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Esophageal hypermotility disorders

Last updated: March 5, 2021

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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. [1]

  • 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 [2][3]
  • Premature esophageal contractions: contractions with a distal latency of < 4.5 seconds between them (see ''Diagnostics'')

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. [4]

  • Distal esophageal spasm is thought to be caused by impaired inhibitory innervation. [1][3]
  • Hypercontractile esophagus is thought to be caused by excessive cholinergic drive. [3]
  • GERD may play a causative and/or aggravating role in esophageal hypermotility disorders. [5]

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) [1][4]

  • Intermittent dysphagia to liquids (and potentially, solids) [6]
  • 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.

Approach [1][3][4]

Imaging

Imaging is the mainstay of diagnosis for esophageal hypermotility disorders. Laboratory studies may be considered to rule out other diagnoses (see “Diagnostics” in acute chest pain).

Upper endoscopy [1][4][7]

  • Indication: preferred first-line test in the workup of dysphagia predominantly to solids [7]
  • Findings: typically normal in hypermotility disorders

Esophageal barium swallow [1][3][4]

High-resolution esophageal manometry (HRM) [1][4][8]

Conventional esophageal manometry [1][2]

  • 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 [2]

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!

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 [4]

  • 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 [3][9][10]

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

Invasive therapy [3][4][10]

  • 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.
  • Surgery: extended/long LES myotomy [4][10] [3]
    • 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. [3]
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  2. John Cameron, Andrew Cameron. Current Surgical Therapy 13th Edition. Elsevier ; 2019
  3. Rohof WOA, Bredenoord AJ. Chicago Classification of Esophageal Motility Disorders: Lessons Learned. Curr Gastroenterol Rep. 2017; 19 (8). doi: 10.1007/s11894-017-0576-7 . | Open in Read by QxMD
  4. Roman S, Kahrilas PJ. Management of Spastic Disorders of the Esophagus. Gastroenterol Clin North Am. 2013; 42 (1): p.27-43. doi: 10.1016/j.gtc.2012.11.002 . | Open in Read by QxMD
  5. Abdel Jalil AA, Katzka DA, Castell DO. Approach to the Patient with Dysphagia. Am J Med. 2015; 128 (10): p.1138.e17-1138.e23. doi: 10.1016/j.amjmed.2015.04.026 . | Open in Read by QxMD
  6. Bredenoord AJ, Fox M, Kahrilas PJ, et al. Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography1. Neurogastroenterology & Motility. 2012; 24 : p.57-65. doi: 10.1111/j.1365-2982.2011.01834.x . | Open in Read by QxMD
  7. Clément M, Zhu WJ, Neshkova E, Bouin M. Jackhammer Esophagus: From Manometric Diagnosis to Clinical Presentation. Canadian Journal of Gastroenterology and Hepatology. 2019; 2019 : p.1-7. doi: 10.1155/2019/5036160 . | Open in Read by QxMD
  8. Carlson DA, Ravi K, Kahrilas PJ, et al. Diagnosis of Esophageal Motility Disorders: Esophageal Pressure Topography vs. Conventional Line Tracing. Am J Gastroenterol. 2015; 110 (7): p.967-977. doi: 10.1038/ajg.2015.159 . | Open in Read by QxMD
  9. Khalaf M, Chowdhary S, Elias PS, Castell D. Distal Esophageal Spasm: A Review. Am J Med. 2018; 131 (9): p.1034-1040. doi: 10.1016/j.amjmed.2018.02.031 . | Open in Read by QxMD
  10. Kahrilas PJ, Bredenoord AJ, Carlson DA, Pandolfino JE. Advances in Management of Esophageal Motility Disorders. Clinical Gastroenterology and Hepatology. 2018; 16 (11): p.1692-1700. doi: 10.1016/j.cgh.2018.04.026 . | Open in Read by QxMD
  11. Achem SR, Gerson LB. Distal Esophageal Spasm: An Update. Curr Gastroenterol Rep. 2013; 15 (9). doi: 10.1007/s11894-013-0325-5 . | Open in Read by QxMD
  12. Chumpitazi BP, Kearns GL, Shulman RJ. Review article: the physiological effects and safety of peppermint oil and its efficacy in irritable bowel syndrome and other functional disorders.. Aliment Pharmacol Ther. 2018; 47 (6): p.738-752. doi: 10.1111/apt.14519 . | Open in Read by QxMD
  13. Viazis N, Karamanolis G, Vienna E, Karamanolis DG. Selective-serotonin reuptake inhibitors for the treatment of hypersensitive esophagus.. Therapeutic advances in gastroenterology. 2011; 4 (5): p.295-300. doi: 10.1177/1756283X11409279 . | Open in Read by QxMD