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AchalasiaHighlights

Summary

Overview

Basics

Definition

EpidemiologyAetiology

Pathophysiology

Diagnosis

History & examination

Tests

Differential

Step-by-step

Guidelines

Case history

Treatment

Details

Step-by-stepEmerging

Guidelines

Evidence

Follow Up

Recommendations

Complications

Prognosis

Resources

References

Images

Patient leaflets

CreditsEmail

Print

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Add notes

History & exam

Key factors

dysphagia

Other diagnostic factors

posturing to aid swallowing

retrosternal pressure/pain

heartburn

regurgitation
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slow eating

coughing/choking while recumbent

recurrent chest infections

gradual weight loss

sensation of a lump in the throat (globus)

hiccups

History & exam details

Diagnostic tests

1st tests to order

upper gastrointestinal endoscopy

barium swallow

oesophageal manometry

Tests to consider

chest x-ray

radionucleotide oesophageal emptying studies

CT chest

endoscopic ultrasound

Emerging tests

multi-channel intraluminal impedance

Diagnostic tests details

Treatment details

Presumptive

patients awaiting definitive treatment

pharmacological therapy

Acute

good surgical candidate

pneumatic dilatation

laparoscopic cardiomyotomy

poor surgical candidate

pharmacological therapy

injection of botulinum toxin A

gastrostomy
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Treatment details

Summary

An oesophageal motor disorder characterised by a loss of peristalsis in the distal third of the

oesophagus and failure of the lower oesophageal sphincter to relax in response to swallowing.

The most common presenting symptoms are dysphagia to solids and liquids, regurgitation, and

retrosternal pain. These can be slowly progressive over months or years.

The first investigation for any patient with dysphagia is endoscopy to exclude malignancy, but

subsequent barium swallow studies and oesophageal manometry are usually required to establish the diagnosis

of achalasia.

Treatment is symptomatic, not curative, and is primarily aimed at relieving dysphagia; options include

pharmacological, endoscopic, and surgical procedures.

Other related conditions

Assessment of dysphagia

Aspiration pneumonia

Chagas disease

Gastro-oesophageal reflux disease

Herpes simplex infection

Herpes zoster infection

Measles infection

Oesophageal cancer

DefinitionAchalasia, from the Greek for 'does not relax', is an oesophageal motordisorder of unknown aetiology, characterised by oesophageal aperistalsisand insufficient lower oesophageal sphincter (LOS) relaxation in response toswallowing. This results from loss of inhibitory nitrinergic neurons in theoesophageal myenteric plexus.

EpidemiologyAchalasia may occur at any age; however, incidence peaks in individualsover the age of 60 or 70 years. There is equal gender distribution. Althoughseveral series have a female predominance, this may be due to greaternumbers of older women.

In British studies the incidence ranges between 0.4 and 1 per 100,000, risingto 3 per 100,000 for people aged >70 years. The prevalence is around 8 per100,000. Incidence rates in New Zealand, Israel, and Canada[1] are similar,
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at around 1 per 100,000, but achalasia seems less common among someAsian and African populations. For example, in Singapore the prevalence is0.3 per 100,000[2] and in Zimbabwe only 0.03 per 100,000. In theseinstances, however, case identification may be a factor.[3] The annualincidence of achalasia in the US, approximately 0.6 per 100,000, has

remained stable in recent decades.[3] [4]Racial differences have been highlighted in some studies, with a NewZealand study showing a higher incidence of achalasia in Pacific Islandersand people of Maori descent than in white people. In Singapore, achalasiawas more common in Chinese and Indian people compared with Malaysianpeople. In the US, achalasia occurs equally frequently in white and non-whitepopulations.[5] Regional and ethnic variations in the incidence of achalasiasuggest a role for both environmental and genetic factors in its aetiology.

AetiologyInflammatory destruction of inhibitory nitrinergic neurons in the oesophagealmyenteric (Auerbach) plexus results in loss of peristalsis and incompletelower oesophageal sphincter relaxation. The exact cause of this inflammatoryprocess is unknown, but possible triggers include:

Infection. Infectious diseases such as Chagas disease can manifest in a similar way to achalasia.

Herpes and measles viruses have been associated with achalasia, and increased antibody titres against these

viruses have been found in patients with achalasia compared with healthy controls. However, a causal

relationship has not been established.

Autoimmunity. Patients with achalasia are more likely to suffer from autoimmune conditions.[6] This

association is supported by the presence of myenteric plexus antibodies in many patients with achalasia, a T-cell

infiltrate in the inflamed myenteric plexus, and increased prevalence of HLA class II antigens.

