Nutcracker esophagus

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Nutcracker esophagus
Other namesJackhammer esophagus, hypercontractile peristalsis, nutcracker achalasia
Peristaltic.jpg
Normal peristalsis in time space graph. Nutcracker esophagus shows higher amplitude contractions (Z-axis) that take longer to pass (X-axis)
Specialty Gastroenterology   OOjs UI icon edit-ltr-progressive.svg

Nutcracker esophagus, jackhammer esophagus, or hypercontractile peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or duration. Nutcracker esophagus is one of several motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. It causes difficulty swallowing (dysphagia) with both solid and liquid foods, and can cause significant chest pain; it may also be asymptomatic. Nutcracker esophagus can affect people of any age but is more common in the sixth and seventh decades of life.

Contents

The diagnosis is made by an esophageal motility study (esophageal manometry), 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 likened 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] [2]

Signs and symptoms

Nutcracker esophagus is characterized as a motility disorder of the esophagus, meaning that it is caused by abnormal movement, or peristalsis of the esophagus. [2] People with motility disorders present with two main symptoms: chest pain or difficulty with swallowing. Chest pain is the more common. The chest pain is very severe and intense, and mimics cardiac chest pain. [3] [4] [5] [6] It may spread into the arm and back. The symptoms of nutcracker esophagus are intermittent, and may occur with or without food. [2] Rarely, patients can present with a sudden obstruction of the esophagus after eating food (termed a food bolus obstruction, or the 'steakhouse syndrome') requiring urgent treatment. The disorder does not progress to produce worsening symptoms or complications, unlike other motility disorders (such as achalasia) or anatomical abnormalities of the esophagus (such as peptic strictures or esophageal cancer). Many patients with nutcracker esophagus do not have any symptoms at all, as esophageal manometry studies done on patients without symptoms may show the same motility findings as nutcracker esophagus. [2] Nutcracker esophagus may also be associated with metabolic syndrome. The incidence of nutcracker esophagus in all patients is uncertain. [7]

Pathophysiology

Pathology specimens of the esophagus in patients with nutcracker esophagus show no significant abnormality, unlike patients with achalasia, where destruction of the Auerbach's plexus is seen. 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. [2] [8] As GERD is associated with nutcracker esophagus, the alterations in nitric oxide and other released chemicals may be in response to reflux. [9]

Diagnosis

In patients who have dysphagia, testing may first be done to exclude an anatomical cause of dysphagia, such as distortion of the anatomy of the esophagus. This usually includes visualization of the esophagus with an endoscope, and can also include barium swallow X-rays of the esophagus. 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. [10] Barium swallow in nutcracker esophagus is also typically normal, [2] but may provide a definitive diagnosis if contrast is given in tablet or granule form. Studies on endoscopic ultrasound show slight trends toward thickening of the muscularis propria of the esophagus in nutcracker esophagus, but this is not useful in making the diagnosis. [11]

Esophageal motility studies

Diagram of esophageal motility study in nutcracker esophagus: The disorder shows peristalsis with high-pressure esophageal contractions exceeding 180 mmHg and contractile waves with a long duration exceeding 6 sec. Nutcracker manometry.jpg
Diagram of esophageal motility study in nutcracker esophagus: The disorder shows peristalsis with high-pressure esophageal contractions exceeding 180 mmHg and contractile waves with a long duration exceeding 6 sec.
Normal esophagus in (A). Nutcracker esophagus in (C): high-pressure waves in blue; cross-sectional areas (CSA) in fucsia. Esofago Distencion Contraccion Nutcracker.png
Normal esophagus in (A).
Nutcracker esophagus in (C): high-pressure waves in blue; cross-sectional areas (CSA) in fucsia.

