Achalasia Flashcards
What is achalasia?
Achalasia is a rare neurodegenerative disorder characterized by the inability of the lower esophageal sphincter (LES) to relax and an aperistaltic esophagus.
What are the potential contributing factors to the development of achalasia?
Genetic predisposition
environmental factors
and autoimmune destruction of the myenteric plexus, possibly triggered by a viral infection.
What are common symptoms of achalasia?
Regurgitation of food, retrosternal chest pain, and dysphagia
What symptoms might manifest in advanced cases of achalasia?
Recurrent aspiration pneumonia, weight loss, and failure to thrive
How can the effectiveness of treatment for achalasia be assessed?
By using the Eckardt scoring system to monitor symptom improvement
What classic appearance might a barium swallow reveal in achalasia?
The classic “bird’s beak” appearance
Why is endoscopy recommended in the evaluation of achalasia?
To rule out mechanical obstruction such as malignancy, benign stricture, or pseudoachalasia
What is the main diagnostic tool for achalasia?
High-resolution manometry (HRM)
What might be seen on endoscopy in cases of long-standing achalasia?
A markedly dilated esophagus and retained food within the esophageal lumen
What characterizes type I achalasia on high-resolution manometry (HRM)?
No esophageal contraction or pressurization, and an integrated relaxation pressure (IRP) greater than 15 mm Hg.
What characterizes type II achalasia on HRM?
Panesophageal pressurization and no peristaltic contraction.
What characterizes type III achalasia on HRM?
At least 20% premature (spastic) contractions.
What treatment may be useful for frail elderly patients or those with contraindications to general anesthesia?
Intrasphincteric injection of botulinum toxin
How is botulinum toxin administered for achalasia?
It is injected endoscopically into four quadrants of the lower esophageal sphincter (LES), with 20–25 units per quadrant (80–100 units total).
What is the mechanism of action of botulinum toxin in treating achalasia?
It irreversibly inhibits acetylcholine release from presynaptic terminals, reducing the neurogenic component of LES pressure.
How long does symptom relief from botulinum toxin injections typically last?
Up to 1 year, but symptoms often recur due to new axonal growth
What are the limitations of repeat botulinum toxin injections?
Local inflammatory reactions, fibrosis, and side effects such as chest discomfort and rash
What pharmacologic therapies have been used for patients unfit for general anesthesia?
Smooth muscle relaxants like nitrates and calcium channel blockers, such as nifedipine and sildenafil
What are the side effects of nitrates and nifedipine in treating achalasia?
Nitrates can cause headaches, while nifedipine can cause headaches, orthostasis, dizziness, and has limited efficacy
What is the most effective nonsurgical option for achalasia?
Endoscopic dilation.