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| Image = Nutcracker-esophagus-004.jpg| | | Image = Nutcracker-esophagus-004.jpg| |
| Caption = Image courtesy of RadsWiki and copylefted| | | Caption = Image courtesy of RadsWiki and copylefted| |
| DiseasesDB = 32060 |
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| ICD10 = {{ICD10|K|22|4|k|20}} |
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| ICD9 = {{ICD9|530.5}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = |
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| MeshID = D015154 |
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| }} | | }} |
| {{Nutcracker esophagus}} | | {{Nutcracker esophagus}} |
| '''For patient information, click [[Nutcracker esophagus (patient information)|here]]''' | | '''For patient information, click [[Diffuse esophageal spasm (patient information)|here]]''' |
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| {{CMG}} | | {{CMG}} |
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| {{SK}}: Diffuse esophageal spasm, corkscrew esophagus. | | {{SK}} Diffuse esophageal spasm; corkscrew esophagus |
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| ==Overview== | | ==[[Nutcracker esophagus overview|Overview]]== |
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| '''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 [[esophageal spasm|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 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.<ref name=Castell>{{cite journal | author = Tutuian R, Castell D | title = Esophageal motility disorders (distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): modern management | journal = Curr Treat Options Gastroenterol | volume = 9 | issue = 4 | pages = 283-94 | year = 2006 | id = PMID 16836947}}</ref>
| | ==[[Nutcracker esophagus historical perspective|Historical Perspective]]== |
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| ==Symptoms== | | ==[[Nutcracker esophagus classification|Classification]]== |
| [[Image:Peristaltic.jpg|left|thumb|Time space graph of normal [[peristalsis]]. '''Nutcracker esophagus''' shows higher amplitude contractions (Z-axis) that take longer to pass (X-axis)]]
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| Nutcracker esophagus is characterized as a [[motility]] disorder of the [[esophagus]], meaning that it is caused by abnormal movement, or [[peristalsis]] of the esophagus.<ref name=Castell/> Patients with motility disorders present with two key symptoms: either with ''[[chest pain]]'' (typically given termed as non-cardiac chest pain as it is esophageal in origin), which is usually found in disorders of spasm, or with ''[[dysphagia]]'', or difficulty swallowing. Nutcracker esophagus can present with either of these, but chest pain is the more common presentation.<!--
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| --><ref name=Fass>{{cite journal | author = Fass R, Dickman R | title = Nutcracker esophagus--a nut hard to swallow | journal = J Clin Gastroenterol | volume = 40 | issue = 6 | pages = 464-6 | year = 2006 | id = PMID 16825926}}</ref> <!--
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| -->The symptoms of nutcracker esophagus are intermittent, and may occur with or without food.<ref name=Castell/> 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 [[endoscopic foreign body retrieval|treatment]].<!--
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| --><ref name=Impaction>{{cite journal | author = Breumelhof R, Van Wijk H, Van Es C, Smout A | title = Food impaction in nutcracker esophagus. | journal = Dig Dis Sci | volume = 35 | issue = 9 | pages = 1167-71 | year = 1990 | id = PMID 2390932}}</ref><ref>{{cite journal | author = Chae H, Lee T, Kim Y, Lee C, Kim S, Han S, Choi K, Chung I, Sun H | title = Two cases of steakhouse syndrome associated with nutcracker esophagus. | journal = Dis Esophagus | volume = 15 | issue = 4 | pages = 330-3 | year = 2002 | id = PMID 12472482}}</ref> <!--
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| -->The disorder also does not progress to produce worsening symptoms or complications, unlike other motility disorders, such as [[achalasia]], or anatomical abnormalities of the esophagus, such as [[stricture|peptic strictures]] or [[esophageal cancer]].<!--
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| --><ref name=Castell2>{{cite journal | author = Dalton C, Castell D, Richter J | title = The changing faces of the nutcracker esophagus | journal = Am J Gastroenterol | volume = 83 | issue = 6 | pages = 623-8 | year = 1988 | id = PMID 3376915}}</ref>
