Nutcracker esophagus

Nutcracker esophagus, jackhammer esophagus, or

Nutcracker esophagus
hypercontractile peristalsis, is a disorder of the
movement of the esophagus characterized by Other Jackhammer esophagus,
contractions in the smooth muscle of the esophagus in a names hypercontractile peristalsis,
normal sequence but at an excessive amplitude or nutcracker achalasia
duration. Nutcracker esophagus is one of several
motility disorders of the esophagus, including achalasia
and diffuse esophageal spasm. It causes difficulty
swallowing, or 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.

The diagnosis is made by an esophageal motility study

(esophageal manometry), which evaluates the pressure
Normal peristalsis in time space graph.
of the esophagus at various points along its length. The
term "nutcracker esophagus" comes from the finding of Nutcracker esophagus shows higher amplitude
increased pressures during peristalsis, with a diagnosis contractions (Z-axis) that take longer to pass
made when pressures exceed 180 mmHg; this has been (X-axis)
likened to the pressure of a mechanical nutcracker. The Specialty Gastroenterology
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

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. Diagram of esophageal motility study
Castell. The Castell criteria include one major criterion: a mean in nutcracker esophagus: The
peristaltic amplitude in the distal esophagus of more than 180 mm disorder shows peristalsis with high-
Hg. The minor criterion is the presence of repetitive contractions pressure esophageal contractions
(meaning two or more) that are greater than six seconds in duration. exceeding 180 mmHg and contractile
Castell also noted that the lower esophageal sphincter relaxes waves with a long duration exceeding
normally in nutcracker esophagus, but has an elevated pressure of 6 sec.
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,
Normal esophagus in (A).
given before meals, may also help relax the LES and improve Nutcracker esophagus in (C): high-
symptoms.[2] The inexpensive generic combination of belladonna pressure waves in blue; cross-
and phenobarbital (Donnatal and other brands) may be taken three sectional areas (CSA) in fucsia.
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
Esophageal spasm

References
 1. Esophageal Spasm (https://emedicine.medscape.com/article/174975-overview) at
eMedicine
2. 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 (https://doi.org/10.1007%2Fs11938-006-0010-y).
PMID 16836947 (https://pubmed.ncbi.nlm.nih.gov/16836947). S2CID 37079411 (https://api.s
emanticscholar.org/CorpusID:37079411).
3. "Heartburn or Heart Attack? How to Tell the Difference" (http://www.health.com/health/conditi
on-article/0,,20278772,00.html). health.com. Retrieved 17 April 2018.
4. "Esophageal spasms - Symptoms and causes" (http://www.mayoclinic.org/diseases-conditio
ns/esophageal-spasms/basics/symptoms/con-20025653). 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" (https://www.nytimes.com/1989/
01/19/us/health-symptoms-diagnosis-when-chest-pains-have-nothing-with-heart-attack.htm
l). The New York Times. Retrieved 17 April 2018.
6. "Heart Attack and Conditions That Mimic Heart Attack: Learn About Chest Pain" (http://www.
secondscount.org/heart-condition-centers/info-detail-2/nonheart-attack-sources-of-chest-pai
n#.WB21uPl97IU). 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" (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7685127).
Neurogastroenterology and Motility. 32 (11): e14010. doi:10.1111/nmo.14010 (https://doi.or
g/10.1111%2Fnmo.14010). PMC 7685127 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7
685127). PMID 33043556 (https://pubmed.ncbi.nlm.nih.gov/33043556).
8. Kahrilas, P. J. (2000). "Esophageal motility disorders: Current concepts of pathogenesis and
treatment" (https://doi.org/10.1155%2F2000%2F389709). Canadian Journal of
Gastroenterology. 14 (3): 221–31. doi:10.1155/2000/389709 (https://doi.org/10.1155%2F200
0%2F389709). PMID 10758419 (https://pubmed.ncbi.nlm.nih.gov/10758419).
9. 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 (https://do
i.org/10.1023%2FA%3A1015343119090). PMID 12064816 (https://pubmed.ncbi.nlm.nih.go
v/12064816). S2CID 20665774 (https://api.semanticscholar.org/CorpusID:20665774).
10. 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 (https://doi.org/10.1016%2FS0002-9270%2802%2904159-X).
PMID 12094884 (https://pubmed.ncbi.nlm.nih.gov/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 (https://doi.org/10.1016%2FS0016-5107%2897%2970090-7).
PMID 9378208 (https://pubmed.ncbi.nlm.nih.gov/9378208).
12. Cockeram, A. W. (1998). "Canadian Association of Gastroenterology Practice Guidelines:
Evaluation of dysphagia" (https://doi.org/10.1155%2F1998%2F303549). Canadian Journal
of Gastroenterology. 12 (6): 409–13. doi:10.1155/1998/303549 (https://doi.org/10.1155%2F1
998%2F303549). PMID 9784896 (https://pubmed.ncbi.nlm.nih.gov/9784896).
13. Ott, D. J. (1994). "Motility disorders of the esophagus". Radiologic Clinics of North America.
32 (6): 1117–34. PMID 7972703 (https://pubmed.ncbi.nlm.nih.gov/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 (https://pubmed.ncbi.nlm.nih.gov/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" (https://doi.
org/10.1046%2Fj.1365-2306.2003.01788.x). Alimentary Pharmacology and Therapeutics.
18 (11–12): 1129–35. doi:10.1046/j.1365-2306.2003.01788.x (https://doi.org/10.1046%2Fj.1
365-2306.2003.01788.x). PMID 14653833 (https://pubmed.ncbi.nlm.nih.gov/14653833).
S2CID 31592531 (https://api.semanticscholar.org/CorpusID:31592531).
16. "Esophageal spasm: MedlinePlus Medical Encyclopedia" (https://www.nlm.nih.gov/medline
plus/ency/article/000289.htm). Nlm.nih.gov. Retrieved 2013-01-15.
17. "Esophageal Spasm Causes, Symptoms, Treatments, and More" (http://www.webmd.com/di
gestive-disorders/tc/esophageal-spasm-topic-overview). 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" (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC177324
9). Gut. 50 (6): 758–64. doi:10.1136/gut.50.6.758 (https://doi.org/10.1136%2Fgut.50.6.758).
PMC 1773249 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1773249). PMID 12010875
(https://pubmed.ncbi.nlm.nih.gov/12010875).
19. "Sildenafil in Esophageal Motility Disorders" (http://www.dpic.org/article/professional/sildena
fil-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" (http://www.cghjournal.org/article/S1542356513004618/pdf).
Clinical Gastroenterology and Hepatology. 11 (9): 1115–1121.e2.
doi:10.1016/j.cgh.2013.03.021 (https://doi.org/10.1016%2Fj.cgh.2013.03.021).
PMID 23591282 (https://pubmed.ncbi.nlm.nih.gov/23591282).
21. "Is there any objective benefit from therapeutic dilatation in patients with nutcracker
esophagus ?" (http://www.hon.ch/OESO/books/Vol_4_Prim_Motility/Articles/ART280.HTML
#R18).
22. "Achalasia" (https://www.lecturio.com/concepts/achalasia/). The Lecturio Medical Concept
Library. 14 October 2020. Retrieved 26 June 2021.

External links

Retrieved from "https://en.wikipedia.org/w/index.php?title=Nutcracker_esophagus&oldid=1164131280"