Esophageal motility disorders

1. MOTILITY DISORDERS OF ESOPHAGUS
Moderator: Dr A I Mazumdar (Asst Prof. Dept of Surgery)
Presenter : Dr Monitosh Paul (2nd Yr PGT)

2. Brief Anatomy Of Esophagus:
• The esophagus is a hollow muscular organ, approximately 25cm in length that extend from the
pharynx to the stomach
• Extends from lower end of cricoid cartilage (C6 vertebra) to esophagogastric junction (T12)
• Layers: mucosa-basement membrane –lamina propria-submucosa(strongest)-muscularis
mucosa(outer longitudinal, inner circular muscle fibre) #lacks serosa
• Parts: Cervical; Thoracic; Abdominal. Upper esophageal sphincter & lower esophageal sphincter.
• Constrictions: Cervical- cricopharyngeus, 15cm from upper incisor, f/b impaction
Bronchoaortic-T4, 25cm, endoscopic perforation
Diaphragmatic-T10, 40cm
• The esophageal wall: The proximal esophagus is predominantly striated muscle. The distal
esophagus is predominantly smooth muscle. The mid esophagus contained a graded transition of
striated and smooth muscle.

4. Physiology of Esophagus:
• The function of the esophagus is to transport the ingested material from the
pharynx to the stomach by peristaltic waves.
• Primary peristalsis: Progressive, triggered by the swallowing center in the
brain stem and the contraction wave travel at speed 2cm/s.
• Secondary peristalsis: Progressive, generated by esophageal
distension/irritation from retained bolus, refluxed material. Its role is to
clear the esophagus form retained bolus.
• Tertiary peristalsis: non-propulsive and uncoordinated and their non-
peristaltic nature means they move the bolus up as well as down the
esophagus.
They are seen -as intermittent ripples along the wall of the esophagus lasting
only a few seconds, -as multiple simultaneous contraction rings or -as a
segmented barium column producing a corkscrew appearance (in DES).

5. Mechanism of swallowing:
• During the pharyngeal phase of swallowing, a primary
peristalsis is created, that relax the UES and forces the
food bolus through it.
• The UES remain constricted and has resting pressure
of 20-60 mmHg. The peristaltic waves travel at the
speed 2cm/s and reach the stomach in 5-10 sec.
• Secondary peristalsis get initiated if the primary
peristalsis failed to get food to the stomach and the
esophagus became distended.

6.  Classification of Esopageal Motility Disorder:
Clinical Classification: Primary & Secondary
 Primary:
Achalasia
Spastic esophageal motility disorders, such as
1. Diffuse esophageal Spasm
2. Nutcracker Esophagus(Jackhammer)
3. Hypertensive lower esophageal sphincter
4. Ineffective Esophageal Motility(IEM)
5. Presbyoesophagus

7.  Secondary:
Neurological- stroke, bulbar palsy, motor neuron disease, multiple sclerosis, Parkinson's
disease, poliomyelitis
Collagen or Neuromuscular disorder: myasthenia gravis, Dermatomyositis, muscular
dystrophy
Autoimmune- systemic sclerosis, Scleroderma, Polymyositis, SLE, CREST syndrome
Chagas disease, alcoholic neuropathy
Endocrine and metastatic disease

8. Classification based on part involved:

10. A. Diffuse Esophageal Spasm:
• f > m
• Motor abnormality of lower 2/3rd of esophagus
• Muscular hypertrophy and degeneration of vagus branches
• Repetitive simultaneous and high amplitude esophageal contractions
• C/f: Chest pain (mimics angina), dysphagia
• Aggravated by acid reflux, dinking cold liquids, heightened
emotional stress
• 10% LES relaxation on wet swallow
Diagnosis: Radiograph- Corkscrew esophagus or pseudo-
diverticulosis on esophagogram,
distal bird beak sign or normal peristalsis can also be present
Manometry- Simultaneous multipeaked contractions and
intermittent normal peristalsis of high amplitude (>120mmHg) or long
duration (>2.5 secs)

11. Treatment:
• Psychiatric evaluation is done along with elimination of trigger factor
• Acid suppression, nitrates, calcium channel blockers, sedatives or anticholinergics is used
• Bougie dilatation of esophagus (50-60 fr)
• Botulinum toxin injection
Surgery:
Indication- severe chestpain, dysphagia or pulsion diverticula of thoracic esophagus
Procedure- long Esophagomyotomy through left thoracotomy or video assisted technique
(proximally, from thoracic inlet to distal extent to LES)

12. B. Nutcracker Esophagus (hypercontractile esophagus):
• M/c esophageal hypermotility disorder
• m = f
• most painful, dysphagia, chest pain
• Hypertrophic musculature with high amplitude contractions(DCP > 8000 or >180mmHg), long
duration (>6 secs)
• The LES pressure is normal
• LES relaxation occurs with each wet swallow.
• Ambulatory monitoring can help distinguish this disorder from DES
• Treatment- mainly medical with CCB, Nitrates, Antispasmodics
to avoid caffeine, cold & hot drinks

