Nutcracker Esophagus

Nutcracker esophagus (hypertensive peristalsis) is a condition associated with poor swallowing of both solid and liquid foods due to very high pressures in the esophagus during the swallowing process. This condition is associated with gastroesophageal reflux disease in many patients.

This disorder stems from the following process: endocrine.

Presentation

The two main presentations for esophageal spasm and nutcracker esophagus are chest pain and dysphagia. The retrosternal pain caused by esophageal spasm can be severe and crushing, which is similar to cardiac pain. This makes it difficult to distinguish between the two conditions and many patients are misdiagnosed with angina pectoris instead of esophageal spasm. The pain is usually felt in the anterior chest, epigastrium, or throat. There might be radiation to the back, upper arms, or neck. Dysphagia may present in patients with diffuse esophageal spasm, which will manifest as regurgitation, cough, heartburn, and hoarseness. However, it is less common to present in nutcracker esophagus patients.

Workup

Esophageal spasm and nutcracker esophagus present similarly to cardiac problems. Therefore, it is important to distinguish between them in order to reach the right diagnosis.

Laboratory studies do not suffice in the diagnosis of esophageal spasm, especially in patients with unremarkable history and physical findings. Esophageal spasm is thought to be triggered by gastroesophageal reflux disease, which makes it important to monitor patient's pH. It is also essential to rule out diabetes by measuring blood glucose and hemoglobin A1C levels.

The best imaging study in the diagnosis of esophageal spasm is esophagram or barium swallow, which evaluates for nonpropulsive contractions in the esophagus and shows a corkscrew appearance with segmentation caused by a characteristic appearance of multiple simultaneous contractions. However, barium swallow studies in nutcracker esophagus may reveal only minimal nonspecific findings. 

Patients with diffuse esophageal spasm or nutcracker esophagus may demonstrate thickening of the esophagus and muscular hypertrophy on CT scans. However, this may be seen in many other conditions as well and should prompt further workup.

Both the sensory and the motor functions of the esophagus can be assessed by catheter-based high-frequency intraluminal ultrasound imaging, which is useful in differentiating between achalasia, nutcracker esophagus, and diffuse esophageal spasm.

Associated disorders such as reflux esophagitis, strictures, and hiatal hernia may be revealed using upper endoscopy [6]. However, it is not helpful in diagnosing motility disorders of the esophagus.

The preferred method for the diagnosis of esophageal spasms is esophageal manometry [7]. High-resolution manometry will reveal continuous spatiotemporal representations of pressure through the esophagus, which offers better accuracy and greater detail for many of the important measurements of esophageal motor function [8] [9].

Treatment

Management of nutcracker esophagus consists of non-drug and drug treatment, as well as invasive or surgical intervention.

Non-drug treatment includes dietary modifications, reassurance of the patient that the chest pain is not related to a heart disease, and avoiding exacerbating factors, such as very cold and hot liquids.

Drug treatment involves several options, such as nitrates, calcium-channel blockers, antidepressants, peppermint oil, theophylline, phosphodiesterase inhibitors, and ruling out gastroesophageal reflux disease via a trial of proton pump inhibitor.

Invasive or surgical intervention may comprise several procedures, such as injection of Botox at the gastroesophageal junction, esophageal dilatation or myotomy. However, surgical treatment seems to be more effective in diffuse esophageal spasm than in nutcracker esophagus [10].

Prognosis

Evidence suggests that the prognosis of both nutcracker esophagus and diffuse esophageal spasm is good, and they are unlikely to progress to the more severe condition known as achalasia. Even though the prognosis is good, it is difficult to treat esophageal spasm and only a minority of patients found the treatment actually beneficial.

Etiology

The exact cause of nutcracker esophagus is unknown; however, different factors are involved, including nervous system abnormalities, visceral hypersensitivity, defect in the nitric oxide pathway, smooth muscle thickening in the esophageal wall and gastroesophageal reflux disease [2]. Several conditions have been reported to be associated with diffuse esophageal spasm, including depression and anxiety, alcoholic neuropathoes, diabetes mellitus, amyloidosis, scleroderma, and pseudo-obstruction [3].

Epidemiology

The incidence of nutcracker esophagus in the United States is about 1 case per 100,000 population per year [4]. Many patients may be misdiagnosed with reflux instead of esophageal spasm because of the similarity of the symptoms. Patients with mild simple symptoms may not seek medical intervention.

International statistics are not available for nutcracker esophagus because the symptoms are usually mild or even absent in most patients. The risk of developing esophageal spasm increases with age and it is rare in children. It is reported to be more common in females than in males, and white populations are affected to a higher extend. Nutcracker esophagus was diagnosed for the first time in the 1970s [4] [5].

Sex distribution
Age distribution

Pathophysiology

Dysphagia of solid or liquid food suggests the presence of a neuromuscular disorder; however, mechanical obstruction by a structural problem is suggested in case of only solid food dysphagia. Dysphagia due to esophageal spasm may be exacerbated by several factors, including loud noises, stress, and very cold or hot liquids, which stimulate muscular contractions.

When the amplitude of esophageal contractions exceed 2 standard deviations from normal, this may result in the developing of nutcracker esophagus. The most common presentation in patients suffering from this condition is chest pain; however, dysphagia is less presented in patients with nutcracker esophagus than in diffuse esophageal spasm patients.

Esophageal motor activity is initiated and controlled by different mechanisms, which are located peripherally within the intramural muscles and nerves, as well as within the central nervous system at different levels.

