Achalasia Goals and Assessment of Therapy

Minimally Invasive Therapies

for Achalasia

Siva Raja MD, PhD

What is Achalasia?

• Achalasia (a- and -chalasia "no

relaxation") is a failure of smooth

muscle fibers (at the end of the

esophagus) to relax, which can cause a

sphincter to remain closed…

Wikipedia, 2014

What is Achalasia?

• Achalasia – failure to relax

- Lower esophageal sphincter (LES) has

impaired relaxation and dysregulation of

esophageal peristalsis

• Sir Thomas Willis 17th century treated

starving patient with a sponge-tipped

whale bone

• Rare – annual incidence 1/100,000

Traditional manometry

LES

20 cm

15 cm

10 cm

5 cm

-6

-5

-4

-3

-2

-1

0

1

2

3

4

5

6

7

8

9

10

11

12

13

15

14

16

18

17

20

19

22

21

24

23

25

27

26

28

30

29 High Resolution • 36 circumferential

pressure sites, 1-cm

apart

• 0 Impedance sites

• 4.4 mm diameter

LES

A. Traditional manometry:5 pressure sites, 5-cm apart

B. Impedance manometry:5 pressure sites, 5-cm apart, 4 impedance sites, 5-cm apart

A B

20 cm

15 cm

10 cm

5 cm

MANOMETRY CATHETER EVOLUTION 1960-2010

1960- 2001- 2007-

Conventional

manometry

mmHg

High Resolution Manometry

Free selection mode Topographic display

Hypotensive Peristalsis: Assess Bolus Transit ?

Mild Severe Normal

Normal peristalsis > 30 mmHg Simultaneous >30 mmHg

DIFFUSE ESOPHAGEAL SPASM

Type I

Phary

nxPressure

Scale

UE

S13-c

m8-c

m3-c

mLE

SG

astr

ic

75

25

50

75

25

50

75

25

50

75

25

50

75

25

50

75

25

50

75

25

50

Type 2

Pressure

Scale

Phary

nx

UE

S13-c

m8-c

m3-c

mLE

SG

astr

ic

100

75

25

50

100

75

25

50

100

75

25

50

100

75

25

50

100

75

25

50

100

75

25

50

100

75

25

50

Type 3

PressureScale

CCF © 2009

Type 1 Type 2 Type 3

THREE TYPE OF ACHALASIA

Motiltiy Disorders

Disorders of EGJ Relaxation

Achalasia

Type I Classic

Type II Esophageal compression

Type III Spastic

Functional Obstruction

Normal propagation (CFV)

Intrabolus pressure > 15 mmHg

≥ 30% of swallows compartmentalized

Pandolfino JE, et al. Neurogast & Mot 2009;21:796

Evolution of Achalasia

Aperistalsis

IncompleteLESR

Low AMPContractions

MyentericInflammation

GanglionCell Number

MyentericFibrosis

Manometry

Histology

Early

(Vigorous)

Late

(Classic)

Goals of Therapy

Symptom relief

Palliation

Esophageal empting

Timed Barium Esophagram

• 250 ml low density barium

• Upright

• Drink the volume they can tolerate without regurgitation (recorded for future testing) in 30-60 seconds

• Left posterior oblique position

• 3 on 1 spot films (35 cm x 35 cm) at 1, 2 and 5 minutes

De Olivera JAM et al. AJR 1997;169:473

Therapy

• Medical

- Calcium channel blockers, nitrates

• Interventional

- Botox

- Pneumatic Dilation

• Surgical

- Heller Myotomy

- Per Oral Endoscopic Myotomy

Medical Therapy

Medical Therapy

• • Calcium channel blockers

- nifedipine 10 – 20 mg sublingual before meals

compared to PD in 30 patients over mean

21months provided good or excellent response

in77% and 75%, respectively

- LES pressure reduced by 28%

• Oral nitrates

- isosorbide dinitrate 5 mg sublingual before meals

- May be more potent than nifedipine

Coccia Gut 1991;32604-6

Traube Am J Gastroenterol 1989;84:1259-62

Gelfond Gastroenterology 1982;83:963-9

Interventional Therapy

Botulinum Toxin (BT)

• Inhibitor of acetylcholine release from neurons

promoting LES contraction

• 80 u injected into LES with sclerotherapy needle

- 20 units / cc into 4 quadrants

- High safety profile - mild chest pain

- BT retreatment possible after BT, PD, myotomy

- Negative predictors – age <55 or LESP >50

mmHg

Pasricha Ann Intern Med 1994;121:590-1

Storr BMC Gastroenterol 2002;Aug 13;2:19

Neubrand Endoscopy 2002;34:519-23

Pneumatic Dilation (PD)

Pneumatic Dilation (PD)

• Per-oral balloon disruption of the LES

- High pressure 5 – 12 psi to obliterate waist

- Large diameter 30, 35 and 40 mm

- Repeated sessions 1 day – 3 weeks apart

with size progression

• Negative predictors

- Age <40 years

- Higher LES pressures after treatment

- Pretreatment chest painHulselmans Clin Gastro Hepatol 2010;8:30-5

Eckart Gut 2004;53:629-33

Pneumatic Dilation (PD)

• Perforation 3 – 7%

• Severe chest pain 15%

• Fever

• Intramural hematoma

• Bleeding

• GERD 15%

Surgical Therapy

Robotic Heller

Narration

Best Operation

• Heller/Dor is obstructive decreasing the

adequacy of myotomy

• Heller/Dor does not adversely effect

esophageal emptying as assessed by

TBE

• Heller/Dor decreases reflux (?Normal)

150

120

90

60

30

0

Lo

ng

es

t E

pis

od

e p

H <

4 (

min

)

Upright Supine

No Dor Dor No Dor Dor

pH – Longest Episodes

P=.17 P=.004

Clinical Experiences

of

Per-Oral Endoscopic Myotomy

POEM

Inoue H, et al. Endoscopy 2010;42:265

Details of the Cases

Sex male: female = 15: 15

Age 18~75y.o. (mean 46.2 y.o.)

History 1~23years

Type Spindle 13 cases, Flask 9 cases

Sigmoid 8 cases

POEM – Entry

After proper injection

of normal saline,

small incision as an

entry is made.

POEM – Myotomy

Cutting only

inner circular

muscles with

triangle knife.

POEM – Myotomy complete

Cutting through the narrow segment of EGJ.

POEM – Clipping

S

before POEM after POEM

LES pressure

55.2mmHg 23.5mmHg

Subjective Symptom

Score

10 mean 1.3

Mean Hospital Stay After

The Procedure

4.9 days (3-8 days)

100

80

60

40

20

0before after

(mmHg)

When minimally invasive therapies fail,

there is maximally invasive therapy