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## Complete Summary

#### GUIDELINE TITLE

Esophageal achalasia.

#### BIBLIOGRAPHIC SOURCE(S)

Society for Surgery of the Alimentary Tract (SSAT). Esophageal
achalasia. Manchester (MA): Society for Surgery of the Alimentary Tract
(SSAT); 2003. 3 p.

#### GUIDELINE STATUS

This is the current release of the guideline.

This guideline updates the previously issued version: Society for
Surgery of the Alimentary Tract. Achalasia. Manchester (MA): Society for
Surgery of the Alimentary Tract (SSAT); 1996-2000. 4 p.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Esophageal achalasia

#### GUIDELINE CATEGORY

Diagnosis\
Risk Assessment\
Treatment

#### CLINICAL SPECIALTY

Family Practice\
Gastroenterology\
Internal Medicine\
Surgery

#### INTENDED USERS

Physicians

#### GUIDELINE OBJECTIVE(S)

To guide primary care physicians to the appropriate utilization of
surgical procedures on the alimentary tract or related organs

#### TARGET POPULATION

Patients with symptomatic esophageal achalasia

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis**

1.  Barium swallow

2.  Endoscopy

3.  Esophageal manometry

4.  Prolonged pH monitoring

5.  Endoscopic ultrasound

6.  Computed tomography

**Treatment**

1.  Pneumatic dilatation

2.  Intrasphincteric injection of botulinum toxin (Botox)

3.  Laparoscopic Heller myotomy and partial fundoplication

4.  Esophagectomy (reserved for failures after myotomy)

5.  Surveillance endoscopy as follow-up

#### MAJOR OUTCOMES CONSIDERED

-   Symptom relief

-   Symptom recurrence, gastroesophageal reflux following interventions

-   Mortality rates associated with esophageal myotomy

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

Not stated

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Not stated

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

Not applicable

#### METHODS USED TO ANALYZE THE EVIDENCE

Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not applicable

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

The Society for Surgery of the Alimentary Tract (SSAT) guidelines are
based on statements and recommendations that were overwhelmingly
supported by clinical evidence. Each represents a consensus of opinion
and is considered a reasonable plan for a specific clinical condition.

(See companion document Gadacz TR, Traverso LW, Fried GM, Stabile B,
Levine BA. Practice guidelines for patients with gastrointestinal
surgical diseases. J Gastrointest Surg 1998;2:483-484.)

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Not applicable

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

The guidelines were reviewed by several committee members and then by
the entire committee on several occasions. Each guideline was then sent
back to the original author for final comment and reviewed again by the
committee. Each guideline was approved by the Board of Trustees of the
Society for Surgery of the Alimentary Tract and final comments were
reviewed by the committee.

(See companion document Gadacz TR, Traverso LW, Fried GM, Stabile B,
Levine BA. Practice guidelines for patients with gastrointestinal
surgical diseases. J Gastrointest Surg 1998;2:483-484.)

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

**Diagnosis**

In addition to careful symptomatic evaluation, the following tests
should be routinely performed: Barium swallow usually shows narrowing at
the level of the gastroesophageal junction (\"bird beak\") and various
degrees of esophageal dilatation. Endoscopy is important to rule out the
presence of a peptic stricture or cancer and gastroduodenal pathology.
Esophageal manometry is the key test for establishing the diagnosis. The
classic manometric findings are: (a) absence of esophageal peristalsis
and (b) hypertensive or normotensive lower esophageal sphincter (LES)
that fails to relax completely in response to swallowing.

Prolonged pH monitoring may be helpful preoperatively in patients who
have previously failed treatment with pneumatic dilatation, Botulinum
toxin (Botox), or surgical myotomy, for whom a myotomy is planned.
Demonstration of reflux clearly indicates the need for a fundoplication
in addition to the myotomy.

In patients older than 60 years of age with recent onset of dysphagia
and excessive weight loss, secondary or pseudo-achalasia should be ruled
out. Because a cancer of the gastroesophageal junction is the most
common cause of pseudo-achalasia, an endoscopic ultrasound or a computed
tomography (CT) scan of the gastroesophageal junction can help to
establish the diagnosis.

**Treatment**

Treatment is palliative, and it is directed toward elimination of the
outflow resistance at the level of the gastroesophageal junction. The
following treatment modalities are available to achieve this goal:

Pneumatic dilatation has a success rate between 70 and 80%.
Gastroesophageal reflux occurs after dilatation in 25 to 35% of
patients. Up to 5% of patients may sustain a perforation at the time of
a dilatation. These patients may require open surgery to close the
perforation and perform a myotomy.

Intrasphincteric injection of botulinum toxin results in initial relief
of symptoms in about 60% of patients, but this is transitory and
symptoms will return in the majority of patients within a year.
Subsequent injections are less effective and the benefit is of briefer
duration. In addition, this treatment may cause an inflammatory reaction
at the level of the gastroesophageal junction, which obliterates the
anatomic planes. Consequently, a myotomy is more difficult, a mucosal
perforation occurs more frequently, and the relief of dysphagia is less
predictable. Because of these shortcomings, botulinum toxin should be
reserved for elderly or high-risk patients who are poor candidates for
dilatation or surgery.

Traditionally, pneumatic dilatation has been the first line of treatment
for esophageal achalasia, while surgery was reserved for patients who
had persistent dysphagia after multiple dilatations or who had suffered
a perforation during dilatation.

