Analysis and Classification of Esophageal

Motor Disorders using HRM/EPT

John E. Pandolfino, MD, MSci

Department of Medicine
Feinberg School of Medicine
Northwestern University
Pressure Topography of Esophageal Motility
The Chicago classification
IRP ≥ upper limit of normal AND
absent peristalsis
No
IRP ≥ upper limit of normal AND
some instances of intact or weak
peristalsis
No
IRP is normal AND
absent peristalsis
OR reduced distal latency
OR DCI > 8,000 mmHg-cm-s
No
IRP is normal AND
Minor Peristaltic Abnormalities
*outside of normative range/clinical
significance less clear
No
Normal
Achalasia
•Subtypes I,II,III
Yes
EGJ Outflow Obstruction
•Achalasia variant versus
Yes mechanical obstruction
 Absent Peristalsis
Diffuse esophageal spasm (DES)
• ≥ 20% of swallows with reduced DL(<4.5s)
Jackhammer esophagus [Extreme Nutcracker]
Yes • ≥ 20% of swallows with DCI > 8,000 mmHg-s-cm
and normal DL
Rapid contraction
• ≥ 20% of swallows with rapid CFV (>9 cm/s) and normal DL
Hypertensive Peristalsis
• ≥ 20% of swallows with DCI > 5,000 mmHg-s-cm and normal DL
Weak Peristalsis [TZ vs IEM]
Yes • ≥ 30% of swallows with small (2-5 cm) breaks in the 20-mmHg IBC
• ≥ 20% of swallows with large (>5 cm) breaks in the 20-mmHg IBC
Frequent Failed Peristalsis
• ≥ 30% of absent swallows
 Pressure Topography of Esophageal Motility
Crucial Measurements for Classification

Pressure Topography Metrics

IRP (mmHg) Lowest mean EGJ pressure for 4 contiguous or non-contiguous
Integrated Relaxation Pressure seconds of relaxation
Defect Size (cm) Length of gap (vertical) in the isobaric contour set at 20 mmHg
Peristaltic Integrity

DCI (mmHg-s-cm) Amplitude x duration x length (mmHg-s-cm) of the distal esophageal

Distal Contractile Integral
CDP (time, position)
Contractile Deceleration Point
CFV (cm/s)
Contractile Front Velocity
DL (s)
Distal Latency
contraction greater than 20 mmHg from proximal (P) to distal (D)
pressure troughs
The inflection point along the 30 mmHg isobaric contour where
propagation velocity slows demarcating the tubular esophagus from
the phrenic ampulla
Slope of the tangent approximating the 30 mmHg isobaric contour
between P and the CDP
Interval between UES relaxation and the CDP
 Figure 3
Pressure Topography of Esophageal Motility
Measure: Integrated Relaxation Pressure
1
mmHg
50

40

10

Length along the esophagus (cm)

30
15

20
20

10
25
5

0
30
-5

-10
I E I E I E I E I 35

0 5 10 15 20
NU IRB Seconds
 Pressure Topography of Esophageal Motility
Measure: Peristaltic Integrity [Break Size]

Fox M et al. Neurogastroenterol Motil. 2004 Oct;16(5):533-42.

Pohl D et al.Am J Gastroenterol. 2008 Oct;103(10):2544-9
NU IRB Ghosh SK. Neurogastroenterol Motil. 2008 Dec;20(12):1283-90
Pressure Topography of Esophageal Motility
Measure: Contraction pattern [DCI]
0

UES
≥110
5
proximal
esophagus

Transition 90
10
Zone

70
15
Location
along Length Contractile 50
Activity
lumen 20 Above
(cm) 20 mmHg IBC

30

25
EGJ 10

30 Time

-10
DCI = contractile activity above 20 mmHg X Time X length

35
0 5 10 15 20
Time (s)
 Figure 4b
Pressure Topography of Esophageal Motility
Measure: Define the CDP
1

5
mmHg

Length along the esophagus (cm)

150
140 10
Contractile Front
Velocity-fast= 3.3 cm/s
120 Stripping wave

15
100

80
Contractile Front
20
60 Velocity-slow = 0.5 cm/s
Emptying phase

40 25
30 CDP
20
10
0
30
-10

35
0s 5s Globular 10s 15s 20s
Formation
NU IRB Pandolfino et al. Neurogastroenterol Motil. 2009 Dec 27. [Epub]
Figure 1
Pressure Topography of Esophageal Motility
Measure: Contraction Pattern [Latency]

EPT: normal latency swallow

Latency measured with

conventional manometry 21
mmHg

Axial position (cm)

