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Neuromuscular esophogeal disorders

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NEUROMUSCULAR
ESOPHOGEAL
DISORDERS
Dr. Abdullah Bahadi GS R2
Zenker’s Diverticula
• It consist of three locations pharyngoesophageal ,
midesophageal , epiphrenic
• Historically known to be caused by motor disturbances of
ues and les
• What is the difference between true and false diverticulum
• Most common area for zenker diverticulum ??
• Classic patients are about 70 years old , this is has been
thought to be caused by losing the tone and elasticisty
with age
• You name the symptoms
• Most serious is
• aspiration and lung abscess
• Diagnosis is done by barium swallow
• <2cm diverticulum myotomy is suffuciant , in frail pt do
diverteculopexy due to higher risk of leak
• >5 cm or good tissue >> do excision and keep pt npo 2-3
days post op
• 2-5 cm >> endoscopic , needs extension of neck (not
good for cervical stenosis) , 1 day stay , NPO post op day
,
• Study wise <3cm surgical is suprorior to endoscopic ,
>3cm both are same , inanition and stay is shorter in the
endoscopic
Midesophageal Diverticula
• Historically caused by tuberculosis or fibrosing medistinitis
• Nowadays belived to be from motility disorders , tend to
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be right sided due to left crowding
Diagnosis by barium swallow with lateral views to identify
sac place before surgery
CT and manometry ??
<2 cm >> no need
symptomatic or 2 or more cm>> intervention required with
diverteculopexy to thoracic vertebral fascia
severe symptoms>> long esophagomyotomy is done
Epiphrenic Diverticula
• Tend to be right sided and its within 10 cm from the GEJ ,
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its pulsion diverticula and can be
caused due to motility disorder most commonly NEM ,
other causes like trauma or ehlers danlon can be
assosciated
< 2cm suspend to the vertebral fascia
Severe chest symptoms and documented motor disorder
>> long esophagomyotomy
If there is large hiatal hernia during the repair it must be
repaired
Motility disorders
• Primary : achalasia . DES , Hypertensive LES ,
Nutcracker , Ineffective esophageal motility
• Secondary : collagen vascular disease , NEM disorders
Achalasia
• Means failure to relax
• 6/100000
• Young women – middle aged man
• Can be caused by nerve degeneration , trauma , infection
(trypanosome cruzi) , stress
• Nerveous degeneration of the LES >> makes it
hypertensive and fail to relax with swallowing >>
pressurization >> dilatation
• After 20 years the risk is __% to develop squamous cell
carcinoma but no specific survilance is there mostly occur
at middle third of the esophagous below level of airfluid
• Classic triad is dysphagia + regurgitation + weight loss
• Patient often experience liquid dysphagia that progress to
solid
• Patient try to adapt by drinking water and experience
relieve with LES opened
• Patient present late or with complication such pneumonia
and abscess
• Lack of gastric bubble means advanced disease
• It can lead to megaesophagous
• Manometry is gold standard it will show 5 findings
• 2 in les and 3 in esophageal body
• LES pressure higher than 35 and failure to relax with
degultation
• Esophageal body will show , pressure above baseline due
to air in esophagous, low amplitude due to lack of tone ,
mirrored simultaneous contraction with no progressive
prestalsis
• Endoscopy is done for esophagitis and cancer but not
diagnosis
• Non surgical treatment , ccb , nitrates , nitroglycerine
• Botox treatment prevent acetylcholine release but
recurrence is 50% in 6 month
• Bougie dilatation until 54fr can be done but needs serial
dilatations to sustain
• Gruntzig type ballon effective in 60% and risk of
perforation is 4%
• Surgical heller myotomy can be done laparoscopic and is
treatment of choice lower risk of perforation than ballon ,
pt can have reflux post surgery and its controversy to do
partial antireflux surgey with it like topet or dor
• Esophagectomy is endstage treatment , for those with
megaesophagous , failed myotomy (<60% will benefit
from repeated surgery) , reflux strictures
Different treatments
• 1 year remission ballon dilataion vs botox 89%vs38%
• Perforation surgery vs ballon 1%vs4%
• Mortality surgery vs ballon 0.2%vs 0.5%
• Laparoscopic proved shorter stay , less pain , better relief
of dysphagia , improved heartburn
Diffuse esophageal spasms
• Young women
• Simultaneous contractions of esophageal body with high
amplitude due to what it could be nerve degeneration and
muscular hypertrophy
• 5 times less common than achalasia
• Chest pain plus dysphagia , squeezing CP , related to
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stress
Can be exacerbated by cold liquids
IBS , pyloric spasm, gallstones , pancreatitis , can all
trigger DES
Picture of corkscrew esophagous or pseudodivertucolosis
on esophagogram
Manometry findings , multipeaked contraction with high
amplititude >120mmhg
Or long duration >2.5
• Introducing bethancol or ambulatory manometry can help
catch spontenous contractions
• Treatment is non surgical is the best , antireflux if needed,
nitrates and ccbs , conrolloing triggers , psychiatric
problems , reassurance
• Botox= non sustainable
• Dilatation = 70-80% of success
• Peppermint of some symptomatic relief
• Patient for long esophagomyotomy if failed medical tx ,
pulsion diverticulum, failed endoscopic therapy ,
incapacitating symptoms
• Surgery can provide 80% relieve , extend must be
determined by Manometry
• Dor fundo performed with it
Nutcracker esophagous
• All ages , no specific gender , most painful disorder
• Diagnosed by chest pain + contraction of esophagous
more than 2 SD than normal up to 400 mmhg , normal
LES
• Mainly medical tx nitrates ,ccb, antispasmodics
• Avoid triggers , dialation maybe of benefit
Hypertensive LES
• Hypertensive LES but normal relaxation
• Hyperprestaltic esophageal body contraction but
sometimes normal
• Symp As usual nothing new
• In manometry there is hypertensive of LES with normal
relaxation
• Medical or ballon or botox could be tried but procedure of
choice is modified laparoscopic heller myotomy
IES
• Distal esophagous noncontraction leading to decrease in
the clearance of gastric acid
• Caused by GERD destroying the distal esophagous
• Treatment is by prevention
Non specific esopheageal Motility
disorders
• Anything else falls in this category
• Conditions like dermatomyositis , scleroderma , SLE
causes esophageal motility disorders
• More reflux more regurgitation
• Treated by treatment of underlaying disease
• No specific findings in LES , but in esophageal body it will
be either non transmitted , triple-peaked, retrograde , low
amplititude <35mm hg, prolonged >6
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