ESOPHAGEAL MOTILITY

DISORDERS
Approach to Diagnosis & Management

Dr Selvakumar B

Moderators: Prof U C Ghoshal(GEM) & Prof Anu Behari(SGE)

 Introduction

 Esophageal dysmotility – a significant cause of dysphagia

 Abnormalities of smooth muscle (Distal 2/3rd Esophagus)

 Major changes in definitions since the introduction of High

Resolution Manometry(HRM) - Esophageal Pressure
Tomography(EPT) and the Chicago classification of 2009.

 Of relevance to the surgeon

 Achalasia cardia
 Motility disorders in surgically treated GERD patients
 Agenda for today’s talk

 Approach to diagnosis of Eso dysmotility

 Clinical approach to dysphagia
 Investigations used in daily practice
 EPT – parameters and definitions
 Chicago classification

 Approach to Management – SGE point of view

 Achalasia cardia
 GERD with dysmotility & other disorders of peristalsis
 Swallowing Physiology
 3 phases
 Oral - Voluntary
 Pharyngeal – Swallowing reflex Involuntary
 Esophageal – Progressive peristalsis & LES relaxation

 Centrally initiated due to sensory stimuli from oro-pharynx

 Co-ordinated by Vagus N. and Auerbach plexus

 Stimulation – cholinergic; inhibition – NO, VIP

 Esophageal phase
 UES relaxation
 Propogative peristalsis – Primary (vs Secondary/Tertiary)
 LES relaxation – dLESR (vs tLESR)
*Shackelford’s 7th Edition
 Involuntary phase of swallowing

*Shackelford’s 7th Edition

 Dysphagia
Oropharyngeal vs Esophageal**
 HISTORY:
 Difficulty in initiation of swallow
 Localised to supra-sternal notch*
 Associated nasal regurgitation, coughing*
 Hoarseness of voice preceding dysphagia
 H/o CVA, neck surgery, neck radiation, neurologic diseases

 EXAMINATION:
 e/o neurologic deficits – facial palsy, dysarthria, ptosis etc
alsoInspection
* May ofesophageal
be present in mouth/pharynx for structural lesions
dysphagia
**Harrison’s 19th Ed & Slazinger 10th Ed
 Esophageal dysphagia
 HISTORY**:
 Localised to chest
 Stickiness of food after initiation of swallow
 H/o associated chest pain
 H/o odynophagia – s/o ulcerated mucosa
 Voice change/coughing while swallowing?

 EXAMINATION:
 Usually unremarkable
 Skin changes may be seen in scleroderma and other connective
tissue disorders

**Harrison’s 19th Ed & May be10mechanical

Slazinger th
Ed or motor
Abdel Jalil AA et al Approach to dysphagia. AJM 2015 128; 1138e17-23
 Mechanical or Motor dysphagia
 Dysphagia to solids/both solids and liquids?
 Progressive/intermittent dysphagia?
 Food impaction and liquid intake to clear it?
 Difference with type of foods Eg. hot vs cold liquids?
 Bland regurgitation hours after food intake?
 H/o Compensatory mechanisms – eating slowly, avoidance of
restaurants, avoiding certain foods.
“Before investigating, the clinician should watch the patient
swallow at office eg a glass of water/a bite of solid food*”

*Abdel Jalil AA et al Approach to dysphagia. AJM 2015 128; 1138e17-23

Navaneethan U et al Approach to Esophageal dysphagia Surg Clin N Am 95(2015) 483-9
Approach to Dysphagia
 Investigations - dysphagia
 Initial investigation: Ba swallow/UGIE?*

 Ba Swallow
 Cheaper, easy to do.**
 Superior for motility disorders, esophageal strictures, rings/webs
 Helps plan UGIE – site of stricture, length of stricture
 Best inv for tracheoesophageal fistula, oropharyngeal dysphagia

 UGIE – usually to r/o a mechanical cause

 Can observe mucosal abnormality, take biopsies
 Can carry out therapeutic interventions viz dilatation

 Manometry – if UGIE is normal & high suspicion of motor

dysphagia
*Abdel Jalil AA et based on clinical
al Approach or barium
to dysphagia. picture
AJM 2015 128; 1138e17-23
Navaneethan U et al Approach to Esophageal dysphagia Surg Clin N Am 95(2015) 483-9
**Esfandyari T et al. Dysphagia: Cost analysis of Dx Am J Gastroenterol 2002 97(11): 2733-7
 Ba swallow – Eso. dysmotility

