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Nutcracker esophagus

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Diffuse esophageal spasm; corkscrew esophagus

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Nutcracker esophagus (diffuse esophageal spasm or corkscrew esophagus) is a disorder of the movement of the esophagus, and is one of many motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. It causes difficulty swallowing, or dysphagia, to both solid and liquid foods, and can cause chest pain; it may also have no symptoms. Nutcracker esophagus can affect people of any age, but is more common in the 6th and 7th decades of life. The diagnosis is made by an esophageal motility study, which evaluates the pressure of the esophagus at various points along its length. The term “nutcracker esophagus” comes from the finding of increased pressures during peristalsis, with a diagnosis made when pressures exceed 180 mmHg; this has been linked to the pressure of a mechanical nutcracker. The disorder does not progress, and is not associated with any complications; as a result, treatment of nutcracker esophagus targets control of symptoms only.[1]

Historical Perspective

Classification

Pathophysiology

Causes

The cause of diffuse esophageal spasm is unknown. Very hot or very cold foods may trigger an episode in some people.

Differentiating Nutcracker esophagus overview from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Endoscopy

Endoscopy is typically normal in patients with nutcracker esophagus; however, abnormalities associated with gastroesophageal reflux disease, or GERD, which associates with nutcracker esophagus, may be seen.[2] Studies on endoscopic ultrasound show a slight trend towards thickening of the muscularis propria of the esophagus in nutcracker esophagus, but this is not useful in making the diagnosis.[3]

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Pneumatic dilatation of the esophagus, which is an endoscopic technique where a high-pressure balloon is used to stretch the muscles of the LES, can be performed to improve symptoms.[1][4] In patients who have no response to medical or endoscopic therapy, surgery can be performed. A Heller myotomy involves an incision to disrupt the LES and the myenteric plexus that innervates it. It is used as a final treatment option in patients who do not respond to other therapies.[1][5][6]

Prevention

References

  1. ↑ 1.0 1.1 1.2 Tutuian R, Castell D (2006). “Esophageal motility disorders (distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): modern management”. Curr Treat Options Gastroenterol. 9 (4): 283–94. PMID 16836947.
  2. ↑ Fang J, Bjorkman D (2002). “Nutcracker esophagus: GERD or an esophageal motility disorder”. Am J Gastroenterol. 97 (6): 1556–7. PMID 12094884.
  3. ↑ Melzer E, Ron Y, Tiomni E, Avni Y, Bar-Meir S (1997). “Assessment of the esophageal wall by endoscopic ultrasonography in patients with nutcracker esophagus”. Gastrointest Endosc. 46 (3): 223–5. PMID 9378208.
  4. ↑ Kahrilas P (2000). “Esophageal motility disorders: current concepts of pathogenesis and treatment”. Can J Gastroenterol. 14 (3): 221–31. PMID 10758419.
  5. ↑ Traube M, Tummala V, Baue A, McCallum R (1987). “Surgical myotomy in patients with high-amplitude peristaltic esophageal contractions. Manometric and clinical effects”. Dig Dis Sci. 32 (1): 16–21. PMID 3792178.
  6. ↑ Richter J, Castell D (1987). “Surgical myotomy for nutcracker esophagus. To be or not to be?”. Dig Dis Sci. 32 (1): 95–6. PMID 3792184.

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Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Historical Perspective

Discovery

  • [Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].
  • The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
  • In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
  • In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

Outbreaks

  • There have been several outbreaks of [disease name], which are summarized below:

Landmark Events in the Development of Treatment Strategies

  • In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Impact on Cultural History

Famous Cases

References

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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

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Overview

Classification

References

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Pathology specimens of the esophagus in patients with nutcracker esophagus show no significant abnormality, unlike patients with achalasia where destruction of the myenteric plexus is seen. This has led to the thought that the pathophysiology of nutcracker esophagus may be related to abnormalities in neurotransmitters or other mediators in the distal esophagus. Abnormalities in nitric oxide levels, which have been seen in achalasia are postulated as the primary abnormality.[1][2] As GERD is associated with nutcracker esophagus, it has also been hypothesized that the alterations in nitric oxide and other released chemicals may be a response to reflux.[3]

