Podcast
Questions and Answers
Which of the following accurately describes the pathophysiology of achalasia?
Which of the following accurately describes the pathophysiology of achalasia?
- Absence of esophageal peristalsis, increased LES pressure, and impaired LES relaxation (correct)
- Increased lower esophageal sphincter (LES) tone due to excessive cholinergic stimulation.
- Decreased LES pressure secondary to smooth muscle atrophy.
- Relaxation of the LES due to increased levels of nitric oxide.
A 25-year-old patient is diagnosed with achalasia. What is the most likely underlying cause of this condition?
A 25-year-old patient is diagnosed with achalasia. What is the most likely underlying cause of this condition?
- Degeneration of ganglion cells in the myenteric plexus. (correct)
- Chronic acid reflux leading to esophageal stricture.
- Long-term use of NSAIDs.
- Infection with H. pylori.
A patient with achalasia experiences regurgitation of undigested food. What percentage of achalasia patients typically experience this symptom?
A patient with achalasia experiences regurgitation of undigested food. What percentage of achalasia patients typically experience this symptom?
- 40%
- 60% (correct)
- 20%
- 80%
What is the classic finding on manometry in a patient with achalasia?
What is the classic finding on manometry in a patient with achalasia?
Match each motility disorder with its anatomical location in the esophagus:
- Cricopharyngeal Spasm a. LES
- Nutcracker Esophagus b. UES
- Achalasia c. Body
Match each motility disorder with its anatomical location in the esophagus:
- Cricopharyngeal Spasm a. LES
- Nutcracker Esophagus b. UES
- Achalasia c. Body
Which of the following is a potential complication of achalasia?
Which of the following is a potential complication of achalasia?
A 65-year-old patient presents with recent onset dysphagia and significant weight loss. What condition should be suspected to differentiate it from achalasia?
A 65-year-old patient presents with recent onset dysphagia and significant weight loss. What condition should be suspected to differentiate it from achalasia?
Which of the following is considered the procedure of choice for esophageal achalasia?
Which of the following is considered the procedure of choice for esophageal achalasia?
A patient with achalasia undergoes pneumatic dilation. What is the approximate incidence of post-dilation reflux in these patients?
A patient with achalasia undergoes pneumatic dilation. What is the approximate incidence of post-dilation reflux in these patients?
Which of the following best describes the primary goal of treatment for achalasia?
Which of the following best describes the primary goal of treatment for achalasia?
What is the typical length of the myotomy during a Heller myotomy procedure for achalasia, involving both the lower esophagus and proximal stomach?
What is the typical length of the myotomy during a Heller myotomy procedure for achalasia, involving both the lower esophagus and proximal stomach?
What is the main purpose of performing a partial fundoplication in conjunction with a Heller myotomy for achalasia?
What is the main purpose of performing a partial fundoplication in conjunction with a Heller myotomy for achalasia?
For which patient population are calcium-channel blockers primarily considered in the management of achalasia?
For which patient population are calcium-channel blockers primarily considered in the management of achalasia?
What differentiates secondary achalasia from primary achalasia?
What differentiates secondary achalasia from primary achalasia?
What is the mechanism of action of botulinum toxin when used in the treatment of achalasia?
What is the mechanism of action of botulinum toxin when used in the treatment of achalasia?
Which diagnostic finding is characteristically observed on a barium swallow study in achalasia?
Which diagnostic finding is characteristically observed on a barium swallow study in achalasia?
Compared to healthy individuals, what is the effect of unopposed cholinergic stimulation on LES resting pressure in patients with achalasia?
Compared to healthy individuals, what is the effect of unopposed cholinergic stimulation on LES resting pressure in patients with achalasia?
What is the incidence of achalasia, as described in the text?
What is the incidence of achalasia, as described in the text?
A patient with achalasia is at increased risk of developing which type of esophageal cancer due to chronic irritation?
A patient with achalasia is at increased risk of developing which type of esophageal cancer due to chronic irritation?
A patient is diagnosed with hypertensive Lower Esophageal Sphincter (LES). Which conditions could be associated?
A patient is diagnosed with hypertensive Lower Esophageal Sphincter (LES). Which conditions could be associated?