Genetic factors. Familial cases of achalasia are rare. However, a number of cases of achalasia have

been reported among children of consanguineous couples.[7] The triple-A (Allgrove) syndrome, characterised

by achalasia, alacrima, and adrenocorticotrophic hormone-resistant adrenal insufficiency, is an autosomal

recessive disorder that has been mapped to chromosome 12.[8]

PathophysiologyThe smooth muscle of the distal oesophageal wall and lower oesophagealsphincter are innervated by vagal pre-ganglionic fibres arising in the dorsalmotor nucleus. These synapse in the myenteric plexus ganglia with post-ganglionic fibres consisting of cholinergic excitatory neurons and inhibitoryneurons releasing nitric oxide (NO) and vasoactive intestinal peptide.
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In achalasia, the loss of ganglion cells in the myenteric (Auerbach) plexus isaccompanied by an inflammatory response, consisting of T lymphocytes,eosinophils, and mast cells, and neural fibrosis. The end result of thesechanges is a selective loss of post-ganglionic inhibitory neurons containingNO and vasoactive intestinal peptide. Stimulatory cholinergic neurons remain

intact, resulting in a high basal lower oesophageal sphincter pressure andinsufficient relaxation.

Aperistalsis is caused by loss of the latency gradient that normally permitssequential contractions along the oesophageal body, a process that ismediated by NO. Studies measuring NO synthase activity have confirmedloss of NO innervation in patients with achalasia, as have histological studiesof oesophagectomy specimens from patients with achalasia.[8]

History & examination

Key diagnostic factorsshow alldysphagia (common)Other diagnostic factorsshow allposturing to aid swallowing (common)

retrosternal pressure/pain (common)

regurgitation (common)

gradual weight loss (common)

heartburn (uncommon)

slow eating (uncommon)

coughing/choking while recumbent (uncommon)

recurrent chest infections (uncommon)

sensation of a lump in the throat (globus) (uncommon)

hiccups (uncommon)

Risk factorsshow all

Strong

Allgrove syndrome

Weak

herpes and measles viruses

autoimmune disease

HLA class II antigens

consanguineous parents

History & examinationKey diagnostic factorshide alldysphagia (common)

The key symptom is dysphagia to solids and liquids. Dysphagia to liquids is uncommon in structural

obstruction unless disease is very advanced.

Requires prompt investigation with upper GI endoscopy.

Other diagnostic factorshide allposturing to aid swallowing (common)
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To manage dysphagia, patients may adopt particular postures such as arching the neck and

shoulders or raising the arms.

They may sit straight up or stand during meals or need to walk around after meals.

retrosternal pressure/pain (common)

Pressure may be precipitated by drinking fluids but eased by continued drinking.

Retrosternal pain may be relieved by drinking cold water and typically affects younger people. It is

often described as cramp-like in nature and may wake the individual from sleep.

This may persist even when dysphagia has improved following successful dilatation.

regurgitation (common)

Regurgitation results from retention of food and liquids in the oesophagus, and is more common

later in the course of the disease when the oesophagus becomes dilated.

Bland, undigested food or saliva retained in the oesophagus regurgitates when patient is in the

recumbent position.gradual weight loss (common)

Weight loss caused by achalasia tends to be gradual and mild; rapid weight loss should trigger the

suspicion of malignancy.heartburn (uncommon)

This may occur secondary to fermentation of food retained in the oesophagus, rather than gastric

acid reflux past a high-pressure lower oesophageal sphincter.slow eating (uncommon)

As a result of dysphagia, eating may be slower. This is sometimes noted by friends or relatives.

coughing/choking while recumbent (uncommon)

Secondary to regurgitation of retained food and liquids in the oesophagus.

recurrent chest infections (uncommon)

As a result of regurgitation of retained food, aspiration may result in chest infections with symptoms

of coughing and choking, which can wake patient up.sensation of a lump in the throat (globus) (uncommon)

Patients with achalasia may complain of a lump in the throat.

hiccups (uncommon)

Secondary to delayed transit of food in the oesophagus and diaphragmatic irritation.

Risk factorshide all

Strong

Allgrove syndrome

One of the characteristic features of Allgrove syndrome is achalasia, along with alacrima and

adrenal insufficiency.

Weak

herpes and measles viruses

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Herpes and measles viruses have been associated with achalasia, and increased antibody titres

against these viruses have been found in patients with achalasia compared with healthy controls.autoimmune disease

The association is supported by the presence of antibodies against the myenteric plexus in many

patients, although they are not found in everyone.

HLA class II antigens

Increased prevalence of HLA class II antigens has been found in patients with achalasia, and this

may support an autoimmune aetiology, although this has not yet been established.consanguineous parents

A number of cases of achalasia have been identified among children of parents who are related.

Overall familial cases are rare.