The diagnosis of nutcracker esophagus is typically made with an esophageal motility study, which shows characteristic features of the disorder. Esophageal motility studies involve pressure measurements of the esophagus after a patient takes a wet (fluid-containing) or dry (solid-containing) swallow. Measurements are usually taken at various points in the esophagus. [12]

Nutcracker esophagus is characterized by a number of criteria described in the literature. The most commonly used criteria are the Castell criteria, named after American gastroenterologist D.O. Castell. The Castell criteria include one major criterion: a mean peristaltic amplitude in the distal esophagus of more than 180 mm Hg. The minor criterion is the presence of repetitive contractions (meaning two or more) that are greater than six seconds in duration. Castell also noted that the lower esophageal sphincter relaxes normally in nutcracker esophagus, but has an elevated pressure of greater than 40 mm Hg at baseline. [2] [12] [13] [9]

Three other criteria for the definition of the nutcracker esophagus have been defined. The Gothenburg criterion consists of the presence of peristaltic contractions, with an amplitude of 180 mm Hg at any place in the esophagus. [10] [9] The Richter criterion involves the presence of peristaltic contractions with an amplitude of greater than 180 mm Hg from an average of measurements taken 3 and 8 cm above the lower esophageal sphincter. It has been incorporated into a number of clinical guidelines for the evaluation of dysphagia. [9] The Achem criteria are more stringent, and are an extension of the study of 93 patients used by Richter and Castell in the development of their criteria, and require amplitudes of greater than 199 mm Hg at 3 cm above the lower esophageal sphincter (LES), greater than 172 mm Hg at 8 cm above the LES, or greater than 102 mm Hg at 13 cm above the LES. [9] [14]

Treatment

People are usually reassured that the disease is unlikely to worsen. However, the symptoms of chest pain and trouble swallowing may be severe enough to require treatment with medications, and rarely, surgery.

The initial step of treatment focuses on reducing risk factors. While weight reduction may be useful in reducing symptoms, the role of acid suppression therapy to reduce esophageal reflux is still uncertain. [15] Very cold and very hot beverages may trigger esophageal spasms. [16] [17]

Medications

Medications for nutcracker esophagus includes the use of calcium-channel blockers, which relax the lower esophageal sphincter (LES) and palliate the dysphagia symptoms. Diltiazem, a calcium-channel blocker, has been used in randomized control studies with good effect. Nitrate medications, including isosorbide dinitrate, given before meals, may also help relax the LES and improve symptoms. [2] The inexpensive generic combination of belladonna and phenobarbital (Donnatal and other brands) may be taken three times daily as a tablet to prevent attacks or, for patients with only occasional episodes, as an elixir at the onset of symptoms. Phosphodiesterase inhibitors, such as sildenafil, can be given to reduce symptoms, particularly pain, but small trials have not been able to demonstrate clinical improvement. [18] [19]

Procedures

Endoscopic therapy with botulinum toxin can also be used to improve dysphagia which stabilizes unintentional weight loss, but the effect has limited effect on other symptoms, including pain, while also being a temporary treatment lasting a few weeks. [20] Finally, 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, but again no clinical improvement is seen in regards to motility. [21]

In people 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. The Heller myotomy is used as a final treatment option in patients who do not respond to other therapies. [22]

Prognosis

Nutcracker esophagus is a benign, nonprogressive condition, meaning it is not associated with significant complications.

See also

Related Research Articles

<span class="mw-page-title-main">Gastroenterology</span> Branch of medicine focused on the digestive system and its disorders

Gastroenterology is the branch of medicine focused on the digestive system and its disorders. The digestive system consists of the gastrointestinal tract, sometimes referred to as the GI tract, which includes the esophagus, stomach, small intestine and large intestine as well as the accessory organs of digestion which include the pancreas, gallbladder, and liver.

<span class="mw-page-title-main">Esophageal achalasia</span> Rare, incurable, progressive motility disorder due to failure of esophogeal motor neurons

Esophageal achalasia, often referred to simply as achalasia, is a failure of smooth muscle fibers to relax, which can cause the lower esophageal sphincter to remain closed. Without a modifier, "achalasia" usually refers to achalasia of the esophagus. Achalasia can happen at various points along the gastrointestinal tract; achalasia of the rectum, for instance, may occur in Hirschsprung's disease. The lower esophageal sphincter is a muscle between the esophagus and stomach that opens when food comes in. It closes to avoid stomach acids from coming back up. A fully understood cause to the disease is unknown, as are factors that increase the risk of its appearance. Suggestions of a genetically transmittable form of achalasia exist, but this is neither fully understood, nor agreed upon.

