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aCHALASIA
ACHALASIA
14-Sep-24
PRESENTED
BY
MUTEGEKI ADOLF
Definition
•Achalasia is a rare esophageal motility disorder
characterized by the inability of the lower
esophageal sphincter (LES) to relax properly and
the absence of normal peristalsis in the
esophageal body, leading to difficulty in
swallowing (dysphagia), regurgitation, and
sometimes chest pain.
•Achalasia results from a malfunction of the nerves
that control the movement of food through the
esophagus.
•It makes it difficult to swallow food and liquid.
•It is derived from Greek term which means “ does not
relax”.
•It is primarily esophageal motility disorder associated
with the spasm of the lower esophageal sphincter due
to neuromuscular incoordination characterized by:
•A) Spasm of the cardiac end of the esophagus
•B) Dilation of the lower two thirds of the esophagus.
AETIOLOGY
•The exact cause of achalasia is unknown, but it is thought to result from the
degeneration of the myentericplexus (Auerbach'splexus) in the esophagus.
•This degeneration affects the nerve cells responsible for coordinating the
relaxation of the LES and peristalsis.
Possible contributing factors include:
•Autoimmune factors:The body's immune system may attack nerve cells in
the esophagus.
•Genetic predisposition:There may be a hereditary component in some
cases.
•Infections:A viral infection (such as herpes or measles) could potentially
trigger the autoimmune response.
•Chagasdisease:This infection by the parasite Trypanosomacruzican cause
achalasia-like symptoms due to damage to the esophageal nerves.
•The esophagus is a hollow muscular tube that is about 10
inches long.
•It has a two valve-like muscles, one at each end.These
muscles are called the upper and the lower esophageal
sphincters.
•These sphincters control the passage of food. In achalasia,
there is a problem with esophageal muscles and the
lower esophageal sphincter.
•Normally, after you swallow food, rhythmic contractionsof
the esophagus push food downward into the stomach.
How does esophagus work???
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•At he lower end of the esophagus, where it meets
the stomach, the lower esophageal sphincter is
normally closed to prevent stomach contents from
coming back up into the esophagus.
•Howevermwhen you swallow, the lower
esophageal sphincter opens to let the swallowed
food into the stomach.
•This pattern of swallowing is called peristalsis. It is
controlled by nerves in the wall of the esophagus.
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PATHOPHYSIOLOGY:
•The primary dysfunction in achalasia is the loss of
inhibitory ganglion cells in the myentericplexus.
•This leads to the failure of the LES to relax during
swallowing and the absence of coordinated
peristaltic contractions in the esophageal body.
•As a result, food and liquids cannot pass easily from
the esophagus into the stomach, causing the
esophagus to dilate and the LES to remain tonically
contracted.
CLINICAL PRESENTATIONS
•Dysphagia:Difficulty swallowing solids and liquids, often starting
with solids and progressing to liquids.
•Regurgitation:Backflow of undigested food, which may occur hours
after eating.
•Chest pain:Often due to esophageal spasms or distension, typically
intermittent and unrelated to exertion.
•Heartburn:Sensation similar to acid reflux but caused by fermenting
food in the esophagus rather than gastric acid.
•Weight loss:Due to difficulty eating and retaining food.
•Coughing or aspiration:Due to regurgitation of food into the airway.
•In the early stages of achalasia, the main symptoms are
trouble swallowing or chest pain.
•If achalasia is not treated, the food that collects in the
esophagus can cause complications.
•This material can sometimes spill into the lungs causing
bronchitis, pneumonia, or chronic lung disease.
•The retained food can also cause chronic irritation of the
esophageal lining, sometimes with fungal infections.
•Poor nutrition may lead to weight loss or malnutrition.
•There is also a small increased risk of esophageal cancer.
COMPLICATIONS OF ACHALASIA
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INVESTIGATIONS
•Esophageal manometry:The gold standard for diagnosing
achalasia; it measures the pressure within the esophagus and
the LES, showing lack of peristalsis and incomplete LES
relaxation.
•Barium swallow (esophagram):This X-ray study shows a
dilated esophagus with a narrow LES (bird-beak appearance).
•Endoscopy:Used to rule out other causes of dysphagia, such as
esophageal cancer or strictures, and to assess the esophageal
mucosa.
•CT scan or endoscopic ultrasound:May be used if there is a
suspicion of malignancy or other structural abnormalities.
ACHALASIA TREATMENT
•Currently, the nerve damage in the esophagus cannot be repaired.
Fortunately, there are several medical and surgical treatments that
improve swallowing function.
•However, esophageal function never completely returns to normal.
Following are the few techniques to be tried to improve the
swallowing function:
Eat slowly, drink plenty of liquids while you eat.
Chew food thoroughly.
Stay upright while you eat and for atleast1 hour after eating.
Drink a full glass of water with pills.
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Medical management:
•Nitrates and calcium channel blockers:Used to relax the LES but
are often not very effective.
•Botulinumtoxin (Botox) injections:Injected into the LES to
temporarily relax the muscle, offering short-term relief.
Endoscopic interventions:
•Pneumatic dilation:A balloon is inflated at the LES to disrupt the
muscle fibers, offering relief in many cases but may need to be repeated.
•Peroralendoscopic myotomy(POEM):A minimally invasive
endoscopic procedure where the muscle fibers of the LES are cut to
allow better passage of food.
Surgical management:
•Heller myotomy:A surgical procedure where the
muscles of the LES are cut to allow food to pass more
easily into the stomach, often combined with a partial
fundoplication to prevent reflux.
•Fundoplication, its where the upper portion of the
stomach (fundus) is wrapped around the lower end of
the esophagus.
•This procedure helps to reduce the risk of too much acid
reflux from the stomach.
•After surgical procedures, patients may need to take
medicine (usually proton pump inhibitor) to reduce the
secretion of stomach acid.
•In very advanced cases of achalasia, or if traditional
treatments fail, it may be necessary to remove the
diseased part of the esophagus.
NOTE:There are no known preventive measures for
primary achalasia due to its unclear etiology.
However, early diagnosis and treatment can help
manage symptoms and prevent complications such
as severe esophageal dilation, aspiration
pneumonia, or esophageal cancer in chronic cases.
•HerbellaFA, Colleoni R, Bot L, Vicentine FP, Patti MG. High-resolution manometry
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•Smits M, van LennepM, VrijlandtR, et al. Pediatric achalasia in the Netherlands:
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•FerriLE, Cools-LartigueJ, Cao J, et al. Clinical predictors of achalasia.Dis Esophagus. 2010
Jan. 23(1):76-81. [Medline].
•PandolfinoJE, GawronAJ. Achalasia: a systematic review.JAMA. 2015 May 12.
313(18):1841-52. [Medline].
•Inoue H, Minami H, Kobayashi Y, et al. Peroralendoscopic myotomy(POEM) for
esophageal achalasia.Endoscopy. 2010 Apr. 42(4):265-71. [Medline].
•FamiliariP, GiganteG, Marchese M, et al. Peroralendoscopic myotomyfor esophageal
achalasia: Outcomes of the first 100 patients with short-term follow-up.Ann Surg. 2016
Jan. 263(1):82-7. [Medline].
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REFERENCE