achalasia 05 2015 - home | gastroenterology und … · • degeneration of ganglion cells in the...

ACHALASIA 05 2015 ACG 2013

Titel der Präsentation / Name Referent/-in 2

Gastroenterologie

• 1674 Sir Thomas Willis, mechanical dilatation with

whalebone

• 1929 “does not relax” “Achalasia” Sir Arthur Hurst

• Primary motor disorder of the esophagus characterized by

insufficient (incomplete or absent) lower esophageal

sphincter relaxation and loss of esophageal peristalsis

Definition ?


Gastroenterologie

Pathophysiology ?

Park und Vaezi, 2005


Gastroenterologie

• Autoimmune, viral immune, or neurodegenerative etiology

• Degeneration of ganglion cells in the myenteric plexus of the esophageal body

and the LES by inflammatory process

• Loss of inhibitory neurotransmitters nitrous oxide and vasoactive intestinal

peptide and consequently imbalance between the excitatory and inhibitory

neurons

unopposed cholinergic activity

incomplete relaxation of the LES and aperistalsis due to loss of latency

gradient along the esophageal body

Pathophysiology


Gastroenterologie


Gastroenterologie

• Equally in men and women

• Incidence of 1 in 100,000 individuals annually

• Prevalence of 10 in 100,000

• Peak incidence occurs between 30 and 60 years of age

Vaezi et al, 1999

Francis DL et al, 2010

Epidemiology?


Gastroenterologie

• Dysphagia to solids and liquids 90 %

• Regurgitation (of undigested food) 76–91 %

• Chest pain 25-64 %

• Weight loss 35-91 %

• Heart burn 18-52 %

• Aspiration 38 %

• Long vs short duration of symptoms

• “unresponsive to an adequate trial of proton pump inhibitor

(PPI) therapy”

–Compliance, Changing PPI, trial bid dosing

Symptoms/classic presentation?


Gastroenterologie

Score?


Gastroenterologie

• Pseudoachalasia from tumors (not only gastroscopy, also CT)

• Secondary achalasia

–Fundoplicatio, gastric banding

• Chagas disease (Trypanozoma cruzi)

• Esophagitis with or without stenosis/Schatzki

• Motility disorders (primary Achalasie, Jackhammer

esophagus, diffuse esophageal spasms)

• Neuromuscular diseases eg Apoplex (Oropharyngeal),

AML, Myasthenia gravis

• EOE

• Amyloidose, Kollagenosen, Sklerodermie

• ……

Differentialdiagnosis of Dysphagia


Gastroenterologie

• Gastroscopy

• Contrast studies

• Manometry

Diagnosis


Gastroenterologie

Gastroscopy


Gastroenterologie

• Ruling out a mechanical obstruction or pseudoachalasia

• Bx EOE…

• Red flags:

–Short duration of symptoms, Age, Weight loss

• Strong resistance at the EGJ suspect of

pseudoachalasia further imaging (CT)

• EUS? Ziegler et al, 1990

Mittal et al, 2003

Gastroscopy


Gastroenterologie

• Barium column height 1 and 5 min = “ timed barium

esophagram ” (TBE)

De Oliveira et al, 1997

• Nondiagnostic in up to one-third of patients, but no false

positive Ott et all, 1987

• TBS recommended in those with equivocal motility testing

• Objective assessment of esophageal emptying after

therapy!

Esophagogram/Contrast studies


Gastroenterologie

• Dilation of the esophagus

• “ bird beak”

• Aperistalsis

• Poor emptying of barium

• Late- or end-stage achalasia

changes (tortuosity,

angulation, megaesophagus)

Esophagogramm/TBS


Gastroenterologie

• Gold standard

• Normal findings in gastroscopy and esophagramm should

prompt manometry

Esophageal Manometry


Gastroenterologie

Figure 1: Esophageal conventional manometry using 4-5 pressure sensors located in the lower

esophageal sphincter (LES) high pressure zone (HPZ) and esophageal body 5, 10, 15 and 20cm

above the LES.


Gastroenterologie

Figure 2: Esophageal high-resolution manometry. Esophageal pressure data is collected from

sensors placed with 1-1.5cm from each other (a). In pressure topography the pharyngeal

contraction, upper esophageal sphincter relaxation (normal 300-500 msec), proximal esophageal

peristalsis, transition zone (TZ), distal esophageal peristalsis and lower esophageal sphincter

(LES) relaxation (normal 6-10 sec) can be quantified for each swallow (b).


