophageal sphincter (LES) pressures and then
defining peristalsis of the esophageal body. Motility disorders fall into
four categories: (1) achalasia, (2) esophagogastric junction outflow
obstruction (EGJOO), (3) major disorders of peristalsis, and (4) minor
disorders of peristalsis. (Achalasia is discussed further on.) Esophageal
dysmotility may occur primarily or secondary to other diseases.
EGJOO may be a precursor to achalasia or to benign or malignant
infiltrative disorders. EGJOO has the same pathophysiology as achalasia.
In these patients the LES cannot relax and there is an elevated LES
pressure. It is an uncommon manometric abnormality found in patients
with dysphagia and chest pain that is sometimes associated with gas-
troesophageal reflux disease (GERD). Absent contractility occurs most
often with GERD and collagen vascular diseases such as scleroderma.
Failed peristalsis may result from a 360-degree fundoplication that is
“too tight.”
Diffuse esophageal spasm (DES) is a disease of the esophageal body
characterized by rapid wave progression down the esophagus. It has an
incidence of 1 in 100,000 individuals per year and is found in 4% of
patients undergoing manometry. DES is unique in that it is distinguished
by a nonperistaltic response to swallowing and may be closely related
to achalasia in that 33% of individuals have an elevated LES pressure
with poor relaxation.
Hypercontractile (jackhammer) esophagus or nutcracker esophagus
(NE) was first diagnosed in the 1970s. It is found in 12% of individu-
als undergoing manometry. Patients are symptomatic with dysphagia.
It is believed to occur from overexcitation of the esophageal smooth
muscle or as a response to esophagogastric junction outflow obstruc-
tion (EGJOO).
Ineffective esophageal motility (IEM) is a monometrically defined
disorder associated with severe GERD, obesity, respiratory symptoms,
delayed acid clearance, and mucosal injury. IEM may occur secondary
to other diseases, including alcoholism, diabetes mellitus, multiple scle-
rosis, rheumatoid arthritis, scleroderma, and systemic lupus erythema-
tosus. Fragmented esophageal muscle segments may indicate the presence
66
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of hypomotility, which can lead to the inability to clear refluxed acid
from the distal esophagus.
Unfortunately pathologic distinction between these disorders is
usually not helpful because muscles and neural plexuses cannot be
properly biopsied. The degree of increase in muscle mass may be an
important determinant of the type and severity of esophageal motor
dysfunction. The LES and esophageal muscles are thickest in patients
with achalasia, thicker in patients with esophageal spasm disorders, and
least thick in patients with DES and NE. In some studies no specific
change in ganglion cells, vagus nerve, or disease progression has been
found. However, a nerve defect is suspected because many patients may
be sensitive to cholinergic stimulation.
Clinical Picture
Classic symptoms of esophageal motility disorders include chest pain
(80%–90% of patients), dysphagia (30%), and heartburn (20%). Dys-
phagia of liquids and solids indicates a functional disorder of the
esophagus; dysphagia of solids alone indicates a physical lesion. Very
hot or cold liquids and stress may exacerbate dysphagia. The pain is
usually retrosternal and frequently radiates to the back. Patients describe
a pain more severe than angina that is intermittent and variable from
day to day. It may last from minutes to hours. Usually a disparity exists
between symptoms and manometric findings, and the chest pain may
be unrelated to the dysmotility. Anxiety and depression are common
in these patients. Stress, loud noises, and ergonovine maleate may stimu-
late muscular contractions. The cause may be a sensory abnormality,
and psychiatric illness may alter patients’ sensory perceptions.
Patients with EGJOO have dysphagia (71%) and chest pain (49%).
Other common symptoms are regurgitation (75%) and heartburn (71%).
Patients with absent contractility complain of dysphagia to liquids and
solids. Patients with a low LES pressure and aperistalsis have severe
GERD and its complications.
Patients with DES complain of chest pain and dysphagia. The pain
may be associated with eating quickly or drinking hot, cold, or carbon-
ated beverages. Anxiety is common. Patients with jackhammer esophagus
or NE usually present with chest pain; dysphagia is present in only
10%. There is a 30% incidence of associated psychiatric disorders.
Patients with IEM present with typical symptoms of heartburn and
reflux and rarely have nonobstructive dysphagia with impaired bolus
transit through the esophagus. Patients with fragmented peristalsis are
likely to complain of coughing and have reflux symptoms due to hypo-
motility and poor acid clearance.
Diagnosis
Several tests may be helpful with the diagnosis of motility disorders.
Barium esophagraphy may detect nonpropulsive contractions with
 CHAPTER 13  Esophageal Motility Disorders and Diagnosis 67