13. C. Hypertensive Lower Esophageal Sphincter
• In Chicago classification, esophagogastric junction (EGJ) outflow obstruction
• Defined as a median integrated relaxation pressure greater than 15 mm Hg (hypertensive, poorly
relaxing sphincter).
• Evidence of effective peristalsis that is not present in classic achalasia
• dysphagia, chest pain
• Manometry- elevated LES pressure, infrequently acid reflux and regurgitation. LES pressures
above normal (>26 mm Hg), and relaxation will be incomplete.
• Esophagogram : Narrowing at the gastroesophageal junction (GEJ) with delayed flow and
esophageal contraction abnormality. 50% of the time, peristalsis in esophageal body is normal. In
remainder, abnormal contractions are noted to be hypertensive peristaltic or simultaneous
waveforms. The pathogenesis is not well understood.

14. Treatment:
• Botox injections alleviate symptoms temporarily, and hydrostatic balloon dilation may provide
long-term symptomatic relief.
Surgery:
• Indication- failure to respond to interventional treatments, significant symptoms.
• A laparoscopic modified Heller esophagomyotomy is the operation of choice. In patients with
normal esophageal motility, a partial antireflux procedure (e.g., Dor or Toupet fundoplication) is
added.
Per-oral endoscopic myotomy may be done.

15. D. Ineffective Esophageal Motility:
• Irreversible contraction abnormality of distal esophagus, associated with GORD
• Due to secondary inflammation of body of esophagus following more exposure to gastric juices
• Reflux, dysphagia, heartburn, chestpain
• Hypo-contraction in distal esophagus with at least 30% of wet swallows exhibiting any
combination of the followings
- Distal esophageal peristaltic wave amplitude <30 mm Hg
- Simultaneous contractions with amplitudes <30 mm Hg
- Failed peristalsis wave: not traverse entire length of distal esophagus
- Absent peristalsis
Patients often have LES hypotension

16. E. Presbyoesophagus:
• Abnormal motility in the elderly
• Although an underlying cause, for example diabetes, can often be identified in such
patients.
• Elderly patients with severely disordered motility may become symptomatic with chest
pain or dysphagia.

17. F. Non-specific Esophageal Motility Disorder:
This term is used to describe the remaining abnormalities of motility, such as
• Slow transit incomplete emptying or hypertensive LES pressure
• incomplete lower esophageal relaxation and
• loss of peristalsis
• Dysphagia, chest pain, reflux & regurgitation
• solitary abnormal contractions
• Decreased amplitude pressure <35 mmHg
• >20% nontransmitted prolonged waves of >60 secs with abnormal peristalsis

18. Achalasia Cardia:
• Achalasia means failure to relax
• Incidence 6/100000 person/yr
• Young women
 Primary- destruction of LES
 Secondary- degeneration of neuromuscular function of
body of esophagus
 Vigorous (or spastic/type III) achalasia is seen in a subset
of patients presenting with chest pain. In these patients,
the LES is hypertensive and fails to relax.
 Premalignant condition, 8% chance over 20yr period,
m/c sq cell carcinoma
 There is pencil-shaped narrowing of cardia with
enormous dilatation of proximal esophagus, which
contains foul smelling fluid

19. Etiology:
There is absence or less numbered ganglions in myenteric plexus(Auerbach’s),
due to-
• Stress, Vit B1 deficiency
• Chaga’s disease, caused by Trypanosoma cruzi
• Diffuse esophageal spasm
• m/c idiopathic, There is degeneration off Auerbach's plexus along entire length
of esophagus predominantly LOS

20. Symptoms:
• Triad of dysphagia, regurgitation & wt loss;
• post-prandial coughing & nocturnal coughing.
• Heart burn
• Dysphagia, first to liquids then to solids
• Regurgitation of undigested, foul-smelling food is common, and with progressive disease,
aspiration can become life-threatening.
-Walking while eating, chin trust, neck & shoulder extension, Valsalva maneuver facilitates
emptying of food from the esophagus.
-Pneumonia, lung abscess, and bronchiectasis often result from long-standing achalasia.

21. Stagging:
I. Proximal dilatation < 4cm
II. Dilation between 4-6 cm
III. Dilatation >6 cm
IV. Sigmoid dilatation (>10cm, tortous course & angulation)
Clinical Scoring:
0. No weight loss or dysphagia or retrosternal pain or regurgitation
1. Weight loss <5 kg; occasional dysphagia, retrosternal pain, regurgitation
2. Weight loss 5-10 kg, daily dysphagia, retrosternal pain, regurgitation
3. Weight loss >10 kg, dysphagia and regurgitation during each meal, retrosternal pain several times a day

22. Investigaions:
Barium swallow Esophagogram (diagnostic) – bird beak
appearance.
Dilatation of proximal esophagus (cucumber esophagus)
Lack of a gastric air bubble is a common finding on the upright portion
of the esophagram and is a result of the tight LES not allowing air to
pass easily into the stomach.
In the more advanced stage of disease, massive esophageal dilation,
tortuosity, and sigmoidal esophagus (megaesophagus) are seen

23. Chest Xray
• Mediastinal widening, with double
contour of mediastinal borders (outer
border represent dilated esophagus).
• Mediastinal air fluid level.
• Displacement of the azygo-esophageal
recess.
• Anterior bowing of the trachea.
• Absent gastric air bubbles.