Gastroenterologie

Diagnostik


Gastroenterologie

• HRM criteria:

– Impaired EGJ relaxation (Mean 4 s IRP >/= 10 mmHg over

test swallows)

–Aperistalsis

–Sensitivity of 98 % and a specificity of 96 % (Pandolfino et al)

• Increased basal LES pressure is not requiered

Katz et al, 1986

Esophageal Manometry


Gastroenterologie

Different pressure patterns of aperistalsis

Pandolfino, 2008

•Typ I Absent peristalsis (classic achalasia)

•Typ II Pan-esophageal pressurization

•Typ III Spastic achalasia

Chicagoklassifikation subtyps achalasia?

Kahrlias et al


Gastroenterologie

type I classic achalasia –

impaired EGJ relaxation and absent esophageal peristalsis


Gastroenterologie

type II with pressurization -

impaired EGJ relaxation and

non-peristaltic pressure changes exceeding 30mmHg


Gastroenterologie

type III with spasms –

more than 20% spastic contractions in the distal esophagus

with impaired EGJ relaxation


Gastroenterologie

Clinically relevant!

•Typ I Absent peristalsis (classic achalasia)

•Typ II Pan-esophageal pressurization

best outcome (100 % by Heller myotomy and 91 %

with PD

•Typ III Spastic achalasia

poorest overall response (29 %)

Pandolfino JE, Kwiatek MA, Nealis TJ, Bulsiewicz W, Post J,

Kahrilas PJ.Achalasia: it’s not all one disease., Gastroenterology

2008; 135: 1526–33

Chicagoklassifikation subtyps achalasia -

Why?


Gastroenterologie

• Pharmacologic therapy

–Nitrates, Ca-channel blockers

–BoTox

• Endoscopic treatment

–Pneumatic dilatation

–Temporary Stents

–POEM

• Surgical options

–Myotomie (open vs. laparoscopicaly) ± Fundoplicatio (Dor

vs. „floppy Nissen“)

–Esophagectomy

Treatment


Gastroenterologie

Treatment •Chronic non curable disease, often recurrence

•The choice of initial therapy should be guided by patients age, gender,

preference, and local institutional expertise


Gastroenterologie

• Least effective, noninvasive

• Side effects (headache, hypotension, pedal edema)

• Not complete relief of symptoms

–Phosphodiesterase-5-inhibitor, sildenafil also effective ia

Bartolotti et al, 2000

• Ca-Channel blockers

• Sublingual Isosorbide dinitrate

Pharmacologic therapy


Gastroenterologie

Ca-channel blockers

• Decrease LES pressure by 13 – 49 %

• Improve symptoms by 0 – 75 %

–Nifedipine time to maximum effect after ingestion of 20 – 45

min, duration from 30 to 120 min

–10 – 30 mg sublingually 30– 45 min before meals



Gastroenterologie

Sublingual isosorbide dinitrate

• symptomatic improvement ranging from 53 to 87 %

–Time to maximum reduction in LES pressure (3 –27 min)

duration 30 – 90 min

–5 mg 10 – 15 min before meals

Indication only if Patient cannot or refuse to undergo more

definitive therapies (PD or surgical myotomy)

+

those who have failed botulinum toxin injections



Gastroenterologie

• 12 month success rate 35 -41 %, after 1 month > 75 %

• 50 % relaps in 6- 24 months Pasricha et al, 1995

Vaezi, 1999; Annese et al, 2000

• 100 units botox just above the squamocolumnar junction in

at least 4 quadrants (diluted in preservative-free saline and

injected in 0.5 – 1 ml aliquots)

• Complications:

–25% chest pain, rare mediastinitis, allergy to egg protein

• Repeated injections…eventually higher rate of surgical

complications in subsequent myotomy Smith , 2006

Pharmacologic therapy II:

BoTox


Gastroenterologie

Endoscopic treatment Pneumatic dilatation

Treatment


Gastroenterologie

PD

• Rigiflex (polyethylene ballons)

• Ballon 3–3.5-4 cm

• Pressure 5-10-20 PSI

• Distention time 6-15 sec–5 min

• Sessions 1 – 5

• Ballon distention more important

(no waist) than distention time

–8-15 psi, held 15-60 s


Gastroenterologie

• Esophageal perforation overall median rate of 1.9 %

(range 0 – 16 % )

Campos, 2009; Eckardt, 1997

Must be candidates for surgical intervention

• GERD may occur after PD in 15 – 35 %

• Recurrence of dysphagia should exclude GERD-related

distal esophageal stricture as a potential contributing

complication PPI

PD Complications


Gastroenterologie

• Success rate of

–Single PD ~ 62 % at 6 months and 28 % at 6 years

–Serial dilation ~ 90 % at 6 months and 44 % at 6 years

Vela, 2006

Graded dilatator approach...50-93 % response

– Initial dilation 3-cm balloon

–Followed by symptomatic and objective assessment in 4 – 6

weeks next size dilator

• After treatment a third relapses over 4 – 6 years follow-up

PD


Gastroenterologie

Favorable clinical response to PD:

