Achalasia Flashcards

1
Q

what is achalasia

A

oesophageal motility disorder :

  • absence of peristalsis
    and in the distal 2/3 (thoracic) of the esophagus
    uncoordinated oesophageal contractions
  • failure of lower esophageal sphincter relaxation
  • elevated resting LES pressure
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2
Q

what is the innervation of the esophagus

A
parasympathetic(NO, VIP) - vagus nerve 
in the muscle - myenteric (auerbach plexus) lies between the longitudinal and circular layers of the esophagus giving motor innervation- peristalsis , wall relaxation 
in submucosa - messier plexus -
gland stimulation 
LES relaxation 

sympathetic
cervical and thoracic sympathetic ganglions
contaraction of sphincters

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3
Q

what is the pathophysiology of achalasia ?

A

loss of preganglionic parasympathetic vagus nerve input resulting in loss of post ganglionic parasympathetic NITERGEGIC neurones (myeneric plexus) = NO release subsides

unopposed cholinergic sympathetic stimulation = high LES pressure

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4
Q

what is the signs and symptoms of achalasia ?

A

regurgitation of food - non bilious and non acidic

dysphagia more prominent for solid foods
- progressive going to liquids

chest pain in the xiphoid area 
especially when eating 
retrosternal angina like pain 
 due to esophgeal spasms 
decreases in advanced stages as oesophagus expands 

nocturnal cough

later stages :
aspiration pneumonia
weight loss

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5
Q

what’re the treatment options for Achalasia?

A

diet - high protein and less n spicy and hot food

pharmacological

forceful dilation

endoscopic botulinum toxin injection

peroral endoscopic myotomy

laparoscopic heller cardiomyotomy

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6
Q

what are the pharmacological treatment methods ? and side effects ?

A

calcium channel blockers sublingual before meals - nifedipine
= short term relief
:( low blood pressure and headache

nitroglycerin sublingual before meals

phosphodiesterase inhibitors

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7
Q

how is there forceful dilation achieved ?

A

endoscopic balloon catheter - rupture the circular muscle fibres and reduce LES pressure

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8
Q

what are the side effects of botox ?

A

short lived needs reinjection after one year

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9
Q

etiology of achalasia ?

A

higher incidence in family members

ACTH insensitivity

infectious neurotropic factors - diphtheria pertussis , clostridia , TB

viral - antibodies to measles and varicella booster

chagas disease- in chronic condition - arrhythmia of the heart but can also have achalasia

esophageal trauma

ischemic oesophageal damage in utero

autoimmune process infiltration of myenteric plexus with T lymphocytes , antibodies to neurones , and association with HLA -DQ alleles in combo with sjoren syndrome

neurological and psychiatric disorders such as - Parkinson disease, heridtory cerebral ataxia

von recklinhause’s neurofibromatosis

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10
Q

there is histopathology in the myenteric plexus in achalasia such as ?

and in other part oesophagus

A
ganglionitis 
perinueritis 
neuritis 
lewy body in ganglions - protein accumulation - seen in Parkinson aswell 
neural fibrosis 
ganglion cell loss - In LES 
migration , differentiation and growth of neurones 
 degeneration of neurones 

most marked changes in inner circular layer :
muscular hypertrophy
fibrosis of SMC
non specific intracytoplasmic inclusions

mucosal and submucosal :
hyperplasia
dysplasia
atrophy of submucosal glands

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11
Q

what is diagnosis of achalasia ?

A

barium fluoroscopy - rat tail and absence of gastric air bubble - signs
can have pulmonary infiltrates due to aspiration

endoscopy - contains retained food

esophageal manometry manometry -

electromyography

Ntroglycerin test - nitrates relax the LES

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12
Q

what is the normal LES pressure ?

A

10-45mmhg

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13
Q

how can we differentiate achlasia from esophgeal carcinoma based on clinical history

A

Esophegal carcinoma :

age greater than 60

FIRST DYSPHGIA THEN PAIN (opposite for achlasia)

assymterical narrowing ( achalasia - smooth funnels shaped narrowing)

peristalsis preserved above narrowing (in achalasia it is not)

not positive to ng test

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14
Q

complications of achlasia ?

A
esophagitis 
ulcer 
hiatal hernia 
esophgeal rupture 
fistula 
carcinoma 
aspiration pneumonia
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15
Q

the content of muscle change in the esophagus describe it ?

A

striated muscle in the upper 5 cm
striated and smooth muscle in the middle part
and smooth muscle in the lower part

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16
Q

achalasia is part of what syndrome ?

A

AAA syndrome

Alacrima
Achlorhydria
ACTH insensitivity
Achalasia

low hieght
microcephaly
deafness

17
Q

what can cause secondary achalasia ?

A

trypanasoma cruzi

Cancer

18
Q

patents with achlasia is prone to what ?

A

candida infections - seeing white plaques

19
Q

achalasia is most common in whom ?

A

young people

20
Q

oesophageal myotomy- laparoscopic heller cardiomyotomy side effects

A

GERD reflux disease after surgery

21
Q

what is the differential diagnosis of achalasia ?

A

oesophageal carcinoma

oesophageal strictures , webs , rings foreign bodies

diffuse oesophageal spasm

scleroderma

paraneoplastic nerve degen

gerd

22
Q

what is the complication of peroral endoscopic myotome ?

A

perforation , mediastinitis