References

  1. ↑ Tutuian R, Castell D (2006). “Esophageal motility disorders (distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): modern management”. Curr Treat Options Gastroenterol. 9 (4): 283–94. PMID 16836947.
  2. ↑ Kahrilas P (2000). “Esophageal motility disorders: current concepts of pathogenesis and treatment”. Can J Gastroenterol. 14 (3): 221–31. PMID 10758419.
  3. ↑ Pilhall M, Börjesson M, Rolny P, Mannheimer C (2002). “Diagnosis of nutcracker esophagus, segmental or diffuse hypertensive patterns, and clinical characteristics”. Dig Dis Sci. 47 (6): 1381–8. PMID 12064816.

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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

The cause of diffuse esophageal spasm is unknown. Very hot or very cold foods may trigger an episode in some people.

Causes

References

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Differentiating Nutcracker esophagus from Other Diseases

Differentiating Nutcracker esophagus from Other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

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Overview

Differential Diagnosis

Nutcracker esophagus must be differentiated from other causes of dysphagia, odynophagia and food regurgitation such as GERD, esophageal adenocarcinoma and esophageal stricture.

Manifestations Diagnostic tools
Achalasia
  • Dysphagia for solids and liquids is the most common feature, being seen in 91 % and 85% of patients respectively[2]
  • Regurgitation of undigested food occurs in 76-91% of patients[2]
  • Cough mainly when lying down in 30%[2]
  • Esophagogastroduodenoscopy findings include a dilated esophagus with residual food fragments, normal mucosa and occasionally candidiasis (due to the prolonged stasis).
  • Barium swallow shows the characteristic bird’s beak appearance.
Barium swallow showing bird’s beak appearance – By Farnoosh Farrokhi, Michael F. Vaezi. – Idiopathic (primary) achalasia. Orphanet Journal of Rare Diseases 2007, 2:38(http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2040141), CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=2950922
GERD
  • Retrosternal burning chest pain.
  • Cough and hoarseness of voice.
  • May present with complications such as strictures and dysphagia.[3]
  • Upper GI endoscopy shows the complications such as esophagitis and barret esophagus.
  • Esophageal manometry may show decreased tone of the lower esophageal sphincter.
  • 24-hour esophageal pH monitoring may be done to confirm the diagnosis.
Barret’s esophagus – By Samir धर्म – taken from patient with permission to place in public domain, Copyrighted free use, https://commons.wikimedia.org/w/index.php?curid=1595945
Esophageal carcinoma
  • Dysphagia
  • Odynophagia– fluids and soft foods are usually tolerated, while hard or bulky substances (such as bread or meat) cause much more difficulty[4]
  • Weight loss
  • Pain, often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in character
  • Nausea and vomiting[4]
  • Upper GI endoscopy and esophageal biopsy the gold standard for the diagnosis of esophageal
CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=2587715
Corckscrew esophagus
  • Retrosternal chest pain that presents with or without food intake.[5]
  • The condition is not progressive and not causing complications.[6]
  • Barium swallow shows the characteristic corckscrew appearance of the esophagus.
Corckscrew esophagus – Case courtesy of Radswiki, Radiopaedia.org, rID: 11680
Esophageal stricture
  • Patient may present with the symptoms of the underlying GERD.
  • Dysphagia and odynophagia.[7]
  • Barium esophagography provides information about the site and the diameter of the stricture before the endoscopic intervention.[8]
Peptic stricture – By Samir धर्म – From en.wikipedia.org, Public Domain, https://commons.wikimedia.org/w/index.php?curid=1931423
Plummer-Vinson syndrome Common symptoms of Plummer-Vinson syndrome include:[9][10][11]
  • Difficulty swallowing (more for solids)
  • Weakness
  • Pain
  • Burning sensation in mouth
  • Dry tongue
  • Painful cracks in the angles of a dry mouth
  • Pale color of the skin

Less cmmon symptoms

  • Cold intolerance
  • Reduced resistance to infection
  • Altered behavior
  • Craving for for unusual items (such as ice or cold vegetables)
 Lab tests are consistent with the diagnosis of iron deficiency anemia.