Diagnostic tests1st tests to ordershow all

Test Result

upper gastrointestinal endoscopy mucosa obscured by retained saliva with frothy aoesophagus may be dilated and tortuous and con

oesophagus)

barium swallow loss of peristalsis and delayed oesophageal emptsmoothly to a beak-like narrowing at the gastro-

oesophageal manometry incomplete relaxation of the lower oesophageal oesophageal aperistalsis; typically high resting lo

are followed by simultaneous contraction waves

high-amplitude (vigorous achalasia)

Tests to considershow all

Test Result

chest x-ray absence of the gastric gas bubble or unusual sha

radionucleotide oesophageal emptying studies delayed oesophageal transit

CT chest dilatation of the oesophagus; often concentric th

endoscopic ultrasound normal in achalasia

Emerging testsshow all

Test Result

multi-channel intraluminal impedance identifies chronic fluid retention in the oesophag

Diagnostic tests1st tests to orderhide all

Test

upper gastrointestinal endoscopy

Gastroscopy has low sensitivity for the diagnosis of achalasia and is often reported to be normal in early

an essential first-line investigation to exclude malignancy.[11]
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In patients with a dilated oesophagus, a clear fluid diet for a prolonged period or oesophageal lavage ma

before endoscopy to avoid aspiration and to obtain adequate views. The gastro-oesophageal junction ca

gives way with sustained gentle pressure with the gastroscope.

Biopsies from the cardia are indicated if pseudoachalasia is suspected.

barium swallow

This test is often done with fluoroscopy.

Timed barium studies enable assessment of oesophageal emptying and have been used to evaluate res

pneumatic dilatation or surgical myotomy.[18]

In early disease the swallow may be reported as normal. In advanced disease the dilated oesophagus m

and sigmoid-shaped with diverticula. View image

oesophageal manometry

Incomplete relaxation of the lower oesophageal sphincter with wet swallows and oesophageal aperistals

important manometric criteria for the diagnosis of achalasia.[12] [19]

In some patients with achalasia it might not be possible to pass the manometry catheter through the low

sphincter.

Longitudinal muscle spasm and consequent oesophageal shortening can result in apparent pseudorelax

High-resolution manometry has been shown to be more accurate than conventional manometry in identi

and differentiating it from other forms of oesophageal motility disorders.[13] [14]

Tests to considerhide all

Test

chest x-ray

Appearance may suggest achalasia, but test is not diagnostic.
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radionucleotide oesophageal emptying studies

This is the investigation of choice for monitoring response to therapy.[16]

CT chest

Used to rule out gastro-oesophageal infiltration by invading extrinsic or intrinsic malignancy.

Asymmetrical thickening may suggest pseudoachalasia.

endoscopic ultrasound

This test may be performed to exclude gastro-oesophageal infiltration by an intrinsic or extrinsic maligna

process.

If pseudoachalasia is suspected, this modality can increase diagnostic yield and help in staging.

Emerging testshide all

Test

multi-channel intraluminal impedance

Used in conjunction with oesophageal manometry.[17] This modality is not yet established for the diagn

and is not generally available at the present time.

Differential diagnosis

Condition

Differentiating

signs/symptoms Differentiating tests

Oesophageal carcinoma Dysphagia is

mainly for

solids, although

difficulty in

swallowing

liquids develops

with advanced

disease.

Weight loss

may be severe.

Barium swallow and endoscopy will show oesoph

Reflux oesophagitis Can give rise to

dysphagia

through

inflammatory

swelling or a

Endoscopy usually shows reflux oesophagitis, wi

hiatus hernia may be present below the stricture.

Barium swallow has low sensitivity for oesophagi
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fibrotic peptic

stricture,

sometimes even

in the absence

of endoscopic

abnormalities.

The patient will

usually also

report heartburn

and/or

regurgitation in

addition to

dysphagia.

hiatus hernias. Gastro-oesophageal reflux will lik

Lower oesophageal pH studies will demonstrate

reflux.

Connective tissue disorders

(e.g., systemic sclerosis) Muscle and joint

pain, Raynaud's

phenomenon,

skin changes

(e.g., rash, skin

swelling or

thickening).

Antinuclear antibodies, rheumatoid factor, creatin

initial screening tests for connective tissue patho

Oesophageal spasm Chest pain is

often more

prominent than

dysphagia,

which tends to

be intermittent.

Manometry shows high-amplitude oesophageal c

aperistalsis usually seen in achalasia.

Eosinophilic oesophagitis Presents with

intermittent

dysphagia,

often in young

men with history

of atopy.

Oesophageal biopsy shows eosinophilic infiltratio

power field).