80
Patients may present with:

mm Hg
•Nonperistaltic
40 18 cm contractions
•Waves have increased
0 amplitude and duration
80
mm Hg
40 13 cm

0
160
mm Hg

80 8 cm

0
160
mm Hg

80 3 cm

0
IEM ineffective
WS esophageal motility
Manometric tracing showing repetitive
contraction seen in diffuse esophageal
spasm. WS = wet swallow.
Poor
Symptoms Include: esophageal
motility
Reflux

Weak LES

Chest pain

Regurgitation, heart burn

Dysphagia

Pain lasting minutes to hours

radiates to back (restrosternal)
Fig. 13.1  Esophageal Spasm Syndromes. IEM, Ineffective esophageal motility; LES, lower esophageal
sphincter.

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68 SECTION I Esophagus

segmentation, which are diagnostic for “corkscrew” esophagus or DES.
NE and other spastic disorders may present with minimal findings.
Endoscopy is not diagnostic but should be performed to exclude malig-
nancy or associated disorders such as hiatal hernia, reflux esophagitis,
and strictures. Degeneration of esophageal vagal branches may be seen
on biopsy in DES.
Of patients with GERD and respiratory symptoms, 30% to 50%
have IEM, and 75% of IEM patients and 25% of EGJOO patients have
an abnormal DeMeester score on 24-hour pH monitoring. In DES
patients, computed tomography (CT) has been found to be sensitive
in detecting esophageal wall thickening in the distal 5 cm of the esopha-
gus; thus CT shows promise as a diagnostic test.
In the past most esophageal motility disorders have been diagnosti-
cally nonspecific.
Manometry is the definitive test for evaluating esophageal motility
disorders, but symptoms correlate poorly with findings. Traditionally
classic manometry has been used. HRM with EPT differs from tradi-
tional manometry; it has 36 sensors, each 1 cm apart, compared with
3 to 5 cm apart in traditional manometry (Fig. 13.2). Indications for
usage, transnasal placement, and the number of swallows needed are
the same, but HRM is more tolerable as only one placement of the
catheter is needed. In HRM, sensors are located longitudinally and
radially and pressure values between the sensors are extrapolated by
software to create a complete pressure continuum, which is converted
into a color three-dimensional graph. The red color tones indicate high
pressures and blue color tones indicate low pressures. A topographic
plot combining both the anatomy and physiology of the esophagus is
developed from the data. The graph plots time and location on one
axis and pressure as color on the other axis, creating an isobaric contour
(IBC) map. The map demonstrates physiologic changes in the variables
of space, contraction, and velocity, tracking the movement along the
esophageal anatomy.
HRM and EPT both define esophageal motor function but EPT is
more sensitive in determining peristalsis and LES function. EPT plots
demonstrate the anatomy, physiology, and pathophysiology of the
esophagus. The UES, LES, and passage through the crura are indicated
by sudden increases in pressure along the EPT map, indicating tightness
in the esophageal lumen. The LES is categorized as a type I at the LES,
type II slightly separate, and type III, complete separation defined only
by a respiratory inversion point (RIP).
Esophageal peristalsis comprises three contractile segments. The
first is the UES, second the tubular esophagus in two places, and third
the LES. There is a transition zone, a pressure trough between the first
and second zones of the esophagus, where control of peristalsis shifts
from the central nervous system to the enteric nervous system (myenteric
plexus).
The contractile deceleration point (CDP) is measured on EPT by
the slope of the IBC at 30 mm Hg. In the esophagus, conduction occurs
faster proximally, correlating with the presence of a transient phrenic
ampulla distally, which stretches, then the LES valve is elevated and is
responsible for the slower emptying. The CDP is located within 3 cm
of the upper aspect of the LES. Integrated relaxation pressure (IRP)
measures the ability of the LES to relax. Manometry cannot differentiate
between LES abnormalities of stenosis versus compression, which can
cause outflow obstruction. The e-sleeve is a 10-second period, averaging
10 to 20 mm Hg at the LES, which includes crural contraction pres-
sures. The IRP is the average pressure measured during the 4 seconds
when the e-sleeve value is lowest. Distal latency (DL)—median 6.2
seconds and minimum of 4.6 seconds—measures the time at the end
of a swallow between UES relaxation and the CDP.
The distal contractile integral (DCI) combines distal esophageal
length, time of contraction, and mean amplitude of contraction. DCI
measures the distance from the proximal to the distal pressure troughs
excluding the first 20 mm Hg. According to the CC, contractile vigor
relies on the DCI. Lower than 100 mm Hg/s/cm indicates failed peri-
stalsis, whereas between 100 and 450 mm Hg/s/cm indicates weak
peristalsis; however, both are ineffective. Normal is between 450 and
8000 mm Hg/s/cm, whereas hypercontractility is defined as above
8000 mm Hg/s/cm. Fragmented peristalsis is defined as a large break
(>5 cm) in the 20 mm Hg IBC and is commonly found in patients with
dysphagia. A premature contraction has a DL less than 4.5 seconds and
a DCI greater than 450 mm Hg/s/cm.