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| 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.<ref name=Castell/><!--
| | ==[[Nutcracker esophagus pathophysiology|Pathophysiology]]== |
| --><ref name=Adler>{{cite journal | author = Adler D, Romero Y | title = Primary esophageal motility disorders | journal = Mayo Clin Proc | volume = 76 | issue = 2 | pages = 195-200 | year = 2001 | id = PMID 11213308}}</ref><!--
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| Nutcracker esophagus may also be associated with the [[metabolic syndrome]],<!--
| | ==[[Nutcracker esophagus causes|Causes]]== |
| --><ref>{{cite journal | author = Börjesson M, Albertsson P, Dellborg M, Eliasson T, Pilhall M, Rolny P, Mannheimer C | title = Esophageal dysfunction in syndrome X. | journal = Am J Cardiol | volume = 82 | issue = 10 | pages = 1187-91 | year = 1998 | id = PMID 9832092}}</ref> <!--
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| -->[[obesity]],<!--
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| --><ref>{{cite journal | author = Hong D, Khajanchee Y, Pereira N, Lockhart B, Patterson E, Swanstrom L | title = Manometric abnormalities and gastroesophageal reflux disease in the morbidly obese. | journal = Obes Surg | volume = 14 | issue = 6 | pages = 744-9 | year = | id = PMID 15318976}}</ref><!--
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| --> and [[gastroesophageal reflux disease]].<!--
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| --><ref name=Bjorkman>{{cite journal | author = Fang J, Bjorkman D | title = Nutcracker esophagus: GERD or an esophageal motility disorder. | journal = Am J Gastroenterol | volume = 97 | issue = 6 | pages = 1556-7 | year = 2002 | id = PMID 12094884}}</ref> <!--
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| -->It is uncertain what the effects of treating the underlying conditions will have on improvement of symptoms.<!--
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| --><ref name=PPI>{{cite journal | author = Borjesson M, Rolny P, Mannheimer C, Pilhall M | title = Nutcracker oesophagus: a double-blind, placebo-controlled, cross-over study of the effects of lansoprazole. | journal = Aliment Pharmacol Ther | volume = 18 | issue = 11-12 | pages = 1129-35 | year = 2003 | id = PMID 14653833}}</ref> The incidence of nutcracker esophagus in all patients is uncertain.
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| ==Diagnosis== | | ==[[Nutcracker esophagus differential diagnosis|Differentiating Nutcracker esophagus from Other Diseases]]== |
| In patients who have [[dysphagia]], testing may first be done to exclude an anatomical cause of dysphagia, where there is a distortion of the anatomy of the [[esophagus]]. This usually includes visualization of the [[esophagus]] with an [[esophagogastroduodenoscopy|endoscope]], and can also include [[barium swallow]] x-rays of the esophagus.<!--
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| --><ref name=Canguidelines>{{cite journal | author = Cockeram A | title = Canadian Association of Gastroenterology Practice Guidelines: evaluation of dysphagia. | journal = Can J Gastroenterol | volume = 12 | issue = 6 | pages = 409-13 | year = 1998 | id = PMID 9784896}}</ref> <!--
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| -->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.<ref name=Bjorkman/> Barium swallow in nutcracker esophagus is also typically normal.<ref name=Castell/> Studies on [[endoscopic ultrasound]] show slight trend toward thickening of the [[esophagus|muscularis propria]] of the esophagus in nutcracker esophagus, but this is not useful in making the diagnosis.<ref>{{cite journal | author = Melzer E, Ron Y, Tiomni E, Avni Y, Bar-Meir S | title = Assessment of the esophageal wall by endoscopic ultrasonography in patients with nutcracker esophagus. | journal = Gastrointest Endosc | volume = 46 | issue = 3 | pages = 223-5 | year = 1997 | id = PMID 9378208}}</ref>
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| ===Esophageal motility studies=== | | ==[[Nutcracker esophagus epidemiology and demographics|Epidemiology and Demographics]]== |
| [[Image:Nutcracker manometry.jpg|left|thumb|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 seconds.]] | |
| 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 (food-containing) [[swallow]]. Measurements are usually taken at various points in the esophagus.<ref name=Canguidelines/>