Findings on an x-ray (barium esophagogram) suggestive of esophageal web/strictures associated with Plummer-Vinson syndrome appear as either:

Plummer-Vinson syndrome (Source: Case courtesy of Dr Hani Salam, <a href=”https://radiopaedia.org/“>Radiopaedia.org</a>. From the case <a href=”https://radiopaedia.org/cases/14029“>rID: 14029</a>)

References

  1. ↑ Ferri, Fred (2015). Ferri’s clinical advisor 2015 : 5 books in 1. Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751.
  2. ↑ 2.0 2.1 2.2 Boeckxstaens GE, Zaninotto G, Richter JE (2013). “Achalasia”. Lancet. doi:10.1016/S0140-6736(13)60651-0. PMID 23871090.
  3. ↑ Badillo R, Francis D (2014). “Diagnosis and treatment of gastroesophageal reflux disease”. World J Gastrointest Pharmacol Ther. 5 (3): 105–12. doi:10.4292/wjgpt.v5.i3.105. PMC 4133436. PMID 25133039.
  4. ↑ 4.0 4.1 Napier KJ, Scheerer M, Misra S (2014). “Esophageal cancer: A Review of epidemiology, pathogenesis, staging workup and treatment modalities”. World J Gastrointest Oncol. 6 (5): 112–20. doi:10.4251/wjgo.v6.i5.112. PMC 4021327. PMID 24834141.
  5. ↑ Matsuura H (2017). “Diffuse Esophageal Spasm: Corkscrew Esophagus”. Am. J. Med. doi:10.1016/j.amjmed.2017.08.041. PMID 28943381.
  6. ↑ Lassen JF, Jensen TM (1992). “[Corkscrew esophagus]”. Ugeskr. Laeg. (in Danish). 154 (5): 277–80. PMID 1736462.
  7. ↑ Ruigómez A, García Rodríguez LA, Wallander MA, Johansson S, Eklund S (2006). “Esophageal stricture: incidence, treatment patterns, and recurrence rate”. Am. J. Gastroenterol. 101 (12): 2685–92. doi:10.1111/j.1572-0241.2006.00828.x. PMID 17227515.
  8. ↑ Shami VM (2014). “Endoscopic management of esophageal strictures”. Gastroenterol Hepatol (N Y). 10 (6): 389–91. PMC 4080876. PMID 25013392.
  9. ↑ López Rodríguez MJ, Robledo Andrés P, Amarilla Jiménez A, Roncero Maíllo M, López Lafuente A, Arroyo Carrera I (2002). “Sideropenic dysphagia in an adolescent”. J. Pediatr. Gastroenterol. Nutr. 34 (1): 87–90. PMID 11753173.
  10. ↑ Chisholm M (1974). “The association between webs, iron and post-cricoid carcinoma”. Postgrad Med J. 50 (582): 215–9. PMC 2495558. PMID 4449772.
  11. ↑ Larsson LG, Sandström A, Westling P (1975). “Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden”. Cancer Res. 35 (11 Pt. 2): 3308–16. PMID 1192404.

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Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

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Overview

Epidemiology and Demographics

References

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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

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Overview

Risk Factors

References

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Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

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Overview

Screening

References

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Natural History, Complications, and Prognosis

Natural History, Complications, and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Natural History

Complications

The condition may not respond to treatment.

Prognosis

A diffuse esophageal spasm may come and go (intermittent) or last for a long time (chronic). Medicine can help relieve symptoms.

References

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1


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