Fig. 13.2  High-Resolution Manometry. IBP, Isobaric pressure; LOS, lower oesophageal sphincter; UOS,
upper oesophageal sphincter. (Reused with permission from Fox MR, Bredenoord AJ: Oesophageal high-
resolution manometry: moving from research into clinical practice, Gut 57:405-423, 2008, F2.)

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 CHAPTER 13  Esophageal Motility Disorders and Diagnosis
Esophageal contraction and pressurization differ in that a contrac-
tion must squeeze the muscle and close the lumen whereas pressurization
occurs with the UES, LES, and esophageal lumen all open. Pressurization
is visualized on EPT with vertical lines indicating increased pressure
obstructed at both ends by a greater pressure. Pressure along the entire
length of the esophagus is termed panesophageal pressurization and is
diagnostic of type II achalasia. Compartmentalized pressurization can
be found in postsurgical patients after a fundoplication surgery, hiatal
hernia repair, or bariatric surgery.
The CC, version 3.0, enables the diagnosis of dysmotility disorders
using EPT. The prevalence of esophageal dysmotility disorders is 5%
of the general population. HRM has been used to characterize EPT
findings in motility disorders. (Achalasia is explained further on.) EGJOO
is defined by HRM as having a median IRP above the upper limit of
normal with evidence of weak peristalsis, not meeting the threshold of
severity of achalasia. The treatment for both may be similar. Initial
therapy is botulinum toxin (Botox) injection and dilation, reserving
surgical myotomy or peroral endoscopic myotomy (POEM) for cases
where less invasive treatments have failed.
The first of the major motility disorders, absent contractility, occurs
with a normal average IRP and 100% failed peristalsis. Distal esophageal
spasm (DES) is defined as a normal median IRP with greater than 20%
percent premature contractions and a DL less than 4.5 seconds. HRM
has defined DES more accurately than traditional manometry. Diag-
nostically, DL is specific for DES. When DL is reduced, which is rarely
found, symptoms of dysphagia or chest pain are almost always present
and are consistent with either DES or type III achalasia. Hypercontractile
(jackhammer) esophagus was previously called NE with diagnosis by
traditional manometry. It is defined as two swallows or more with a
DCI greater than 8000 mm Hg/s/cmin conjunction with single-peaked
or multipeaked contraction. When hypercontractile esophagus is present
with EGJ outflow obstruction, the motility resolves when the obstruc-
tion is corrected.
The minor disorders of peristalsis include IEM and fragmented
peristalsis. IEM is defined by an average IRP less than 15 mm Hg and
≥50% ineffective swallows. IEM is associated with GERD. Nontransmit-
ted contractions are present and ineffective at propelling food through
a normal LES. Promotility drugs are recommended for IEM, but there
is little evidence that they resolve symptoms. Fragmented peristalsis is
defined as an average IRP less than 15 mm Hg with a normal DL, ≥50%
fragmented contractions, and a DCI above 450 mm Hg/s/cm. Large
breaks, defined as greater than 5 cm in the 20 mm Hg IBC, are signifi-
cantly more common in patients complaining of dysphagia. The evidence
of normal DL, or contraction vigor, distinguishes “fragmented peristalsis”
from IEM.
Treatment and Management
Despite the categorization of motility disorders into separate entities
based on HRM and EPT, disorders involving different pathophysiologic
mechanisms and manometric findings do not always correlate with
symptoms. The advancement of HRM and EPT has been extremely
beneficial in diagnosis, but there remains few successful therapies with
which to treat motility disorders. Treatment focuses on symptom reduc-
tion and begins with reassurance, because many disorders may have a
psychiatric component. Patients’ most frequent complaint is pain, which
may be related to GERD and not the motor disorder; therefore treat-
ment should also include proton pump inhibitors (PPIs). It is unknown
whether IEM is the cause or the effect of GERD, but resolving reflux
helps to improve IEM. Unfortunately effective motility medications for