Heartburn, also known as pyrosis, cardialgia or acid indigestion, is a burning sensation in the central chest or upper central abdomen. Heartburn is usually due to regurgitation of gastric acid into the esophagus. It is the major symptom of gastroesophageal reflux disease (GERD).

<span class="mw-page-title-main">Dysphagia</span> Difficulty in swallowing

Dysphagia is difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right.

An esophageal motility disorder (EMD) is any medical disorder resulting from dysfunction of the coordinated movement of esophagus, which causes dysphagia.

<span class="mw-page-title-main">Gastroesophageal reflux disease</span> Flow of stomach contents into the esophagus

Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is a chronic upper gastrointestinal disease in which stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/or complications. Symptoms include dental corrosion, dysphagia, heartburn, odynophagia, regurgitation, non-cardiac chest pain, extraesophageal symptoms such as chronic cough, hoarseness, reflux-induced laryngitis, or asthma. In the long term, and when not treated, complications such as esophagitis, esophageal stricture, and Barrett's esophagus may arise.

<span class="mw-page-title-main">Esophageal motility study</span> Medical test

An esophageal motility study (EMS) or esophageal manometry is a test to assess motor function of the upper esophageal sphincter (UES), esophageal body and lower esophageal sphincter (LES).

<span class="mw-page-title-main">Hiatal hernia</span> Entrance of abdominal organs into the middle chest through the diaphragm

A hiatal hernia or hiatus hernia is a type of hernia in which abdominal organs slip through the diaphragm into the middle compartment of the chest. This may result in gastroesophageal reflux disease (GERD) or laryngopharyngeal reflux (LPR) with symptoms such as a taste of acid in the back of the mouth or heartburn. Other symptoms may include trouble swallowing and chest pains. Complications may include iron deficiency anemia, volvulus, or bowel obstruction.

<span class="mw-page-title-main">Nissen fundoplication</span> Surgical procedure to treat gastric reflux and hiatal hernia

A Nissen fundoplication, or laparoscopic Nissen fundoplication when performed via laparoscopic surgery, is a surgical procedure to treat gastroesophageal reflux disease (GERD) and hiatal hernia. In GERD, it is usually performed when medical therapy has failed; but, with a Type II (paraesophageal) hiatus hernia, it is the first-line procedure. The Nissen fundoplication is total (360°), but partial fundoplications known as Thal, Belsey, Dor, Lind, and Toupet fundoplications are alternative procedures with somewhat different indications and outcomes.

<span class="mw-page-title-main">Esophageal web</span> Abnormal membrane in the esophagus

Esophageal webs are thin membranes occurring anywhere along the esophagus.

<span class="mw-page-title-main">Eosinophilic esophagitis</span> Allergic inflammatory condition of the esophagus

Eosinophilic esophagitis (EoE) is an allergic inflammatory condition of the esophagus that involves eosinophils, a type of white blood cell. In healthy individuals, the esophagus is typically devoid of eosinophils. In EoE, eosinophils migrate to the esophagus in large numbers. When a trigger food is eaten, the eosinophils contribute to tissue damage and inflammation. Symptoms include swallowing difficulty, food impaction, vomiting, and heartburn.

Esophageal dysphagia is a form of dysphagia where the underlying cause arises from the body of the esophagus, lower esophageal sphincter, or cardia of the stomach, usually due to mechanical causes or motility problems.

Esophageal spasm is a disorder of motility of the esophagus.

<span class="mw-page-title-main">Diffuse esophageal spasm</span> Uncoordinated contractions of the esophagus

Diffuse esophageal spasm (DES), also known as distal esophageal spasm, is a condition characterized by uncoordinated contractions of the esophagus, which may cause difficulty swallowing (dysphagia) or regurgitation. In some cases, it may cause symptoms such as chest pain, similar to heart disease. In many cases, the cause of DES remains unknown.

