achalasia cardia a pathology for differential in patient with dysphagia

1. ACHALASIA CARDIA
Dr Saurabh Dhanda
Registrar Minimal Access Surgery
Department
Apollo Greams Road ,Chennai

2. Objectives
● Review the etiopathogenesis of achalasia
● Discuss the clinical features of achalasia
● Discuss the workup of achalasia
● Discuss the current options for the management of achalasia
● Describe a new development in the treatment of achalasia

3. History
● In 1913 Ernest Heller performed the first surgical
intervention for achalasia and the procedure still
bears his name
● It was not actually called achalasia until a 1927
article by Arthur Hurst
◦ The treatment of achalasia of the cardia: so-called
‘cardiospasm’
◦ Achalasia is Greek for lack of relaxation
● Ellis et al described the first transthoracic approach
in 1958

4. History continues
• The first laparoscopic Heller myotomy by Sir Alfred Cuschieri
in 1991

5. What is achalasia?
● Aperistalsis of the esophageal body
● Incomplete relaxation of LES
● Hypertonic lower esophageal sphincter (NOT diagnostic)
● Due to a degeneration of the inhibitory neurons of the esophageal wall
● Second most common benign disorder of the esophagus requiring surgical
intervention

7. Etiopathogenesis
• PRIMARY –
• Autoimmune
• Class -2 MHC antigen – HLA-DQw 1,
HLA –DQB1 , HLA-DRB1
• SECONDARY
• Infectious
• HSV -1
• HSV-2
• Polio
• Human papilloma virus
• Measles
• Chagas disease
• .Malignancy of GE junction ,
lymphoma , chagas disease
• Postvagotomy states
• Amyloidosis
• Sarcoidosis

8. Clinical features
• There is classic triad of symptoms consist of dysphagia , regurgitation
, and weight loss
• 1. Regurgitation because of stasis ( 70%-80% )
• 2. Dysphagia (100%)
• 3. GERD
• 4. Heart burn (50%)
• 5.Aspiration , cause nocturnal cough , pneumonia , pulmonary
abscess. (8-46% )
• 6. Chest pain /Retrosternal pain (50%)
• 7. Weight loss .(30%-90% )
• In clinical history taking it is important to find out the past hx of
pneumatic dilatation and history of BT Injection .

9. Eckardt score

10. INVESTIGATION
• 1. Chest X Ray - Widened mediastinum , airfluid level in esophagus ,
Absent gastric air bubble , and aspiration pneumonia .
• 2. Barium meal – Distal esophageal narrowing ( bird beak ) , airfluid level ,
slow emptying of barium and esophageal dilatation .
• 3. Esophageal manaometry Three classic manometry findings in achalasia
• 1. Aperistalsis of esophageal body
• 2. Elevated LES pressure
• 3. Impaired relaxation of LES during swallowing
• 4. 24hr –ph monitering
• To find out the evidence of gastroesophageal reflux disease .
• To distinguish between the real and false reflux
• 5. Endoscopy
• 1. To rule out several disease that mimic achalasia eg, GE junction CA
• 2. Evaluation of esophageal mucosa before therapeutic manipulation .
• 6.EUS
• 7.CECT ABDOMEN

11. High resolution manometry
• It is the latest investigation in
esophageal motility disorders
• Type -1 Achalasia with
minimal esophageal
pressurization ( wave
amplitude <30 mmHg)
• Type –II Achalasia with
rapidly propagating
compartamentalized
pressurization (
panesophageal pressurization
> 30 mmHg )
• Type-III Achalasia with
pressurization attributable to
spasm .

13. Endoscopy

14. Diagnosis
• Alogrithm suggested by ACG

15. MANAGEMENT
MEDICAL MANAGEMENT
SURGICAL MANAGEMENT
• OPEN
• LAPAROSCOPIC
• THORACOSCOPIC
ENDOSCOPIC MANAGEMENT
• Pneumatic dilatation
• POEMS

16. Medical management
● Medical therapy with calcium channel blockers or
nitrates
● They are taken 10-30 minutes before meals
● While they have been shown to have moderate success, they
require the patient to take them perpetually
● They are not recommended as first-line therapy

