Description of esophageal motility disorders according to criteria of Chicago classification version 3.0 by high resolution manometry

1. Esophageal motility disorders addressed
by Chicago classification version 3.0
Samir Haffar M.D.

2. Chicago classification
International High Resolution Manometry Working Group
Year City, Country Version
20081 San Diego, USA v1.0
20112 Ascona,
Switzerland
v2.0
20143 Chicago, USA v3.0
1- Pandolfino JE et al. Neurogastroenterol Motil. 2009; 21(8):796–806.
2- Bredenoord AJ et al. Neurogastroenterol Motil. 2012; 24(Suppl 1):57–65.
3- Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

4. Participating members in CC v3.0
Shobna Bhatia
Guy Boeckxstaens
Serhat Bor
Donald Castell
Minhu Chen
Daniel Cisternas
Jeffrey Conklin
Ian J Cook
Kerry Dunbar
Geoffrey Hebbard
Ikuo Hirano
Richard H Holloway
Phil Katz
David Katzka
Meiyun Ke
Jutta Keller
Anthony Lembo
Ravinder K Mittal
Taher Omari
Jeff Peters
Joel Richter
Nathalie Rommel
Renato Salvador
Edoardo Savarino
Felice Schnoll-Sussman
Daniel Sifrim
Stuart Spechler
Rami Sweis
Jan Tack
Radu Tutuian
Miguel Valdovinos
Marcelo F Vela
Yinglian Xiao
Frank Zerbib

5. Endorsement of CC v3.0
CC v3.0 were reviewed & endorsed by
• American Neurogastroenterology and Motility Society
• European Neurogastroenterology and Motility Society
• Latin American Society of Neurogastroenterology
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

6. Chicago classification v3.0
No previous foregut surgery
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.
• Individual scoring of at least ten 5-ml swallows in supine position
• Metrics of EGJ at rest EGJ morphology & LES-CD separation
EGJ tone
• Metrics of each swallow Integrated relaxation pressure (IRP)
Contraction vigor
Contraction pattern
Intra-bolus pressure pattern (pressurization)
• Absent in CC v3.0 Contractile front velocity (CFV)
Small break (2 – 5 cm)
No more nutcracker

7. Chicago classification v3.0
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.
(1) Incomplete LES relaxation Achalasia (type I - II - III)
EGJ outflow obstruction (EGJOO)
(2) Major motility disorders Absent contractility
never seen in asymptomatic controls Distal esophageal spasm
Hypercontractile esophagus
(3) Minor motility disorders Ineffective esophageal motility
can be seen in asymptomatic controls Fragmented peristalsis
(4) Normal esophageal motility Not fulfilling any of the above

8. Achalasia type I / Classic Achalasia
• Elevated median IRP (> 15 mmHg)
• 100% failed peristalsis (DCI < 100 mmHg)
• DL < 4.5 sec with DCI < 450 mmHg.s.cm meet criteria
for failed peristalsis
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

9. Achalasia type I / Classic Achalasia
IRP 17.6 mmHg, nadir LES pressure 23.3 mmHg
Absent peristalsis
Carlson DA and Pandolfino JE. Gastroenterol Clin North Am 2013; 42(1): 1–15.

10. Achalasia type II / with esophageal compression
• Elevated median IRP (>15 mmHg)
• 100% failed peristalsis
• Panesophageal pressurization with at least 20% of swallows
Contractions may be masked by esophageal pressurization
& DCI should not be calculated
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

11. Achalasia type II / with esophageal compression
IRP 26.5mmHg
Pressurization spanning the entire length of esophagus without peristalsis
Carlson DA & Pandolfino JE. Gastroenterol Clin North Am 2013; 42(1): 1–15.

12. Achalasia type III / Spastic achalasia
• Elevated median IRP (>15 mmHg)
• No normal peristalsis
• Premature contractions with DCI > 450 mmHg.s.cm
for ≥ 20% of swallows
• May be mixed with panesophageal pressurization
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

13. Carlson DA & Pandolfino JE. Gastroenterol Clin North Am 2013; 42(1): 1–15.
IRP 46.5 mmHg, nadir LES pressure 42.3 mmHg
Fragments of distal peristalsis and/or premature contractions
Elevated wave amplitudes on CM labeling as “vigorous achalasia"
Achalasia type III / Spastic achalasia

14. HRM in achalasia / Correlation with barium swallow
Type I Type II Type III
Associated esophagogram in type III interpreted as esophageal spasm
Extreme corkscrew with small diverticulum above distal contraction
Pandolfino JE & Kahrilas PJ. Clin Gastroenterol Hepatol 2013; 11:887–897.

15. Achalasia & EUS
thickened esophageal wall (circular muscle) - traditional type
Esophageal HRM
Very thick esophageal inner circular muscle
2.5 mm at 40 cm from upper incisor
EUS
Achalasia type II
Increased IRP: 26.8 mmHg
Panesophageal pressurization
Purple color means 150 mmHg
Kim HH and Choi MG. Neurogastroenterol Motil 2013; 19(3): 407-408.

