Achalasia Flashcards

1
Q

What is achalasia?

A

Achalasia is a rare neurodegenerative disorder characterized by the inability of the lower esophageal sphincter (LES) to relax and an aperistaltic esophagus.

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2
Q

What are the potential contributing factors to the development of achalasia?

A

Genetic predisposition
environmental factors
and autoimmune destruction of the myenteric plexus, possibly triggered by a viral infection.

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3
Q

What are common symptoms of achalasia?

A

Regurgitation of food, retrosternal chest pain, and dysphagia

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4
Q

What symptoms might manifest in advanced cases of achalasia?

A

Recurrent aspiration pneumonia, weight loss, and failure to thrive

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5
Q

How can the effectiveness of treatment for achalasia be assessed?

A

By using the Eckardt scoring system to monitor symptom improvement

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6
Q

What classic appearance might a barium swallow reveal in achalasia?

A

The classic “bird’s beak” appearance

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7
Q

Why is endoscopy recommended in the evaluation of achalasia?

A

To rule out mechanical obstruction such as malignancy, benign stricture, or pseudoachalasia

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8
Q

What is the main diagnostic tool for achalasia?

A

High-resolution manometry (HRM)

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9
Q

What might be seen on endoscopy in cases of long-standing achalasia?

A

A markedly dilated esophagus and retained food within the esophageal lumen

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10
Q

What characterizes type I achalasia on high-resolution manometry (HRM)?

A

No esophageal contraction or pressurization, and an integrated relaxation pressure (IRP) greater than 15 mm Hg.

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11
Q

What characterizes type II achalasia on HRM?

A

Panesophageal pressurization and no peristaltic contraction.

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12
Q

What characterizes type III achalasia on HRM?

A

At least 20% premature (spastic) contractions.

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13
Q

What treatment may be useful for frail elderly patients or those with contraindications to general anesthesia?

A

Intrasphincteric injection of botulinum toxin

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14
Q

How is botulinum toxin administered for achalasia?

A

It is injected endoscopically into four quadrants of the lower esophageal sphincter (LES), with 20–25 units per quadrant (80–100 units total).

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15
Q

What is the mechanism of action of botulinum toxin in treating achalasia?

A

It irreversibly inhibits acetylcholine release from presynaptic terminals, reducing the neurogenic component of LES pressure.

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16
Q

How long does symptom relief from botulinum toxin injections typically last?

A

Up to 1 year, but symptoms often recur due to new axonal growth

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17
Q

What are the limitations of repeat botulinum toxin injections?

A

Local inflammatory reactions, fibrosis, and side effects such as chest discomfort and rash

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18
Q

What pharmacologic therapies have been used for patients unfit for general anesthesia?

A

Smooth muscle relaxants like nitrates and calcium channel blockers, such as nifedipine and sildenafil

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19
Q

What are the side effects of nitrates and nifedipine in treating achalasia?

A

Nitrates can cause headaches, while nifedipine can cause headaches, orthostasis, dizziness, and has limited efficacy

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20
Q

What is the most effective nonsurgical option for achalasia?

A

Endoscopic dilation.

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21
Q

What is the principle behind endoscopic dilation for achalasia?

A

Disruption of the circular muscle fibers of the lower esophageal sphincter (LES) through dilation.

22
Q

Which type of dilator is more effective for achalasia, pneumatic or rigid?

A

Pneumatic dilators are more effective at disrupting the muscle fibers

23
Q

What is a significant risk during endoscopic dilation for achalasia?

A

Aspiration, especially due to retained food within the esophageal lumen

24
Q

What is recommended before performing endoscopic dilation in achalasia patients?

A

Implementing a clear liquid diet for more than 1 day.

25
Q

What tools are used to guide balloon inflation during endoscopic dilation?

A

Gastroscopy and fluoroscopy

26
Q

What complication is most worrisome during pneumatic dilation?

A

Esophageal perforation, with an incidence of 1%.

27
Q

What are the signs of esophageal perforation after pneumatic dilation?