Pseudoachalasia (or secondary Underlying Gastroscopic biopsy of gastro-oesophageal junct

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achalasia)malignancy that

mimics

idiopathic

achalasia.

Patients tend to

be older,

duration of

symptoms

shorter,[21] and

weight loss

greater and

more rapid.

Dysphagia is

clinically

indistinguishabl

e.

malignancy.

Findings at endoscopy, barium swallow, and man

from achalasia.

Chagas' disease Endemic to

Latin America;

multiple-organ

involvement

probably

causing atonic

colon,

myocarditis, and

Romana sign;

swelling of the

eyelids in acute

disease.

Microscopic examination of fresh blood showing

PCR for precise identification of trypanosome sub

Giemsa staining of thick and thin blood films dete

Step-by-step diagnostic approachAchalasia cannot be diagnosed on the basis of the history alone. Symptomsare often slowly progressive, during which time many patients may adapt tosignificant symptoms by slowly altering their diet or eating habits. A minorityof patients present with heartburn, regurgitation, or slow eating comparedwith other family members, rather than dysphagia.[9]
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Furthermore, barium swallow or endoscopy can appear normal in patientswith early disease. Therefore, many patients with achalasia have beensymptomatic for months or years before they seek medical attention andbefore the correct diagnosis is made.[9] [10]

History Dysphagia to solids and liquids is the key symptom of achalasia. Dysphagia to liquids is uncommon in

structural causes of oesophageal obstruction, except in advanced disease. Therefore, its occurrence at

presentation suggests an oesophageal motility disorder.[9]

Individual patients often develop a range of strategies to live with their dysphagia, such as arching the

neck and shoulders, raising the arms, standing or sitting up straight during the meal, and walking around after a

meal.[9] These manoeuvres increase intrathoracic pressure to overcome the increased lower oesophageal

sphincter pressure, allowing oesophageal contents to empty into the stomach.

Drinking fluid may initially cause a sensation of retrosternal pressure, which is relieved by continued

drinking.

Regurgitation results from retention of food and liquids in the oesophagus, and is more common in

established disease when the oesophagus has become dilated. Bland, undigested food or saliva retained in the

oesophagus regurgitates when the patient is in the recumbent position and may wake the patient from sleep with

coughing and choking, sometimes leading to chest infections.

Retrosternal pain typically affects younger patients and may be relieved by drinking cold water. It is often

described as cramp-like in nature and may wake the patient from sleep. This may persist even when dysphagia

has improved following successful dilatation.

Heartburn can also occur, possibly secondary to fermentation of food retained in the oesophagus rather

than gastric acid reflux past a high-pressure lower oesophageal sphincter.

Weight loss is usually gradual and mild, unlike in pseudoachalasia where the underlying malignancy

often causes rapid and profound weight loss.[10]

Atypical symptoms include the sensation of a lump in the throat and hiccups.

Physical examinationThere are no specific physical signs in achalasia, but features of recent, rapidweight loss should alert the clinician to the possibility of underlyingmalignancy, sometimes presenting as pseudoachalasia.

InvestigationsDysphagia should always be promptly investigated.
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Upper gastrointestinal endoscopy, to exclude malignancy, is usually the first investigation for

dysphagia,[11] although barium swallow may be the best initial test for high dysphagia in older individuals, in

case there is a pharyngeal pouch. In early achalasia, endoscopic abnormalities can be subtle and the endoscopy

may be normal.

Chest x-ray may offer some clues: absence of the gastric gas bubble and an unusually shaped

oesophagus may be noted, but is not diagnostic.

Following endoscopy, people with suspected achalasia will usually undergo abarium swallow and oesophageal manometry.

Barium swallow may also be normal in the early stages of the disease, but may demonstrate loss of

peristalsis and delayed oesophageal emptying. In advanced disease, the typical appearance is that of a dilated

oesophagus that tapers smoothly to a beak-like narrowing.View imageIn severe disease, a dilated oesophagus

may be tortuous and sigmoid-shaped with diverticula.

Oesophageal manometry: incomplete relaxation of the lower oesophageal sphincter and oesophageal

aperistalsis are the two most important manometric criteria for the diagnosis of achalasia.[12] High-resolution

manometry has been shown to be more accurate than conventional manometry in identifying achalasia and

differentiating it from other forms of oesophageal motility disorders.[13] [14] Three subtypes of achalasia have

been described using high resolution manometry and this may help to predict treatment response.[15]

CT and endoscopic ultrasound are useful subsequent investigations toexclude gastro-oesophageal infiltration by invading extrinsic or intrinsicmalignancy. Asymmetrical thickening on CT may suggest pseudoachalasia.In an older individual or if there is rapid or profound weight loss, either CT orendoscopic ultrasound is recommended.

