Esophageal Motility

Disorders
 Anatomy
;dikk' Active muscular organ with a complex
neuromuscular structure and integration.

rR The sequential muscular contractions push

food from above and clear acid and bile
reflux from below.

;'1 Specialized sphincter at each end.

UES and LES

 Anatomy
UES contracts during inspiration preventing air
from entering into the GI tract, while the LES
maintains a steady baseline tone to prevent gastric
juice from refluxing into the esophagus.
n LES also contracts during periods of increased
intraabdominal pressure, preventing reflux due to
pressure in the abdomen.
r4 Inner circular layer, outer longitudinal layer of
muscle. (ring occlusions and shortens) =>
peristalsis.
 Peristalsis
v‘' Sequential, coordinated contraction wave that
travels along the whole length of the esophagus,
propelling intraluminal contents downstream.
Primary wave strips from proximal to distal,
triggered by swallowing center, 2cm/sec.
Secondary wave induced by distension of bolus,
acts to clear esophagus of retained food.
Tertiary contractions are dysfunctional and have
no role.
 Motility Disorders
rs Achalasia
lfi` Primary spastic motility disorders, including
DES, nutcracker esophagus, hypertensive
LES
Secondary esophageal motility
disorders
related to DM, scleroderma, alcohol,
psychiatric disorders, etc.
 Achalasia
Loss of ganglion cells from the wall of the
esophagus, starting at the LES and going
proximally.
Loss of inhibitory nerves at LES.
Circular muscle layer thickened at LES
but
microscopically cells appear normal,
 ▪

Achalasia
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 Achalasia
Loss of the inhibitory nerves at the LES
causes failure of the LES to completely
relax, and a hypertensive LES pressure over
40mmHg in 60% of patients.
rR Loss of nerves along the body of esophagus
causes aperistalsis, stasis, dilatation.
 Achalasia
'fit Non-peristaltic isolated contractions or low-
amplitude simultaneous contractions occur.
rR If high-amplitude (>60mmlig) simult
contractions occur it is called Vigorous
Achalasia.
 Achalasia
Edrophonium (acet cholesterase inhib)
increases LES pressure.
Atropine reduces the LES pressure in
achalasia, which is why botulinum toxin
can
be therapeutic (ach release inhibitor).
 Spastic Motility Disorders
Diffuse fragmentation of vagal filaments,
mitochondrial fragmentation results in
functional
imbalance between excitatory and inhibitory
pathways.
When DES occurs, diffuse muscular hypertrophy
as much as 2cm has been described in the distal
2/3 of the esophagus, but wall thickening is also
found in asymp patients, absent in patients with
typical sympt and manometric findings too.
 Diffuse Esophageal Spasm
--0-- -0- -0-

Diffuse esophageal spasm.

Scieroderma Esophagus
;1:: Primary defect here is related to smooth
muscle atrophy and fibrosis.

;1, The dysmotility here an absence of

peristalsis in the esoph body and an atonic
LES occur.