IEM, such as cisapride, are no longer available.
Besides PPI medications for associated acid reflux, limited treatments
have been developed to address the symptoms and not the specific
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69
diseases. If dysphagia is the initial complaint, patients are initially treated
with a calcium channel blocker such as diltiazem 80 to 240 mg/day. If
chest pain is the primary complaint, treatment should be initiated with
diltiazem. In NE, diltiazem, 60 to 90 mg four times daily significantly
reduces chest pain more than a placebo. In DES, diltiazem reduces both
chest pain and dysphagia.
Tricyclic antidepressants such as imipramine 25 to 50 mg/day or
trazadone, 100 to 150 mg/day administered at bedtime may also be
used for chest pain symptoms. Tricyclic antidepressants have not been
demonstrated to improve dysmotility but are believed to improve the
visceral sensory perception to chest pain. Tricyclic antidepressants, which
have proven benefit for chest pain, have produced the most success
with motility disorders. A 70% incidence of concomitant psychiatric
disorders has been observed with motility disorders.
In patients who fail to respond to primary treatments, secondary
options for NE, DES, and EGJOO include botulinum toxin injection
above the EGJ, which relieves dysphagia but not chest pain, regurgita-
tion, or heartburn. The effects last 6 months and therefore the injection
must be repeated. Other options to reduce LES pressure include: a nitric
oxide–contributing drug such as isosorbide 10 mg or sildenafil 50 mg,
which is given for chest pain. Bougie dilation may also be used and
may be beneficial in 40% of patients with severe manometric abnor-
malities. In DES, peppermint oil, as in Altoids, can improve manometric
abnormalities and chest pain.
Surgical therapy is reserved for patients who have failed medical
treatment with severe dysphagia and/or chest pain. Surgical options
include a long myotomy from the arch of the aorta across the LES, with
an added antireflux procedure to address severe esophageal dysmotility.
Thoracoscopy is the preferred technique for long myotomy and a viable
alternative to open surgery. Myotomy with partial fundoplication for
isolated EGJOO relieves dysphagia and chest pain, suggesting a primary
sphincter dysfunction. Some data support POEM for patients with NE
and DES, but long-term results are not documented and patients can
develop recurrent dysphagia and reflux. Because medication for IEM
is used only to treat associated reflux, patients may be offered partial
fundoplication to treat GERD, with expected relief of reflux in 79% of
patients.
Course and Prognosis
Motility disorders rarely progress and are not known to be fatal.
Time alone has demonstrated improvement in DES symptoms and
manometry findings. Up to 75% of patients treated with tricyclic anti-
depressants for chest pain may experience prolonged remission of
symptoms. Achalasia with worsening dysphagia and regurgitation devel-
ops in 5% of patients. Open surgical therapy has been successful
in 50% of patients when dysmotility is associated with dysphagia.
Thoracoscopic myotomy for NE and DES has resulted in a good or
excellent result in 80% of patients, compared with only 26% of patients
treated with medication or dilation. Minimally invasive surgery offers
patients with NE and DES the best opportunity to become asymptomatic.
Patients with IEM should undergo laparoscopic partial fundoplication
for relief of reflux.
ACHALASIA
With an incidence of 1 to 6 per 100,000 population in North America,
achalasia is the most common motor disorder of the esophagus (Fig.
13.3). It affects both genders equally and most commonly occurs in
people 25 to 60 years of age. With the advent of HRM and EPT findings
in motility disorders, normal and vigorous achalasia subtypes have now
been more specifically classified into three subtypes. The traditional
form, characterized by extensive esophageal dilatation, aperistalsis,
70 SECTION I Esophagus

Cardiospasm with
hypertrophy of circular
muscle layer

Esophagoscopic view

"Thin-walled" type of
musculature in cardiospasm

Fig. 13.3  Achalasia (Cardiospasm or Achalasia Cardiae).

