Nutcracker esophagus, Jackhammer esophagus, or hypercontractile peristalsis, is a disorder of the movement of the

esophagus The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...

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 Motility is the ability of an organism to move independently, using metabolic energy. Definitions Motility, the ability of an organism to move independently, using metabolic energy, can be contrasted with sessility, the state of organisms th ...

disorders of the esophagus, including

achalasia 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 esoph ...

and

diffuse esophageal spasm 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 sym ...

. It causes difficulty swallowing, or

dysphagia Dysphagia is difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, in some contexts it is classified as a disease#Terminology, condition in its own right. It may be a sensation that suggests difficulty in the passag ...

, to both solid and liquid foods, and can cause significant

chest pain Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with n ...

; 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 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). Indications An EMS is typically done to evaluate suspecte ...

(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 Peristalsis ( , ) is a radially symmetrical contraction and relaxation of muscles that propagate in a wave down a tube, in an anterograde direction. Peristalsis is progression of coordinated contraction of involuntary circular muscles, which ...

, with a diagnosis made when pressures exceed 180 mmHg; this has been likened to the pressure of a mechanical

nutcracker A nutcracker is a tool designed to open nuts by cracking their shells. There are many designs, including levers, screws, and ratchets. The lever version is also used for cracking lobster and crab shells. A decorative version portrays a person w ...

. The disorder does not progress, and is not associated with any complications; as a result, treatment of nutcracker esophagus targets control of symptoms only.

Signs and symptoms

Nutcracker esophagus is characterized as a

disorder of the

, meaning that it is caused by abnormal movement, or

of the esophagus. People with motility disorders present with two main symptoms:

or difficulty with swallowing. Chest pain is the more common. The chest pain is very severe and intense, and mimics cardiac chest pain. It may spread into the arm and back. The symptoms of nutcracker esophagus are intermittent, and may occur with or without food. 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

) or anatomical abnormalities of the esophagus (such as peptic strictures or

esophageal cancer Esophageal cancer is cancer arising from the esophagus—the food pipe that runs between the throat and the stomach. Symptoms often include difficulty in swallowing and weight loss. Other symptoms may include pain when swallowing, a hoarse voice ...

). 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. Nutcracker esophagus may also be associated with

metabolic syndrome Metabolic syndrome is a clustering of at least three of the following five medical conditions: abdominal obesity, high blood pressure, high blood sugar, high serum triglycerides, and low serum high-density lipoprotein (HDL). Metabolic syndrome ...

. The incidence of nutcracker esophagus in all patients is uncertain.

Pathophysiology

Pathology specimens of the esophagus in patients with nutcracker esophagus show no significant abnormality, unlike patients with

, where destruction of the

Auerbach's plexus The myenteric plexus (or Auerbach's plexus) provides motor innervation to both layers of the muscular layer of the gut, having both parasympathetic and sympathetic input (although present ganglion cell bodies belong to parasympathetic innervatio ...

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 Nitric oxide (nitrogen oxide or nitrogen monoxide) is a colorless gas with the formula . It is one of the principal oxides of nitrogen. Nitric oxide is a free radical: it has an unpaired electron, which is sometimes denoted by a dot in its che ...

levels, which have been seen in achalasia, are postulated as the primary abnormality. As

GERD Gerd or GERD may refer to: * Gerd (given name), a list of people with the given name or nickname * Gerd (moon), a moon of Saturn * Gerd Island, South Orkney Islands, Antarctica * Gastroesophageal reflux disease, a chronic symptom of mucosal damage ...

is associated with nutcracker esophagus, the alterations in nitric oxide and other released chemicals may be in response to reflux.

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 An endoscope is an inspection instrument composed of image sensor, optical lens, light source and mechanical device, which is used to look deep into the body by way of openings such as the mouth or anus. A typical endoscope applies several modern t ...

, and can also include

barium swallow An upper gastrointestinal series, also called a barium swallow, barium study, or barium meal, is a series of radiographs used to examine the gastrointestinal tract for abnormalities. A contrast medium, usually a radiocontrast agent such as barium ...

X-rays of the esophagus. Endoscopy is typically normal in patients with nutcracker esophagus; however, abnormalities associated with

gastroesophageal reflux disease Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is one of the upper gastrointestinal chronic diseases where stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/ ...