17. Medical management
1.Oral nitrates (GTN, Isosorbide dinitrate)
2.Calcium channel blockers (Nifedipine, verapamil)
3. Anticholinergics
4.Opioids (loperamide)
5.Phosphodiesterase inhibitors
6.Β2 agonists
7.Nitric oxide agonists

18. Botox injection

19. Surgical management
Heller’s Myotomy
S
● First described by Ernest Heller in 1913 where
he used an abdominal approach to perform an
anterior and posterior esophagomyotomy
● Surgical therapy now involves usually
performing only an anterior myotomy, via
either abdominal or thoracic approach
● In addition to laparoscopic myotomy,
thoracoscopic myotomy has also been described
● Thoracic approach does have certain drawbacks
V
M
C

20. Heller’s
Myotomy
S ● At this point in time, laparoscopic myotomy
is considered the standard operation
● When compared to open techniques,
similar rates of complications with much
shorter hospital stay and recovery times
V
M
C

21. Heller’s
Myotomy
S
● In performing Heller’s myotomy, there are
a few important questions to consider…
● To do a fundoplication?
● If so, what kind of fundoplication?
● What to do with the sigmoid esophagus?
● Length of myotomy?
● Any benefit to the robot?
V
M
C

22. To fundoplicate, or not to
fundoplicate that is the
question…
S
● 30% of pts complained of significant heartburn
● 24 hr pH probe or endoscopy demonstrated that
60% of pts had significant reflux
● “Objective analysis reveals an unacceptable
rate of gastroesophageal reflux in laparoscopic
Heller’s myotomy without an antireflux
procedure. We therefore recommend
performing a concurrent antireflux procedure.”
V
M
C

23. Dor vs Toupet
fundoplication
S
● Dor fundoplication is an anterior
180 degree wrap
● Toupet fundoplication is a posterior
270 degree wrap
V
M
C

24. Dor
fundoplication

25. Toupet
fundoplication

26. Dor vs Toupet
fundoplication
S
● They showed no significant difference
in outcome
◦ Looked at dysphagia
◦ Looked at GER and use of PPIs
● To date, there has been no randomized
controlled trial comparing the two
procedures
V
M
C

27. LAPAROSCOPIC MANAGEMENT
• 1. Patient positioing and preparation
• 2. Port Positioning
• 3.Creation of pneumoperitoneum
• 4. Surgical dissection
• 1. Retraction of liver
• 2. Hiatal dissection
• 3. Mobilization of esophagus and Exposure of Anterior Wall of
Esophagus
• 4. Myotomy
• 5. Antireflux Fundoplication

28. POSITIONING OF PATIENTAND SURGEON
1. Supine position with split leg
with slightly flexed knees
2. 30 degree reverse trendelenberg
position .

29. PORTPOSITION

30. SURGERY
Laparoscopic Esophageal mobilizationand myotomy

31. FUNDOPLICATION
The procedure is completed by performing a
partial anterior (Dor) or a posterior (Toupet)
fundoplication.
This was demonstrated in a prospective study
that compared total (Nissen) versus partial
anterior (Dor) fundoplication after Heller
myotomy; it showed equal GERD control but
higher dysphagia rate (15% versus 2.8%) for
the total fundoplication group after 5 years of
followup .
Partial fundoplication is the procedure of
choice after Heller myotomy .
The differences between anterior 180°
(Dor) and posterior 270° (Toupet)
fundoplication that it might prevent
recurrent dysphagia by keeping the edges
of myotomy apart and even more
effecting in preventing reflux .

32. POSTOPERATIVE MANAGEMENT
• First postoperative day
• Gastrograffin – to rule out mucosal perforation
• Second postoperative day
• Liquid diet on 2 nd to 48 hr
• Soft diet for 8 -10 days than normal diet allowed
• Endoscopy after 6 month to rule out GERD
• Endoscopy every 2 year to rule out any neoplasia .

33. POSTOPERATIVE COMPLICATIONS
1. Mucosal Injury ( Perforation)
Incidence 2%-4%
2. Pneumothorax
Commonly in left side .
Incidence 1-2%
3. Postoperative Gastroesophageal reflux
Incidence 10-15%
4. Postoperative dysphagia
Early postoperative dysphagia is the result of incomplete
myotomy or periesophageal scarring and manifest within 3
year
Late dyspahgia is result from reflux induced peptic stricture

34. Cardiomyotomy failure
1. Late occurrence of carcinoma,
2. Presence of a decompensated “sigmoid-shaped”
megaesophagus before surgery,( grade IV achalasia),
3. Gastroesophageal reflux,
4. Tight fundoplication,
5. Inadequate myotomy (caused either by incomplete
section of the muscle fibers or by healing with fibrosis
of the myotomy edges).

