Prof. Amgad Fouad Prof. Amgad Fouad

Gastroenterology Gastroenterology

centercenter

Mansoura UniversityMansoura University

Achalasia of the esophagus, Achalasia of the esophagus,

different line for therapydifferent line for therapy

Achalasia

–Greek word

–Failure to relax

–Willis (1672)

Zenker & Vonziemssen (1877): Diminished

contractile power of the esophageal

musculature.

Meltzer & Miklicz (1888): Spasmodic contraction

of the cardiac sphincter.

Einhorn (1888): Failure of relaxation of the

cardia on swallowing.

Horst (1929): Established the term achalasia

(failure to relax).

Achalasia:

–The most recognised motor

disorder of the esophagus.

–Cardinal features:

• Poorly relaxing LES

• Prolonged esophageal transit

• Defective esophageal body peristalsis .

• 1-2 / 200.000

• ♂ =♀ .

• Any age.

• Onset 3rd – 5th decade.

• Duration of symptoms at presentation 2

years average.

(Mayberry & Atkinson, 1985)

Presentation

• Dysphagia (almost 100%)

• Regurgitation (60-90%)

• Chest pain (30 – 50%)

• Wt loss (Advanced disease).

• Pulmonary symptoms.

– Bronchopneumonia

– Lung abscess

– Ht burn (rare presentation )

Diagnosis

– Compatible clinical history.

– Radiography.

– Endoscopy.

– Manometry.

Radiographic studies Plain X ray :

Widened mediastinum .

Air fluid level

Absence of gastric air bubble

Evidence of pulmonary complications

Radiographic studies (continue)(continue)

Barium swallow

Screening test

Esophageal body dilated

Lower end esophagus → tapered point.

(birds Beak )

Nitrite test → Diagnostic.

Radiographic staging

Stage I : Slight dilatation of the body not >3.5cm.

Stage II : Moderate dilatation 3.5-6cm.

Stage III : Marked dilatation >6cm.

Stage IV : Marked dilatation elongation and

tortiousity of the esophagus (sigmoid

esophagus).

Endoscopy

Important diagnostic tool

• Rule out several diseases that mimic achalasia.

• Evaluate esophageal mucasa before therapeutic

manipulation.

Typical finding

• Dilated esophgeal body

• Puckered closed LES

• No organic stricture

Mamometry

•Confirms & establishes the diagnosis

•Features:

Essential features Absence of esophgeal body peristalsis (1ry peristaltic waves) ↑ intraesophageal resting pressure. Abnormal LES relaxation.

Supportive Features Hypertensive LES pressure Low amplitude esophageal contractions

(Castell, 1996)

☺Treatment of Achalasia Treatment of Achalasia

Medical TreatmentMedical TreatmentNitrates .

Ca ++ channel blockers

Anticholinergic drugsAll have been shown to reduce the force of

contraction of esophageal body smooth muscles.

May be of value in reducing chest pain & improving dysphagia

(Gelfond et al ., 1982)

Botulinum toxinBotulinum toxin

Neurotoxin produced by clostridium

botulinum.

Only serotype A+B have been approved

for clinical use.

Two preparations of BTX (A) available:Dysport (UK)

Botox (USA)

Efficacy of BTX in achalasia Efficacy of BTX in achalasia

AuthorYearStudy

designNo. of

patientsDoseResponse 1

mo (%)Response 6

mo (%)

Adults

   Pasricha 1994OL1080 U9060

   Pasricha 1995DBPC3180 U9066

   Annese 1996DBPC8100 U10053

   Prakash 1999OL4280U8041

   Annese 2000MCRT40200 U8836

   D'Onofrio 2002OL37100 U8465

Children

   Hurwitz 2000OL2380-100U8343

   IP 2000OL730U10033

Pneumatic dilatation Pneumatic dilatation

The most effective non-surgical treatment

option.

Forceful dilatation using air or water

pressure can be applied to the lower

esophageal segment and controlled to

secure further stretching to the point of

rupture of the circular ms fibers.

Recommended technique for pneumatic balloon dilatationRecommended technique for pneumatic balloon dilatation

(Voizi et al, 1994)

1Fasting for at least 12 h before procedure .

2Esophageal lavage with a large-bore tube (if needed).

3Sedation and endoscopy in Rt lateral position.

4Guidewire positioned in stomach and balloon passed over the guidewire.

5Initial dilatation with 3-cm diameter balloon; subsequent progression to 3.5-cm and 4-cm balloons may be required at separate sessions.

6Accurate placement of balloon across gastroesophageal junction fluoroscopically.

7Balloon distention to obliterate the waist, which usually requires 7-10 psi (this is the key to a successful dilatation)

8Gastrograffin study followed by barium swallow to exclude esophageal perforation.

9Observation for 4 h for chest pain and fever.

10Discharge with follow-up in 1 mo.

*Before proceeding with pneumatic dilatation, it is important to ensure that a cardiothoracic surgeon is available in case of an esophageal perforation.