Radionucleotide oesophageal emptying studies are the investigation ofchoice for monitoring response to therapy.[16] They can demonstratedelayed transit time but are not first-line for the initial diagnosis of achalasia.The use of multi-channel intraluminal impedance values is an emerginginvestigation used in conjunction with oesophageal manometry to identifychronic fluid retention in the oesophagus.[17]Click to view diagnostic guideline references.Diagnostic guidelinesEuropehide allGuidelines for oesophageal manometry and pH monitoring (external link)Published by: British Society of Gastroenterology

Last published: 2006

Summary

Oesophageal manometry is indicated in the diagnosis of achalasia.

North Americahide all
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ACR appropriateness criteria: dysphagia (external link)Published by: American College of Radiology


Summary

Imaging study recommendations for variants of dysphagia

Diagnosis and management of achalasia (external link)Published by: American College of Gastroenterology Practice Parameters Committee


Summary

Comprehensive clinical guidelines encompassing definition, diagnosis, and management of

achalasia in adults.

1

Case historyA 52-year-old man presents with a 6-month history of heartburn and atypicalchest pain, both unrelated to food. He also described 'gurgling' sounds in his

chest. A month before presentation he developed intermittent dysphagia toboth solids and liquids, regurgitation, and weight loss of 3 kg.

Other presentationsPatient may need to adopt certain positions or manoeuvres to easeswallowing. There may be a history of previously treated achalasia. Atypicalsymptoms include nocturnal cough, recurrent chest infecctions, and thesensation of a lump in the throat.

Treatment Options

Patient group

Treatment

line Treatmentshow all

patients awaiting definitive

treatment

1st pharmacological therapy

Presumptive

Patient group

Treatment


good surgical candidate1st pneumatic dilatation

1st laparoscopic cardiomyotomy
http://www.acr.org/Quality-Safety/Appropriateness-Criteria/Diagnostic/Gastrointestinal-Imaginghttp://gi.org/clinical-guidelines/clinical-guidelines-sortable-list/http://www.acr.org/Quality-Safety/Appropriateness-Criteria/Diagnostic/Gastrointestinal-Imaginghttp://gi.org/clinical-guidelines/clinical-guidelines-sortable-list/

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Patient group

Treatment



treatment


Presumptive

Patient group

Treatment


poor surgical candidate1st pharmacological therapy

2nd injection of botulinum toxin A

3rd gastrostomy

Acute

Treatment Options

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Patient group

Treatment

line Treatmenthide all


treatment


This can be used as a bridge treatment while

awaiting definitive intervention. The agents used are

either calcium-channel blockers (i.e., nifedipine,

verapamil) or nitrates, taken prior to meals.

Patients report a variable improvement in symptoms

of dysphagia and chest pain.

Sublingual isosorbide dinitrate is more potent and

has a faster onset of action compared with

nifedipine. It has also been shown to improve

oesophageal emptying. Although nitrates are probably more effective they

are less well tolerated and are often replaced with

nifedipine.[46]

Maximum effect occurs in 5 to 30 minutes with

isosorbide dinitrate and 30 to 120 minutes with

calcium-channel blockers.

With long-term use, patients may become tolerant

to the effects. Adverse effects of either treatment,

such as hypotension and headaches, may limit theiruse.

Primary Options

isosorbide dinitrate: 5-20 mg orally (immediate-

release) three times daily; 2.5 to 5 mg sublingually

three times daily

OR

nifedipine : 10-30 mg orally (immediate-release)

three times daily

OR

verapamil : 80-160 mg orally (immediate-release)

three times daily

Presumptive
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Patient group

Treatment



Pneumatic dilatation is usually performed under

conscious sedation on an outpatient basis. It involves

the mechanical dilatation of the lower oesophageal

sphincter using a balloon with a sufficiently forceful

mechanical stretch to rupture the muscle fibres. The

balloon is inserted endoscopically or by a combined

endoscopic-radiological approach.

There is a perforation rate of up to 5%. All patients

considered for pneumatic dilatation should be

suitable surgical candidates, so that perforation can

be surgically repaired if required. Patients in whom

the oesophagus is particularly dilated or tortuous, and

those with oesophageal diverticula or previous

surgery, may be at particular risk of perforation.

Pneumatic dilatation is also a second-line option if

cardiomyotomy is unsuccessful.

1st laparoscopic cardiomyotomy

Cardiomyotomy may be a first-line treatment

depending on local expertise, especially in youngerpatients, or second-line after failed pneumatic

dilatation.