Prevention

It is not easy to prevent esophageal spasm because the causing factors are still not understood. However, changes in habits appear to decrease the risk of developing the condition, such as avoiding very hot or cold drinks. Treating conditions associated with the developing of esophageal spasm, such as gastroesophageal reflux disease, will decrease the risk of developing motility disorders of the esophagus.

Summary

There are two major variants of esophageal spasms; hypertensive peristalsis and diffuse esophageal spasm. The first one, which is also known as nutcracker esophagus, is characterized by esophageal contractions in a coordinated manner but with an excessive amplitude. The second variant is diagnosed when contractions are uncoordinated and with normal amplitude.

Nutcracker esophagus results in different symptoms including chest pain, difficulty swallowing both liquid and solid foods, and regurgitation. The clinical picture may be similar to that seen in cardiac patients, which makes it difficult to distinguish.

The best modality for diagnosing nutcracker esophagus is high-resolution manometry. Other diagnostic tools, such as upper endoscopy, ultrasound imaging and computed tomography (CT) scans, may be used. The diagnosis of nutcracker esophagus is established when the pressures on manometry exceed 180 mmHg.

Management of nutcracker esophagus targets control of symptoms and includes non-drug treatment with dietary modifications and reassurance; drug treatment with antidepressants, nitrates, and calcium-channel blockers; and invasive or surgical intervention with Botox injection, esophageal dilatation or myotomy [1].

Nutcracker esophagus may develop in all age groups; however, the risk increases with age, with most patients being in the sixth or seventh decade of their lives.

There are not enough statistics about the international incidence of nutcracker esophagus; however, it is reported to be 1 per 100,000 per year in the United States. It also seems to be more common among women and in white populations.

Nutcracker esophagus has a good prognosis and no complications; however, it is difficult to treat and patients rarely benefit from medication.

There is an association between gastroesophageal reflux disease (GERD) and the development of nutcracker esophagus. The management of GERD will minimize the risk of developing the condition. Very hot or cold drinks should also be avoided to prevent esophageal spasms.

Patient Information

Nutcracker esophagus is an abnormality in which the swallowing process is associated with very high pressures and powerful contractions in the esophagus, resulting in poor swallowing of both liquid and solid foods.

Causes

The cause of nutcracker esophagus us unknown; however, it could be connected to other conditions such as gastroesophageal reflux disease, diabetes mellitusdepression, and anxiety.

Presentation

Symptoms displayed in nutcracker esophagus are similar to the ones seen in heart diseases, such as chest pain, heartburn, and difficulty swallowing. Even though there is no physical blockage of the esophagus, symptoms of esophageal obstruction may develop.

Diagnosis

The best modality used in the diagnosis of nutcracker esophagus and other esophageal motility disorders is high-resolution manometry, which includes measurement of pressures in the esophagus. Gastroesophageal endoscopy may be needed to rule out gastroesophageal reflux disease and other causes of chest pain and dysphagia.

Management

Different treatment options may be incorporated in the management of nutcracker esophagus including drugs, such as nitroglycerin, antidepressants, and calcium-channel blockers. However, the condition is considered benign and usually does not require treatment. In rare cases, invasive or surgical intervention may be needed.

Outcome

Many patients do not find medical treatment useful and the prognosis for esophageal spasm is considered moderately good. It usually does not progress and it does not develop complications. Surgical intervention in patients with diffuse esophageal spasm relieves dysphagia and shows very good outcomes. However, the results are not as promising in patients with nutcracker esophagus.

Prevention

The main cause of esophageal spasm is still unknown, which makes prevention difficult. Avoiding very hot or cold drinks seems to decrease the risk of developing the condition. Successful management of conditions associated with esophageal spasm, such as gastroesophageal reflux disease, will also minimize the risk.

Self-assessment

References

  1. Tutuian R, Castell DO. Esophageal motility disorders (distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): modern management. Curr Treat Options Gastroenterol. July 2006; 9(4): 283-94.
  2. Lacy BE, Weiser K. Esophageal motility disorders: medical therapy. J Clin Gastroenterol. May-June 2008; 42(5): 652-8.
  3. Konturek T, Lembo A. Spasm, nutcracker, and IEM: real or manometry findings? J Clin Gastroenterol. May-June 2008; 42(5): 647-51.
  4. Floch M, et al. Esophageal Motility Disorders. In: Netter's Gastroenterology. 2nd ed. Philadelphia, Pa: Saunders; 2010: Chapter 14.
  5. Ferguson TB, Woodbury JD, Roper CL, et al. Giant muscular hypertrophy of the esophagus. Ann Thorac Surg. September 1969; 8(3): 209-18. 
  6. Roman S, Kahrilas PJ. Management of spastic disorders of the esophagus. Gastroenterol Clin North Am. March 2013; 42(1): 27-43. 
  7. Tutuian R, Castell DO. Review article: oesophageal spasm - diagnosis and management. Aliment Pharmacol Ther. Aliment Pharmacol Ther. May 15, 2006; 23(10): 1393-402.
  8. Carlson DA, Pandolfino JE. High-resolution manometry and esophageal pressure topography: filling the gaps of convention manometry. Gastroenterol Clin North Am. March 2013; 42(1): 1-15. 
  9. Carlson DA, Pandolfino JE. The Chicago criteria for esophageal motility disorders: what has changed in the past 5 years? Curr Opin Gastroenterol. July 2012; 28(4): 395-402. 
  10. Patti MG, Gorodner MV, Galvani C, et al. Spectrum of esophageal motility disorders: implications for diagnosis and treatment. Arch Surg. May 2005; 140(5): 442.

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