• Postdilation LES pressure < 10 mmHg

• Chicago classification (pattern of contraction)

–Best effect for all treatments in typ II

• Age > 40/45 years

• Female

• No dilatation

Boeckxstaens 2011, Pandolfino 2008

Farhoomand, 2004; Eckardt, 1992; Pratap, 2011

Predictors of outcome PD


Gastroenterologie

POEM peroral esophageal myotomy

–Cut the muscle over a minimum length of 6 cm into the

esophagus and 2 cm below the squamocolumnar junction

onto the cardia

–Overall, the success rate in prospective cohorts > 90%

Inoue, 2010; von Renteln, 2012; Swanstrom, 2011

–Since no long term + comparison with standards only within

trials

Endoscopic perspectives

Inoue 2010


Gastroenterologie

Endoscopic treatment

• Pneumatic dilatation

• PEG

• POEM

• Temporary Stents

–30 mm stents success rate of 83 % at 10 years

Li, 2010

Cai, 2013

Treatment


Gastroenterologie

• Surgical options

–Myotomie (open vs. Laparoscopicaly, thoracoscopic) ±

Fundoplicatio (Dor vs. „floppy Nissen“, Toupet)

–Esophagectomy

Treatment


Gastroenterologie

Modified Heller myotomy

• Myotomie extending at least 6 cm above

Gastroesophageal Junction and 1.5 cm

Over stomach + 180° Fundoplication

Of Dor

• Symptome improvement in

• 77-100 %, decreases with follow up

period Campos, 2009

• Complications?

–GERD!

Treatment


Gastroenterologie

Modified Heller myotomy

• GERD but with antireflux procedure possible postoperative

dysphagia?

–GERD without fundoplicatio 29-31 %

–GERD with added fundoplicatio 9-14 %

Campos, 2009, Torquati , 2006

• Anterior Dor vs posterior Toupet vs floppy Nissen

Treatment


Gastroenterologie

Endstage achalasia: megaesophagus, sigmoid esophagus

• PD less effective

• Two recent studies documented symptomatic improvement

after myotomy in 72 - 92 % Sweet, 2008; Mineo, 2004

• Unresponsive esophageal resection with gastric or

colonic interposition (greater morbidity/mortality (0-5.4 %)

good candidates for surgery) response in 80 %

• Dysphagia requiring dilation may occur in up to 50 % of

patients after esophagectomy

Duranceau, 2012

Options in endstage achalasia?


Gastroenterologie

• PD vs Botox 70 vs 32 % after 12 months, no differences

after 1 months Vaezi, 1999; Leyden, 2006

• PD vs Heller

–Boeckxstaens only prospective randomized multicentre 3

series of 3 sets of dilatation! No significant differences: 92 vs

87 %

–Systematic review 2006 by Campos found after 12 months

and 36 months First PD vs Heller: 68 vs 89 % and 56 vs 89

%, not graded PD!

• PD more cost-effective

Comparative effectiveness


Gastroenterologie

• After 5-7 years 1/3 needs retreatment after Heller and PD

• Those who failed with the first intervention have a worse

outcome

• Botox, PD, re-do myotomy, esophagectomy

Treatment in case of recurrence


Gastroenterologie

• Assessment of symptoms and esophageal emptying by

barium esophagogram

Aim avoid developement of endstage achalasia

Short-term and lifelong follow-up (recurrence,

megaesophagus, carcinoma), 1-3 months

Postintervention LES pressure 10 mmHg higher remission,

but controversy… Eckardt, 1992

More aggressive follow-up in those with abnormal barium

height at 5 min TBS Vaezi, 1999

Early recurrence(no response) vs. Late recurrence (after

iniial response)

Follow-up I + II ?


Gastroenterologie

Megaesophagus (Diameter > 6 cm)

• Sigmoidesophagus

• Of treatet patients 15 % develop megaesophagus

Of those 5 % need esophagektomy

No data for aggressive treatment, even with post treatment

LES > 10 mmHg, TBS height 5 min abnormal

Endstage disease


Gastroenterologie

• Surveillance endoscopy is not recommended

• Risk of esophageal squamous cell carcinoma 10-50 times

increased in achalasia: incidence rate 1 cancer per 300

patient years (+ adenocarcinoma)

Leeuwenburgh, 2010; Dunaway, 2001

• Overall number of cancers remains low

• Many experts favor endoscopic surveillance after 10 - 15

years, interval of every 3 years Eckardt, 2010

Follow-up II


Gastroenterologie

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