16. Achalasia & EUS
thickened esophageal wall of mucosal/submucosal layer
Fluoroscopy
Achalasia pattern
Esophageal HRM
Achalasia type III
Early latency contraction
EUS
Thickened esophageal wall
Majority on mucosal/
submucosal layer
Krishnan K et al. Neurogastroenterol Motil. 2014; 26(8): 1172–1178.

17. Achalasia & EUS
thin esophageal wall
Esophageal HRM
Achalasia type I
High IRP
No pressurization in esophagus
Markedly dilated esophagus
Thin muscle thickness
Different from traditional achalasia type
High-frequency intraluminal US
Kim JH. J Neurogastroenterol Motil 2011; 17(2): 195-196.

18. Diagnosis of achalasia
ACG Clinical Guideline 2013
For achalasia diagnosis, esophageal motility testing, EGD,
and barium esophagram play complementary roles
Vaezi MF et al. ACG Clinical Guideline: Diagnosis and Management of Achalasia.
Am J Gastroenterol advance online publication, 23 July 2013; doi:10.1038/ajg.2013.196

20. Treatment of achalasia according to its subtype
Type II achalasia Most frequent form
Best treatment response
Type III achalasia Least common form
Worst treatment response
Least responsive to pneumatic dilation
Most responsive to Heller myotomy
Conceptual benefit for POEM
POEM: peroral endoscopic myotomy
van Hoeij FB & Bredenoord A. J Neurogastroenterol Motil 2016; 22(1):6-13.
Subtype can serve as criterion for optimal treatment

21. Achalasia secondary to cancer (pseudoachalasia)
• Squamous cell carcinoma of esophagus
• Adenocarcinoma of esophagus
• Gastric adenocarcinoma
• Lung carcinoma
• Leiomyoma
• Lymphoma
• Breast adenocarcinoma
• Hepatocellular carcinoma
• Reticulum cell sarcoma
• Lymphangioma
• Leiomyoma
• Metastatic renal cell carcinoma
• Mesotheliomau
• Metastatic prostate carcinoma
• Pancreatic adenocarcinoma

22. Achalasia secondary to cancer
• Clinic Advance age
Rapidly progressive dysphagia
Weight loss
• HRM None of manometric parameters distinguishes
idiopathic achalasia from cancer-associated achalasia

23. Achalasia secondary to cancer
Fluoroscopy
Typical achalasia like
pattern
High resolution manometry
Achalasia type II
Normal standard endoscopy
Asymmetric thickening
of distal esophagus
FNA confirmed SCC
EUS
Krishnan K et al. Neurogastroenterol Motil 2014; 26(8): 1172–1178.

24. EGJ outflow obstruction (EGJOO)
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.
• Criteria
• Elevated median IRP (>15 mmHg)
Sufficient peristalsis (criteria of achalasia not met)
• Potential etiologies
Incompletely expressed achalasia (achalasia variant)
Manifestation of hiatal hernia
Vascular compression of distal esophagus
Esophaygeal wall stiffness (infiltrative disease or cancer)
• Other investigations: EUS or CT to clarify etiology

25. EGJ outflow obstruction / Achalasia phenotype
Abnormal EGJ relaxation (IRP: 21.3 mmHg)
Some preserved normal peristalsis (DL: 5.0 sec)
Diagnostic criteria for types I, II, or III achalasia are not met
Patient proved to have achalasia
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

26. EGJ outflow obstruction / Large hiatal hernia
5 out of 90 patients with hiatal hernia > 5 cm1
(1) Roman S et al. Arch Sur 2012; 147(4):352–357.
(2) Pandolfino J & Roman S. Thorac Surg Clin 2011; 21(4): 465–475.
LES-CD separation
Pressurization between contractile wave & CD
IRPLES = 4.0 mmHg - IRPCD = 16.6 mmHg
Obstruction related to anatomical angulation of intra-thoracic EGJ

27. EGJ outflow obstruction / Vascular compression
Fluoroscopy
Typical achalasia
like pattern
Esophageal HRM
Achalasia type I
Vascular artifact above LES
EUS
Compression of EGJ by aorta
Loss of typical plane
between aorta & esophagus
Krishnan K et al. Neurogastroenterol Motil. 2014; 26(8): 1172–1178.