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| 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 2 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.<ref name=Castell/> <ref name=Canguidelines/> <!-- | | ==[[Nutcracker esophagus risk factors|Risk Factors]]== |
| --><ref name=Ott>{{cite journal | author = Ott D | title = Motility disorders of the esophagus. | journal = Radiol Clin North Am | volume = 32 | issue = 6 | pages = 1117-34 | year = 1994 | id = PMID 7972703}}</ref><ref name=Pilhall>{{cite journal | author = Pilhall M, Börjesson M, Rolny P, Mannheimer C | title = Diagnosis of nutcracker esophagus, segmental or diffuse hypertensive patterns, and clinical characteristics. | journal = Dig Dis Sci | volume = 47 | issue = 6 | pages = 1381-8 | year = 2002 | id = PMID 12064816}}</ref><!--
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| Three other criteria for 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.<ref name=Bjorkman/><ref name=Pilhall/> The ''Richter'' criterion involves the presence of peristaltic contractions with an amplitude of greater than 180 mmHg from an average of measurements taken 3 and 8 centimetres above the lower esophageal sphincter.<!--
| | ==[[Nutcracker esophagus screening|Screening]]== |
| --><ref>{{cite journal | author = Richter J, Wu W, Johns D, Blackwell J, Nelson J, Castell J, Castell D | title = Esophageal manometry in 95 healthy adult volunteers. Variability of pressures with age and frequency of "abnormal" contractions. | journal = Dig Dis Sci | volume = 32 | issue = 6 | pages = 583-92 | year = 1987 | id = PMID 3568945}}</ref> <!--
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| -->It has been incorporated into a number of clinical guidelines for the evaluation of dysphagia.<ref name=Pilhall/> The ''Achem criteria'' are more stringent criteria that 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.<ref name=Pilhall/><!--
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| --><ref>{{cite journal | author = Achem S, Kolts B, Burton L | title = Segmental versus diffuse nutcracker esophagus: an intermittent motility pattern. | journal = Am J Gastroenterol | volume = 88 | issue = 6 | pages = 847-51 | year = 1993 | id = PMID 8503378}}</ref>
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| ===Esophagography=== | | ==[[Nutcracker esophagus natural history, complications and prognosis|Natural History, Complications, and Prognosis]]== |
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| Images shown below are courtesy of RadsWiki and copylefted
| | ==Diagnosis== |
| | | [[Nutcracker esophagus history and symptoms|History and Symptoms]] | [[Nutcracker esophagus physical examination|Physical Examination]] | [[Nutcracker esophagus laboratory findings|Laboratory Findings]] | [[Nutcracker esophagus electrocardiogram|Electrocardiogram]] | [[Nutcracker esophagus x ray|X Ray]] | [[Nutcracker esophagus CT|CT]] | [[Nutcracker esophagus MRI|MRI]] | [[Nutcracker esophagus other imaging findings|Other Imaging Findings]] | [[Nutcracker esophagus other diagnostic studies|Other Diagnostic Studies]] |
| <div align="left">
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| <gallery heights="225" widths="200">
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| Image:Nutcracker-esophagus-001.jpg
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| Image:Nutcracker-esophagus-002.jpg
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| </gallery>
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| </div>
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| <div align="left">
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| <gallery heights="225" widths="200">
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| Image:Nutcracker-esophagus-003.jpg
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| Image:Nutcracker-esophagus-004.jpg
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| </gallery>
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| </div>
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| ==Pathophysiology==
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| 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 the 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.<ref name=Castell/><ref name=Kahrilas>{{cite journal | author = Kahrilas P | title = Esophageal motility disorders: current concepts of pathogenesis and treatment. | journal = Can J Gastroenterol | volume = 14 | issue = 3 | pages = 221-31 | year = 2000 | id = PMID 10758419}}</ref> As GERD is associated with nutcracker esophagus, it has also been hypothesized that the alterations in nitric oxide and other released chemicals may be response to reflux.<ref name=Pilhall/>