 Timed Barium Swallow(TBS) – Eso emptying

 Upright position with Left posterior oblique(LPO) projection
 100-250 ml liquid barium(45% wt/vol)
 Serial/spot radiographs taken at 1, 2 and 5 min
 Height, width and area of Ba column at 1 & 5 min compared

 Supine Ba swallow - Videoflouroscopy, Eso peristalsis

 Semi-prone Rt Ant Oblique(RAO) – eso. in ‘supine’ position
 5-10 ml 68% wt/vol Ba x 5 swallows, separated by 25-30s
 Rapid distal progress of inverted ‘V’ Ba column

*Baker ME et al GERD: Ba esophagogram and antireflux Sx. Radiology 2007; 243(2); 329-39
Ba swallow
 Conventional Manometry(CM)

 Water perfused system – resistance to water flow assessed

 8 capillary tubes with water perfused continuously at 0.5ml/min
 4 tubes open circumferentially at LES zone, 4 at 5 cm intervals in
Esophageal body
 Intra-gastric pressure is baseline
1. LES length and pressure– by Station/Rapid pull through(SPT/RPT)
2. Eso Body motility - 10 wet swallows at 30s intervals

*Ghoshal UC et al Eso function tests in clinical practice. Trop Gastroenterol 2010; 31(3): 145-54
 CM

*Ghoshal UC et al Eso function tests in clinical practice. Trop Gastroenterol 2010; 31(3): 145-54
Sabiston’s 19th Ed Chapter 43, pg 1021
 EPT - HRM
 High Resolution Manometry(HRM) catheter
 30-32 Radial solid state sensors placed at </= 1cm distance
 Static pressure measurement
 Simultaneous recording in Eso body, UES and LES

 HRM - using 10 swallows of 5 ml aliquots of water, supine position, no

prior h/o esophagogastric surgery

 EPT – Real time 3D(time, distance in eso body and pressure) graph during a
swallow “The Clouse plot”

 Baseline – Atmospheric pressure

HRM Catheter
Chicago classification(CC)
 Chicago classification(CC)

 1st conceptualised in 2009* – by International High resolution

manometry(HRM) working group
 Aim: to simplify EPT, define various motility disorders(based
on EPT metrics) and apply it for clinical practice
 Latest - version 3, 2015** Changes
 Contractile Front Velocity(CFV) abolished
 Metrics for EGJ morphology and 4 types defined
 Weak and failed peristalsis combined into Ineffective
motility(IEM)
*Pandolfino JE et al(2009) Neurogastroenterol Motil.;21(8):796-806
**Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74
 Parameters in EPT – CC v3
 Basic:
 P & D – Proximal and distal troughs in isobaric curve; P at Eso transition zone
and D just above LES
 Contractile Deceleration Point(CDP) – Inflexion point at esophageal
ampulla; must be within 3 cms of LES

 Contractile Vigor and propogation:

 Distal Latency(DL) = Time interval between UES relaxation and CDP
 Distal Contractile Integral(DCI) = Space-time box to calculate amplitude-
duration-length of contraction between P and D for pressure > 20mm Hg

 EGJ & LES:

 EGJ morphology, LES-CD separation – 4 types
 Integrated Relaxation pressure(IRP) = Mean of 4s maximal relaxation in
10s window after UES relaxation

 Peristaltic integrity: Breaks in Isobaric curve between P & D

A normal EPT –the ‘Clouse’ plot
Isobaric curve – 30 mm Hg(green color)
A normal EPT
 Parameters in EPT – CC v3
 Basic:
 P & D – Proximal and distal troughs in isobaric curve; P at Eso transition zone
and D just above LES
 Contractile Deceleration Point(CDP) – Inflexion point at esophageal
ampulla; must be within 3 cms of LES

 Contractile Vigor and propogation:

 Distal Latency(DL) = Time interval between UES relaxation and CDP
 Distal Contractile Integral(DCI) = Space-time box to calculate amplitude-
duration-length of contraction between P and D for pressure > 20mm Hg

 EGJ & LES:

 EGJ morphology, LES-CD separation – 4 types
 Integrated Relaxation pressure(IRP) = Mean of 4s maximal relaxation in
10s window after UES relaxation

