Esophageal hypermotility disorders

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Esophageal hypermotility disorders are a group of uncommon conditions that include hypercontractile peristalsis, which is caused by vigorous esophageal contractions, and distal esophageal spasm, which is caused by premature esophageal contractions. While the precise etiology is unknown, the disorders are thought to be due to autonomic dysfunction of the esophagus. Consumption of hot/cold food, stress, and gastroesophageal reflux can trigger an acute episode of esophageal hypermotility, which typically manifests as intermittent retrosternal chest pain and dysphagia, predominantly to liquids (but also, in some cases, to solids). It is important to rule out more common causes of acute chest pain and dysphagia before considering high-resolution esophageal manometry (HRM) to confirm a diagnosis of esophageal hypermotility. Treatment is symptomatic and typically includes smooth muscle relaxants, such as nitrates and calcium channel blockers. A trial of PPIs may be considered, as GERD often accompanies esophageal hypermotility disorders and may even trigger an acute episode. Endoscopic botox injection into the esophageal body or the spastic segment may be considered if there is an inadequate response to pharmacological therapy. Long myotomy (endoscopic or surgical) may be considered as a last resort option.

Esophageal hypermotility disorders are caused either by hypertensive or premature esophageal contractions. ^[2]

• Hypertensive esophageal contractions: vigorous esophageal contractions with distal contractile integral (DCI) > 8000 mm Hg/sec/cm (see ''Diagnostics'')
  • Hypercontractile esophagus (jackhammer esophagus): hypertensive propagative esophageal contractions in which at least 20% of swallows have a DCI > 8000 mm Hg/sec/cm
  • Hypertensive peristalsis (nutcracker esophagus): an obsolete term according to the Chicago classification; previously used to describe hypertensive propagative esophageal contractions involving progressive peristaltic waves with an amplitude ≥ 220 mm Hg on conventional esophageal manometry ^[3][4]
• Premature esophageal contractions: contractions with a distal latency of < 4.5 seconds between them (see ''Diagnostics'')
  • Distal esophageal spasm (DES; corkscrew esophagus): premature and uncoordinated nonpropagative esophageal peristalsis in at least 20% of swallows

Hypertensive and propagative esophageal contractions are seen in hypercontractile esophagus. Premature and nonpropagative esophageal contractions are seen in distal esophageal spasm.

The precise etiology of esophageal hypermotility is not known. ^[5]

• Distal esophageal spasm is thought to be caused by impaired inhibitory innervation. ^[2][4]
• Hypercontractile esophagus is thought to be caused by excessive cholinergic drive. ^[4]
• GERD may play a causative and/or aggravating role in esophageal hypermotility disorders. ^[6]

Esophageal motility disorders include disorders of hypomotility (e.g., achalasia) and hypermotility (e.g., diffuse esophageal spasm). The Chicago classification divides esophageal motility into four categories according to findings on high-resolution manometry.

Chicago Classification of Esophageal Motility Disorders (version 3.0) ^[2][5]

• Incomplete LES relaxation
  • Achalasia
  • Esophagogastric junction outflow obstruction (EGJ outflow obstruction)
• Major motility disorders
  • Absent contractility
  • Distal esophageal spasm
  • Hypercontractile or jackhammer esophagus
• Minor motility disorders: a group of esophageal disorders that, in contrast to the major motility disorders, typically remain asymptomatic or mildly symptomatic and usually resolve spontaneously
  • Ineffective esophageal motility: a minor esophageal motility disorder characterized by acontractile breaks > 5 cm within normally occurring peristalsis in > 50% of swallows on high-resolution manometry
  • Fragmented peristalsis: a minor esophageal motility disorder characterized by failed/weak peristalsis in > 50% of swallows on high-resolution esophageal manometry
• Normal esophageal motility

• Intermittent dysphagia to liquids (and potentially, solids) ^[7]
• Episodic retrosternal chest pain
• Reflux symptoms (e.g., heartburn, regurgitation)
• Globus sensation
• Upper respiratory symptoms (e.g., hoarseness, recurrent cough)
• Symptoms aggravated by stress and/or hot and cold food/drinks

Dysphagia predominantly to liquids is suggestive of an esophageal hypermotility disorder.

Manometry is the mainstay of diagnosis for esophageal hypermotility disorders but should be considered only after the more common structural and inflammatory esophageal conditions have been ruled out. Laboratory studies may be considered to rule out other diagnoses.

Approach ^[2][4][5]

• Rule out immediately life-threatening causes of chest pain (see ''Diagnostics'' in acute chest pain).
• Investigate for more common causes of dysphagia (see “Diagnostics” in “Dysphagia”).
  • Upper endoscopy with biopsies ^[2]
  • Esophageal pH monitoring to evaluate for GERD (see ''Diagnostics'' in GERD) ^[4][5]
  • Esophageal barium swallow
• Obtain high-resolution esophageal manometry (HRM) if the index of suspicion for esophageal hypermotility disorders is high or if endoscopy and esophageal barium swallow are inconclusive.