Which condition is NOT regarded as a primary motility disorder of the oesophagus?
Which condition is NOT regarded as a primary motility disorder of the oesophagus?
Which of the following secondary motility is associated with Chagas' disease?
Which of the following secondary motility is associated with Chagas' disease?
Why does food stagnate in the oesophagus in patients with achalasia?
Why does food stagnate in the oesophagus in patients with achalasia?
What symptom besides regurgitation and dysphagia, do some achalasia patients experience?
What symptom besides regurgitation and dysphagia, do some achalasia patients experience?
When should secondary achalasia be highly suspected?
When should secondary achalasia be highly suspected?
Why is an endoscopy performed when diagnosing achalasia?
Why is an endoscopy performed when diagnosing achalasia?
What causes heartburn (40%) in Achalasia patients?
What causes heartburn (40%) in Achalasia patients?
What advice should an achalasia patient be given to ease the passage of food to the stomach?
What advice should an achalasia patient be given to ease the passage of food to the stomach?
The surgery success rate (SR) for Heller's Myotomy is 90%. What does success mean here?
The surgery success rate (SR) for Heller's Myotomy is 90%. What does success mean here?
Which pathophysiological mechanism causes the hypertensive LES observed in achalasia?
Which pathophysiological mechanism causes the hypertensive LES observed in achalasia?
What explains the palliative nature of achalasia treatments?
What explains the palliative nature of achalasia treatments?
What is the rationale behind adding a partial fundoplication to the Heller myotomy procedure for achalasia?
What is the rationale behind adding a partial fundoplication to the Heller myotomy procedure for achalasia?
Why do patients with achalasia develop pulmonary complications such as aspiration pneumonia?
Why do patients with achalasia develop pulmonary complications such as aspiration pneumonia?
Which aspect of esophageal physiology is least likely to be restored by current achalasia treatments?
Which aspect of esophageal physiology is least likely to be restored by current achalasia treatments?
In older adults presenting with dysphagia and significant unintentional weight loss, which of the following is the most important reason to rule out pseudoachalasia?
In older adults presenting with dysphagia and significant unintentional weight loss, which of the following is the most important reason to rule out pseudoachalasia?
What role might a viral infection play in the pathogenesis of achalasia?
What role might a viral infection play in the pathogenesis of achalasia?
A patient with achalasia is being considered for pneumatic dilation. What factor would be most concerning about this approach?
A patient with achalasia is being considered for pneumatic dilation. What factor would be most concerning about this approach?
What is the primary risk associated with longstanding achalasia?
What is the primary risk associated with longstanding achalasia?
Which of the following best reflects the mechanism of action of botulinum toxin in treating achalasia?
Which of the following best reflects the mechanism of action of botulinum toxin in treating achalasia?
What are the components determining the effectiveness of gravity in aiding esophageal emptying following treatment for achalasia?
What are the components determining the effectiveness of gravity in aiding esophageal emptying following treatment for achalasia?
In a patient with achalasia who develops gastroesophageal reflux after pneumatic dilation or myotomy, there is an increased risk to which type of esophageal cancer as a result?
In a patient with achalasia who develops gastroesophageal reflux after pneumatic dilation or myotomy, there is an increased risk to which type of esophageal cancer as a result?
Which clinical scenario should most strongly raise suspicion for secondary achalasia?
Which clinical scenario should most strongly raise suspicion for secondary achalasia?
If a patient that has achalasia also has heartburn (40%), what is the most likely cause?
If a patient that has achalasia also has heartburn (40%), what is the most likely cause?
What is the importance of performing an endoscopy in patients diagnosed with achalasia?
What is the importance of performing an endoscopy in patients diagnosed with achalasia?
What is the relationship between the Auerbach's plexus and achalasia?
What is the relationship between the Auerbach's plexus and achalasia?
Which patient population would benefit from calcium-channel blockers in the management of achalasia?
Which patient population would benefit from calcium-channel blockers in the management of achalasia?
How does Chagas' disease cause secondary achalasia?
How does Chagas' disease cause secondary achalasia?
What does the term SR= 90% mean in this scenario?