 Peristaltic integrity: Breaks in Isobaric curve between P & D

 Chicago v3 - definitions
LES relaxation: IRP – Normal < 15 mm Hg or Upper limit of Normal(ULN)

Spasm
EPT vs CM
 The ‘Chicago’ Hierarchy

 Paste from Chicago_Latest article

Pseudoachalasia

*Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74

 Major Peristaltic disorders

 Not present in normal individuals

 They are 3 types:

 Distal esophageal spasm(DES)
 Hypercontractile esophagus
 Aperistalsis

 “Cork-screw” esophagus is the Ba swallow appearance seen

in both DES and hypercontractile esophagus
*Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74
 Distal Esophageal Spasm(DES)
 Diffuse esophageal spasm

 DL < 4.5s

 Management: ‘Improves with time’

 Medical: Trazodone, imipramine,
sildenafil
 Failed Medical Rx: Endoscopic
Botulinum toxin

 For the Surgeon: GERD + DES

 Dysphagia prominent, avoid Nissen’s
 Start medical Rx after antireflux Sx
 Counsel patient about partial symptom
relief post-op
*Bowers SP Eso Motility Disorders Surg Clin N Am 95(2015) 467-82
Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74
 Hypercontractile Esophagus
 Jack-Hammer/
Nut-cracker esophagus
 20% swallows with
DCI > 8000

 Problems:
 Chest pain
 Dysphagia

 Management:
 Medical(Diltiazem, Sildenafil)/Endoscopic(Botulinum)

 For the Surgeon:

 Failed Medical therapy – Heller’s myotomy
SPPlace
*Bowers of PerOral
Eso Motility Endoscopic
Disorders Myotomy(POEM)?
Surg Clin N Am 95(2015) 467-82
 Aperistalsis
 Seen in Scleroderma, Connective tissue disorders

 100% failed peristalsis

(DCI < 100)

 No Dysphagia

 Problems:
 Recurrent aspiration pneumonia
 Peptic esophageal stricture

 For the Surgeon:

 Partial Fundoplication with feeding access via gastrostomy
*Bowers SP Eso Motility Disorders Surg Clin N Am 95(2015) 467-82
Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74
 Minor peristaltic disorders
 Present in 5% normal population(absent in 95% i.e., 2 SD)

 They are
 Ineffective Motility (50% swallows with DCI < 450)
 Fragmented peristalsis(50% swallows with breaks > 5 cms)

 For the Surgeon:

 In cases of GERD, if they are present – then one should go for
*Bowers SPpartial
Eso Motility Disorders Surg Clin N Am
fundoplication(Preferred – 95(2015)
Toupet) 467-82
Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74
 Achalasia
 Absent dLESR +/- non-propogative peristalsis

 Epidemiology: Type II > Type I > Type III

Chicago Type Common criteria Other criteria

Achalasia Type I IRP > 15mm Hg/ULN with No esophageal pressurisation

(Classic) 100% failed peristalsis( DCI <
100)
Achalasia Type II Panesophageal pressurisation > 20%
swallows
Achalasia Type III IRP > 15mm Hg/ULN Premature contractions > 20%
(Spastic) swallows(DL <4.5s, DCI atleast 450)
EGJ Obstruction IRP > 15mm Hg/ULN Peristalsis not meeting above criteria

 Treatment outcome: Type II > Type I > Type III

 Type I: PD < LHC; Type II: PD = LHC
*Kahrilas PJ et al(2015 Feb) Neurogastroenterol Motil.;27(2): 160-74
Bowers SP Eso Motility Disorders Surg Clin N Am 95(2015) 467-82
EPT – Achalasia types
 Management - Achalasia
Aim: To relieve LES obstruction so that esophageal emptying
and dysphagia improve
 Medical – transient relief with side effects
 CCBs, Isosorbide dinitrate, Sildenafil
 Used in patients unfit for any procedure/as bridging therapy

 Endoscopic Botulinum toxin injection(EBTI)

 Pneumatic dilatation(PD)

 Division of LES circular muscle

 Laparoscopic Heller cardiomyotomy(LHC)
 POEM
*Allaix ME et al. Treatment modalities for Achalasia. Surg Clin N Am 95(2015) 567-78.
Vela MF Mx strategies for Achalasia. Neurogastroenterol Motil (2014) 26; 1215-21.
 EBTI
 Safe, easy technique
 Immediate relief in 80%; 12 mths: 40%.
 Repeated sessions -Less effective than 1st.