28. EGJ outflow obstruction / Obstructive stricture
Patient have distal esophageal stenosis
Based on compartmentalized pressurization & elevated IRP (18.4mmHg)
Normal DL (6.0 sec)
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

29. EGJ outflow obstruction (EGJOO)
clinically unclear disorder
(1) van Hoeij FB & Bredenoord A. J Neurogastroenterol Motil 2016; 22(1):6-13.
(2) Perez-Fernandez MT et al. Neurogastroenterol Motil 2016; 28(1),116–126.
• Symptoms Symptoms of achalasia: dysphagia - chest pain
Symptoms not related to EGJOO: epigastralgia - GERD
Symptoms may disappear spontaneously
• Attitude Subsequent diagnostic tests to identify “true” EGJOO
Achalasia symptoms: treated as achalasia
Epigastralgia or GERD & UES-RP < 50 mmHg:
spontaneous resolution2

30. Absent contractility
Rare
• Criteria 100% failed peristalsis
Normal median IRP
Consider achalasia if borderline IRP & pressurization
• Etiologies Typically associated with scleroderma
Systemic diseases: diabetes, myxedema, MS, ….
In the absence of systemic disease
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

31. Absent contractility
Failed peristalsis - Normal median IRP
Kahrilas PJ. J Clin Gastroenterol 2008; 42(5): 627-635.

32. Clinical significance of absent contractility
• Symptomatic subject
Fundoplication or gastric bypass contra-indicated
Higher chance of post-surgical dysphagia is expected
• Healthy subject
Clinical significance not completely clear
van Hoeij FB & Bredenoord A. J Neurogastroenterol Motil 2016; 22(1):6-13.

33. Distal esophageal spasm
previously known as "diffuse esophageal spasm"
≥ 20% premature contractions with DCI > 450 mmHg.s.cm
Some normal peristalsis may be present
Normal median IRP
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

34. Distal esophageal spasm
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.
Premature contraction (DL < 4.5 sec) with DCI > 450 mmHg.s.cm
Premature contractions uniformly associated with chest pain/dysphagia

35. Hypercontractile esophagus (jackhammer)
• ≥ 20% of swallows with DCI > 8000 mmHg.s.cm & normal latency
• Hypercontractility can involve LES or even be restricted to LES
Expanding DCI measurement to include EGJ in such instances
• Hypercontractile esophagus can be a manifestation of other
esophageal abnormalities such as EGJOO, GERD, or EE
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

36. Hypercontractile esophagus (jackhammer)
restricted to the esophagus
At least two swallows with a DCI ≥ 8000 mmHg.s.cm
Roman S & Tutuian R. Neurogastroenterol Motil 2012; 24 (Suppl. 1), 32–39.

37. Hypercontractile esophagus (jackhammer)
can involve the LES
In case of borderline DCI including LES in the measurement
(dashed box) results in dg of hypercontractility
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

38. Hypercontractile esophagus (jackhammer)
restricted to the LES
Normal esophageal contraction followed by hyperconctractility of LES
Including LES in measurement (dashed box) results in diagnosis
of hypercontractility
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

39. Ineffective esophageal motility (IEM)
popularized in conventional manometry
• ≥ 50% ineffective swallows
• Ineffective swallow can be failed or weak
No distinction need to be made between failed and weak swallows
• Multiple repetitive swallows (MRS) may helpful to determine
peristaltic reserve
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

40. Ineffective esophageal motility (IEM)
failed contractions
DCI < 100 mmHg.s.cm DCI < 450 mmHg.s.cm
+ DL < 4.5 cm
Roman S et al. Gastrointest Endosc Clin N Am. 2014; 24(4): 545–561.

41. Ineffective esophageal motility (IEM)
weak contraction
DCI : 100 - 450 mmHg.s.cm
Roman S et al. Gastrointest Endosc Clin N Am 2014; 24(4): 545–561.

42. Ineffective esophageal motility (IEM)
Conklin JL. J Neurogastroenterol Motil 2013; 19(3):281-294.
Isobaric contour line set at 20 mmHg

43. Multiple repetitive swallow (MRS)
proposed to evaluate peristaltic reserve
• Five swallows of 2-ml water each separated by 2–3 sec interval
Results in profound inhibition of esophageal body & LES
Followed by esophageal contraction of increased amplitude
• Lack of contraction after MRS associated with higher likelihood
of late postoperative dysphagia following anti reflux surgery
• HRM Working Group acknowledged utility of MRS in IEM
but not convinced that it should yet be incorporated into the CC

44. Normal multiple rapid swallows (MRS)
healthy control subject
Strong inhibition of LES tone & esophageal peristalsis during MRS
followed by robust contraction sequence & return of LES tone
Mello M & Gyawali CP. Gastroenterol Clin North Am 2014; 43(1):69-87.

45. Abnormal multiple rapid swallows (MRS)
Lack of contraction response after MRS associated with higher
likelihood of late postoperative dysphagia following anti-reflux surgery
Mello M & Gyawali CP. Gastroenterol Clin North Am 2014; 43(1):69-87.

46. Fragmented peristalsis
> 50% fragmented contractions not meeting IEM criteria
break >5 cm in 20-mmHg ICL & mean DCI >450 mmHg.s.cm
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

47. Fragmented peristalsis
Large break > 5 cm in the 20 mmHg isobaric contour
& DCI > 450 mmHg.s.cm
Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

48. Kahrilas PJ et al. Neurogastroenterol Motil 2015; 27:160-174.

49. Thank You