Open cardiomyotomy (through a thoracic or

abdominal procedure) has been superseded by the

minimally invasive laparoscopic procedure, which has

comparable success rates, lower post-operative

morbidity, 90% overall improvement post procedure,

and >80% of patients remaining dysphagia-free at 5

years.[55] Recurrence of dysphagia aftercardiomyotomy should be carefully evaluated and is

often secondary to gastro-oesophageal reflux rather

than achalasia. Prophylactic fundoplication at the

time of cardiomyotomy has been advocated[56] and

is variably practised.
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Patient group

Treatment



















poor surgical candidate1st pharmacological therapy

Patients who are not suitable candidates or are

unwilling to undergo surgery can be maintained on

pharmacological therapy, but tolerance can develop

with long-term use. Patients report a variable

improvement in symptoms of dysphagia and chest

pain.

Typically either calcium-channel blockers (i.e.,

nifedipine, verapamil) or nitrates are used. Sublingual

isosorbide dinitrate is more potent and has a faster

onset of action compared with nifedipine. It has been

shown to improve oesophageal emptying. However,

although nitrates are probably more effective, they

are less well tolerated and are often replaced with

nifedipine.[46]

Adverse effects of either treatment, such as

hypotension and headaches, may limit their use.

Maximum effect occurs in 5 to 30 minutes with
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Patient group

Treatment



















isosorbide dinitrate and 30 to 120 minutes with

calcium-channel blockers.

Primary Options

isosorbide dinitrate: 5-20 mg orally (immediate-

release) three times daily; 2.5 to 5 mg sublingually

three times daily

OR

nifedipine : 10-30 mg orally (immediate-release) three

times daily

OR

verapamil : 80-160 mg orally (immediate-release)

three times daily

2nd injection of botulinum toxin A

Injection of botulinum toxin into the lower

oesophageal sphincter improves dysphagia in about
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Patient group

Treatment



















85% of patients.[48] [57]

Older patients and those with vigorous achalasia are

more likely to respond; however, dysphagia invariably

recurs.[48]Although repeat injections can be given,

efficacy wanes over time due to development of

antibodies against the botulinum toxin.

Botulinum toxin injections cause severe inflammation

and scarring of the gastro-oesophageal junction,

which may increase the technical difficulties and risks

of cardiomyotomy.[22] [32] By contrast, prior

botulinum toxin injection does not increase the

complication rate of subsequent pneumatic dilatation.

[23] [58]

Two different formulations of similar efficacy are

commercially available, and are injected in divided

doses into the 4 quadrants of the lower oesophageal

sphincter.[59]

Primary Options
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Patient group

Treatment



















botulinum toxin type A : Botox:100 units injected in

divided doses into the four quadrants of the lower

oesophageal sphincter; Dysport: 250 units injected

in divided doses into the four quadrants of the lower

oesophageal sphincter

3rd gastrostomy

In a frail, older patient who is a poor operative risk, a

gastrostomy may be considered if previous therapy

with botulinum toxin and pharmacological agents has

failed or if severe oesophageal dilatation is present.

Acute

Treatment approachThere is no known cure for achalasia and treatment is symptomatic to reducedysphagia. The aim is to decrease lower oesophageal sphincter pressure andimprove oesophageal emptying. There are no interventions that can restore
http://bestpractice.bmj.com/best-practice/druglink.html?component-id=283781-7&optionId=expsec-5&dd=MARTINDALEhttp://bestpractice.bmj.com/best-practice/druglink.html?component-id=283781-7&optionId=expsec-5&dd=MARTINDALE

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oesophageal peristalsis. Although swallowing usually improves significantlywith treatment, it never returns completely to normal.

Treatment includes pharmacological, endoscopic, and surgical modalities.Each modality has specific advantages and disadvantages, and choice

depends on local expertise and patient preference. As the clinicalcircumstances change, different treatment modalities may becomeappropriate. Initial treatment will depend on whether the patient is a surgicalcandidate. All patients considered for pneumatic dilatation should be fitenough to undergo surgery so complications can be managed surgically ifrequired.[22] Surgery can be considered after failed dilatation, andpneumatic dilatation can be effective following cardiomyotomy failures.[23] [24]

Good surgical candidates: pneumatic dilatationMany gastroenterologists advocate pneumatic dilatation as the first-linetherapy. It is performed on an outpatient basis, under sedation. Air-inflatedballoons are used to apply mechanical stretch to the lower oesophagealsphincter to tear its muscle fibres.

The most commonly used dilators are the Rigiflex (Microvasive) and theWitzel. Rigiflex dilators are balloon dilators that enable graded dilatationunder fluoroscopic guidance. The 'over-the-scope' Witzel balloon dilator doesnot require fluoroscopic guidance.

