Nutcracker esophagus

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

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]

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

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]

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]

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

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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 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]

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

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

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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	Dyspnea[1] 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.
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.
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
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.
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]
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: Thin projections on the anterior esophageal wall. Multiple upper (cervical) esophageal constrictions consistent with esophageal webs.

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The condition may not respond to treatment.

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

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