Today, minimally invasive surgery has completely changed this treatment
algorithm and a laparoscopic Heller myotomy and partial fundoplication
is preferred by most gastroenterologists and surgeons as the primary
treatment modality. Critical details of the operation include a generous
myotomy of the lower esophagus, extending well onto the gastric wall.
Because of the lack of esophageal peristalsis, a partial (Dor or
Toupet), rather than a total fundoplication is frequently added to
prevent reflux. Patients can usually eat the morning of the first
postoperative day and can be discharged home after one or two days.

The need for esophagectomy for achalasia is very uncommon, even in the
presence of a dilated esophagus, and should be reserved for failures
after myotomy.

All patients undergoing treatment for achalasia should be followed by
surveillance endoscopy, because they are at increased risk for
development of both squamous and adenocarcinoma.

**Expected Outcomes**

About 90% of patients have long-term relief of dysphagia after a
myotomy, with a low incidence of symptomatic acid reflux. Patients
should undergo 24-hour pH testing routinely after surgery, as reflux is
often asymptomatic, and should be treated with proton pump inhibitors if
abnormal acid reflux is present.

**Qualifications for Performing Operations for Achalasia**

At a minimum, surgeons who are certified or eligible for certification
by the American Board of Surgery, the Royal College of Physicians and
Surgeons of Canada, or their equivalent should perform operations for
achalasia. The qualifications of a surgeon performing any operative
procedure should be based on training, experience, and outcomes.

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of supporting evidence is not specifically stated for each
recommendation.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

-   *Pneumatic dilatation* has a success rate between 70 and 80%.

-   *Intrasphincteric injection of botulinum toxin* results in initial
    relief of symptoms in about 60% patients, although relief is
    transitory and symptoms will return in the majority of patients
    within a year.

-   About 90% of patients have long-term relief of dysphagia after a
    myotomy, with a low incidence of symptomatic acid reflux.

#### POTENTIAL HARMS

-   *Pneumatic dilatation*. Gastroesophageal reflux occurs after
    dilatation in 25 to 35% of patients. Up to 5% of patients may
    sustain a perforation at the time of a dilatation.

-   *Intrasphincteric injection of botulinum toxin* results in initial
    relief of symptoms, but this relief is transitory and symptoms will
    return in the majority of patients within a year. Subsequent
    injections are less effective and the benefit is of briefer
    duration. In addition, this treatment may cause an inflammatory
    reaction at the level of the gastroesophageal junction, which
    obliterates the anatomic planes. Consequently, a myotomy is more
    difficult, a mucosal perforation occurs more frequently, and the
    relief of dysphagia is less predictable.

-   Aspiration of retained food in the esophagus at the time of
    induction of anesthesia and perforation of the esophageal mucosa are
    the most common operative complications. Persistent or recurrent
    dysphagia occurs in 5 to 10% of patients.

-   Up to 15% patients may experience gastroesophageal reflux after
    myotomy. The mortality rate after elective myotomy is less than 1%.

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

These guidelines have been written by the Patient Care Committee of the
Society for Surgery of the Alimentary Tract (SSAT). Their goal is to
guide primary care physicians to the appropriate utilization of surgical
procedures on the alimentary tract or related organs and they are based
on critical review of the literature and expert opinion. Both of the
latter sources of information result in a consensus that is recorded in
the form of these Guidelines. The consensus addresses the **range** of
acceptable clinical practice and should not be construed as a standard
of care. These Guidelines require periodic revision to ensure that
clinicians utilize procedures appropriately, but the reader must realize
that clinical judgment may justify a course of action outside of the
recommendations contained herein.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Society for Surgery of the Alimentary Tract (SSAT). Esophageal
achalasia. Manchester (MA): Society for Surgery of the Alimentary Tract
(SSAT); 2003. 3 p.

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

1996 (revised 2003 Feb 1)

#### GUIDELINE DEVELOPER(S)

Society for Surgery of the Alimentary Tract, Inc - Medical Specialty
Society

#### SOURCE(S) OF FUNDING

Society of Surgery of the Alimentary Tract, Inc.

#### GUIDELINE COMMITTEE

Patient Care Committee

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

Not stated

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

This guideline updates the previously issued version: Society for
Surgery of the Alimentary Tract. Achalasia. Manchester (MA): Society for
Surgery of the Alimentary Tract (SSAT); 1996-2000. 4 p.

#### GUIDELINE AVAILABILITY

Electronic copies: Available from the [Society for Surgery of the
Alimentary Tract, Inc. Web
site](http://www.ssat.com/cgi-bin/guidelines.cgi).

Print copies: Available from the Society for Surgery of the Alimentary
Tract, Inc., 900 Cummings Center, Suite 221-U, Beverly, MA 01915;
Telephone: (978) 927-8330; Fax: (978) 524-0461.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following is available:

-   Gadacz TR, Traverso LW, Fried GM, Stabile B, Levine BA. Practice
    guidelines for patients with gastrointestinal surgical diseases. J
    Gastrointest Surg 1998;2:483-484.

Electronic copies: Not available at this time.

Print copies: Available from the Society for Surgery of the Alimentary
Tract, Inc., 900 Cummings Center, Suite 221-0, Beverly, MA 01915;
Telephone: (978) 927-8330; Fax: (978) 524-8890.

#### PATIENT RESOURCES

None available

#### NGC STATUS

This summary was completed by ECRI on March 28, 2000. The information
was verified by the guideline developer as of May 30, 2000. This summary
was updated by ECRI on September 8, 2004.

#### COPYRIGHT STATEMENT

For terms governing downloading, use, and reproduction of these
guidelines, please contact: <ssat@prri.com>.

### DISCLAIMER

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© 1998-2008 National Guideline Clearinghouse

Date Modified: 11/3/2008