<span class="mw-page-title-main">Esophageal food bolus obstruction</span> Blockage of the esophagus by an ingested foreign body

An esophageal food bolus obstruction is a medical emergency caused by the obstruction of the esophagus by an ingested foreign body.

Cricopharyngeal spasms occur in the cricopharyngeus muscle of the pharynx. Cricopharyngeal spasm is an uncomfortable but harmless and temporary disorder.

<span class="mw-page-title-main">Esophageal intramural pseudodiverticulosis</span> Medical condition

Esophageal intramucosal pseudodiverticulosis (EIPD) is a rare condition wherein the esophagus wall develops numerous small outpouchings (pseudodiverticulae). Individuals with the condition typically develop difficulty swallowing. The outpouchings represent the ducts of the submucosal glands of the esophagus. It typically affects individuals in their sixth and seventh decades of life. While it is associated with certain chronic conditions, particularly alcoholism, diabetes and gastroesophageal reflux disease, the cause of the condition is unknown. Treatment involves medications to treat concomitant conditions such as reflux esophageal spasm, and dilation of strictures in the esophagus.

<span class="mw-page-title-main">Lymphocytic esophagitis</span> Medical condition

Lymphocytic esophagitis is a rare and poorly understood medical disorder involving inflammation in the esophagus. The disease is named from the primary inflammatory process, wherein lymphocytes are seen within the esophageal mucosa. Symptoms of the condition include difficulty swallowing, heartburn and food bolus obstruction. The condition was first described in 2006 by Rubio and colleagues. Initial reports questioned whether this was a true medical disorder, or whether the inflammation was secondary to another condition, such as gastroesophageal reflux disease.

Functional Lumen Imaging Probe (FLIP) is a test used to evaluate the function of the esophagus, by measuring the dimensions of the esophageal lumen using impedance planimetry. Typically performed with sedation during upper endoscopy, FLIP is used to evaluate for esophageal motility disorders, such as achalasia, diffuse esophageal spasm, etc.

Esophagogastric junction outflow obstruction (EGJOO) is an esophageal motility disorder characterized by increased pressure where the esophagus connects to the stomach at the lower esophageal sphincter. EGJOO is diagnosed by esophageal manometry. However, EGJOO has a variety of etiologies; evaluating the cause of obstruction with additional testing, such as upper endoscopy, computed tomography, or endoscopic ultrasound may be necessary. When possible, treatment of EGJOO should be directed at the cause of obstruction. When no cause for obstruction is found, observation alone may be considered if symptoms are minimal. Functional EGJOO with significant or refractory symptoms may be treated with pneumatic dilation, per-oral endoscopic myotomy (POEM), or botulinum toxin injection.