24. Manometry(gold standard): manometry tracings show five classic
findings,
• Abnormalities of the LES
– Failure to relax with deglutition
– Hypertension >35 mm-Hg
• Abnormalities of the esophageal body
– Pressure above the baseline (pressurisation)
– Simultaneous mirrored contractions with no e/o
progressive peristalsis
– Low amplitude waveforms

25. Endoscopy: To evaluate the mucosa for evidence of esophagitis or cancer. It shows totally
Closed LES (rossete like) with atonic dilated proximal esophagus

26. THE TRANSIT TEST (Radionuclitide)
Scintigraphic tests, shown to be more sensitive than endoscopy radiography and manometry in the
identification of patients with motility problems.
Indications
patients with atypical chest pain (pain of cardiac type with normal ECG and enzymes)
patients with dysphagia but normal endoscopy/ barium studies
patients with suspected muscular or neuromuscular dysfunction

27. • It demonstrates oesophageal function by visualising the passage of a swallowed bolus into the
stomach.
• The labelled material may be prepared in either liquid or solid form – for example, orange
juice labelled with 99mTc-DTPA, or scrambled egg labelled with 99m Tc colloid.
• Typically, three consecutive swallows may be obtained in the supine position, and a further
three swallows in the sitting position. Between each swallow the oesophagus is rinsed with an
unlabeled drink in order to clear residual activity
• Transit through the upper third of the oesophagus usually takes about I s, through the middle
third about 2 s, and through the lower third about 6 s, giving a transit time through the whole
oesophagus of 8-10 s.

28. Qualitatively, several different patterns can be recognised:
Normal. The bolus traverses the oesophagus in a single wave of peristalsis in 8-10 s or so, with no delay,
no fragmentation of the bolus, and no reflux
Transfer dysphagia. Once initiated, transit shows a normal progression but there is delay in initiating
swallowing, and sometimes fragmentation of the bolus in the pharynx
Step-delay' pattern. The initial peristaltic wave dies out in the middle third of the oesophagus and the
bolus then remains stationary until it is stripped down the lower third by the next peristaltic wave.
Associated with reflux oesophagitis

29. Intraoesophageal reflux. The swallowed bolus proceeds normally to the lower third, then part or all of
it refluxes back to the middle third, before being cleared by further peristalsis
Associated with reflux oesophagitis
Incoordinate. After swallowing, the bolus is immediately fragmented by dystonic contractions, and no
peristaltic wave develops
Patients with diffuse oesophageal spasm, frequent tertiary contractions, or presbyoesophagus typically
show an incoordinate pattern with delayed transit
Adynamic. Swallowing is initiated normally, but peristalsis is weak or absent and bolus remains in the
middle third of the oesophagus if the patient is supine, or clears only very slowly from the lower
oesophagus if the patient is sitting
Patients with autonomic neuropathy associated with diabetes, and those with systemic sclerosis
involving the oesophagus, typically show delayed clearance with adynamic patterns

30. Condensed image of a
normal swallow.
Timescale (x axis) is 30 s,
y axis corresponds to the
length of the oesophagus
with the mouth at the top
and gastric fundus at the
bottom.
M = mouth; D =
distance; S = stomach.

31. Treatment of Achalasia:
1. Medical: Sublingual NTG, nitrates, CCB’s
2. Botulinum toxin (endoscopic injection to sphincter)
3. Forcible dilatation (Bougie/Plummer pneumotic/ Negus hydrostatic)
4. Surgery:
• Modified Heller’s cardiomyotomy with Toupet’s/Dor’s fundoplication
-Open abdominal/thoracic approach
-Laparoscopic approach
-Robotic assisted laparoscopic aproach
• Resection of OG junction/transhiatal total esophagectomy in severe
cases(megaesophagus/metaplasia)
5. Per-Oral Endoscopic Myotomy(POEM)

32. Pneumatic Dilatator
• 30-40mm diameter
• Risk if perforation (5%)
• 30mm diameter for 3mins
• High risk of perforation

35. Hellers Motomy:

36. POEM (Per Oral Endoscopy Myotomy):
It is a natural orifice approach to perform the myotomy. With use of an operating endoscope, the mucosa of
the esophagus is divided around the mid to distal third, and a submucosal tunnel is created after injection of
methylene blue diluted saline . Through this tunnel, the muscular layer of the distal esophagus, LES, and
cardia is visualized and divided, effectively performing an endoscopic myotomy.

37. #Thank you