Cumulative effectiveness of pneumatic dilatation in Achalasia Cumulative effectiveness of pneumatic dilatation in Achalasia

ReferenceNumber

of Patients

Study DesignDilator

(Size/cm)

Objective Assessments

% Sx Improvement

Follow-up (yr) Mean (Range)

Pe

rfora

tion

(%) %LES

PressureExcellent/

Good

Cox 7Prospective3860.8(0.5-1) 0

Gelfand 24Prospective3.460,68 70,93 0

Barkin 50Prospective3.5901.3(0.1-3.4) 0

Stark 10Prospective3.5740.50

Makela 17Retrospective3, 3.5, 4 50,75,75 0.55.9

Levine 62Retrospective3, 3.5 85,88 0

Kadakia 29Prospective3, 3.5, 4 67 62,79,93 4(0.3-6) 0

Kim 14Prospective3, 3.5 39 750.3

Lee 28Prospective3, 3.5, 4 7

Reference

Number of

Patients

Study DesignDilator

(Size/cm)

Objective Assessments

% Sx Improvement

Follow-up (yr) Mean (Range)

Pe

rfora

tion

(%) %LES

PressureExcellent/Good

Levine 62Retrospective3, 3.5 85,88 0

Kadakia 29Prospective3, 3.5, 4 67 62,79,93 4(0.3-6)0

Kim 14Prospective3, 3.5 39 750.3

Lee 28Prospective3, 3.5, 4 7

Abid 36Retrospective3.5, 4 88,89 2.3(1-4) 6.6

Wehrmann 40Retrospective3, 3.5 42 89 2.52.5

Lambroza 27Retrospective3 67 1.8(0.1-4.8) 0

Bhatnagar 15Prospective3, 3.5 73,93 1.2(0.3-3) 0

Total359size 3125/168=74% 1.6(0.1-6)yr 7/345=2%

Size 3.5184/214=86%

size 490/100=90%

Continue

Surgical management of AchalasiaSurgical management of Achalasia

• Rationale of surgery is to weaken the lower

esophageal sphincteric pressure but in

controlled Faison avoiding subsequent reflux

(Earlam, 1976)

Heller (1913)

First performed extra mucosal

cardiorytomy.

Two 8cm incisions one ant & one post.

Incisions extending 2cm into the dilated

part cranially & into the fundus of the

stomach caudally.

Modified Heller’s myotomy

(Zaaijer, 1985)• Most widely used technique.• Single anterior myotomy.• Transabdominal.

However

Two problems →poor results

1. Incomplete myotomy

2. Reflux esophagitis .

Anti – Reflux Anti – Reflux

Laparoscopic Heller’s Laparoscopic Heller’s

Most preferred by gastrointestinal

laparoscopic surgeon.

Easy access.

Good result

Anti – reflux +

Results of HellerResults of HellerAuthor (Year)ProcedureNo.

%SAT

%Dys

%RegF/U

Mort )%(

Morb )%(

Esoph Perf

Laparotomy

Black et al (1976)Heller/Post1086625194 y0NANA

Csendes et al (1989)Heller/D4295212862 mo02.30

Paricio et al (1990)Heller/Post4892445.4 yr08.30

Pinotti et al (1991)Heller/Post72295NRNR6 mo-15 yr

0NANA

Bonavina et al (1992)Heller/D206944964.5 mo01.90

Thoracotomy

Menzies-Gow et al (1978)

Heller10280768 yr05.60

Jara et al (1979)Heller145INR114885 mo0NANA

Okike et al (1979)Heller/B or A

46885336.5 yr0.2NA1%

Yong-xian (1982)Heller/DF4493INR

INR

3 mo-19 yr

02.30

Ellis et al (1984)Heller10391546.75 yr09.60

Little et al (1988)Heller/B5788694.8 yr1.8NANA

Laparoscopy

Ancona et al (1995)Heller/D1794607 mo000

Rosati et al (1995)Heller/D25964INR12 mo040

Delgado et al (1996)Heller/D12INR16INR3 mo01.68%

Swanstrom and Pennings (1994)

Heller or Heller/T

129281716 mo000

Hunter et al (1997)Heller/D or T40INR10512.5 mo07.50

Thoracoscopy

Pelligrini et al (1993)Heller228812INR2 yr0180

Aim of work

The aim of this work was to evaluate Heller’s

myotomy and preumatic balloon dilatation as two

alternative lines of therapy for patients with

achalasia of the esophagus.

Patients and MethodsPatients and Methods

o Our study is a retrospective non- randomised study

conducted at GEC during the period between October

1979- November 2002.

o The study included 310 cases with achalasia.

o 169 ♂ & 141♀

o According to the line of management the study included

two groups: Group A: 150 patients treated with myotomy +

fundoplication. Group B: 160 patient treated by pneumatic balloon

dilatation.

Preoperative work up

• Thorough history and clinical examination.

• Patients were divided into 4 groups according to

Demeester's grading for dysphagia.

(Cuschieri et al., 2002)

• No dysphasia

• Mild : occasional episodes.

• Moderate: requires fluids to clear.

• Severe : episodes of solid food impaction &

require medical treatment.