and a thickened LES that does not relax to baseline pressure affects
75% of patients with achalasia. The remaining 25% have “vigorous”
achalasia. Compared with patients with traditional achalasia, those
with vigorous achalasia seek treatment at an earlier stage of disease
and have higher muscle contraction amplitude, minimal esophageal
dilatation, higher LES pressure, and prominent tertiary contractions.
Patients with achalasia lack ganglion cells in the myenteric plexus of
Auerbach in the distal esophagus. Degeneration of the vagal motor
dorsal nucleus and destruction of the vagal nerve branches have been
observed.
The etiology of primary or idiopathic achalasia remains unknown.
Achalasia may be an immune-mediated inflammatory disease in which
esophageal neurons are destroyed by herpes simplex virus type 1 (HSV-1)
reactive T cells present in the LES muscles. Myenteric antiplexus anti-
bodies are present in 100% of women and 67% of men with achalasia.
The response occurs after HSV-1 infection and is believed to have a
genetic predisposition.
Secondary achalasia results from diseases that cause esophageal motor
abnormalities that are similar to achalasia. These diseases include amy-
loidosis, chronic idiopathic intestinal pseudo-obstruction, eosinophilic
esophagitis, Fabry disease, juvenile Sjögren syndrome, sarcoidosis, mul-
tiple endocrine neoplasia type 2B, and neurofibromatosis.
Clinical Picture
Almost all patients with achalasia have dysphagia of solids, and 66%
to 85% have dysphagia of liquids. Patients initially feel heaviness or
constriction in the chest when under stress. Food itself causes some
stress, eventually resulting in obstruction. Retrosternal chest pain may
occur in 40% to 60% of patients but improves over time. Patients
eventually become afraid to eat as symptoms of dysphagia, chest pain,
and regurgitation of food develop. Regurgitation of undigested food
occurs in 60% to 90% of patients. Difficulty belching occurs in up to
85% of patients due to poor relaxation of the UES. Most patients main-
tain their nutritional status with little weight loss. Pneumonia is common
in elderly patients, approximately 8%, from the regurgitation and aspi-
ration of food.
Neither the severity nor the total number of achalasia-related symp-
toms correlates with the severity of radiographic findings. Although it