References

  1. ↑ Esophageal Spasm at eMedicine
  2. 1 2 3 4 5 6 7 8 Tutuian, Radu; Castell, Donald O. (2006). "Esophageal motility disorders (Distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): Modern management". Current Treatment Options in Gastroenterology. 9 (4): 283–94. doi:10.1007/s11938-006-0010-y. PMID   16836947. S2CID   37079411.
  3. ↑ "Heartburn or Heart Attack? How to Tell the Difference". health.com. Retrieved 17 April 2018.
  4. ↑ "Esophageal spasms - Symptoms and causes". mayoclinic.org. Retrieved 17 April 2018.
  5. ↑ Schmeck, Harold M. Jr. (19 January 1989). "HEALTH: SYMPTOMS AND DIAGNOSIS; When Chest Pains Have Nothing to Do With a Heart Attack". The New York Times . Retrieved 17 April 2018.
  6. ↑ "Heart Attack and Conditions That Mimic Heart Attack: Learn About Chest Pain". www.secondscount.org. 30 November 2008. Retrieved 17 April 2018.
  7. ↑ Savarino, E; Smoutcorresponding, A (November 2020). "The hypercontractile esophagus: Still a tough nut to crack". Neurogastroenterology and Motility. 32 (11): e14010. doi:10.1111/nmo.14010. PMC   7685127 . PMID   33043556.
  8. ↑ Kahrilas, P. J. (2000). "Esophageal motility disorders: Current concepts of pathogenesis and treatment". Canadian Journal of Gastroenterology. 14 (3): 221–31. doi: 10.1155/2000/389709 . PMID   10758419.
  9. 1 2 3 4 5 Pilhall, M.; Börjesson, M.; Rolny, P.; Mannheimer, C. (2002). "Diagnosis of nutcracker esophagus, segmental or diffuse hypertensive patterns, and clinical characteristics". Digestive Diseases and Sciences. 47 (6): 1381–8. doi:10.1023/A:1015343119090. PMID   12064816. S2CID   20665774.
  10. 1 2 Fang, J; Bjorkman, D (2002). "Nutcracker esophagus: GERD or an esophageal motility disorder". The American Journal of Gastroenterology. 97 (6): 1556–7. doi:10.1016/S0002-9270(02)04159-X. PMID   12094884.
  11. ↑ Meizer, Ehud; Ron, Yishay; Tiomni, Elisa; Avni, Yona; Bar-Meier, Simon (1997). "Assessment of the esophageal wall by endoscopic ultrasonography in patients with nutcracker esophagus". Gastrointestinal Endoscopy. 46 (3): 223–5. doi:10.1016/S0016-5107(97)70090-7. PMID   9378208.
  12. 1 2 Cockeram, A. W. (1998). "Canadian Association of Gastroenterology Practice Guidelines: Evaluation of dysphagia". Canadian Journal of Gastroenterology. 12 (6): 409–13. doi: 10.1155/1998/303549 . PMID   9784896.
  13. ↑ Ott, D. J. (1994). "Motility disorders of the esophagus". Radiologic Clinics of North America. 32 (6): 1117–34. PMID   7972703.
  14. ↑ Achem, S. R.; Kolts, B. E.; Burton, L (1993). "Segmental versus diffuse nutcracker esophagus: An intermittent motility pattern". The American Journal of Gastroenterology. 88 (6): 847–51. PMID   8503378.
  15. ↑ Borjesson, M.; Rolny, P.; Mannheimer, C.; Pilhall, M. (2003). "Nutcracker oesophagus: A double-blind, placebo-controlled, cross-over study of the effects of lansoprazole". Alimentary Pharmacology and Therapeutics. 18 (11–12): 1129–35. doi: 10.1046/j.1365-2306.2003.01788.x . PMID   14653833. S2CID   31592531.
  16. ↑ "Esophageal spasm: MedlinePlus Medical Encyclopedia". Nlm.nih.gov. Retrieved 2013-01-15.
  17. ↑ "Esophageal Spasm Causes, Symptoms, Treatments, and More". Webmd.com. 2011-06-01. Retrieved 2013-01-15.
  18. ↑ Eherer, A. J.; Schwetz, I; Hammer, H. F.; Petnehazy, T; Scheidl, S. J.; Weber, K; Krejs, G. J. (2002). "Effect of sildenafil on oesophageal motor function in healthy subjects and patients with oesophageal motor disorders". Gut. 50 (6): 758–64. doi:10.1136/gut.50.6.758. PMC   1773249 . PMID   12010875.
  19. ↑ "Sildenafil in Esophageal Motility Disorders".
  20. ↑ Vanuytsel, Tim; Bisschops, Raf; Farré, Ricard; Pauwels, Ans; Holvoet, Lieselot; Arts, Joris; Caenepeel, Philip; De Wulf, Dominiek; Mimidis, Kostas; Rommel, Nathalie; Tack, Jan (2013). "Botulinum Toxin Reduces Dysphagia in Patients with Nonachalasia Primary Esophageal Motility Disorders". Clinical Gastroenterology and Hepatology. 11 (9): 1115–1121.e2. doi:10.1016/j.cgh.2013.03.021. PMID   23591282.
  21. ↑ "Is there any objective benefit from therapeutic dilatation in patients with nutcracker esophagus ?".
  22. ↑ "Achalasia". The Lecturio Medical Concept Library. 14 October 2020. Retrieved 26 June 2021.
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