 EBTI vs PD: RCT Vaezi et al Gut 1999; 44:231-9

 Relief @ 12 mths: 7/22(32%) vs 14/20(70%)

 EBTI vs LHC: RCT Zaninotto et al Ann Surg 2004; 239: 364-70

 2EBTI at 1 mth gap vs LHC; n = 40
 Relief @ 24 mths: 34% vs 87.5%
 Pneumatic dilatation(PD)
 30 – 40 mm balloons, 7-12 psi, 15 - 60s
 0 - 8% Eso perf; Risk: > 60yrs & 1st PD with 35mm
 15-33% abnormal reflux, mostly manageable medically
 25% need repeated procedures
 Relief: 1 mth 90%, 3 yrs 60%; 5 yrs 44%*; 10 yrs 36%
 3rd PD onwards, No long term improvement over 2 PDs**
 Best results: Age > 40 yrs, female, Type II achalasia, Post PD LES press <
10mm Hg
*Karamanolis G et al. Long term outcome of PD. Am J Gastroenterol 2005;100: 270-4
**Eckardt VF et al. PD: Late results. Gut 2004; 53: 629-33
PD
Lap Heller Cardiomyotomy(LHC)
 Current ‘gold standard’ treatment for achalasia
 Dysphagia free: 5 yrs 90-95%, 10 yrs 80-90%*
 Best: < 40 yrs, Type II, initial LES press > 30mm, straight axis
 Thoracoscopic vs Laparoscopic: RCT Lap. is better**
 UGIE needed to complete myotomy; No gastric myotomy
 No fundoplication, so higher postop GER(60% vs 10%)
 Post op ICD needed; Longer Hosp. stay (4 d vs 2 d)
 Fundoplication decreases post-op reflux
 RCT: Richards WO et al*** LHC(n= 21) vs LHC + Dor(n=22)
 Post-op pathologic GER: 47.6% vs 9.1%
*Zaninotto G et al. 400 LHC from a single center. Ann Surg (2008) 248: p986
**Patti MG et al. J Gastrointest Surg 1998;2:561-6
***Richards WO et al. Ann Surg 2004;240:405-15
LHC + Dor fundoplication
Myotomy – usually at 11 o’ clock position, 6
cms above GEJ and 3 cms on gastric wall
 Controversies in LHC
 Total or Partial fundo? RCT Rebecchi F et al. Ann Surg 2008; 248:1023-30.
 Similar post-op GER but higher dysphagia(15% vs 2.8%)

 Dor or Toupet fundoplication? Debatable

 Toupet: Better wrap; Keeps edges of myotomy separated
 Dor: Simpler; Covers eso mucosa; Myotomy separated by suturing left
edge to crus; Leaves posterior attachments intact
 Reflux on pH monitoring: Non significant trend favoring Toupet(41% vs
21%) RCT Rawlings A et al. Surg Endosc 2012;26:18-26

 Increased
Allaix MEage
et al.and Eso diameter
Treatment modalities for–Achalasia.
no poorer Surgoutcome
Clin N Am 95(2015) 567-78.

 Sigmoid Esophagus( > 6cm with deviated axis)

Mineo 65-72%
TC et relief LHC
al LT outcome: at 7yrs; Esophagectomy
for sigmoid only for
esophagus J Thorac failedSurg
Cardiovac LHC 2004; 128: p402
 PerOral Endosc. Myotomy

Incision:12-
13 cms
above GEJ
(3 cms)
3 cm distal to
mucosal incision
to 3 cm distal to
GEJ
POEM – a NOTES procedure
 POEM outcomes
 Von Renteln D et al Gastroenterol 2013;145:309-11. & Gut 2015 Apr 30 Online 1st
 N = 80; Median myotomy: 13 cm
 Significant mean LES pressure fall: 28 to 9 mm
 Esophagitis on UGIE: 42%
 Symptomatic GER: 37% at 1 yr, managed with PPI
 Remission: 3m 97%, 6m 89%, 12m 82%, 24m 78%
 Total Failure Rate at 24m: 22%
“POEM = LHC without fundoplication via a mucosal incision without GA”

 Points to ponder about this promising new technique

 Needs advanced endoscopic skills
 Pathologic GER common after POEM
 Most studies are small series with short F/U (commonly 6m)
 Surgical revision for failure may be challenging due to adhesions
 LHC vs Others
 LHC vs PD: RCT 106 vs 95 “Comparable in short term”
 Success: 1 yr - 93% vs 90%; 2 yrs – 90% vs 86%
 Eso emptying, LES pressure, QOL similar
 Eso perf: more in LHC - 12% vs 4%
 LHC superior in < GE
Boeckxstaens 40yrs
et al.age
European Achalasia Trial N Engl J Med 2011;364:1807–16.

