ESOPHAGEAL MOTILITY DISORDERS

Question 1

Achalasia (4)

Answer 1

Most common primary motility disorder, due to degeneration of the myenteric plexus, characterized by:
- aperistalsis of esophageal body
- failure of relaxation of the LES (in more than 50% of cases)
- increased LES pressure >6 mmHg

Question 2

Achalasia- Causes (3)

Answer 2

1- Idiopathic
2- Adenocarcinoma of proximal stomach
3- Chagas disease

Question 3

Achalasia- Presentation (3)

Answer 3

1- Triad of dysphagia (liquids/solids), regurgitation, and weight loss
2- +/- Halitosis (due to stagnant food) +/- Retrosternal chest pain
3- Complications: aspiration —> pneumonia/bronchitis

Question 4

Achalasia- Diagnosis (4)

Answer 4

1- Chest X-ray: Dilated esophageous + air-fluid levels & absence of gastric air bubble
2- Barium swallow: bird beak sign (tapering of distal esophageous and dilation of proximal lower esophageous, and impaired peristalsis)
3- Manometery (gold-standard): absence of peristalsis, absence of LES relaxation with swallowing, increased LES tone
4- Endoscopy to rule out other causes of chronic distal obstruction e.g. cancer

Question 5

Achalasia- Management (5)

Answer 5

Mainly palliative and symptomatic relief
1- Medical: nitroglycerin, sildenafil (PDEI), nifedipine (CCB) —> decrease LES tone
2- Endoscopic balloon dilation (2% risk of perforation)
3- Injection of LES with botulinum toxin (botox injection)
4- Surgery: Heller cardiomyotomy (esophagomyotomy) + concomitant anti-reflux procedure (fundoplication to prevent reflux Esophagitis)
5- Indications for surgery: persistent pain, GI or biliary obstruction, pseudocyst infection, hemorrhage, or rupture, and enlarging pseudocyst

Question 6

What is Achalasia associated with?

Answer 6

A higher risk of squamous cell carcinoma in both treated and untreated patients

Question 7

Systemic sclerosis (4)

Answer 7

• Smooth muscle layer of esophageous replaced by fibrous tissue
• Presents as chronic heartburn, patient has history of scleroderma
• Manometry: Low LES pressure, aperistalsis, esophageal hypomobility
• Treatment is with PPIs for reflux

Question 8

Diffuse Esophageal Spasm

Answer 8

• Loss of normal peristaltic coordination of esophageal smooth muscle —> simultaneous contraction of segments of esophageal body, mainly in distal end

Question 9

Diffuse Esophageal Spasm- Presentation (2)

Answer 9

1- Substernal spastic chest pain, sudden onset, not related to exertion, precipitated by drinking cold liquids
2- Dysphagia to both solids and liquids, but NO regurgitation (unlike achalasia)

Question 10

Diffuse Esophageal Spasm- Diagnosis (3)

Answer 10

1- Barium swallow —> cork-screw appearance
2- Manometery (gold-standard): spontaneous activity, repetitive waves, prolonged high-amplitude contractions, LES tone is normal
3- Endoscopy to rule out mass/ stricture/ esophagitis

Question 11

Diffuse Esophageal Spasm- Management

Answer 11

Medical therapy with nitrates and calcium channel blockers or surgical management (if severe symptoms) with an esophageal myotomy from the aortic arch level to the stomach