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 CHAPTER 13  Esophageal Motility Disorders and Diagnosis
is the most common symptom, dysphagia is the initial symptom in
only 39% of patients. Heartburn occurs in 25% to 75%. Slow eating
occurs in 79% of patients, regurgitation occurs in 76%, and 60% engage
in characteristic movements such as arching the neck and shoulders,
raising the arms, standing and sitting straight, and walking to assist in
swallowing food. Rapid progression of weight loss may be a sign of
pseudoachalasia from a malignancy.
Diagnosis
Barium esophagraphy shows dilatation of the distal esophagus, aperi-
stalsis, and poor relaxation of the LES. There is a classic bird-beak
appearance as a dilated portion of the LES esophagus tapers to a point.
Fluoroscopy may visualize spasms in the esophagus as it attempts to
empty its contents through the LES. Epiphrenic diverticula are often
associated with achalasia. Esophagraphy is not sensitive and may appear
normal in 33% of patients.
Despite its unique visual appearance, the diagnosis of achalasia is
made through manometric evaluation. Traditional manometry has
revealed simultaneous low-amplitude contractions of the esophageal
body that do not propagate. The LES narrows to 2 cm, and the LES
relaxes incompletely.
HRM has become the standard as it is able to more specifically
characterize motility disorders by the EPT findings. As a result, achalasia
may now be diagnosed and described in thee subtypes by this technol-
ogy. Type I achalasia has a median IRP that is greater than the upper
limit of normal, 100% failed peristalsis with a DCI less than 100 mm
Hg/s/cm, and minimal pressurization within the lumen of the esophagus.
Type II achalasia has a median IRP that is greater than the upper limit
of normal, there is no normal peristalsis, and panesophageal pressuriza-
tion is seen with ≥20% of swallows. Type III achalasia has a median
IRP that is greater than the upper limit of normal, there is no normal
peristalsis, preserved fragments of nonpropagating distal peristalsis or
premature contractions with a DCI greater than 450 mm Hg/s/cm with
≥20% of swallows.
In the past, achalasia and vigorous achalasia had no measurable
diagnostic definitions. HRM and EPT have provided a measurable
technique to categorize the subtypes, thus allowing for different thera-
peutic options and resultant outcome results.
Esophagoscopy is performed to rule out malignancy and other dis-
eases that are a part of the differential diagnosis and to evaluate the
mucosa before any procedure is undertaken. Endoscopic findings may
include dilatation and atony of the esophageal body and LES closing
that is difficult to open; a pop may be detected as the scope passes
through the LES. Small particles of food may be retained early and
large amounts retained late in the disease process. Inspissated food
particles may adhere to the thickened mucosa, causing leukoplakia, and
there may be resultant erythema, inflammation, or ulceration. The
esophagus may also become elongated before dilatation.
Endoscopic ultrasound (EUS) may reveal a LES with a thickened
circular muscle layer at the LES and along the esophagus. It may also
detect a distal esophageal or gastric cardia tumor which then may be
biopsied; 10 mm thick or greater is suspicious. The differential diagnosis
of achalasia includes GERD, pseudoachalasia from a malignancy, or
other esophageal dilation disorders such as DES and jackhammer
or NE.
Treatment and Management
Therapy consists of medications, local injections, pneumatic dilatation,
and surgery and is directed at palliation of symptoms and prevention
of complications. Medications have had limited success in relieving
achalasia symptoms and are a last resort if botulinum toxin does not
relieve symptoms.
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71
Sublingual medications that relieve some symptoms of achalasia
include nifedipine 10 to 30 mg, isosorbide dinitrate 5 mg, and nitro-
glycerin 0.4 mg; nifedipine is taken 30 minutes before meals; isosorbide
dinitrate and nitroglycerin are taken 15 minutes before meals. These
medications relax the smooth muscle of the LES. Oral calcium channel
blockers have resulted in some symptom improvement, as has sublingual
nitroglycerin, in 53% to 87%.
Botulinum toxin A (Botox) inhibits acetylcholine release from the
nerve endings within the myenteric plexus and at the nerve-muscle
junction. It decreases LES pressure in patients with achalasia and has
limited adverse effects. It is successful in 30% to 75% of patients but
the results last only 6 to 9 months so the injection must be repeated.
Only 50% of patients respond for more than 1 year, and 70% experi-
ence relapse at 2 years. Long-term success is highest in elderly patients
and in those with LES pressure that exceeds normal by only 50%. Botu-
linum toxin injection is a good option for elderly, debilitated patients
who are not candidates for more invasive procedures as well as for
patients who prefer this option.
Pneumatic dilatation is another option that is less invasive than
surgery. Dilatation with a 50-Fr dilator provides temporary relief for
only 3 days. Forceful dilatation with a balloon is more successful because
the circular muscles must be torn to achieve long-term relief. The balloon
creates pressure to 300 mm Hg for 1 to 3 minutes and distention to a
diameter of 3 cm. After dilatation, a meglumine diatrizoate (Gastro-
grafin) swallow is performed and the patient is observed for 6 hours
before discharge. The most severe complication after dilatation is esopha-
geal perforation, which occurs in 3% of patients. Small tears with free
flow of contrast back into the esophagus may be treated conservatively.
If there is free flow into the mediastinum, emergency thoracotomy is
indicated. Surgery is usually necessary for 50% of perforations. Symptom
relief is successful in 55% to 70% of achalasia patients with an initial
dilatation and up to 93% with multiple dilatations. Symptom relief is