35. TREATMENT
• Early postoperative dysphagia
1. Dilation,
2.Botulinum toxin injection,
3.Redo- fundoplication.
• Recurrence of symptoms after myotomy is mainly
related to incomplete myotomy or sclerosis of the
distal site of the myotomy; it can be treated by
dilations after surgery in about 80% of cases.
ANNALS OF SURGERY Vol. 235, No. 2, 186–192

36. Identification of the precise cause of failure requires
1. Careful analysis of the patient's symptoms
2. barium studies, esophagoscopy and esophageal function tests, including 24-hour pH
monitoring.
1. Dysphagia
The treatment :of patients with an inadequate myotomy is generally a second
myotomy.
The myotomy is sited away from the previous myotomy to decrease the risk of
mucosal perforation, and is carried under direct vision onto the stomach for one or
two cm.
2. Gastroesophageal reflux following esophagomyotomy varies according to the
surgical approach utilized (transthoracic vs. abdominal)
1. Mild uncomplicated reflux, Medical treatment require .A satisfactory response to
medical therapy in the setting of an aperistaltic esophagus is highly unlikely.
2. Redo myotomy is not essential under those circumstances, unless there is any doubt
regarding the underlying etiology of the recurrence.

37. 1.Undilatable peptic stricture -Resection and reconstruction using
an isoperistaltic segment of colon or jejunum
2. Obstructive antireflux repair because of Total fundoplication
of the Nissen type performed after an esophagomyotomy and
aperistaltic esophageal body- Partial fundoplication is therefore
not only desirable
3. Dilated tortuous megaesophagus - segmental or subtotal
esophageal resections

38. Length of
myotomy
S
● Often quoted as needing 5 cm of
esophageal myotomy with 1 cm of
myotomy onto the cardia
● Long-term outcomes confirm the superior
efficacy of extended Heller’s myotomy
with Toupet fundoplication for achalasia
◦ 2007 article from Surgical Endoscopy
◦ By Wright et al from Unversity of Washington
◦ Retrospective review
V
M
C

39. Laparoscopic versus Thoracoscopic Myotomy
• Laparoscopic Heller myotomy with Dor fundoplication was found to
be superior to thoracoscopic Heller myotomy
• . J Gastrointest Surg. 1998 Nov-Dec;2(6):561-6
• These two methods compared with respect to
• (1) relief of dysphagia,
• (2) incidence of postoperative gastroesophageal reflux,
• (3) hospital course.
• Sixty patients with esophageal achalasia were operated on
between 1991 and 1996.
• Both operations relieved dysphagia, but the laparoscopic approach
avoided postoperative reflux.
• Patients left the hospital earlier following a laparoscopic myotomy.
• Thoracoscopic approach is superior as it helps to confirm the
diagnosis , allow careful palpation of esophagus , and enable the
surgeon to proximally as far as necessory .

40. What about the
robot?
S
● Laparoscopic Heller myotomy for
achalasia facilitated by robotic assistance
◦ Galvani et al from University of Illinois,
Chicago
◦ 2006 article from Surgical Endoscopy
● Showed it to be safe an effective
V
M
C

41. Pneumatic
dilatation
S
● Considered the most effective
nonsurgical treatment of achalasia
● Involves passing the pneumatic device to
the LES, using both endoscopy and
fluoroscopy to properly place the balloon
● The balloon is inflated to a pressure
between 7 to 15 psi
● Patients are usually observed for six
hours and then discharged home
V
M
C

42. Pneumatic
dilatation
S
V
M
C

43. Pneumatic
dilatation
S
● The two best predictors of success:
◦ Post-dilation pressure (or some report
the difference between pre- and post-
dilation pressures)
◦ Older age
● The biggest concern with pneumatic dilation is
esophageal perforation, which has been reported
to be as low as 1.6% while other studies have
reported an incidence of around 10% (one study
reported 21% perforation rate)
V
M
C