21 150
19
17
Axial position (cm)

15
100
13
11 0
2s
9
7 50
EPT: short latency swallow
5 30
3
1 21
0
0 5 10 Axial position (cm)
Time (s)

0
2s
Figure 2
PressureSwallow
Topography of Esophageal Motility
Measure: Contraction Pattern [Velocity]
0

5 UES
mmHg
Length along the esophagus (cm)

150
10 P (transition zone)

15 100

CFV 30 mmHg
20 tangent isobaric contour
50
CDP
30
25 D
DL
0

30 EGJ
EGJ relaxation
5s
35

Time (s)
 Pressure Topography of Esophageal Motility
The Chicago classification
• Step 1-EGJ 25
I

– Assess EGJ
• PIP
30
• EGJ Morphology
• Basal pressure
35
IIIa IIIb
Length along esophagus (cm)

20 20

25 25 RIP

30 30

35 35
0 3 6 9 12 15
time (sec) time (sec)
Pressure (mmHg)

NU IRB 0 10 20 30 40 50
 Pressure Topography of Esophageal Motility
The Chicago classification
• Step 1-EGJ
– EGJ Relaxation Pressure
• IRP

EGJ relaxation Median [IQR] 95th percentile

measure (mmHg) (high)

HRM nadir 3.6 [1.9 – 5.8] ≥ 10 mmHg

4s Integrated
7.9 [6.4 – 10.0] ≥ 15 mmHg
Relaxation Pressure
NU IRB
 Pressure Topography of Esophageal Motility
Single Swallow Classification

Peristaltic Integrity
Intact peristalsis 20 mmHg isobaric contour without large or small break (< 2cm)

Weak peristalsis a) Large break in the 20 mmHg isobaric-contour (>5 cm in length)

b) Small break in the 20 mmHg isobaric-contour (2-5 cm in length) and DCI<
5,000 mmHg-s-cm

Failed peristalsis Minimal (<3 cm) integrity of the 20 mmHg isobaric contour distal to the proximal
pressure trough (P) in any swallow

Contraction Pattern (for intact or weak peristalsis with small breaks)

Hypercontractile DCI >8,000 mmHg-s-cm
Premature contraction DL < 4.5 s
Rapid contraction CFV >9 cm/s
Normal contraction Not achieving any of the above diagnostic criteria

Intrabolus Pressure Pattern (30 mmHg isobaric contour)

Panesophageal pressurization Uniform pressurization extending from the UES to the EGJ

Compartmentalized esophageal Pressurization extending from the contractile front to a sphincter

pressurization
EGJ Pressurization Pressurization restricted to zone between the LES and CD in conjunction with
hiatus hernia
Normal pressurization No bolus pressurization >30 mmHg
 Pressure Topography of Esophageal Motility
The Chicago classification
• Step 2
– Assess Peristalsis
• Characterize Peristaltic Integrity
– Using the IBC set at 20 mmHg
» Intact
» Weak
» Small versus large
» Failed
• Characterize Contractile Pattern
– Calculate DCI, Latency and CFV for each swallow
» Jackhammer
» Hypercontractile
» Spasm
» Rapid

NU IRB
 Pressure Topography of Esophageal Motility
The Chicago classification
• Step 3
– Assess Pressurization Pattern
• Using the IBC set at 30 mmHg
-Panesophageal versus Compartmentalized

Panesophageal Pressurization Compartmentalized Pressurization

NU IRB
 Pressure Topography of Esophageal Motility
The Chicago classification

DIAGNOSIS DIAGNOSTIC CRITERIA

Achalasia
Classic achalasia Mean IRP > upper limit of normal, 100% failed peristalsis
Achalasia with esophageal compression Mean IRP > upper limit of normal, no normal peristalsis, panesophageal pressurization with
≥20% of swallows
Spastic achalasia Mean IRP > upper limit of normal, no normal peristalsis, premature contractions with ≥20% of
swallows
EGJ outflow obstruction Mean IRP > upper limit of normal, some instances of intact peristalsis or weak peristalsis with
small breaks such that it does not meet criteria for achalasia

Distal esophageal spasm* Normal mean IRP, ≥20% premature contractions

Jackhammer esophagus At least one swallow with multipeaked contraction and DCI > 8,000 mmHg-s-cm

Absent peristalsis Normal mean IRP, 100% of swallows with failed peristalsis
Weak peristalsis with large peristaltic defects Mean IRP <15 mmHg and >20% swallows with large breaks in the 20 mmHg isobaric contour
(>5 cm in length)