A graded approach, starting with the smallest Rigiflex dilator (30 mm indiameter), with wider balloons used at subsequent sessions in non-responders, is as effective as using larger balloons and is thought to result inlower perforation rates. The risk of perforation is greatest during the firstdilatation.[25] [26] In one retrospective study, perforation was found to bemore common with the Witzel balloon than Rigiflex dilators,[25] but a recentreview found an overall perforation rate for pneumatic dilatation of 2%,whether Rigiflex or other types of balloons were used.[27] Patients with a

dilated or tortuous oesophagus, oesophageal diverticula, or previous surgeryat the gastro-oesophageal junction may be at higher risk of perforation. Mostpatients with perforation after pneumatic dilatations can be treatedconservatively.[28] The proportion of patients experiencing a good initialresponse averaged 66% with the Witzel balloon and 82% with the Rigiflexballoon.[29] Older patients have better response rates.
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The remission rate after a single pneumatic dilatation decreases with time(40% at 5 years and only 36% after 10 to 15 years).[30] In a retrospectivestudy, repeated dilatations (median of 4) achieved sustained clinicalremission in 60% of patients followed up for 5 to 9 years, 50% of patientsfollowed up for 10 to 14 years, and 40% of patients followed up for more than

15 years.[31]Gastro-oesophageal reflux is a potential adverse effect of pneumaticdilatation, occurring in 4% to 16% of patients who have undergone theprocedure. This is usually mild and responds well to acid suppression.

Good surgical candidates: laparoscopiccardiomyotomy

Surgery to divide the muscle fibres across the lower oesophageal sphincter

relieves dysphagia in 90% of patients.[32] Surgical cardiomyotomy used tobe a second-line treatment, reserved for patients in whom pneumaticdilatation was unsuccessful or whose symptoms recurred followingsatisfactory initial response. The advent of minimally invasive laparoscopiccardiomyotomy, which has lower morbidity compared with the openprocedure, has made surgery a more attractive option. More than 80% ofpatients remain dysphagia-free 5 years after laparoscopiccardiomyotomy.[33] [34]

A 1989 study reported better results over a 5-year follow-up period for open

cardiomyotomy compared to pneumatic dilatation.[35] Two recent reviewsalso suggest that laparoscopic cardiomyotomy is associated with betterremission rates than pneumatic dilatation.[36] [37]However, 3 head-to-headstudies, both retrospective and prospective,have shown comparable resultsfor the two techniques.[38] [39] [40]Another single-centre study reportedhigher remission rates after laparoscopic cardiomyotomy than afterpneumatic dilatation after 1 and 3 years.[41]A recent multi-center,randomised controlled study showed that up to 3 courses of 2 to 3 pneumaticdilatations over a 2-year period was as effective and safe as laparoscopicmyotomy.[42] [A Evidence] In practice most patients treated with pneumaticdilatation require multiple dilatations over a lifetime to maintain symptomrelief.[27]Surgical failure often relates to postoperative gastro-oesophagealreflux.[43]Anti-reflux fundoplication at the time of cardiomyotomy isadvocated to address this problem. Other post-operative complicationsreported include mucosal tear, perforation, or post-operative leakage,occurring in

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seem to affect the outcome of surgery, but the procedure may be moretechnically challenging.[23] [32]

Pharmacological therapyDrug treatment is used as an initial therapy pending definitive treatment or as

a first-line treatment for patients who are poor surgicalcandidates.[44] Calcium-channel blockers (e.g., nifedipine or verapamil) ornitrates have been shown to lower resting or mean oesophageal sphincterpressure.[45] [46] [47] [B Evidence] Variable improvement in dysphagia andchest pain scores are reported.[45] Sublingual isosorbide dinitrate is morepotent and has a faster onset of action compared with nifedipine. Isosorbidedinitrate has been shown to improve oesophageal emptying. Althoughnitrates are probably more effective, they are less well tolerated and are oftenreplaced with nifedipine.[46] With long-term use, patients may become

tolerant to the therapeutic effects of either drug.

Poor surgical candidates: botulinum toxinBotulinum toxin inhibits the release of acetylcholine from nerve terminals,alleviating the effect of the selective loss of inhibitory neurotransmitters thatoccurs in achalasia. Endoscopic injection of botulinum toxin into the loweroesophageal sphincter decreases the pressure and improves dysphagia,regurgitation, and chest pain.[48]The injections are as effective as pneumatic dilatation at relieving symptoms,

but the effect tends to be transient.[49] [50] Older patients respond betterthan younger people, and botulinum toxin is useful in patients who are toofrail for more invasive procedures and who are unable to toleratepharmacological therapy. Initial response is in excess of 80%, but this dropsto 68% to 75% after 2 years, even with repeat treatment sessions.[49] Theeffectiveness of repeat injections may be diminished by the formation ofantibodies to botulinum toxin.[51]Unexpectedly, and seemingly at variance with standard guidelines, a recentUS database study reported that botulinum toxin injection was the most

commonly used first-line endoscopic treatment for achalasia, in 41% ofcases, compared to 25% for pneumatic dilatation.[52]