What does the term SR= 90% mean in this scenario?
Why do patients with achalasia often experience dysphagia for both solids and liquids?
Why do patients with achalasia often experience dysphagia for both solids and liquids?
Which manometric finding, while characteristic of achalasia, is not universally present in all patients with the condition?
Which manometric finding, while characteristic of achalasia, is not universally present in all patients with the condition?
Why is aspiration pneumonia a complication of achalasia?
Why is aspiration pneumonia a complication of achalasia?
What is the most common way to treat Achalasia?
What is the most common way to treat Achalasia?
What is the role of esophageal peristalsis?
What is the role of esophageal peristalsis?
Which is the most common symptom of Achalasia?
Which is the most common symptom of Achalasia?
What is the main way in which an endoscopy helps patients?
What is the main way in which an endoscopy helps patients?
What clinical presentation needs to be carefully explored to avoid a dangerous misdiagnosis?
What clinical presentation needs to be carefully explored to avoid a dangerous misdiagnosis?
Which test would be the first carried out when a patient has difficulty swallowing?
Which test would be the first carried out when a patient has difficulty swallowing?
What percentage of patients suffer reflux after a Heller myotomy when treating achalasia?
What percentage of patients suffer reflux after a Heller myotomy when treating achalasia?
Flashcards
Achalasia Definition
Achalasia Definition
Esophageal motility disorder characterized by absence of esophageal peristalsis and increased LES pressure.
Primary UES motility disorders
Primary UES motility disorders
Cricopharyngeal spasm, Zenker's diverticulum, Plummer Vinson syndrome.
Primary Body motility disorders
Primary Body motility disorders
Nutcracker esophagus, Diffuse esophageal spasm, Non-specific motility disorders
Primary LES motility disorders
Primary LES motility disorders
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Secondary causes of motility disorders
Secondary causes of motility disorders
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Achalasia Incidence
Achalasia Incidence
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Achalasia Pathogenesis
Achalasia Pathogenesis
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Achalasia Complications
Achalasia Complications
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Dysphagia in Achalasia
Dysphagia in Achalasia
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Regurgitation in Achalasia
Regurgitation in Achalasia
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Heartburn in Achalasia
Heartburn in Achalasia
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Achalasia symptom complications
Achalasia symptom complications
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Barium swallow finding
Barium swallow finding
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Manometric findings of achalasia
Manometric findings of achalasia
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Achalasia Diagnosis
Achalasia Diagnosis
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Differential diagnosis of Achalasia
Differential diagnosis of Achalasia
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Achalasia Treatment
Achalasia Treatment
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Target of Achalasia treatment
Target of Achalasia treatment
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Surgical treatment of achalasia
Surgical treatment of achalasia
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Details of Heller myotomy
Details of Heller myotomy
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Non-surgical treatment of achalasia
Non-surgical treatment of achalasia
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Pneumatic dilatation benefits
Pneumatic dilatation benefits
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Function of botulinum toxin
Function of botulinum toxin
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When to use calcium-channel blockers
When to use calcium-channel blockers
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Chest pain in Achalasia
Chest pain in Achalasia
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Purpose of endoscopy
Purpose of endoscopy
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Pseudoachalasia
Pseudoachalasia
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Role of gravity in Achalasia
Role of gravity in Achalasia
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Incidence of postoperative reflux following Heller
Incidence of postoperative reflux following Heller
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Next step after failed pneumatic dilatation
Next step after failed pneumatic dilatation
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Dysphagia causes
Dysphagia causes
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Never take dysphagia in elderly lightly
Never take dysphagia in elderly lightly
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Investigation is mandatory for suspected diagnosis
Investigation is mandatory for suspected diagnosis
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Study Notes
- Achalasia is an esophageal motility disorder.
- This disorder is characterized by the absence of esophageal peristalsis and increased pressure of the lower esophageal sphincter (LES).
- The LES fails to relax completely in response to swallowing.
Classification of Motility Disorders
- Primary motility disorders include cricopharyngeal spasm, Zenker's diverticulum, Plummer Vinson syndrome (UES), Nutcracker esophagus, diffuse esophageal spasm, non-specific motility disorders (Body), Achalasia, hypertensive LES, and hypotensive LES (LES).