 LHC vs POEM: “Comparable in short term” 2 Comparative studies

 Similar success at 6 mths: POEM 89%, LHC not mentioned*
 Longer procedure time(149 vs 120 min)**
 Similar morbidity; Longer/similar hosp stay(2.2 vs 1.1 day)
 Lower LES pressure(7.1 vs 16mm)
 Similar acid exposure(32 vs 39%)
 Esophagitis
*Hungness ES33%
et al. of POEM*;
Comparison of Dysphagia
POEM to LHChigher 29% vs
J Gastrointest 0%**
Surg 2013;17:228–35.
**Bhayani NH et al. LHC vs POEM Ann Surg 2014;259:1098–103.
 Follow-up

 Subjective assessment:
 Symptoms: Dysphagia, chest pain etc
 Symptom scores: Eckardt score </=3 – Weight loss, dysphagia,
retrosternal pain and regurgitation
 QOL scores

 Objective assessment:
 TBS: Eso emptying at 5 min; Most relevant clinically
 Manometry: LES pressure < 10mm - long term success
 LES distensibility: EndoFLIP
 Treatment failures
 Graded PD failure: 23-33% over 5-7 yrs
 LHC/Repeat PD

 LHC failure: 10-15% over 5-6 yrs

 PD/EBTI/Redo myotomy/esophagectomy(sigmoid eso)
 5-8% need esophagectomy eventually

 Veenstra BR et al 2015*; Re-operation: 58 LHC failures

 53% - inadequate myotomy, 19% fibrosis, 26% wrap failure
 46 – 1st reoperation; 10 – 2nd reop; 2 – 3rd reop
 Eso preservation in 51(88%), Esophagectomy 7(4+3)
 Intraop perforation 19%, post-op leak 5%
 34 mths F/U – Failure 63% fibrosis; 26% inadequate myotomy/wrap
*Veenstra BRfailure
et al. Revisional Surgery after failed LHC. Surg Endosc 2015 Aug; Online 1st
 LHC after Endoscopic failure

 Tissue plane obliteration, fibrosis

 Higher complications*
 N = 201**; Endosc failure 154(100 PD, 33 EBTI, 21 both)
 Intra-op complications: 9.7% vs 3.6%**
 Eso perf occurred only in prior endoscopic failure group
 Post op complications: 10.4% vs 5.4%**
 Outcomes: Controversial
 Equivalent outcomes* vs Higher failure(19.5% vs 10.1%)**
*Portale G et al LHC outcomes: Detrimental role of prior endotherapy. J Gastrointest Surg 2005; 9: 1332
*Rosemurgy A et al. LHC salvage after Failed endotherapy. Ann Surg 2005; 241: 725
**Smith CD et al. Endotherapy before LHC leads to worse outcomes. Ann Surg 2006; 243: 579-86
 ACG Clinical Guidelines

Vaezi et al. ACG Clinical Guidelines: Mx of Achalasia Am J Gastroenterol 2013

 Upcoming techniques

 Static High freq USG*: EUS, circular muscle thickness and total muscle
area

 Dynamic USG*: Echo Esophagography, longitudinal muscle contraction

patterns, circular-longitudinal co-ordination

 Impedence Planimetry** - the EndoFLIP system, EGJ distensibility

*Mittal RK. Eso function testing. Gastrointest Endosc Clin N Am 2014; 24(4):667-85
**Familiari et al. EndoFLIP system. United European Gastroenterol J 2014;2(2):77-83
 Summary

 Esophageal Pressure Topography – most accurate tool measure of

esophageal function

 Chicago classification – an evolving consensus to simplify EPT for

clinical use

 LHC + Partial fundoplication - current gold standard for treating

achalasia

 Ba swallow - good follow-up tool even in today’s era

 Esophageal preservation possible in majority of treatment failures