II. Epidemiology

  1. Rare conditions, even in specialty centers
  2. Peak age of onset >60 years old

III. Pathophysiology

  1. Loss of inhibitory Neuron innervation
    1. Unopposed excitatory Neuron overactivity within the esophageal Smooth Muscle
  2. Peristalsis preserved to some extent but is disordered and incoordinated
    1. Waves of peristalsis occur simultaneously
    2. Distal Esophageal Spasm is characterized by premature, forceful contractions
    3. Jackhammer Esophagus has properly timed contractions, but increased forceful contractions

IV. Symptoms

  1. Sudden, severe Chest Pain in typically brief episodes
  2. Patient unable to swallow solids or liquids during the episode

V. Differential Diagnosis

VI. Evaluation

  1. See Esophageal Dysmotility
  2. Esophageal manometry
  3. Esophagram with corkscrew Esophagus

VII. Management

  1. See Esophageal Dysmotility for general measures and overall approach
  2. General Measures
    1. Offer reassurance
      1. Functional disorders and Hypercontractile Esophagus improve or resolve spontaneously in a majority of patients
    2. Optimize GERD Management
      1. Antisecretory therapy (e.g. Proton Pump Inhibitor)
    3. Discontinue Opioids
    4. Functional Disorders (Hypercontractile Esophagus has significant overlap with functional disorders)
      1. Stress management
      2. Consider Cognitive Behavioral Therapy
      3. Consider Antidepressants
        1. Selective Serotonin Reuptake Inhibitors (SSRI)
        2. Tricyclic Antidepressants
    5. Mindful eating
      1. Eat smaller, more frequent meals
      2. Eat slowly
      3. Choose softer foods
      4. Avoid foods and situations that trigger symptoms
  3. Medications
    1. Precautions
      1. These medications decrease lower esophageal sphincter pressure and may worsen GERD
      2. Smooth Muscle relaxants are best limited to hypermotility confirmed by manometry
    2. Agents
      1. Calcium Channel Blockers
      2. Nitrates (Nitroglycerin)
      3. Phosphodiesterase-5 Inhibitors (release nitric oxide)
    3. Other agents which are safe and may be effective
      1. Peppermint Oil (2 mints sublingual before each meal)

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