Preoperative work up (continue )(continue )

• Radiological examination

• According to Olsen scoring system, patients

were divided into 4 groups

(Olsen et al., 1983)

• Endoscopic evaluation

• Manometric study

Method of management

Group (A):• Modified Heller myotomy 35 patient (56.8%).• Myotomy + Dor fundoplication 45 patient (30%).• Myotomy + Nissen fundoplication 9 patient (6%).• Myotomy + Taupet fundoplication 4patient (2.6%).• Laparoscopic mytomy 3 patients (2%).• Laparoscopic mytomy + Dor fundoplication 4patints

(2.6%).

Group (B):

• Pneumatic balloon dilatation (1.8+ 1 set)• One session in 79 patients (60.5%).• Two sessions in 48 patients (30%).• Three or more sessions in only 15 patients

(9.5%).

Method of management Continue

ResultsResults

Dysphagiaaccording to Demeester’s

grading for dysphagia

Mild75 patients (24%)

Moderate186 patients (60%)

Sever49 patients (16%)

Regurgitation213 patients (69%)

Heart burn65 patients (21%)

Weight loss220 patients (71%)

Respiratory complications43 patients (11%)

Dysphagia grading of the patients on presentation.

Mild 24%

Moderate60%

Sever16%

Mild

Moderate

Sever

0

20

40

60

80

100

0 0 : 0 0

Clinical presentation of patients

Dysphagia100%

Weight loss71%

Regurgitation69%

Heart burn

Respiratorycomplications11%

Radiological characters of achalasia patients before management .

First and second degree.according to Olsen et al 1953 scoring system

283 patients (91%)

Third and fourth degree .according to Olsen et al 1953 scoring system

27 patients (8.8%)

Delayed evacuation .279 patients (90%)

Normal esophagus .9 patients (2.8%)

Endoscopic findings in patients with achalasia before management .

Dilated esophagus .273 patients (88%)

Marked spastic cardia .36 patients (12%)

Esophagitis .25 patients(8%)

Gastritis and or duodenitis .50 patients (16%)

Manometric features for achalasia paients before management

Symptomatic evaluation of patients after cardiomyotomy .

Dysphagiaaccording to Demeester’s

grading for dysphagia

Complete resolution

105 patients (70%)

Improvement 25 patients (16%)

Persistant20 patients (14%)

Heart burn and regurgitation37 patients (25%)

Weight gain89 patients (60%)

Symptomatic evaluation of patients after pneumatic ballon dilatation .

Dysphagiaaccording to

Demeester’s grading for dysphagia

Complete resolution96 patients (60%)

Improvement 30 patients (19%)

Persistant34 patients (21%)

Heart burn and regurgitation24 patients (15%)

0%

10%

20%

30%

40%

50%

60%

70%

Dysphagia improvment after cardiomyotomy and ballon dilatation.

Cardiomyotomy

Ballon dilatation

Cardiomyotomy 70% 16% 14%

Ballon dilatation 60% 19% 21%

Complete resolution

Improvement Persistant

BeforeAfter

ValueAverageValueAverage

LESLESP34.5 ±4 mmHg

11 to 95 mmHg

12.9 + 3mmHg

5 to 19 mmHg

%RELAXATION58.5+10 %20to 99%80+8 %69 to 99 %

BODYAMPLITUDEPROX24.1+3.5 mmHg

11to 72% mmHg

24+ 3 mmHg

10 to 54 mmHg

MID23+2 mmHg11to 80% mmHg

27+4 mmHg

10 to 68 mmHg

DISTAL22.5 1.9± mmHg

9to 80 mmHg

34+2 mmHg

11 to 65 mmHg

DURATIONPROX4.1 ± 2.1 sec

1.5to 10 sec

3+0.5Sec

0.9 to 5.4 sec

MID4.1 ± 1sec1.7 to 10.1 sec

3.2+0.3Sec

1.1 to 6.7 sec

DISTAL3.7 ± 1.8 sec

1.6to 9.8 sec

3.1+1.8Sec

1 to 6 sec

VELOCITYSIMULTANEUS55 ± 13.2 %6 to 100 %

41+13.2 %

9 to 100 %

NON TRANSMITTED

32.9+9% 6 to 100 %

25.1+9 %9 to 100 %

Manometric study before and after cardiomyotomy

Manometric features before and after pneumatic dilatation.

0

10

20

30

40

50

60

70

80

L.E.S.P % Relaxation

L.E.S changes with cardiomyotomy and ballon dilatation.

Values onpresentation

Aftercardiomyotomy

After ballondilatation

SummarySummary and conclusionand conclusion

We agree that life long palliation of dysphagia is not

guaranteed but It is obvious that Heller cardiomyotomy

in our study gives good to excellent results as regard

the improvement of dysphagia. As in the largest series

puplished about the management of achalasia.

Pneumatic ballon dilatation is also a good method of

management of patients with achalasia, but it doesn’t

give the same longterm response as surgical

cardiomyotomy besides that some cases may have

early recurrence or persistant dysphagia after

dilatation .