Weak peristalsis with small peristaltic defects Mean IRP <15 mmHg and >30% swallows with small breaks in the 20 mmHg isobaric contour
(2-5 cm in length)

Rapid contractions with normal latency Rapid contraction with ≥20% of swallows, DL <4.5 s

Hypercontractile esophagus Mean DCI > 5,000 mmHg-s-cm but not meeting criteria for jackhammer esophagus

Frequent failed peristalsis >30%, but <100% of swallows with failed peristalsis
Normal Not achieving any of the above diagnostic criteria
 Patients presenting with Dysphagia/Chest Pain- Negative Structural Work-up

Assess Distal Outflow Pressure

4 second IRP/ Intrabolus pressure

Abnormal IRP/ IBP

Achalasia Functional Obstruction

•Type I- Absent peristalsis •Normal peristalsis
•Type II- Panesophageal pressurization •Weak peristalsis
•Type III- Spasm •Hypertensive Peristalsis

Treatment
•Primarily focused on reducing EGJ outflow through
disruption of the LES [Endoscopic/Surgical]
•Smooth muscle relaxants have minimal efficacy
Special Considerations
•Type I- Absent peristalsis- requires complete obliteration of LES
•Type II- Panesophageal pressurization- best prognosis
•Type III- Spasm- will likely required adjunct treatment of spasm
Treatment
•Requires further evaluation to distinguish cause as this
could potentially be an achalasia variant or pseudo-
achalasia.
•EGD-Biopsies to rule out EoE
•Recommend EUS if biopsies are negative
Special Considerations
•Possible achalasia variant [trial of medication- Botox]
•Consider Pneumatic dilation if no response
•Possible EoE- PPI/ Fluticasone/Diet
•Old peptic injury- empiric dilation with Balloon or Bougie
•Hiatus hernia- may require surgery
 Classic Achalasia
Aperistalsis, impaired EGJR, dilated esophagus

Pressure Topography Plot Landscape plot

0 1 2 3 4 5 6 7 8 9 10 11
time (sec)

Pandolfino JE et al, Gastroenterology 2008Nov;135(5):1526-33

NU IRB
 Achalasia with Esophageal Compression
Aperistalsis, impaired EGJR, panesophageal pressurization

Pressure Topography Plot Landscape plot

0 1 2 3 4 5 6 7 8 9 10 11
time (sec)

Pandolfino JE et al, Gastroenterology 2008Nov;135(5):1526-33

NU IRB
 Spastic Achalasia
Impaired EGJR, ≥20% spastic contractions

Pressure Topography Plot Landscape plot

0 1 2 3 4 5 6 7 8 9 10 11
time (sec)

Pandolfino JE et al, Gastroenterology 2008Nov;135(5):1526-33

NU IRB
 Clinical Evolution of Achalasia

Early Chronic Late

Type II Type II--I Type I
NU IRB Netter Atlas
 Response Rates of Achalasia Treatments
83 Patients categorized by pressure topography subtype

Achalasia Type I Type II Type III All

Intervention Classic compression Spasm Types
Botulinum toxin 0% (0/2) 86% (6/7) 22% (2/9) 39% (7/18)

Pneumatic dilation 38% (3/8) 73% (19/26) 0% (0/11) 53% (24/45)

Heller Myotomy 67% (4/6) 100% (13/13) 0% (0/1) 85% (17/20)

All (any) interventions 44% (7/16) 83% (38/46) 9% (2/21) 56% (47/83)

Subsequent Interventions

Number of interventions 1.6 ± 1.5 1.2 ±0.4* 2.4 ± 1.0† 1.8 ± 0.7

Successful last intervention 56% 96%* 29%*† 71%

Last intervention type B-0,P-10,M-6 B-6,P-25,M-15 B-8,P-8,M-5 B-14,P-43,M-26

*P<0.05 vs Type I, †p<0.05 vs Type III

Pandolfino JE et al, Gastroenterology 2008Nov;135(5):1526-33

NU IRB
 EGJ Outflow Obstruction
Abnormal IRP and Compartmentalized IBP
 Increased IRP and IBP
with EGJ Outflow Obstruction

50
*
IRP-mean3
IRP
*
Pressure (mmHg)

IBPIRP
IBP-IRP3
40 Max-IBP
IBP-max3

30 *
* * *
20

10

0
Asymptomatic Symptomatic
Post-Fundoplication Idiopathic Functional
Control Post- Obstruction
Fundoplication

* = p < 0.05 vs. asymptomatic control

NU IRB
Scherer JR et al. J Gastrointest Surg. 2009 Aug 12. [Epub]
 EGJ Outflow Obstruction
Abnormal IRP and evidence of intact or weak peristalsis
[not meeting criteria for Achalasia]

