Achalasia is a motility disorder of esophagus and it has merit as it is the only motility disorder whose etiology is well understood and it responds to treatment. The term achalasia means failure to relax; it is characterized by high lower esophageal sphincter (LES) pressure and failure to relax in response to deglutition.
It is due to loss of ganglionic cells in Auerbach's plexus; the cause of loss of ganglion cells is presumed to presumed to be a neurogenic degeneration which is either idiopathic or due to infection (Varicella Zoster, Trypanosoma Cruzi, which causes Chagas disease). There is selective loss of inhibitory neurons. LES remains contracted and there is ineffective peristalsis in the body of esophagus which dilates progressively and subsequently loss of progressive peristalsis in the body of esophagus. With time this functional disorder results in anatomic alterations seen on radiograph, such as dilated esophagus with tapering of its lower end "bird's beak" appearance
Clinical features:
The disease is more common in middle age (25- 45 years). Patients usually present with
dysphagia, regurgitation is frequent which may over spill into the trachea leading to aspiration
Pneumonia.
Diagnosis:
i. Barium Swallow: "bird's beak" appearance is characteristic of Achalasia seen on barium swallow
ii. Manometry: Esophageal manometry shows hypertensive LES (lower esophageal swallowing sphincter), which does not relax and there may be no peristalsis in the body of esophagus.
iii. Endoscopy: Endoscopy is carried out to rule out stricture or malignancy and it usually shows food particles in the esophagus.
Treatment:
1. Endoscopic baloon dilatation: 85% Carative
A balloon is dilated at level of gastro-esophageal junction which splits the muscle
fibers while leaving the mucosa intact. Balloon of 30-40mm diameter can be passed over the guide wire. Forceful dilatation is curative in 75% to 85% of cases. Best results are obtained in patients of more than 45 years. More than one dilation may be needed.
2. Heller Myotomy: 95% curative.
Heller myotomy involves cutting the muscular fibres of the lower esophagus which
decreases the pressure. This myotomy can be performed by a laparoscope or by open surgery. And it produces excellent results in more than 95% of patients. The major complication of Heller's myotomy is GERD which can be avoided by performing partial Fundoplication.
3. Botulinum toxin: 60% Curative
Intra sphincteric injection of Botulinum toxin may cause relaxation of LES relieving dysphagia. It is usually effective in 60% of patients but the effect is not permanent and injection has to be repeated after a few months.
4. Medical treatment:
Drugs such as calcium channel blocker (Nifedipine) and Nitrates decrease LES tone and may be used for transient relief of symptoms, if definite treatment has to be postponed.
Pseudo achalasia:
It is achalasia like disorder but usually produces from adenocarcinoma of lower esophagus or
sometimes by cancer outside the esophagus like bronchogenic carcinoma (oat cell) cell) and
pancreatic cancers
.
DIFFUSE ESOPHAGEAL SPASM:
It is a condition in which there is uncoordinated contraction of esophagus and pressure of
esophagus may rise up to 400-500 mm Hg and there is corkscrew esophagus on barium swallow.
Treatment is "extended esophageal myotomy".
Nutcracker Esophagus:
It is a condition in which peristaltic pressure of greater than 180 mm Hg develops in the esophagus.
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