ACHALASIA

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It has been a very busy few days. Moving around and preparing for my father's eye surgery. It was later cancelled and fixed on a later date due to logistics. Well, I'm back for now and we'll be discussing Achalasia.

Achalasia is defined as the lack of the lower esophageal sphincter to relax and the presence of abnormal motility in the remainder of the esophagus.

Etymology: A (absent) -chalasia (relaxation).

As a result, the esophagus gradually loses its ability to force food into the stomach and becomes paralyzed and dilated. The food then builds up in the esophagus, where it may ferment or wash back up into the mouth with a bitter flavor. This is sometimes misdiagnosed as gastroesophageal reflux disease (GERD). However, in GERD the food comes from the stomach, whereas in achalasia the food comes from the esophagus.

It is a rare disorder, anc most commonly occurs in middle-aged individuals.

There are two types of achalasia based on the cause.
Primary achalasia (most prevalent) has an unknown cause.
Secondary (or pseudoachalasia) achalasia is the presentation and manometric findings of a mechanical source of blockage (such as a tumor) that mimics achalasia.
Esophageal cancer, Stomach cancer and other extraesophageal cancers (symptoms may be due to mass effect or paraneoplasia), Chagas disease, Amyloidosis, Neurofibromatosis type I, Sarcoidosis.

Excitatory and inhibitory neurohumoral compounds, regulate swallowing.
Atrophy of inhibitory neurons in the Auerbach plexus → lack of inhibitory neurotransmitters (e.g., NO, VIP) → inability to relax and increased resting pressure of the LES, as well as dysfunctional peristalsis → esophageal dilation proximal to LES

While esophageal blockage solely causes dysphagia to solids, achalasia often also causes progressive dysphagia to liquids.Regurgitation, Retrosternal pain and cramps, Weight loss are also features that can be seen.

AcalasiaT2.jpg
Wikimedia commons

In general, upper endoscopy and/or esophageal barium swallow should be performed on all patients with suspected achalasia; results may support the diagnosis.
Regardless of the first imaging results, esophageal manometry is recommended to establish the diagnosis (confirmatory test of choice).
Esophagram can also serve as a confirmatory test if manometry is inconclusive and an esophageal barium swallow (which shows a Bird-beak sign) was not initially obtained.
Because the clinical and manometric results of a mechanical source of blockage (such as a tumor) can mimic achalasia, endoscopy should be done to rule out pseudoachalasia.

The best course of action frequently relies on the surgeon and the patient's circumstances. However, because it is less invasive and the recovery period is shorter, trying pneumatic dilation before myotomy (Heller myotomy and Peroral endoscopic myotomy) is becoming more common. In Europe, this strategy is already more common.

injection of botulinum toxin into the LES
a suitable option for people who are not strong candidates for surgery
Within six to twelve months, more than half of patients need further treatment.
In the event that other attempts fail: Calcium channel blockers or nitrates

Increased risk of esophageal cancer. However, endoscopic surveillance is not recommended.
Pulmonary complications (e.g., pneumonia, abscess, asthma) caused by aspiration, and Megaesophagus are some of the complications.

REFERENCE

  1. Mayo clinic
  2. Wiley online library
  3. The American Journal of Medicine


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