- Achalasia variant
- Subtle mechanical obstruction
• Hiatal hernia
• Eosinophilic esophagitis
• LES hypertrophy
• Old peptic injury
• Infiltrative

NU IRB
 Patients presenting with Dysphagia/Chest Pain- Negative Structural Work-up

Assess Distal Outflow Pressure

4 second IRP/ Intrabolus pressure

Normal IRP/IBP

Severe Motor Abnormality [not found in asymptomatic controls]

Distal esophageal Spasm

Jackhammer esophagus Absent peristalsis

Minor abnormalities outside of the normative range

Rapid Contractions Hypercontractile esophagus Frequent failed Peristaltic Weakness

 Figure 9
Distal Esophageal Spasm
Nutcracker heterogeneity

Abnormal Deglutitive Inhibition-Abnormal Latency

Spastic Achalasia Distal Esophageal Spasm

mmHg C D
150

CBT
100 3.0 cm
IBT

CFV= 13 cm/s
CFV= 30 cm/s
50

5s 5s

0
Time (s) Time (s)

NU IRB Pandolfino JE, et al. Am J Gastroenterol 2008;103:27

 Rapid Contractions
Figure 4

Normal Deglutitive Inhibition-Normal Latency

Intact Swallow Weak Peristalsis

mmHg D Impedance
150

Bolus
present

100

50

Bolus
absent

0
Time (s) Time (s)

NU IRB Behar and Biancani, Gastroenterology 1993;105:111

 Nutcracker Extreme: Jackhammer
Normal propagation, normal IRP, extreme DCI
mmHg Pressure Topography Plot Landscape plot
mmH
150
g

100

50

0 5 10 15 20
time (sec)

0
IRP = 5 mmHg
DCI = 60,300 mmHg-cm-s
CFV = 3.2 cm/s
 Figure 9
Hypercontractile “Nutcracker”
Nutcracker heterogeneity Esophagus
Hypertensive peristalsis DCI >5,000 but <8,000 mmHgscm

Nutcracker Normal

Pandolfino JE, et al. Am J Gastroenterol 2008;103:27

NU IRB
 Absent peristalsis
Normal mean IRP, 100% of swallows with failed peristalsis
 Frequent Failed
>30%, but <100% of swallows with failed peristalsis

Peter J. Kahrilas, M.D.

Northwestern University
 Weak peristalsis
Large: >20% large breaks in the 20 mmHg isobaric contour
-(>5 cm in length)
Small: >30% small breaks in the 20 mmHg isobaric contour
-(2-5 cm in length)
Impedance
isocontour
mmHg
150 Large: major Small: minor Bolus
present

100

2.2 cm CBT
8.1 cm
IBT
50

2.8 cm IBT Bolus

absent
5s 5s
0
Time (s) Time (s)

Time (s) Time (s)

Figure 3
Pressure Topography of Esophageal Motility
The Chicago classification
IRP ≥ upper limit of normal AND
absent peristalsis
No
IRP ≥ upper limit of normal AND
some instances of intact or weak
peristalsis
No
IRP is normal AND
absent peristalsis
OR reduced distal latency
OR DCI > 8,000 mmHg-cm-s
No
IRP is normal AND
Minor Peristaltic Abnormalities
*outside of normative range/clinical
significance less clear
No
Normal
Achalasia
•Subtypes I,II,III
Yes
EGJ Outflow Obstruction
•Achalasia variant versus
Yes mechanical obstruction
 Absent Peristalsis
Diffuse esophageal spasm (DES)
• ≥ 20% of swallows with reduced DL(<4.5s)
Jackhammer esophagus [Extreme Nutcracker]
Yes • ≥ 20% of swallows with DCI > 8,000 mmHg-s-cm
and normal DL
Rapid contraction
• ≥ 20% of swallows with rapid CFV (>9 cm/s) and normal DL
Hypertensive Peristalsis
• ≥ 20% of swallows with DCI > 5,000 mmHg-s-cm and normal DL
Weak Peristalsis [TZ vs IEM]
Yes • ≥ 30% of swallows with small (2-5 cm) breaks in the 20-mmHg IBC
• ≥ 20% of swallows with large (>5 cm) breaks in the 20-mmHg IBC
Frequent Failed Peristalsis
• ≥ 30% of absent swallows