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| ==Treatment== | | ==Treatment== |
| Nutcracker esophagus is a benign, non-progressive condition, meaning that it is not associated with significant complications. Patients are usually reassured by their physicians that the disease is not associated with worsening. However, the symptoms of chest pain and dysphagia may be severe enough to require treatment with medications, and, rarely, surgery. | | [[Nutcracker esophagus medical therapy|Medical Therapy]] | [[Nutcracker esophagus surgery|Surgery]] | [[Nutcracker esophagus primary prevention|Primary Prevention]] | [[Nutcracker esophagus secondary prevention|Secondary Prevention]] | [[Nutcracker esophagus cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Nutcracker esophagus future or investigational therapies|Future or Investigational Therapies]] |
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| The first step in treatment of nutcracker esophagus is the reduction of risk factors. While weight reduction may be useful in reducing symptoms, the role of acid suppression therapy to reduce esophageal reflux, is still uncertain.<ref name=PPI/>
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| Medical therapy for nutcracker esophagus includes the use of [[calcium-channel blocker]]s, which relax the LES and palliate the dysphagia symptoms. [[Diltiazem]] has been used in randomized control studies with good effect.<!-- | |
| --><ref>{{cite journal | author = Cattau E, Castell D, Johnson D, Spurling T, Hirszel R, Chobanian S, Richter J | title = Diltiazem therapy for symptoms associated with nutcracker esophagus. | journal = Am J Gastroenterol | volume = 86 | issue = 3 | pages = 272-6 | year = 1991 | id = PMID 1998307}}</ref> <!--
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| -->Nitrate medications, including [[isosorbide dinitrate]], given before meals may also help relax the LES and improve symptoms.<ref name=Castell/> Phosphodiesterase inhibitors, such as [[sildenafil]] have also been tried in case series for treatment.<ref name=Castell/> Finally, [[trazodone]], an anti-depressant that reduces visceral sensitivity, has also been shown to reduce chest pain symptoms in patients with nutcracker esophagus.<ref name=Castell/><ref>{{cite journal | author = Achem S, Kolts B | title = Current medical therapy for esophageal motility disorders. | journal = Am J Med | volume = 92 | issue = 5A | pages = 98S-105S | year = 1992 | id = PMID 1595773}}</ref><ref name=Kahrilas/>
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| Endoscopic therapy with [[botulinum toxin]], known also as Botox, can also be used to temporarily improve symptoms,<ref>{{cite journal | author = Tutuian R, Castell D | title = Esophageal motility disorders (distal esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter): modern management. | journal = Curr Treat Options Gastroenterol | volume = 9 | issue = 4 | pages = 283-94 | year = 2006 | id = PMID 16836947}}</ref> but the effect is temporarily and may only last for weeks. Finally, pneumatic [[esophageal dilatation|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.<ref name=Castell/><ref name=Kahrilas/>
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| 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.<ref name=Castell/><!--
| | ==Case Studies== |
| --><ref>{{cite journal | author = Traube M, Tummala V, Baue A, McCallum R | title = Surgical myotomy in patients with high-amplitude peristaltic esophageal contractions. Manometric and clinical effects. | journal = Dig Dis Sci | volume = 32 | issue = 1 | pages = 16-21 | year = 1987 | id = PMID 3792178}}</ref><ref>{{cite journal | author = Richter J, Castell D | title = Surgical myotomy for nutcracker esophagus. To be or not to be? | journal = Dig Dis Sci | volume = 32 | issue = 1 | pages = 95-6 | year = 1987 | id = PMID 3792184}}</ref>
| | [[Nutcracker esophagus case study one|Case #1]] |
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| ==References==
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| <div class="references-small"><references/></div>
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| {{Gastroenterology}} | | {{Gastroenterology}} |
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| | | [[Category:Disease]] |
| [[Category:Gastroenterology]] | | [[Category:Gastroenterology]] |
| [[Category:Cardiology]] | | [[Category:Cardiology]] |