44. A new approach to
achalasia
S
● Submucosal endoscopic esophageal
myotomy: a novel experimental approach
for the treatment of achalasia
◦ Published in Endoscopy, 2007
● It has also been referred to as POEM:
Peroral endoscopic myotomy
● It is considered a form of NOTES
V
M
C

45. A new approach to
achalasia
S
● The leading expert in this technique is
Dr. Haruhiro Inoue, from Showa
University Northern Yokohama Hospital
in Japan.
● He has performed over 100 proceduresV
M
C

46. A new approach to
achalasia
S
● Start by entering the submucosal space
approximately 15 cm above the GE
junction
● Uses an endoscope with a special
transparent cap
● Using a solution of saline with indigo dye, a
tunneled dissection is carried distally to
about 2 cm past the GE junction
● Then, myotomy is begun starting 10
cm proximal to GE junction
V
M
C

47. A new approach to
achalasia
S
● Myotomy is carried distally down to 2
cm past the GE junction
● Myotomy only takes the inner circular
fibers while leaving the outer longitudinal
fibers intact
● At the end of the procedure, the scope is
removed from the submucosal tunnel and
the entry site is closed with endoscopic
clips
V
M
C

48. A new approach to
achalasia
POEM
S
V
M
C

49. 2 Diseases of the Esophagus
39
Department of Gastroenterology, University of Bordeaux, Bordeaux, France,
40
ECD Solutions, Atlanta, Georgia, USA,
41
ALMA
(Association of patients with achalasia, ONLUS), Naples, Italy, and
42
Department of Thoracic Surgery Virginia Mason Medical Center,
Seattle, Washington, USA
SUMMARY. Achalasia is a relatively rare primary motor esophageal disorder, characterized by absence of relax- ations of the lower
esophageal sphincter and of peristalsis along the esophageal body. As a result, patients typically present with dysphagia, regurgitation and
occasionally chest pain, pulmonary complication and malnutrition. New diagnostic methodologies and therapeutic techniques have been
recently added to the armamentarium for treating achalasia. With the aim to offer clinicians and patients an up-to-date framework for
making informed decisions on the management of this disease, the International Society for Diseases of the Esophagus Guidelines proposed
and endorsed the Esophageal Achalasia Guidelines (I-GOAL). The guidelines were prepared according the Appraisal of Guidelines for
Research and Evaluation (AGREE-REX) tool, accredited for guideline production by NICE UK. A systematic literature search was
performed and the quality of evidence and the strength of recommendations were graded according to the Grading of Recommendations
Assessment, Development and Evaluation (GRADE). Given the relative rarity of this disease and the paucity of high-level evidence in the
literature, this process was integrated with a three-step process of anonymous voting on each statement (DELPHI). Only statements with an
approval rate >80% were accepted in the guidelines. Fifty-one experts from 11 countries and 3 representatives from patient support
associations participated to the preparations of the guidelines. These guidelines deal specifically with the fol- lowing achalasia issues:
Diagnostic workup, Definition of the disease, Severity of presentation, Medical treatment, Botulinum Toxin injection, Pneumatic dilatation,
POEM, Other endoscopic treatments, Laparoscopic myotomy, Definition of recurrence, Follow up and risk of cancer, Management of end
stage achalasia, Treatment options for failure, Achalasia in children, Achalasia secondary to Chagas’ disease.
KEY WORDS: esophageal achalasia, Chagasdisease.
SUMMARY TABLE OF STATEMENTS AND RECOMMENDATIONS
Consensus
Topic and number Statement agreement score Recommendation
Diagnosis of
achalasia
1 High-resolution manometry is the testof 94.2% We recommend the use of HRM for the
choice for the diagnosis ofachalasia diagnosis of esophageal achalasia.
(compared to conventional manometry) Conditional recommendation; GRADE low.
2 The Chicago Classification is auseful tool 90.4% We recommend classification of achalasia
to define the clinically relevant according to the Chicago Classification.
phenotypes of achalasia. Good practice recommendation
3 The timed barium esophagramoffers an 90%. We recommend the adoption of TBS inthe
objective evaluation of the diseases andof diagnostic pathway of achalasia and to
the outcome after treatment (compared to evaluate the outcome oftreatment.
traditional barium esophagram). Conditional recommendation; GRADE low.
4 Endoscopy should be performedin 98.1% We recommend performing UGI endoscopy
patients with suspected achalasia to in adult with the suspected diagnosisof