- Secondary motility disorders include cerebrovascular disorders, diabetes mellitus (DM), scleroderma, other collagen diseases, myasthenia gravis, presbyoesophagus, and Chagas' disease.
Incidence
- Achalasia is rare, with an incidence of 1 to 2 per 200,000.
- It commonly affects young adults in their 20s and 30s.
Pathogenesis
- The exact cause is unknown.
- Degeneration of the ganglion cells of the myenteric plexus of Auerbach is the mainstay.
- Two theories: degenerative disease of neurons and infections of neurons by a virus (e.g., herpes zoster) or another infectious agent (Chagas' disease).
- Selective degeneration of postganglionic inhibitory neurons mediate LES relaxation.
- Unopposed cholinergic stimulation, as postganglionic cholinergic neurons are spared, which increases LES resting pressure and decreases LES relaxation.
- There is no propagation of peristaltic waves in response to swallowing; instead, simultaneous contractions occur.
Complications
- Esophagitis.
- Aspiration of retained and undigested food leading to pneumonia.
- Epiphrenic diverticulum may occur.
- Esophageal cancer, specifically squamous cell carcinoma (due to continuous irritation) and adenocarcinoma (in patients who develop gastroesophageal reflux after pneumatic dilatation or myotomy).
Clinical Presentation
- Dysphagia, the most common symptom, occurs for both solids and liquids.
- Regurgitation of undigested food occurs in 60% of cases.
- Heartburn occurs in 40% of cases, due to stasis and fermentation of undigested food in the distal esophagus.
- Chest pain (40%) is usually experienced at meal times.
- Symptoms of complications include aspiration pneumonia and malignant dysphagia.
Investigations
- Barium swallow is the first test performed, showing smooth narrowing at the gastroesophageal junction (GEJ), known as the "Parrot beak."
- The esophagus may appear dilated or sigmoid in long-standing achalasia.
- Manometry findings include absence of esophageal peristalsis and hypertensive LES (in about 50% of patients) that relaxes only partially in response to swallowing.
- Endoscopy is performed to rule out a tumor of the gastroesophageal junction.
Differential Diagnosis
- Benign strictures due to gastroesophageal reflux.
- Esophageal carcinoma.
- Endoscopy, endoscopic ultrasound (US), or computed tomography (CT) scans are useful for diagnosis.
- Infiltrating tumors of the GE junction can mimic the clinical, radiological, and manometric profile of achalasia and are suspected in patients older than 60 with recent dysphagia onset and excessive weight loss.
Treatment
- Therapy is palliative, aimed at relieving symptoms by decreasing outflow resistance caused by the dysfunctional LES.
- Peristalsis is absent and does not return after any form of treatment; gravity aids in emptying the esophagus into the stomach.
Surgical Treatment
- Laparoscopic Heller myotomy ± partial fundoplication is the procedure of choice.
- Myotomy involves cutting the lower esophagus (6 cm) and proximal stomach (2 cm), followed by anterior or posterior partial fundoplication to prevent reflux.
- Success rate (SR) is 90% of patients, with a postoperative reflux incidence of around 15%.
- Esophagectomy is reserved for patients with severe dysphagia who have failed both dilatation and myotomy.
Non-Surgical Treatment
- Intrasphincteric injection of botulinum toxin blocks acetylcholine release at the LES level.
- Pneumatic dilatation has been the main non-surgical treatment modality.
- Postdilatation reflux occurs in about 25-35% of cases.
- Patients who fail pneumatic dilatation are typically treated by Heller myotomy.
- Calcium-channel blockers are used to decrease LES pressure, benefiting only 10% of patients.
- These are used primarily in elderly patients with contraindications to pneumatic dilatation or surgery.
Summary
- Difficult swallowing can be due to many causes, either generalized or localized to the esophagus.
- Structural issues or impaired innervation/motility can affect the esophagus.
- Diagnosis based on clinical background is important.
- Never take dysphagia in elderly lightly as it is dangerous.
- Investigations for suspected diagnosis are mandatory.
- Achalasia is the most common motility disorder of the esophagus.
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