ACHALASIA :

INSIGHTS INTO DIAGNOSTIC AND

THERAPEUTIC ADVANCES FOR AN
ANCIENT DISEASE
Mari et al, Rambam Maimonides Med J, 2019(10):1

dr. Satria Mula Habonaran Simatupang

Pembimbing : Dr. dr. Agi Satria Putranto, SpB-KBD
 Lack of
Achalasia Greek
Relaxation
INTRODUCTION

Defective
Neurodegenerative
LES

Incidence 1.63/100.000
PATHOGENESIS
Autoimmune

Inflamatory process

Disruption of inhibitory neuron

within myenteric plexus

Inhibit NO
SYMPTOMPS
Dysphagia Heart burn

Vomit Chest pain

Less
Classic
Typical
Regurgitation Cough

Weight loss Choking

DIAGNOSTIC APPROACH

Mechanical
or
Rule Out Endoscopy
Anatomic
Obstruction
DIAGNOSTIC APPROACH
Barium-swallow

Complementary test

Esophageal Dilation
And
Tapered Distal Esophagus
RECENT ADVANCES IN THE DIAGNOSIS OF DYSPHAGIA
: HIGH-RESOLUTION MANOMETRY (HRM)

• MECHANICAL
EXCLUDED • ANATOMICAL

• ACHALASIA
HRM : • EGJ OUTFLOW OBSTRUCTION
• OTHER MAJOR PERISTALTIC
GOLD STANDARD
DISORDERS
RECENT ADVANCES IN THE DIAGNOSIS OF DYSPHAGIA
: HIGH-RESOLUTION MANOMETRY (HRM)

HRM CATHETER 36 PRESSURE SENSORS

• NARES • CHANGES IN PRESSURE =
COLOR VARIATIONS
• ESOPHAGUS
• EGJ
HRM ADVANTAGES
COMPARED TO THE OLD CONVENTIONAL MANOMETRY
Better LES localization

Shorter study time

Less intra-observer & inter-observer

variation of study analysis

Optimal assessment of esophageal body

peristalsis & ability of detecting even minor
peristaltic defects
T H E C H I C A G O C L A S S I F I C AT I O N
Common
denominator • Impaired EGJ relaxation
Of • Increased IRP
ACHALASIA

HRM CHICAGO 3 Subtypes • Lowest average relaxation

development Classification ACHALASIA IRP pressure within 10 seconds
of LES relaxation window

• Outflow obstruction &

IRP > resistance to flow at the
15mmHg level of the EGJ
 T H E C H I C A G O C L A S S I F I C AT I O N
• Total absence of peristalsis for all swallows.
ACHALASIA
Incomplete LES relaxation, aperistalsis and
Type I absence of esophageal pressurization.

ACHALASIA • Peristalsis is replaced by pan-esophageal

Type II pressurizations throughout the tubular esophagus .

• Premature spastic contractions. Incomplete LES

ACHALASIA
relaxation and premature contractions (DL < 4.5
Type III seconds) in at least 20% of swallows.

RCT STUDY BY ROHOF ET AL :

TREATMENT OUTCOMES BEING BEST IN TYPE II, INTERMEDIATE FOR TYPE I, AND WORST IN TYPE III .
TREATMENT
IRREVERSIBLE
ACHALASIA
OPTIONS DISEASE

THERAPY REDUCE SYMPTOMS &

MAIN AIM IMPROVE QUALITY OF LIFE

RELIEVE EGJ DRUG INDUCING

LES
MECHANICALLY
DISRUPTING SPHINTER
OBSTRUCTION RELAXATION INTEGRITY

ENDOSCOPY
SURGICAL
(PNEUMATIC
(LHM)
DILATATION OR POEM)
TREATMENT
OPTIONS Muscle Relaxant DISADVANTAGES
• Nitric Oxide • Limited efficacy
PHARMACOLOGICAL • Calcium Channel • Temporary effect
AGENTS Blockers • ADVERSE
• Sildenafil EVENTS :
headache,
edema, and
dizziness
TREATMENT
OPTIONS MAIN EFFECT MAIN LIMITATION

• Block the release • TEMPORARY

Botulinum
of acetylcholine EFFICACY
toxin injection
(since 1994) • temporary loss of • a drop in symptom
muscle contraction relief from 78% at
1month to < 40%
after 1year
• BRIDGE THERAPY
TREATMENT
PROCEDURE EFFICIENT & SAVER
OPTIONS • Graded balloon
1.Fluoroscopic
guidance dilation approach
Pneumatic 2.Placed within LES with increasing
3.Balloon inflated up balloon diameters
Baloon
to 10–15 pounds (30 mm, 35 mm, 40
Dilatation mm)
per square inch
4.Maintained for 60’’ • Fewer esophageal
• Leading to muscle perforations
disruption of LES

In most centers :
performed in two treatments with 30 mm and 35 mm
balloon dilatations performed 2 to 4 weeks apart.
TREATMENT
MAIN
OPTIONS PROCEDURE
COMPLICATION

• Success rate : 86% • GERD in 15-35%

Pneumatic
Baloon after 2 years & 85% patients
after 5 years • Controlled by anti-
Dilatation • Long-term acid meds
outcomes are
comparable to those
of LHM after 2 and
5 years
TREATMENT
PARTIAL WRAP
OPTIONS PROCEDURE after LHM
in Achalasia patients

Laparoscopic • Dissection of the 1. Toupet : Posterior

Heller anterior muscle fibers 270° fundoplication
Myotomy • Fundoplication after 2. Tor : Anterior 180°
(LHM) myotomy  to fundoplication
prevent post-surgical
gastro-esophageal
• Success Rate :
acid reflux
Symptom improvement scores
following LHM is estimated to be
85% after 5 years.
• Very low mortality rate :
Less than 1/1000.
NOVEL ADVANCES IN THERAPY : POEM
POEM objective :
perform LES myotomy during an endoscopic procedure

Creating a MYOTOMY
SUBMUCOSAL TUNNEL Passing through the
accessed through a tunnel into the of the circular muscles
mucosal orifice within the mainly with an
EGJ REGION
esophageal wall endoscopic knife
NOVEL ADVANCES IN THERAPY : POEM
Hydrothorax
Meta-analysis Study in 2015 by Barbieri et al :
551 patients showing a pooled success rate of 93%,
though the follow-up period was very diverse (from 3
Late Bleeding
months - 3 years)
Major
adverse events
Retrospective study of 1680 chinese patients : Pneumothorax
major adverse events rate is 3.3%

ICU Admission
NOVEL ADVANCES IN THERAPY : POEM
POEM vs Baloon Indications
dilation vs LHM other than Achalasia

Shorter hospital Diffuse esophageal

Khashab et al :
stay spasm 93% clinical success rate of
POEM after a 8-month follow-up
period in these patients
Shorter recovery Jackhammer
time esophagus refractory

More efficient symptomatic relief for type

III achalasia compared to surgery
THANK YOU