exclude malignancy of the achalasia to exclude neoplastic
esophagogastric junction. pseudoachalasia. Good practice
recommendation.
5 The Eckardt score is a simple toolto 86.5% We recommend the use of the Eckardtscore
measure symptom severity in achalasia as part of the initial andfollow-up assessment
patients, but it should be integratedwith in patients with achalasia. Goodpractice
objective measures such esophagogram recommendation.
and manometry.
Treatment of
achalasia
Medical treatment
with nitrates, calcium
blockers, or
phosphodiesterase
6 There is no convincing evidence that medical
treatment with nitrates iseffective for symptomatic
relief in adults with achalasia.
86.5% We recommend against the use of nitrates, calcium
blockers, or phosphodiesterase treatment for
achalasia. GRADE: low.
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50. Achalasia guidelines 3
Topic and number Statement
Consensus
agreementscore Recommendation
88.2%7 There is no convincing evidence that medical
treatment with calcium blockersis effective for (short
term) symptomatic relief in adults with achalasia.
8 In adults with achalasia, there is no evidence
that medical treatment withphospho-
diesterase inhibitors is effective for
symptomatic relief.
84.3%
Botulinumtoxin
injection (BTI) 9 BTI haslimited application in young
patients (aged less than 50years).
92.3% We recommend against the use of BTI inpatients under 50
years of age, for control of symptoms. GRADE: very low:
We recommend against BTI as an effective therapy (control
of symptoms) for achalasia in patients fit for surgery
(LHM) or pneumatic dilatation GRADE: moderate.94.3%10 BTI should be reserved for patients who are unfit
for surgery or as a bridge to more effective therapies,
such as surgery or endoscopic dilation
11 Repeat treatments with Botox are safe,but
less effective than initial treatment
82.4%
92.1%
Recommendation: Botox injection can be safely repeated,
but the clinician and the patients should be aware that their
efficacy is lower than in initial treatment. Conditional
recommendation.
GRADE: low.
We recommend against BTI in the esophageal body, even
in the presence of type III achalasia. GRADE: very low.
12 There is no evidence for supporting the injection of
Botox in the lower esophageal body (in addition to
injection in the LES) in type III achalasia patients.
13 There is no evidence that patients
undergoing repeat BTI of the LES should be
treated with increasing dosage ofBT.
96.1% We recommend against the use of increasingBT dosage at
retreatment. GRADE: very low.
Pneumaticdilatation
14 In patients with achalasia, graded PD is an effective
treatment in terms of improvement of symptoms
andswallowing function.
90.4%
15 In patients with achalasia who have received PD,
the best postprocedural test to assess if a
perforation occurred is a Gastrografin (iodine
contrast) swallow.
80.8%
82.4%
We recommend graded pneumatic dilatations as an
effective treatment (control of symptoms including
dysphagia) for esophageal achalasia. Strong
recommendation GRADE: moderate. Patients wishing
longer term remission may opt for surgical treatment.
We recommend that after PD patients are
observed for 4 hours. Water-soluble iodine contrast
(Gastrografin) esophagogram (or CT scan with oral
contrast) should be performed if any symptoms, even if
moderate, suggest that perforation is present after
dilatation. We recommend against the routine use of
contrast esophagram or computed tomography shortly
after PD. Conditional recommendation.
GRADE: low.
We make no recommendation about pneumatic dilatation
as first-line therapy in megaesophagus GRADE: very low.
16 There is only limited evidence that pneumatic
dilatation may be used as first-line therapy in
megaesophagus (diameter >6 cm & sigmoid
shaped).
17 There is no evidence that patients
undergoing graded dilation should be treated with
proton pump inhibitors as maintenance therapy
after the procedure, unless symptomatic or positive
at24-hour pH-monitoring.
94.3% We recommend against the prophylactic useof PPI after
PD, unless GERD symptoms are present or objective
evidence of reflux is demonstrated. Conditional
recommendation GRADE: very low.
Peroral endoscopic
myotomy (POEM) 18 Treatment of achalasia patients with POEM,
results in similar outcomes on swallowing
functions compared with alternative treatment
(Heller myotomy or PD), at least at medium term
follow-up (2–4 years).
88.4% We recommend POEM as an effective therapy (control of
symptoms) for achalasia both in short- and medium-term