Progressive disease despite treatmentIn frail and older patients, a gastrostomy is a possibility to allowfeeding.[24] [53]Oesophagectomy is an uncommonly used option for end-stage disease.[54]
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Emerging treatments

Combined botulinum toxin type A injection with pneumatic dilatation

One study on the concurrent use of botulinum toxin injection and pneumaticdilatation showed a positive trend toward greater symptomatic responsecompared with treatment by pneumatic dilatation alone. However, thedifference did not reach statistical significance.[58]

Treatment guidelinesEuropehide allGuidelines on the use of oesophageal dilatation in clinical practice (external link)Published by: British Society of Gastroenterology


Summary

Guidelines on the use of oesophageal dilatation in the treatment of oesophageal disorders including

achalasia.

North Americahide allDiagnosis and management of achalasia (external link)Published by: American College of Gastroenterology Practice Parameters Committee


Summary

Comprehensive clinical guidelines encompassing definition, diagnosis, and management of

achalasia in adults.

Medical position statement on treatment of patients with dysphagia caused by benigndisorders of the distal oesophagus (external link)

Published by: American Gastroenterological AssociationLast published: 1999

Summary

Recommendations on the treatment of patients with benign oesophageal disorders including

achalasia.

SAGES guidelines for the surgical treatment of esophageal achalasia (external link)Published by: Society of American Gastrointestinal and Endoscopic Surgeons


Summary

Systematic, evidence-based statements to assist surgeon (and patient) decisions about the

appropriate use of minimally invasive techniques for the treatment of achalasia in specific clinical

circumstances.

MonitoringAfter initial treatment with pneumatic dilatation, cardiomyotomy, or botulinumtoxin injection, periodic clinical review is recommended to assess the degreeof symptom relief. Follow-up timed barium studies, oesophageal scintigraphy,or manometry enable objective measurements of treatment response. The
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investigation chosen will depend on local resources and expertise. In patientswith long-standing achalasia, clinical vigilance is needed for promptrecognition of oesophageal malignancy, a rare complication with a poorprognosis.

Patient InstructionsIt is important that patients understand their condition and attend follow-upappointments, as treatment is not curative but symptomatic. Patients areencouraged to chew their food well and ensure adequate fluid intake wheneating.

ComplicationsComplicationshow all

aspiration pneumonia

gastro-oesophageal reflux disease (GORD)

oesophageal carcinoma

Complications

Complicationhide all

aspiration pneumonia

see our comprehensive coverage of Aspiration pneumonia

This results from aspiration of retained material in the oesophagus, with nocturnal aspiration being a parproblem.

In the US, pulmonary complications account for approximately 10% of hospitalised cases of achalasia.[

gastro-oesophageal reflux disease (GORD)

see our comprehensive coverage of Gastro-oesophageal reflux disease

GORD is the most common cause for post-surgical treatment failure.

In a 10-year follow-up study, 14 of 67 patients (21%) developed severe reflux disease oesophagitis desp

partial anti-reflux procedure.[43]

The severity of reflux disease increased with increasing duration of follow-up. Nine patients (13%) devel

Barrett's oesophagus.[43] Rarely, a peptic stricture can result.[54]

oesophageal carcinoma

see our comprehensive coverage of Oesophageal cancer

There may be an increased risk of squamous cell oesophageal cancer (up to 140-fold) in patients with

achalasia.[61]
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Three cases of squamous oesophageal cancer were reported in a cohort of 67 patients post cardiomyot

over a 10-year follow-up period.[43]

It is thought to result from chronic injury to the oesophageal mucosa due to retained ingested food and o

noxious compounds.[62]

The diagnosis is often made late, because patients already have a degree of dysphagia and any obstruc

lesion has to be much larger to cause symptoms in a dilated oesophagus.[61] [62]

The outcome is therefore poor; however, endoscopic surveillance is not usual practice.[62]

Last u

PrognosisThere is no known cure for achalasia. Treatment is purely symptomatic,aimed at improving oesophageal emptying and reducing the symptom ofdysphagia. Oesophageal peristalsis remains absent, and swallowing neverreturns totally to normal. Studies comparing outcomes between pneumatic

dilatation and cardiomyotomy show similar outcomes but are limited by thelength of follow-up (

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Classic appearance of dilated oesophagus with tapered beak-like narrowing on barium study

From the personal collection of Dr Jin-Yong Kang

Date post:	08-Aug-2018
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