Initial evaluation

• Upper endoscopy: typically normal in hypermotility disorders ^[2][5][8]
• Esophageal barium swallow: ^[2][4][5]
  • Hypercontractile esophagus: usually normal
  • Distal esophageal spasm: multiple nonperistaltic contractions, which resemble pseudodiverticula (corkscrew appearance; rosary bead esophagus)
  • May appear normal between acute episodes of DES

Manometry

High-resolution esophageal manometry (HRM) ^[2][5][9]

• Indications: normal upper endoscopy and barium swallow in a patient with dysphagia
  • Gold standard for diagnosing esophageal motility disorders ^[2]
• Procedure: measures the amplitude, length, and duration of the peristaltic waves via a nasogastric tube fitted with numerous pressure sensors
• Findings
  • Distal esophageal spasm: premature contractions in at least 20% of swallows ^[2]
  • Hypercontractile esophagus: hypertensive esophageal contractions in at least 20% of swallows ^[2]
  • HRM may be normal between acute episodes

Conventional esophageal manometry ^[2][3]

• Indication: suspected esophageal hypermotility disorder if HRM is not available
• Procedure:
  • Measures the propagation, speed, and vigor of the peristaltic wave via an esophageal catheter fitted with pressure sensors every 3–6 cm
  • Results are presented in a line tracing display.
• Findings ^[3]
  • Distal esophageal spasm: ≥ 10% of swallows have simultaneous (nonprogressive) contractions with a mean amplitude ≥ 30 mm Hg.
  • Hypertensive peristalsis: progressive peristaltic waves with amplitude ≥ 220 mm Hg
  • Findings may be normal between episodes.

Distal esophageal spasm is diagnosed by measuring the duration of latency periods. Hypercontractile esophagus and hypertensive peristalsis are diagnosed by measuring the strength of peristalsis.

Barium swallow and manometry may be normal between episodes of esophageal hypermotility!

• Dysphagia with liquids (and solids): suggestive of a motility disorder; see the Chicago Classification of Esophageal Motility Disorders
• Dysphagia predominantly with solids: suggestive of a structural disorder; examples include
  • Esophageal cancer (older age)
  • Eosinophilic esophagitis (younger age)
  • Esophageal webs or rings ^[7]
  • Esophageal diverticulum
  • Extrinsic esophageal compression (e.g., hilar lymphadenopathy)
  • See also “Causes of dysphagia.”
• Heartburn
  • GERD
  • Hiatal hernia
• Retrosternal chest pain
  • Angina
  • Myocardial infarction
  • Esophagogastric anastomosis insufficiency or rupture
  • Costochondritis
  • Functional chest pain
  • Panic disorder
  • See differential diagnoses of chest pain.

Esophageal motility disorders typically cause dysphagia with both solids and liquids. Dysphagia predominantly with solids is suggestive of a mechanical obstruction (intraluminal, mural, or extrinsic).

The differential diagnoses listed here are not exhaustive.

Symptomatic control is the mainstay of therapy, as the underlying etiology of DES and hypercontractile esophagus is unclear. Abortive therapy for an acute episode includes smooth muscle relaxants and/or visceral analgesic agents. Lifestyle modifications and pharmacological therapy are useful to minimize recurrences. More invasive methods may be considered if there is an inadequate response to pharmacological therapy.

Lifestyle modifications ^[5]

• Sitting upright during and after meals
• Taking small bites, chewing food thoroughly, and eating slowly
• Drinking between bites
• Avoiding extremely hot or cold foods
• Avoiding bread, meat, and rice, as they worsen dysphagia
• Stopping medications that affect esophageal motility (e.g., opioids)

Pharmacological therapy ^[4][10][11]

• Indications
  • Often combined with lifestyle modifications for adequate symptom control
  • Smooth muscle relaxants and visceral analgesic agents are used as abortive therapy for acute episodes.
• Medications for esophageal hypermotility: there is no consensus regarding the best initial therapy ^[10]
  • Smooth muscle relaxants ^[4][10][12]
    • Nitrates (e.g., isosorbide dinitrate ) ^[10]
    • PDE5 inhibitor (e.g., sildenafil ) ^[10]
    • Calcium channel blockers
      • Nifedipine ^[10]
      • Diltiazem ^[10]
  • Consider PPI if GERD is suspected or confirmed (see PPI).
  • Peppermint oil ^[13]
  • Tricyclic antidepressants (e.g., imipramine ) or an SSRI (e.g., trazodone ) ^[4][10][14]
• Additional considerations
  • Titrate drug dosages or try different categories of drugs as needed to achieve the best results.
  • Duration of therapy is not clearly defined.

GERD is a common comorbidity and may trigger acute episodes of hypermotility; consider PPI therapy for any patient suspected of having GERD.

Invasive therapy ^[4][5][11]

• Indications
  • Inadequate symptomatic improvement with pharmacological therapy
  • Intolerable side effects of pharmacological therapy
• Endoscopic options
  • Endoscopic botox injection: first-line invasive procedure for hypermotility disorders
    • Temporarily effective (∼ 6 months)
    • Repeat procedures are required.
    • Infection is a very serious potential complication of botox injection.
  • Second-line options: Consider in patients with DES and impaired esophagogastric junction relaxation.
    • Peroral endoscopic myotomy (POEM) ^[2][4][10]
    • Endoscopic pneumatic dilation ^[4][5][10]
• Surgery: extended/long LES myotomy ^{[5][11] [4]}
  • Indication: persistent symptoms despite pharmacological and endoscopic therapy
  • Procedure
    • May be performed laparoscopically or via an open abdominal/thoracoabdominal approach
    • An incision extending from the LES into the esophageal body is created.
    • Typically combined with a fundoplication procedure to minimize gastroesophageal reflux
  • Evidence is currently lacking on the advantages of surgical intervention over endoscopic therapy. ^[4]

• Rule out immediately life-threatening causes of chest pain.
• Administer a smooth muscle relaxants (e.g., calcium channel blocker, nitrate, or PDE5 inhibitor)
• Consider administering a tricyclic antidepressant and/or a PPI if symptoms persist.
• Consult gastroenterology for further workup.