follow-up with results comparable to Heller myotomy for
symptom improvement. Conditional recommendation.
GRADE: very low. We recommend POEM asan effective
therapy (control of symptoms) for achalasia both in short-
and medium-term follow-up with results comparable to
pneumatic dilations for control of symptoms. Conditional
recommendation. GRADE: low.
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51. 4 Diseases of the Esophagus
ContinuedTopic and number Statement Consensus
agreementscore
Recommendation
19 Treatment of achalasia with POEM is associated
with a higher incidence of GERD compared to
alternative therapies (Heller myotomy with
fundoplication or PD).
96.2%
86.6%
We recommend that pretreatment information on the risk
of GERD should be provided to the patient and follow-up
acid suppression therapy considered after POEM. Good
practice recommendation. Patients who seek a nonsurgical
treatment (PD) or surgical treatment with a lower incidence
of postprocedure GERD (Heller myotomy) should be
counseled that these options exist.
We recommend POEM as feasible and effective
for symptom relief in patients previously treated with
previous endoscopic therapies. Conditional
recommendation; GRADE: verylow.
20 There is no evidence that previous treatment of
patients with achalasia with PD or Botox reduces
the technical feasibility of POEM andresults in
poorer outcomes.
21 POEM is anappropriate treatment for
symptom persistence/recurrence after
laparoscopic myotomy.
88.2%
22 Attaining proficiency with the POEM procedure
involves a stepwise approach to education and a
defined learning curve for both medical and
surgical endoscopists.
90.2%
We recommend the use of POEM for symptom relief, as
an option for treating recurrences after LHM.
Conditional recommendation.GRADE: low.
We recommend that appropriate training within
vivo/in vitro animal model and adequate proctorship
should be considered before starting a clinical program of
POEM. Good practice recommendation.Alternative treatments:
retrievable stents and
intrasphincteric injection
with ethanolamine oleate
or polidocanol
98%23 There is little evidence to support that modified
retrievable stent placement at the LES is an effective
treatment for patients with achalasia.
24 There is no or little evidence to support the
use of endoscopic sclerotherapy with ethanolamine
oleate or polidocanol asan effective first treatment
for patients with achalasia.
96%
We recommend against temporary (retrievable or
absorbable) stents and intrasphincteric injection with
ethanolamine oleate for achalasia.
Conditional recommendation. GRADE: low.
We recommend against temporary (retrievable or
absorbable) stents and intrasphincteric injection with
ethanolamine oleate or polidocanol for achalasia.
Conditional recommendation.
GRADE: low.
Laparoscopic Heller
myotomy 25 The best outcomes for LHM are achieved in
(Chicago) type I & type II achalasia patients.
90.4%
94.2%
We recommend laparoscopic Heller myotomy for control of
symptoms in Chicago type I and type II achalasia. Strong
recommendation. GRADE: moderate.
We recommend that Laparoscopic Heller
cardiomyotomy should be extended at least (6cm proximal
to the GEJ and at least 2 cm distal to the GEJ.
Conditional recommendation.
GRADE: low.
26 Laparoscopic Heller myotomy should include a
myotomy 6 cm into the esophagus and 2 to 3 cm
into the stomach as measured from the GEJ, for
effective symptom control in achalasia
patients.
27 Partial fundoplication should be addedto
laparoscopic myotomy in patients with achalasia
to reduce therisk of subsequent gastroesophageal
reflux.
94.2%
28 Laparoscopic Heller myotomy with a partial
fundoplication is as effective at improving
swallowing function as laparoscopic Heller
myotomy alone.
82.7%
29 LHM (or other therapies as POEM or PD) should
be considered as the first-line treatment optionin
achalasia patients with sigmoid esophagus
(compared to esophagectomy).
86.5%
We recommend that a partial (posterior or anterior
fundoplication) but not a complete 360◦ wrap should be
added to reduce the long-term risk (5 years) of developing
gastroesophageal reflux and dysphagia after myotomy.
Strong recommendation. GRADE: moderate.
We recommend a partial fundoplication should
be used when performing Heller myotomy to prevent
subsequent development of gastro- esophageal reflux
without compromising the adequate control of dysphagia.
We recommend against LHM alone due to the risk
development of gastro-esophageal reflux. Strong
recommendation. GRADE: High.
We recommend standard endoscopic or surgical
