Esophageal motility
disorders (achalasia)
Dr/Mohammed Abdelaty
Oesophageal Anatomy and Physiology
The oesophagus is along muscular tube about 25cm long and ½ inch diameter which start
at level of C6 at crico-pharyngeal sphincter to cardia at level of T11, and divided into
3parts: Cervical: 5cm long Thoracic: 15cm long Abdominal: 5cm
long
The upper esophageal sphincter: is comprised of skeletal muscle and prevents air from
entering the alimentary canal, its formed of cricopharyngeus muscle.
The lower esophageal sphincter (LOS): is composed of smooth muscle and prevents
reflux from the stomach, its physiological rather than anatomical sphincter.
Normal (LESP):15-40 mmHg
Peristaltic waves, controlled by the esophageal myenteric neurones, propel ingested food
down the esophagus. The primary wave is under control of the swallowing center and
the secondary wave is activated in response to distention.
As food descends the esophagus, the lower esophageal sphincter relaxes and allows food
to pass.
Esophageal motility disorders:
Definition: It is a functional disorder of esophagus interferes with the normal
act of swallowing and produce dysphagia.
Classification of:
Primary motility disorders :
Achalasia.
Diffuse esophageal spasm.
Nut cracker esophagus.
Hypertensive LES.
Secondary motility disorders :
Systemic diseases: multiple sclerosis, scleroderma.
Chagas’ Disease
Diffuse esophageal spasm:
Motility disorder causes chest pain and dysphagia ,etiology is unknown, may be due to
emotional disturbance, 50% of cases of DES have associated irritable bowel syndrome, so it is
termed irritable esophagus
Manometric study : (most diagnostic )
Esophageal body contraction: high amplitude spontaneous prolonged
simultaneous contraction (alternate with normal peristalsis).
LES: normal relaxing in response to swallowing and LESP is normal.
Barium swallow :
Typical corkscrew appearance
Treatment:
Avoid emotional stress, antispasmodic: nitrates, calcium channel blocker and diet: small soft
frequent meals.
Long esophegomytomy: extend from level of aortic arch to G/E junction
Nutcracker esophagus:
Causes: unknown
Clinical: chest pain and dysphagia.
Investigations:
Manometry: esophageal body contraction: high amplitude peristaltic contraction > 140 mm
Hg with increased duration >5.5 second.
Treatment: as diffuse esophageal spasm.
Hypertensive LES:
Definition: It is one of esophageal motility disorders characterized by normal esophageal
body contraction, normal relaxation in response to swallowing and high LESP > 45 mm Hg
resulting in dysphagia and chest pain.
Treatment: medical and dilatation as before and myotomy is rarely needed.
Esophageal scleroderma:
As in most autoimmune disorders, the body attacks itself, causing the scarring and
thickening of the esophagus tissues. This causes abnormal functioning of the smooth
muscle of the esophagus, inhibiting its ability to move food toward the stomach,
presented with GERD and its complications.
Chagas’ Disease:
Etiology: Infestation with Trypanosoma cruzi
Pathology: The parasite invades muscles and nervous system with destruction of the
myenteric plexus of esophagus → a condition which simulates achalasia.
Treatment: Dilatation is the preferred method of treatment for patients with esophageal
symptoms due to poor general condition of patients with this chronic infestation
Definition of achalasia:
It is a functional disorder of esophagus characterized by:
a. Absent or weak peristalsis of the body of esophagus.
b. Incomplete or delayed relaxation of LES in response to swallowing.
Incidence:
It may occur at any age, but is most common in middle-aged or older adults
Etiology:
-Degeneration of Auerbach’s plexus ganglia, may be due to:
1. Primary: idiopathic.
2. Secondary:
- Neurogenic disease.
- Infestation with Trypanosoma cruzi (Chagas disease).
- Autoimmune
Pathology: (At – Above – Below)
The cardiac end (At) :
- Spasm of cardia with subsequent fibrosis→ organic stricture.
- Auerbach‘s plexus is deficient.
Esophagus (above) (2 stages) :
(1) Stage of hyper-peristalsis & hypertrophy.
(2) Stage of dilatation:
• The early stage → funnel shaped
• With chronicity→ club shaped
• Last → sigmoid shaped
Stomach (below) :
Collapsed with absent air bubbles (as the air cannot pass through the fluid)
CLINICAL PICTURE: ( 20 - 40 years & neurotic female )
1-Dysphagia :
• Painless, slowly progressive.
• More to fluid than solid
• At 1st intermittent, then constant.
2-Regurgitation :
• Alkaline fluid.
• Foul smell fluid & halitosis
• More Nocturnal.
3Retro-sternal discomfort from retention oesophagitis.
Complications :
1-Aspiration pneumonia.
2-Oesophagitis & ulceration hematemesis.
3-Malignant transformation ( 5 % ) from chronic mucosal irritation.
DD
Achalasia Carcinoma
Age Middle (20 - 40) Old ( > 50 )
Sex More with female More with male
Emotion Aggravates dysphagia. No effect.
Dysphagia More to fluids. More to solids.
Pain May be No or late
General condition Good Very bad
Investigations:
(1) X-ray:
1- Fluid level.
2- Widening of mediastinum due to wide oesophagus.
3- Absence of gastric air bubble.
(2)Esophagoscopy:
1- Dilatation of proximal segment and detection of fluid level .
2- The scope passes the region of distal sphincter with difficulty
3- Exclude the presence of malignancy
(3) Manometry (main investigation):
1- Weak or abscent peristalsis (hypoperistaltic or even apristaltic)
2- Increased LES pressure (in some patients) >40 mmHg
3- Failure of LES to relax in response to swallowing
(4) Barium swallow:
1-Delayed transit of the contrast.
2-Dilatation of the esophagus.
3-Air fluid level in the body of the esophagus.
4-Stenotic area which is short, distal with
central lumen and smooth mucosa.
5- Loss of gas bubble in the fundus of stomach.
6-A smooth pencil-shaped narrowing of lower
segment: giving Parrot’s or Hen‘s beak appearance.
Treatment:
1-Drugs:to induce muscle relaxation e.g nitrates calcium channel blockers
2-Endoscopic injection of botulinum toxin (Botox) into LES :inhibits
release of acetylcholine from presynaptic nerve terminals.
3-Forcible Dilatation by: hydrostatic or pneumatic dilators
- Through esophagoscope → narrow neck is dilated → the bag is inserted &
distended to a diameter of 5 cm for 5min → rupture circular muscle fibres of
the neck
- Dilatation may be complicated by hemorrhage and perforation.
Intersphincteric Injection of
Botulinum Toxin
Pneumatic Dilatation
Surgical treatment:
* Indications:
1. Failure of dilatation or recurrence of symptoms after initial relieve
2. Development of complications.
3. Associated pathology requiring surgical intervention as hiatus hernia.
* Procedure:
- Heller’s esophago-cardiomyotomy:
- Single anteriorly placed longitudinal incision through the longitudinal & circular muscle
fibers of esophagus until mucosa bulges out.
- The incision extends from 1 cm below esophago- gastric junction to 2 cm above
thickened muscle in the esophagus, thus making an incision 8-10 cm long.
- Complications: GERD → to avoid:
1- Incision should not exceed 1cm in stomach
2- Fundoplication
THANKS