the traditional measure used to assess treatment success. The timed
barium study (TBS) is also used to assess esophageal emptying and
correlates with a successful outcome in patients undergoing pneumatic
dilatation. Poor esophageal emptying can be seen on barium esopha-
graphy in almost 30% of achalasia patients reporting complete symptom
relief after pneumatic dilatation; 90% of these patients experience failed
treatment within 1 year.
Laparoscopic Heller myotomy is the procedure of choice for patients
with achalasia. It results in all the same benefits as thoracoscopy
with less dysphagia and less reflux. Surgery is indicated in patients
younger than 40 years or in those who have recurrent symptoms after
botulinum type A treatment or pneumatic dilatation. It is also indi-
cated in those who are at high risk for perforation from dilatation
because of diverticula, previous GEJ surgery, or a tortuous or dilated
esophagus.
Myotomy involves the division of all layers of muscle down to the
mucosa with extension of at least 1 cm onto the stomach. A postopera-
tive esophagram is performed to indicate adequate esophageal clearance
and exclude a leak. Resting pressures of the esophageal body and LES
are lower after surgery. Esophageal transit improves in postoperative
patients but is still slower than in healthy controls. Persistent postopera-
tive dysphagia may occur in up to 5% and may be treated with dilatation
or repeat surgery.
A partial fundoplication, Toupet or Dor, should be performed to
prevent reflux. Median hospital stay is only 2 days. The incidence of
GE reflux in patients who had undergone esophageal myotomy alone
was 64%, but 27% in those who had myotomy and antireflux procedure.
At 15 years after surgery, 11% of patients will develop esophagitis and
more than 40% will have reflux with a partial fundoplication. Good to
excellent long-term results were seen in approximately 90% of patients
72 SECTION I Esophagus
at 3-year follow-up and in 75% to 85% after 15 years. Approximately
2% of patients develop esophageal cancer after surgery.
Thoracoscopic myotomy without fundoplication produces an excel-
lent response in 80% to 90% of patients. Compared with thoracotomy,
surgery is shorter, there is less blood loss and less need for a narcotic,
and recovery to normal activity is faster. Median hospital stay is 3 days,
compared with 2 days after laparoscopic Heller myotomy. GERD may
develop in 60% of patients after thoracoscopic myotomy, and dysphagia
persists in approximately 10%.
POEM is a technique that uses endoscopy to perform an esophageal
myotomy and division of the LES sphincter. The mucosa is divided to
gain access to the underlying muscle; the endoscope is then inserted
down the plane to the gastric cardia, where the muscularis propria of
the LES is divided by cautery. An antireflux procedure is not performed.
Postoperative GERD occurs in 17% to 21% of patients at 2 months
and 3 years, respectively. Small studies have not reported mortality, but
complications of pneumothorax, bleeding, mucosal perforation, and
pleural effusions in 3% of patients have been reported. Results reveal
decreased LES pressures and marked symptomatic improvement. Unique
to POEM are the surprisingly good results in patients with type III
“end stage” achalasia, who usually fail all other treatments.
Course and Prognosis
Achalasia can now be more specifically classified. Type II patients are
most likely to respond to all therapies—such as botulinum toxin with
a response in 71%, pneumatic dilatation with a response in 91%, and
Heller myotomy with a response in 100%—compared with only a 56%
overall response in type I patients and 29% in type III patients. If left
untreated, esophagitis may develop from stasis of retained food; 30%
of patients may aspirate esophageal contents. Coughing attacks and
pulmonary infections may also occur. Carcinoma may develop in 2%
to 7% of patients. Currently there is no recommended surveillance
program for malignancy.
In the primary treatment of achalasia, dilatation is superior to botu-
linum type A treatment in the short term; clinical remission rates at 4
months are approximately 90% (dilatation) versus 40% (Botox). Also,
myotomy is more reliable in reducing LES pressure than pneumatic
dilatation. Good or excellent relief of dysphagia is obtained in 90% of
myotomy patients (85% after thoracoscopic myotomy, 90% after lapa-
roscopic myotomy). Mortality is rare. After documentation that lapa-
roscopic treatment outperforms balloon dilatation and botulinum type
A injection, the laparoscopic Heller myotomy is the preferred treatment
for the appropriately indicated patients diagnosed with achalasia.
Surgery for achalasia can fail in 10% to 15% of patients, more fre-
quently in those who have had previous endoscopic procedures, a longer
duration of symptoms, severely dilated esophagus, and very low LES
pressure. Recurrent dysphagia after myotomy should first be treated
with pneumatic dilatation. If this fails, laparoscopic reoperation for
achalasia is safe and feasible and is the procedure of choice. It is per-
formed in approximately 5% of patients with recurrent or persistent
dysphagia. Repeat surgery improves symptoms in more than 85% of
patients. The surgeon’s experience and recognition of the cause for
failure of the original surgery are the most important factors in predict-
ing outcome.
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The future role of POEM has yet to be defined, as the procedure is
being performed only in centers with significant experience, and the
development of GERD postprocedure remains a potential problem.
Patients with type III achalasia may preferentially benefit from this
procedure.
Over their lifetimes, patients with achalasia develop many complica-
tions that warrant therapy, but their life expectancy and eventual cause
of death do not differ from those in the average population.
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