, or GERD, which associates with nutcracker esophagus, may be seen. Barium swallow in nutcracker esophagus is also typically normal, but may provide a definitive diagnosis if contrast is given in tablet or granule form. Studies on

endoscopic ultrasound Endoscopic ultrasound (EUS) or echo-endoscopy is a medical procedure in which endoscopy (insertion of a probe into a hollow organ) is combined with ultrasound to obtain images of the internal organs in the chest, abdomen and colon. It can be used t ...

show slight trends toward thickening of the muscularis propria of the esophagus in nutcracker esophagus, but this is not useful in making the diagnosis.

Esophageal motility studies

The diagnosis of nutcracker esophagus is typically made with an

, 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 The swallows, martins, and saw-wings, or Hirundinidae, are a family of passerine songbirds found around the world on all continents, including occasionally in Antarctica. Highly adapted to aerial feeding, they have a distinctive appearance. The ...

. Measurements are usually taken at various points in the esophagus. 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 The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...

relaxes normally in nutcracker esophagus, but has an elevated pressure of greater than 40 mm Hg at baseline. 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. 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. 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.

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. Very cold and very hot beverages may trigger esophageal spasms.

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 Diltiazem, sold under the brand name Cardizem among others, is a calcium channel blocker medication used to treat high blood pressure, angina, and certain heart arrhythmias. It may also be used in hyperthyroidism if beta blockers cannot be used ...

, a calcium-channel blocker, has been used in randomized control studies with good effect. Nitrate medications, including

isosorbide dinitrate Isosorbide dinitrate is a medication used for heart failure, esophageal spasms, and to treat and prevent chest pain from not enough blood flow to the heart. It has been found to be particularly useful in heart failure due to systolic dysfunction ...

, given before meals, may also help relax the LES and improve symptoms. The inexpensive generic combination of belladonna and

phenobarbital Phenobarbital, also known as phenobarbitone or phenobarb, sold under the brand name Luminal among others, is a medication of the barbiturate type. It is recommended by the World Health Organization (WHO) for the treatment of certain types of ep ...

(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 ELIXIR (the European life-sciences Infrastructure for biological Information) is an initiative that will allow life science laboratories across Europe to share and store their research data as part of an organised network. Its goal is to bring t ...

at the onset of symptoms.

Phosphodiesterase inhibitors A phosphodiesterase inhibitor is a drug that blocks one or more of the five subtypes of the enzyme phosphodiesterase (PDE), thereby preventing the inactivation of the intracellular second messengers, cyclic adenosine monophosphate (cAMP) and cycli ...

, such as

sildenafil Sildenafil, sold under the brand name Viagra, among others, is a medication used to treat erectile dysfunction and pulmonary arterial hypertension. It is unclear if it is effective for treating sexual dysfunction in women. It is taken by ...

, can be given to reduce symptoms, particularly pain, but small trials have not been able to demonstrate clinical improvement.

Procedures

Endoscopic An endoscopy is a procedure used in medicine to look inside the body. The endoscopy procedure uses an endoscope to examine the interior of a hollow organ or cavity of the body. Unlike many other medical imaging techniques, endoscopes are insert ...

therapy with

botulinum toxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neuromusc ...

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. Finally, pneumatic

dilatation Dilation (or dilatation) may refer to: Physiology or medicine * Cervical dilation, the widening of the cervix in childbirth, miscarriage etc. * Coronary dilation, or coronary reflex * Dilation and curettage, the opening of the cervix and surgic ...

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. In people who have no response to medical or endoscopic therapy, surgery can be performed. A

Heller myotomy Heller myotomy is a surgical procedure in which the muscles of the cardia (lower esophageal sphincter or LES) are cut, allowing food and liquids to pass to the stomach. It is used to treat achalasia, a disorder in which the lower esophageal sphin ...

involves an incision to disrupt the LES and the

myenteric plexus The myenteric plexus (or Auerbach's plexus) provides motor innervation to both layers of the muscular layer of the gut, having both parasympathetic and sympathetic input (although present ganglion cell bodies belong to parasympathetic innervation ...

that innervates it. The Heller myotomy is used as a final treatment option in patients who do not respond to other therapies.

Prognosis

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

References

External links

{{Gastroenterology Diseases of oesophagus, stomach and duodenum Esophagus disorders