Esophageal Motility Disorders

Posted by e-Medical PPT Friday, December 30, 2011
Peristalsis
Sequential, coordinated contraction wave that travels along the whole length of the esophagus, propelling intraluminal contents downstream.
Primary wave strips from proximal to distal, triggered by swallowing center, 2cm/sec.
Secondary wave induced by distension of bolus, acts to clear esophagus of retained food.
Tertiary contractions are dysfunctional and have no role.

Motility Disorders
Achalasia
Primary spastic motility disorders, including DES, nutcracker esophagus, hypertensive LES
Secondary esophageal motility disorders related to DM, scleroderma, alcohol, psychiatric disorders, etc.

Achalasia
Loss of ganglion cells from the wall of the esophagus, starting at the LES and going proximally.
Loss of inhibitory nerves at LES.
Circular muscle layer thickened at LES but microscopically cells appear normal.
Loss of the inhibitory nerves at the LES causes failure of the LES to completely relax, and a hypertensive LES pressure over 40mmHg in 60% of patients.
Loss of nerves along the body of esophagus causes aperistalsis, stasis, dilatation.
Non-peristaltic isolated contractions or low-amplitude simultaneous contractions occur.
If high-amplitude (>60mmHg) simult contractions occur it is called Vigorous Achalasia.

Spastic Motility Disorders
Diffuse fragmentation of vagal filaments, mitochondrial fragmentation results in functional imbalance between excitatory and inhibitory pathways.
When DES occurs, diffuse muscular hypertrophy as much as 2cm has been described in the distal 2/3 of the esophagus, but wall thickening is also found in asymp patients, absent in patients with typical sympt and manometric findings too.

Scleroderma Esophagus
Primary defect here is related to smooth muscle atrophy and fibrosis.
The dysmotility here an absence of peristalsis in the esoph body and an atonic LES occur.
Motility is preserved at the striated muscle part of the esophagus.

Pseudoachalasia
Term used to describe clinical picture of GE junction obstruction.
Present in 5% of patients with clinical and manometric diagnosis of achalasia.
Clinical presentation is more likely to occur with rapidly progressive disease, older age of onset, profound weight loss.
Workup includes upper endoscopy, biopsies should be obtained with any suspicion of malignant process. If suspicious lesion found, image with CT, MRI, EUS if indicated.
In 50% of patients the diagnosis is adenocarcinoma of GE junction.

Diffuse Esophageal Spasm
DES and achalasia can be confused. Manometric criteria require that normal esophageal peristalsis be present intermittently for DES.
LES relaxation, which is incomplete in achalasia, should be normal in DES.

Manometry
Achalasia: aperistalsis of esophageal body is manometric hallmark.
DES: Normal peristalsis, simultaneous contractions in >30% water swallows, incomplete LES relaxation, increased LES pressure (>40mmHg), or repetitive, prolonged(>6sec), high-amplitude contractions (>180mmHg).
Nutcracker: Normal patterned peristalsis with high amplitude contractions(>180mmHg), repetitive contractions, increased LES pressure(>40mmHg).
Hypertensive LES: Increased LES pressure (>40mmHg). Significance of this is questionable.

Endoscopy
Exclude mechanical and inflammatory lesions that are causing dysmotility, structural cause of obstruction.
Endoscopic US still investigational in managing achalasia, used to assist in botulinum toxin injection....

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