ACHALASIA

ICD Case 2
MICHAEL JOHN VALLARIT
JUNIOR INTERN

CHIEF COMPLAINT
Progressive dysphagia to SOLID
foods

HISTORY OF PRESENT
ILLNESS
One year PTC:
Patient started having dull chest pain (5/10) which
seems to go up to her throat.
She was given proton pump inhibitor as maintenance
medication which provided slight relief.

2 weeks PTC:
She had an episode of vomiting of previously
ingested grapes without the characteristic gastric
juice (yellow fluid).

Recurrence prompted her consult hence, was

scheduled for barium swallow.

PHYSICAL EXAM
VS BP 120/80 HR 78 RR 18 T 36.7
Conscious, coherent, not in distress
Symmetrical chest expansion, clear
breath sounds
No palpitations, S1>S2 (apex),
S2>S1 (base), no heaves/lifts/thrills,
no murmurs
No abdominal tenderness

PAST MEDICAL HISTORY

1999 Ankylosing Spondylitis

BARIUM SWALLOW

RADIOGRAPH

RADIOGRAPHIC DIFFERENTIAL
DIAGNOSES
Idiopathic / Primary achalasia
Secondary / Pseudoachalasia
Esophageal Carcinoma

Esophageal mucosal ring

Peptic stricture of the esophagus
Diffuse esophageal spasm (DES)

Chen, M., et al. Basic Radiology 2nd edition. 2011.

Harrisons Principles of Internal Medicine 19 th edition. 2015.

Idiopathic / Primary
achalasia
Secondary /
Pseudoachalasia
Esophageal
Carcinoma
Esophageal mucosal ring
Peptic stricture of the
esophagus
Diffuse esophageal
spasm (DES)

SECONDARY
ACHALASIA /
PSEUDOACHALASIA
Squamous Cell
Carcinoma of the

SECONDARY ACHALASIA /
PSEUDOACHALASIA
Squamous Cell Carcinoma of the
esophagus
A
focal,
irregular
narrowing with abrupt
upper and lower margins,
which
rarely
mimics
peptic stricture.
Occurs in older patients,
who often have a history
of tobacco and alcohol
abuse
May also be multifocal
and
associated
with
similar lesions in the
upper aerodigestive tract.

Schatzki Ring
Lower Esophageal Mucosal Ring
An acquired thin, annular membrane of unknown
cause that demarcates the esophagogastric
junction.
A sign of hiatal hernia.
Symmetric narrowing at lower end of esophagus
Most common cause of solid dysphagia in adults
Best detected by radiographic examination, and
the use of a solid bolus, such as a portion of a
marshmallow, optimizes evaluation of these rings
Chen,
M., et al. Basic
and verifies the
structure
asRadiology
a cause of dysphagia
nd

Peptic
Stricture
of
Esophagu
s

Peptic Stricture of
Esophagus
Complication of reflux
esophagitis
Barrett Esophagus
Second most common
benign cause of
dysphagia
Usually at EG junction;
associated with hiatal
hernia

Diffuse Esophageal Spasm (DES)

Diffuse Esophageal Spasm

(DES)
Dysphagia and chest pain
Due to abnormal
esophageal contractions
with normal deglutitive
LES relaxation
Tertiary contractions or
Corkscrew esophagus,
Rosary esophagus,
Pseudodiverticula
Dx by Manometry
Harrisons Principles of Internal
Medicine 19th edition. 2015.

IDIOPATHIC /
PRIMARY
ACHALASIA
Esophageal
dilatation
Birds beak
appearance
Sigmoid deformity
Sx:
Dysphagia, Chest
pain,
Regurgitation,
Weight loss

RADIOGRAPHIC DIAGNOSIS

IDIOPATHIC / PRIMARY
ACHALASIA

DISCUSSION
What is/are the defining
features of achalasia?

Impaired LES relaxation

Aperistalsis of esophagus
EGJ malformation
Increased LES pressure
Robbin and Cotrans Pathologic basis
of Disease 8th edition

DISCUSSION

Triad of Achalasia?
Impaired LES relaxation
Aperistalsis of esophagus
EGJ malformation
Increased LES tone
Robbin and Cotrans Pathologic basis
of Disease 8th edition

PRIMARY ACHALASIA
Long of ganglion cells within the
esophageal myenteric plexus.
1: 100,000
Usually occurs in 25-60 years old
Long standing achalasia:
Progressive dilatation and sigmoid
deformity of the esophagus with
hypertrophy of the LES.
Harrisons Principles of Internal Medicine 19th

CLINICAL MANIFESTATIONS

Dysphagia
Regurgitation
Chest pain
Weight loss

Harrisons Principles of Internal Medicine 19th

FIGURE 45-1 Approach to the pt with dysphagia. Etiologies in bold

print are the most common. ENT, ear, nose, and throat; GERD,
gastroesophageal reflux disease.

Harrisons Principles of Internal Medicine 19th

DIAGNOSIS
Barium Swallow
Esophageal Manometry
Endoscopy to rule out
pseudoachalasia

Harrisons Principles of Internal Medicine 19th

DIAGNOSIS
BARIUM SWALLOW
Dilated esophagus
with poor emptying
An air-fluid level
Tapering at the LES
Bird Beak
Appearance
Harrisons Principles of Internal Medicine 19th

DIAGNOSIS
MANOMETRY
Total absence of primary esophageal
peristalsis
(aperistalsis),
and
a
dysfunctional
lower
esophageal
sphincter (ie, failure of relaxation).
Evaluation of deglutitive EGJ relaxation
is
probably
the
most
important
measurement made during clinical
esophageal manometry.
Harrisons Principles of Internal Medicine 19th

TREATMENT
GOAL:
Reduce LES pressure
Facilitate esophageal emptying

Treatment options:
Pharmacologic therapy
Pneumatic Balloon Dilatation
Surgical myotomy

Harrisons Principles of Internal Medicine 19th

TREATMENT
Pharmacologic Therapy
Nitrates and Calcium Channel Blockers
Botulinum toxin
Sildenafil and other PDE4 Inhibitors

Harrisons Principles of Internal Medicine 19th

TREATMENT
Pneumatic balloon dilatation
An endoscopic technique using a noncompliant,
cylindrical balloon dilator positioned across the
LES and inflated to a diameter of 3-4 cm.
Complication: Perforation

Myotomy
Laparoscopic Heller
Per esophageal Endoscopic myotomy
Creation of a tunnel within the esophageal wall;
which the circular muscle of the LES and distal
esophagus are transected
Harrisons Principles of Internal Medicine 19th

COMPLICATIONS
Esophageal dilatation
Stasis Esophagitis
Esophageal squamous cell carcinoma

Harrisons Principles of Internal Medicine 19th

Michael John Vallarit

THANK YOU!