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32 Cards in this Set

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Case 1:
- 37 yo AA female complains of difficulty swallowing, vomiting, and weight loss (25 lbs)
- Sleeps with her head up at night, otherwise keeps her
husband up with her coughing

What more do you want to know about?
- Is it liquids or solids? - Started as solids, now also liquids
- Smell? - Does not smell bad, which suggests it is not getting digested; it is trapped in esophagus (regurgitation)
- Nausea? - No sense of nausea (regurgitation)
Case 1:
- 37 yo AA female complains of difficulty swallowing, vomiting, and weight loss (25 lbs)
- Sleeps with her head up at night, otherwise keeps her
husband up with her coughing
- Mild asthma as a child; two uneventful pregnancies (NSVD x2)
- One episode of bronchitis and one episode of pneumonia in past year
- On birth control pills

Differential diagnosis?
- Achalasia
- Esophageal cancer
- Esophageal web
- Zenker's diverticulum
- Esophageal ring
- Eosinophilic esophagitis
- Thoracic mass
- Scleroderma
Case 1:

What should be done next for this patient?
a) Timed Barium Esophagram
b) Chest X-ray
c) CT of the chest
d) Upper endoscopy
e) Esophageal manometery
Upper endoscopy
- If your patient has alarm symptoms, you want to look and see what is in there via endoscopy

A timed barium esophagram would help you diagnosis achalasia but you want to rule out the more severe causes (eg, cancer)
What are the alarm signs and symptoms for upper GI malignancy? What should you do if they have any of these?
- Weight loss
- Anemia
- Dysphagia
- Hematemesis
- Melena (black tarry stools)
- Family history of upper GI malignancy

*Do EGD (upper endoscopy)
*** What does this chest x-ray show?
Achalasia
- The lines demonstrate how wide her esophagus has become
*** What does this endoscopy show?
- Lots of saliva
- Dilated esophagus - you can see all of the way down to the stomach (air is enough to open LES)
*** What does this time barium esophagram show?
Bird's beak appearance (classic for achalasia)
- With time the barium is still not draining into the stomach suggesting that there is a problem with LES
Case 1:

What findings on esophageal manometry would confirm the diagnosis of Achalasia?
a) No peristalsis in the esophageal body and no resting lower esophageal sphincter (LES) pressure.
b) No peristalsis in the esophageal body and normal, but non-relaxing LES pressure
c) Disorganized peristalsis in the esophageal body and normal relaxation of the LES
d) Normal esophageal peristalsis and a normal, but non-relaxing LES pressure
No peristalsis in the esophageal body and normal, but non-relaxing LES pressure

This is characteristic of Achalasia
What are the characteristics of the esophageal sphincters?
- UES and LES are high-pressure zones at either end of esophagus
***
***
***
How does peristalsis propel food through the esophagus?
- Main function of esophagus is to propel swallowed food or fluid into stomach
- Coordinated and propulsive sequential contraction of esophageal muscle
- Primary peristalsis occurs in concert with appropriately timed relaxation of UES and LES
What is the definition of achalasia?
- Impaired relaxation of LES and/or increased LES tone
- Loss of peristaslsis in body of esophagus
What is achalasia also known as?
- Mega-esophagus
- Cardiospasm
- Idiopathic esophageal dilatation
How do patients with Achalasia typically present? When?
- Peak incidence in 7th decade

- Dysphagia to solids and liquids is seen in >90% of patients
- Chest pain, heartburn, regurgitation, and weight loss occur in up to 60% of patients
- Food stasis, bacterial fermentation and acidity results in esophagitis and heartburn

- Subtle symptom development due to slow progression and accommodative behavior is common (slow and stereotypical eating movements; avoid social events with meals)
How is the esophagus innervated?
Peristalsis is generated by:
- Intrinsic: enteric neural plexus
- Extrinsic: Vagus nerve
How is peristalsis controlled differentaly in proximal esophagus vs distal esophagus?
- Top 1/3 of esophagus is striated muscle (all at once)
- Transition zone of both striated and smooth muscle
- Lower 2/3 of esophagus is smooth muscle (in steps)
*** Picture of curves
What are the characteristics of the distal esophageal smooth muscle peristalsis?
- Varicose nerve endings and gap junctions
- Ca2+ influx from outside
- Latency gradient and "dual peripheral innervation": peristalsis in smooth muscle is physiologically regulated as "a wave of inhibition followed by a wave of excitation"
*** Picture of NTs
***
***
- Excitatory NTs:
- Inhibitory NTs:
What is the pathophysiology occurring in achalasia?
- Normally have both excitatory and inhibitory neurons
- In achalasia you initially have impaired and then loss of inhibitory (NO) activity - tells sphincter not to relax

***Pathophysiology picture
What is the histologic appearance of normal esophageal nervous tissue?

*** Pic #1
***
What is the histologic appearance of achalasia esophageal nervous tissue?

*** Pic #2
***
What is the histologic appearance of achalasia esophageal nervous tissue?

*** Pic #3
***
Which of these neurotransmitters MOST LIKELY controls contraction of the lower esophageal sphincter?
a) Acetylcholine
b) Norepinephrine
c) Nitric oxide
d) Vasoactive intestinal peptide
e) GABA
ACh
Which of these neurotransmitters MOST LIKELY controls relaxation of the lower esophageal sphincter?
a) Acetylcholine
b) Norepinephrine
c) Nitric oxide
d) Vasoactive intestinal peptide
e) GABA
Nitric Oxide
How do you diagnose Achalasia?
Need 2 or 3 modalities:
- Manometry
- Radiography
- Endoscopy

*** Pictures of Diagnosis
What is the differential diagnosis for Achalasia?
- Malignancy: adenocarcinoma (esophageal, gastric, pancreatic, or breast via direct infiltration)
- Other infiltrative disorders: amyloidosis or sarcoidosis
- Chagas disease (Trypanosoma cruzi - diffuse enteric myenteric destruction)
- Para-neoplastic syndromes: small cell carcinoma of lung
- Autonomic nerve damage: diabetes, polio, or surgical damage
What malignancies must be ruled out because they present similarly to Achalasia?
***
What other infiltrative disorders must be ruled out because they present similarly to Achalasia?
***
What infection must be ruled out because they present similarly to Achalasia?
***
What other syndromes must be ruled out because they present similarly to Achalasia?
***
What causes of nerve damage must be ruled out because they present similarly to Achalasia?
***
How do you treat Achalasia?
- Pharmacotherapy
- ***