ACHALASIA Flashcards
what type of muscle is the esophagus made up of?
outer longitudinal muscle layer and inner circular muscle layer
what do circular muscle fibers allow?
peristalsis
what is the upper 1/3 of the esophagus predominantly?
skeletal muscles
what is the lower 2/3 of the esophagus mostly?
smooth muscles become more dominant from the middle to the distal esophagus
where is the auerbach plexus located?
between the circular muscle layer and the longitudinal muscle layer in the lower esophagus, stomach, and intestines
what is the auerbach plexus responsible for?
responsible for the peristaltic movement of the bowels
can the auerbach plexus act independently from the CNS?
YES
does the auerbach plexus fail in achalasia?
yessss
what is the epidemiology of achalasia?
rare, neurogenic esophageal motility disorder
25-60 y/o
male=female
what are the 2 pathological features of achalasia?
- failed relaxation in the lower esophageal sphincter (LES) during swallowing (MAJOR)
- impaired peristalsis in the lower 2/3 of the esophagus (minor)
what are the 2 causes of achalasia?
- degeneration of myenteric plexus ganglia in the lower esophagus
- viral and autoimmune factors are suspected; appear there is a genetic predisposition (familial cases)
what are the 3 complications of achalasia?
- aspiration pneumonia
- megaesophagus in 10% of cases -> increase in size affects the transport of food
- increased risk of esophageal cancer -> food stasis, bacterial overgrowth, and chronic inflammation
what is the cause of primary achalasia?
no known cause
what is the cause of secondary achalasia?
d/t disease that causes esophageal abnormalities
what is the number one cause of secondary achalasia?
malignancy (esophageal, gastric, or other extra-esophageal cancers) by mass effect
what are the 4 other causes of achalasia other than malignancy?
- chagas disease
- infiltrative disorders: amyloidosis, sarcoidosis
- eosinophilic esophagitis
- MEN 2B
what is chagas disease caused by
- caused by protozoan parasite (trypanosoma cruzi)
what is the onset of achalasia?
insidious
what are the big 3 sx of achalasia?
- dysphagia (progressive)
- nocturnal regurgitation of undigested food
- weight loss (Mild to mod)
what is involved in dysphagia?
cant swallow solids that slowly progresses to liquids
PROGRESSIVE SX
what can nocturnal regurgitation of undogested food lead to?
may cause nocturnal cough or lead to aspiration pneumonia
what are other sx of achalasia other than the big 3?
heartburn (Pyrosis)
retrosternal pain -> spontaneous or with swallowing
vomitting
what do you START WITH to dx achalasia?
upper endoscopy
what is an upper endoscopy for?
- esophageal dilation
- r/o any obstruction or malignancy
- classic “pop” when the endoscope passes into stomach
what is the gold standard test for achalasia?
HIGH-RESOLUTION MANOMETRY WITH ESOPHAGEAL PRESSURE TOPOGRAPHY (EPT)
what is pressure represented by on EPT?
represented by color (inc. color intensity= inc. pressure)
what 3 things does an EPT demonstrate?
- incomplete relaxation of LES after swallowing
- high LES resting pressure
- aperistalsis in lower 2/3 of esophagus
what is a barium swallow?
a complementary test in equivocal manometric findings
what can a barium swallow show?
the absence of progressive peristaltic contractions during swallowing
on barium swallow, what are upper and lower esophagus doing?
upper esophagus is dilated, but is narrowed and “BEAK LIKE” at the LES
-PATHOGNOMONIC FOR ACHALASIA
what happens to the emptying of barium in body?
delayed emptying
what therapy will restore peristalsis?
none
what is the goal of achalasia tx?
reduce pressure at LES and allow for passage of ingested material
what are the 3 modalities of tx for achalasia?
- pneumatic balloon dilation of the LES
- surgical myotomy of the LES
- botulinum toxin injection
what does a pneumatic balloon dilation of the LES do?
circumferential stretching
what is a surgical myotomy of the LES ?
muscles of the LES are cut to allow food and liquids to pass to the stomach (relieves pressure )
what is a complication of a surgical myotomy of the LES?
esophageal perforation and/or gastroesophageal reflux
who should get the botox treatment?
those who aren’t surgical candidates
what is the botulinum toxin treatment?
direct endoscopic injection into LES to block release of acetylcholine -> decreased LES tone
how long does the botox treatment for achalasia last?
6-12 months