II. Epidemiology
- Rare conditions, even in specialty centers
- Peak age of onset >60 years old
III. Pathophysiology
- Loss of inhibitory Neuron innervation
- Unopposed excitatory Neuron overactivity within the esophageal Smooth Muscle
- Peristalsis preserved to some extent but is disordered and incoordinated
- Waves of peristalsis occur simultaneously
- Distal Esophageal Spasm is characterized by premature, forceful contractions
- Jackhammer Esophagus has properly timed contractions, but increased forceful contractions
IV. Symptoms
- Sudden, severe Chest Pain in typically brief episodes
- Patient unable to swallow solids or liquids during the episode
V. Differential Diagnosis
- See Esophageal Dysphagia
- See Chest Pain Causes
- Exclude Achalasia
VI. Evaluation
- See Esophageal Dysmotility
- Esophageal manometry
- Esophagram with corkscrew Esophagus
VII. Management
- See Esophageal Dysmotility for general measures and overall approach
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General Measures
- Offer reassurance
- Functional disorders and Hypercontractile Esophagus improve or resolve spontaneously in a majority of patients
- Optimize GERD Management
- Antisecretory therapy (e.g. Proton Pump Inhibitor)
- Discontinue Opioids
- Functional Disorders (Hypercontractile Esophagus has significant overlap with functional disorders)
- Stress management
- Consider Cognitive Behavioral Therapy
- Consider Antidepressants
- Mindful eating
- Eat smaller, more frequent meals
- Eat slowly
- Choose softer foods
- Avoid foods and situations that trigger symptoms
- Offer reassurance
- Medications
- Precautions
- These medications decrease lower esophageal sphincter pressure and may worsen GERD
- Smooth Muscle relaxants are best limited to hypermotility confirmed by manometry
- Agents
- Calcium Channel Blockers
- Nitrates (Nitroglycerin)
- Phosphodiesterase-5 Inhibitors (release nitric oxide)
- Other agents which are safe and may be effective
- Peppermint Oil (2 mints sublingual before each meal)
- Precautions