Presptor: Bambang AmAm S.S., dr.

ACHALASIA Sp.B-KBD

Huriyah Hafizhotul ‘Ummah

 Achalasia is primary a disorder of motility of the lower
esophageal or cardiac sphincter. The smooth muscle layer
of the oesophagus has impaired peristalsis and failure of
the sphincter to relax causes a functional stenosis or
functional oesophageal stricture.
The incidence of achalasia 1 in 100.000 persons/year,
men > women
PATHOGENESIS
Degenerative disease of the neurons: vagus nerve and ganglia in myenteric plexus of
esophagus, results in hypertension of the LES, a failure of a sphincter to relax on
swallowing, elevation of intraluminal esophageal pressure, esophageal dilatation,
and loss of peristaltis in the body of esophagus
Infections of the neurons by a virus (herpes zoster)
SIGNS AND SYMPTOMS
Dysphagia (95%)
Regurgitation of undigested food (60%)
Heartburn (40%) due to stasis and fermentation of undigested food
Chest pain (40%) due to esophageal distension, experienced at the time of a meal
 IMAGING STUDIES
Endoscopy
often reveals a dilated esophagus
that contains retained food; it may
also reveal inflammation, small ulcers
caused by residual food or pills, and
candida (yeast) infection

Barium swallows
 ESOPHAGEAL MANOMETRY
Gold standard for achalasia, measures motility and function of esophagus
The test involves the passage of a thin tube through the mouth or nose into the
esophagus. The tube is lined by numerous pressure sensors that convey pressures
within the esophagus to a device that records those pressures. Patients are usually
instructed to have nothing to eat or drink for eight hours before the test, and they are
given sips of water to swallow while the tube is in place.
MANOMETRIC FINDINGS
Absence of esophageal peristalsis
Hypertensive LES that relaxes only partially in response to swallowing
COMPLICATIONS
Aspiration can cause repeated episodes of pneumonia
Malnutrition
Breathlesness
Risk for esophageal squamous cell carcinoma due to continuous irritation of the
mucosa by retained and fermenting food
Weight loss
TREATMENT
Pharmacological: CCB (nifedipine), nitrates (isosorbide dinitrate)
CCB: decreasing calcium entry and reducing pressure in LES
Endoscopic treatment: Intrasphincteric injection of botulinum toxin is used to block
the release of acetylcholine at the level of the LES, thereby restoring the balance
between excitatory and inhibitory neurotransmitters. Causes local relaxation
Pneumatic dilation of the LES. A balloon is inflated at the level of the
gastroesophageal junction to rupture the muscle fibers while trying to leave the
mucosa intact. LES become relaxe and allow the food to pass
SURGICAL TREATMENT
Heller myotomy : open procedure or laparoscopic
Pneumatic dilatation
(The incidence of postoperative reflux is around 25%-35%, and it is usually controlled
by acid reducing medications)
Esophagectomy is reserved for patients with severe dysphagia who have failed both
dilation and myotomy.