therapies in surgically na¨ıveachalasia patients with
sigmoid-shaped esophagus, leaving esophagectomy as
secondary option
in case of failure of first line therapy. Conditional
recommendation. GRADE: verylow.
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52. Achalasia guidelines 5
Continued
Topic and number Statement Consensus
agreementscore
Recommendation
Recurrence of
achalasiaafter
treatment 90.4%
88.4%
We recommend assessment of symptomatic improvement
as the best measure of successafter treatment of achalasia.
Good practice recommendation.
Recommendation: see next statement.
30 Symptom improvement is the most relevant clinical
parameter for defining the success of surgical or
endoscopictreatment for achalasia.
31 In adults with achalasia, thereis no
universal definition of failure afterany
treatment.
32 Recurrent symptoms after achalasia
treatment should routinely undergo repeat objective
testing.
100%
33 The timed barium swallowobjectively
demonstrates the failure of achalasia
treatment in patients with persistent/recurrent
symptoms.
82.7%
We recommend objective testing in patientswho suffer
recurrent symptoms after treatment of achalasia including
UGI endoscopy, barium swallow, manometry, and 24-
hour pH monitoring. Good practicerecommendation.
We recommend TBS as a reliable methodto
assess recurrence of achalasia. Conditional
recommendation. GRADE: Low.Risk ofcancer
34 Achalasia patients carry a moderately increased risk
of development ofsquamous esophageal cancer 10
years or more from the primary treatment of
achalasia.
86.5% We recommend that achalasia patients shouldbe informed
that a moderately increased risk of esophageal cancer is
present in male after atleast 10 years from the initial
treatment of the disease. Good practice recommendation.
We make no recommendation about routine endoscopy or
endoscopy intervals after any treatment.Management of
treatmentfailures 35 Patients with achalasia who do not respond to initial
treatment with graded PD, should be referred for
Heller myotomy or POEM.
94.2%
36 Laparoscopic esophageal myotomy is a safe,
feasible and effective treatment after failed Botox
injection for achalasia.
96.2%
37 PD, compared with repeat myotomy or POEM, is
the first option for treatment after failed Heller
myotomy forachalasia.
80.8%
38 There is insufficient evidence that laparoscopic
myotomy or redo POEM offer better results
than PDs after failed POEM.
82.4%
We recommend that in patients who are fit for surgery
and have symptomatic recurrencesafter several pneumatic
dilations, Heller myotomy, or POEM should be
considered. Conditional recommendation. GRADE: of
evidence
low.
We recommend LHM as an effective therapy for symptom
recurrence after primary treatment with BTI. Conditional
recommendation. GRADE: very low.
We recommend pneumatic dilation as a safeand
effective treatment of symptom recurrences after LHM.
Conditional recommendation. GRADE: Low.
We make no recommendation about laparoscopic
myotomy or redo POEM offering better symptomatic relief
than pneumatic dilationsafter failed POEM. Further
research is recommended to provide high-quality data and
guide clinical decisions.
Diagnosis and
treatmentof end
stage achalasia 94.1%39 Barium swallow esophagram, compared with
manometry, is the best diagnostic method for
defining end stage achalasia (i.e. that which
requiresesophagectomy).
40 Esophagectomy is indicated in patients
with persistent or recurrent achalasiaafter failure of
previous less invasive treatments (PD, POEM,
LHM) and radiologic progression of the disease.
80.8%
We recommend the use of barium swallow asthe most
accurate investigation to properly define end-stage
achalasia. Good practice recommendation.
We recommend esophagectomy in patientswith
end-stage achalasia who have failed otherless invasive
interventions. Conditional recommendation. GRADE:
Low.Achalasia inchildren
41 Children with suspected achalasia should follow the
same diagnostic pathway asthat of adult patients.
96%
42 Surgical or endoscopic myotomy (compared to
dilation) is the preferred treatment for pediatric
patients with idiopathic achalasia (IA),
especially for those aged 5 years or more.
80%
We recommend that children with a provisional diagnosis
of achalasia should undergo thesame work-up as in the
adult population. Good practice recommendation.
We recommend myotomy (either through a
laparoscopic or flexible endoscopy approach as the
preferred treatment in children).Conditional
recommendation. Grade: very low.
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53. 6 Diseases of the Esophagus