A

Fever, chest pain, and subcutaneous emphysema

28
Q

What is the initial management for small esophageal perforations after pneumatic dilation?

A

Conservative measures such as NPO, intravenous antibiotics and antifungals, and inpatient observation.

29
Q

When is surgical therapy recommended for esophageal perforation after pneumatic dilation?

A

If there is worsening fever, chest pain, or an uncontrolled leak, within 6–8 hours

30
Q

Does the number of previous dilations affect the future response to surgical myotomy?

A

No

31
Q

What is the most durable surgical intervention for long-term control of achalasia?

A

Laparoscopic Heller Myotomy (LHM).

32
Q

What is the initial layer of the esophageal muscle to be targeted during myotomy?

A

The outer, longitudinal layer.

33
Q

How far is the myotomy extended cranially and onto the stomach?

A

4 to 6 cm cranially into the mediastinum and 2 to 3 cm onto the stomach

34
Q

What is done at the end of the myotomy before fundoplication?

A

The mucosa is visualized endoscopically, and an insufflation test is performed under saline

35
Q

What test is performed on postoperative day 1 to evaluate for an esophageal leak? post heller

A

A barium swallow.

36
Q

What is the dietary recommendation if the barium swallow is negative for an esophageal leak?

A

The patient is discharged on clear liquids for 3 days, followed by full liquids for another 3 days.
then soft diet till his follow up

37
Q

What are the four main stages of the POEM procedure?

A
  1. Mucosotomy creation 10–15 cm above the GEJ.
  2. Dissection of submucosal fibers.
  3. Tunnel creation using methylene blue dye for muscle fiber delineation.
  4. Myotomy of circular and longitudinal muscle fibers.
38
Q

What complications are associated with POEM?

A

inadvertent mucosal disruption
pneumothorax
pneumomediastinum
pneumoperitoneum
submucosal hematomas
esophageal leaks

39
Q

Why is gastroesophageal reflux (GER) a significant post-procedure issue in POEM patients?

A

GER is common post-POEM because no anti-reflux procedure is performed alongside POEM, with about one-third of patients showing evidence of GER on endoscopy

40
Q

The postoperative course after POEM

A

-A barium esophagram on postoperative day 1

-patient may be discharged on clear liquids and proton pump inhibitor (PPI)

-Ambulatory pH testing and EGD performed between 3 and 6 months to evaluate for gastroesophageal reflux (GER).

41
Q

(SAGES) recommended for LHM vs POEM

A

-either POEM or LHM For type I or type II achalasia in adults and children

-POEM over LHM in adults and children with type III achalasia

42
Q

Proposed treatment algorithm for achalasia

A

see Pic

43
Q

What is dysphagia in the postoperative course of achalasia treatment likely an indication of?

A

indication of treatment failure.

44
Q

What diagnostic tools are used to evaluate dysphagia post-POEM or post-LHM?

A

Endoscopy
fluoroscopy with timed barium swallow
high-resolution impedance manometry,
and functional lumen imaging probe (FLIP) if available

45
Q

What treatments may be considered if postoperative dysphagia is diagnosed?

A

treatments may include pneumatic dilation or repeat myotomy.

46
Q

How does POEM serve as an option for patients with failed laparoscopic myotomy?

A

POEM allows for creating a dissection plane endoscopically, which is remote from the previously created laparoscopic dissection plane

47
Q

What surgical option may be considered for patients with advanced esophageal dysfunction and a dilated esophagus after failed treatments?

A

Esophagectomy may be warranted if the patient is medically fit

48
Q

What are the potential challenges during esophagectomy in patients with end-stage achalasia?

A

-scarring between the esophageal submucosa and the aorta
-large esophageal arterial branches
-esophageal deviation into the right chest

49
Q

What is the preferred surgical approach for esophagectomy in patients with end-stage achalasia?

A

The transhiatal approach is preferred, starting thoracoscopically to mobilize the esophagus and control the large arterial branches.

50
Q

When is it reasonable to perform surveillance upper endoscopy in achalasia patients after the onset of symptoms?

A

Surveillance upper endoscopy may be considered 15 years after the onset of symptoms.