lg Motility is preserved at the striated muscle

part of the esophagus.
 Frequency
;1z Achalasia and DES only a small percentage
of disorders of motility.
aR Achalasia 1 case per 100,000 per year.
;z Familial clustering occurs but not genetic
yet.
Nutcracker esoph is most common motility
disorder, but the most controversial in
significance.
 Mortality and Morbidity
Achalasia associated with significant progressive
discomfort, severe dysphagia, malnutrition, weight
loss, dehydration. Increased incidence of SCC
with long standing disease.
Spastic motility disorders are associated with
sympt discomfort but not the severity of dysphagia
as in achalasia.
r4z• Scleroderma associated with severe acid reflux,
associated complications, including strictures,
Baffetts, adenocarcinoma.
 Race, Sex, and Age
Racial differences not
established.
Achalasia
rs Affectspresents in patients
both sexes equally.25-60 yrs,
although it can affect any age group.
 History
n'. Achalasia: progressive dysphagia for both solids
an liquids is a hallmark. Regurge of food in
dilated
esoph common especially at night. Chest pain,
sensation of heartburn (fermentation). Emotional
stress ordisorders:
Spastic rapid eating makes
Chest painit hallmark,
worse. mimics
angina, may be related to transient esoph
ischemia, distension. Dysphagia to solids and
liquids a common symptom, especially with DES,
intermittent, non-progressive. Heartburn, regurge.
 History
zz• Scleroderma: involves esoph in 75% of patients.
Two forms- PSS a progressive form that is more
fulminant, early involvement with internal organs;
CREST- calcinosis, Raynauds phenomena, esoph
dysfunction, sclerodactyly, telangiectasia.
;'r; Severe acid reflux, regorge, dysphagia, erosive
esophagitis (60%), increased incidence of cancer
Dysphagia from peptic strictures, poor peristalsis.
 Physical
;1z Results of a physical exam are usually
unrevealing.
Pay attention to signs of scleroderma in
proper clinical setting.
:rk A bedside swallowing challenge can be
performed with a glass of water.
4"; Check nutrition and hydration if dysphagia
reported.
 Causes
Primary disorders are idiopathic in nature.
Viral
Infectious
Environmental
Genetic
 Other Problems
rs Differential diagnosis depends on
presenting symptoms.
rR CAD, mechanical obstructing lesions,
benign or malignant should be ruled
out.
g Differential of achalasia includes Chagas
disease secondary to Trypanosoma cruzi
infection and pseudoachalasia from GE
junction tumors.
 Chagas Disease
Mimics achalasia.
Reduviid (kissing) bug bite.
Endemic in SA, A.
Septicemia first, then chronic stage ensues.
Widespread ganglion destruction throughout the
body involving heart, gut, GI tract, urinary tract,
respiratory tract.
Symptoms take years to develop.
Treatment: disrupt LES like in achalasia.
 Pseudoachalasia
;1:, Term used to describe clinical picture of GE
junction obstruction.
rR Present in 5% of patients with clinical and
manometric diagnosis of achalasia.
 Pseudoachalasia
v,z• Clinical presentation is more likely to occur with
rapidly progressive disease, older age of onset,
profound weight loss.
Workup includes upper endoscopy, biopsies
should be obtained with any suspicion of
malignant process. If suspicious lesion found,
image with CT, MRI, EATS if indicated.
In 50% of patients the diagnosis is
adenocarcinoma of GE junction.
 Diffuse Esophageal Spasm
1:4, DES and achalasia can be confused.
Manometric criteria require that normal
esophageal peristalsis be present
intermittently for DES.
LES relaxation, which is incomplete in
achalasia, should be normal in DES.
 Workup
vz• CXR: Dilated esophagus, looks sigmoid like, air
flied level, wide mediastinum, absence of gastric
air bubble.
Esophagram: In achalasia, dilated, A-F level,
tapered LES, bird's beak appearance.
Diverticula
above LES, hiatal hernia. In DES corkscrew or
rosary bead esophagus. In scleroderma, slightly
dilated esophagus, absent peristalsis, free reflux.
i
1
1
Classic "Bird's Beak"
of Achalasia

Distended Esophagus

Sphincter (LES)
 Manometry

rs Achalasia: aperistalsis of esophageal body

is manometric hallmark.
rR DES: Normal peristalsis, simultaneous
contractions in >30% water swallows,
incomplete LES relaxation, increased
LES
pressure (>40mmfig), or repetitive,
prolonged(>6sec), high-amplitude
contractions (>1 80mmHg).
 Manometry

;.1`. Nutcracker: Normal patterned peristalsis

with high amplitude
contractions(> 1 80mmHg),
repetitive
contractions, increased LES
pressure(>40mmfig).
ril` Hypertensive LES: Increased LES pressure
(>40mmlig). Significance of this is
questionable.
 Endoscopy
;dikk' Exclude mechanical and inflammatory
lesions that are causing dysmotility,
structural cause of obstruction.
Endoscopic US still investigational in
managing achalasia, used to assist in
botulinum toxin injection.
 Pharmacological Treatment
Smooth muscle relaxants including Ca
channel Mockers, nitrates. Also used,
anticholinergics, amyl nitrite, NTG,
theophylline„beta-2-agonists. Experience
with these are limited in comparison to the
first two.
 Pharmacological Treatment
vk Spastic Disorders: antireflux therapy, TCA,
trazodone.
Botulinum toxin injection: into the LES
used to treat pts with achalasia. A potent
inhibitor of ach release from nerve
terminals. May be a good alternative for
poor surgical candidates, disadvantage is
high cost and need for repeated injections.
 Endoscopic Therapy
rs EGD with pneumatic dilatation is the
standard for achalasia. Forceful
distension
of the LES to 3cm with disruption of the
circular muscle layer is needed for
symptomatic relief.is perforation (8%)
Complication
If pressure after is less than I °mmHg,
outcome excellent.
Pneumatic Dilation
Pneumatic Dilation
Pneumatic Dilation
Pneumatic Dilation

After balloon dilation

 Surgical Care
vz• Surgical treatments target the LES to relieve the
high pressure.
r4 Heller myotomy is procedure of choice for
achalasia. It decreases the LES pressure across
GE
junction and eliminates
Myotomy dysphagia.
may lead to GE reflux, so a
fundoplication may be necessary with the
myotomy.
Heller Myotomy
Fundoplication After Myotomy
 Surgical Care
;dikk' Heller myotomy is performed thru either
transthoracic or transabdominal approaches.
Efficacy is 60-100% in different series.
 Surgical Care
Esophagectomy with gastric pull up or intestinal
interposition for patients with advanced disease or
refractory cases, unresolved symptoms,
carcinoma, perforation during dilation.
LI Extended Heller Myotomy last resort for DES
when pain or dysphagia is severe.
r4 Operation is a myotomy that starts at the LES and
goes to thoracic inlet.