Achalasia Flashcards
what is achalasia
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
what is the innervation of the esophagus
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
what is the pathophysiology of achalasia ?
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
what is the signs and symptoms of achalasia ?
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
what’re the treatment options for Achalasia?
diet - high protein and less n spicy and hot food
pharmacological
forceful dilation
endoscopic botulinum toxin injection
peroral endoscopic myotomy
laparoscopic heller cardiomyotomy
what are the pharmacological treatment methods ? and side effects ?
calcium channel blockers sublingual before meals - nifedipine
= short term relief
:( low blood pressure and headache
nitroglycerin sublingual before meals
phosphodiesterase inhibitors
how is there forceful dilation achieved ?
endoscopic balloon catheter - rupture the circular muscle fibres and reduce LES pressure
what are the side effects of botox ?
short lived needs reinjection after one year
etiology of achalasia ?
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
there is histopathology in the myenteric plexus in achalasia such as ?
and in other part oesophagus
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
what is diagnosis of achalasia ?
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
what is the normal LES pressure ?
10-45mmhg
how can we differentiate achlasia from esophgeal carcinoma based on clinical history
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
complications of achlasia ?
esophagitis ulcer hiatal hernia esophgeal rupture fistula carcinoma aspiration pneumonia
the content of muscle change in the esophagus describe it ?
striated muscle in the upper 5 cm
striated and smooth muscle in the middle part
and smooth muscle in the lower part