ContinuedTopic and number Statement Consensus
agreementscore
Recommendation
43 BTI is not an appropriate first-line therapy in very
young children with achalasia.
81.6%
44 The long-term outcome of achalasia treatment in
children should beassessed by symptoms, function,
physical growth, and general development.
94.4%
We recommend against BTI as a first-linetherapy in very
young children with achalasia (with exceptions for those
children who are medically frail and at high-risk for
surgical intervention). Conditional recommendation.
Grade: very low. We recommend that the long-term
outcome of achalasia treatment in children should be
closely monitored by symptoms, swallowing function,
physical growth, and general development. Good practice
recommendation.
Diagnosis and
management of
achalasiasecondary to
Chagas disease 45 There are minor differences between the clinical
presentation of IA and achalasia secondary to
Chagasdisease.
86.2%
46 There are no differences in the treatmentof
idiopathic achalasia and achalasia specific to
Chagas disease.
90%
We recommend that diagnostic techniques used for IA
should also be used for CDE, due to the similarities in
manometric and clinical features. Conditional
recommendation. GRADE: low. We recommend that all
treatments for IA may be used for CDE for symptom relief.
Conditional recommendation. GRADE: low.
INTRODUCTION
Achalasia is a relatively rare esophageal motor dis- order
characterized by the absence of swallow-induced relaxation of
the lower esophageal sphincter (LES) and by absence of
peristalsis along the esophageal body. Consequently, the transit
of the food into the stomach is impaired and the patient
typically experiences dysphagia. Other symptoms reported are
regurgitation of saliva or undigested food, respiratory symptoms
(nocturnal cough, recurrent aspiration, and pneumonia),
heartburn, and chest pain.1 The most common form of
achalasia is idiopathic and is mostly observed sporadically. In
idiopathic achalasia (IA), the disease occurs secondary to the
destruc- tion of the myenteric plexus that coordinates both
peristaltic contraction and LES relaxations.2-4 A similar clinical
picture can be present in patients with local or distant cancer
(pseudoachalasia)5,6 or in patients with Chagas’ disease,7 both
characterized by the destruction of the myenteric plexus either
by infiltrating tumors or by circulating autoantibodies or by
Trypanosoma cruzi infection.
The incidence of achalasia is similar in most coun-
tries, with no differences in gender and race, although its
incidence increases with age. It has been consis- tently estimated
that the incidence varies between 0.7 to 1.6 per 100,000
inhabitants/year.8-11 The preva- lence of achalasia was currently
estimated to be 10 per 100,000 inhabitants. Newer studies in the
era of high-resolution esophageal manometry (HRM) suggest
that these numbers are low, and that the actual incidence is 2 to
12-14
cure the disease. As a result, many achalasia patients undergo
multiple treatments throughout their life- time.1
The diagnosis of achalasia is based on tests which include:
esophageal manometry that measures the pressure generated in
the LES and in the esophageal body; barium esophagram and
upper gastrointestinal endoscopy, mainly to rule out the
presence of cancer (pseudoachalasia) and possible
complications of the disease (candidiasis).
Achalasia treatments are aimed at reducing the
pressure of the LES either using medication like botulinum
toxin injection (BTI) into the LES or dis- rupting the LES
muscle by stretching its fibers with dilators or by dividing it
surgically or endoscopically (myotomy).15
However, over the last 10 years, there has been sig-
nificant evolution of the management of achalasia with the
introduction of new diagnostic tools as high- resolution
manometry (HRM)16,17 and treatment options as peroral
endoscopic myotomy (POEM)18, temporary stent insertion and
injection of chemical substances in the LES.
Achalasia is a disease treated by both gastroen-
terologists and surgeons and two American scientific societies of
gastroenterologists and surgeons (ACG & SAGES) have
produced guidelines for achalasia.19,20 This new ISDE Clinical
Guideline for Achalasia (I- GOAL), however, is distinctive in
that it is interdisci- plinary and international. Our guideline
aims to offer all stakeholders (physicians and surgeons, patients,
and health policy managers) a useful and up-to-date resource
for applying the best evidence-based prin- ciples to the diagnosis
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54. •
Thank you