Time space graph of normal peristalsis. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings K00-K93 - Diseases of the digestive system (K00-K14 Diseases of oral cavity salivary glands and jaws ( Disorders of Tooth development and Eruption The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books In the Esophagus After food is chewed into a bolus it is swallowed to move it into the esophagus Nutcracker esophagus shows higher amplitude contractions (Z-axis) that take longer to pass (X-axis)

Nutcracker 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. The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in Motility is a biological term which refers to the ability to move spontaneously and actively consuming energy in the process Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis Diffuse esophageal spasm is a condition in which uncoordinated contractions of the Esophagus occur It causes difficulty swallowing, or dysphagia, to both solid and liquid foods, and can cause chest pain; it may also have no symptoms. Dysphagia should not be confused with the similarly pronounced Dysphasia, a language disorder In Medicine, chest pain is a Symptom of a number of serious conditions and is generally considered a Medical emergency. 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. An esophageal motility study (EMS or esophageal manometry is a study performed to evaluate the pressure of the Esophagus in various stages 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. In the Esophagus After food is chewed into a bolus it is swallowed to move it into the esophagus A nutcracker is a Mechanical device for cracking nuts. It works on the Principle of moments as described in Archimedes ' analysis 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]

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. Motility is a biological term which refers to the ability to move spontaneously and actively consuming energy in the process The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in In the Esophagus After food is chewed into a bolus it is swallowed to move it into the esophagus ^[1] 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. In Medicine, chest pain is a Symptom of a number of serious conditions and is generally considered a Medical emergency. Dysphagia should not be confused with the similarly pronounced Dysphasia, a language disorder Nutcracker esophagus can present with either of these, but chest pain is the more common presentation. ^[2] The symptoms of nutcracker esophagus are intermittent, and may occur with or without food. ^[1] 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. Endoscopic foreign body retrieval refers to the removal of ingested objects from the Esophagus, Stomach and Duodenum by endoscopic techniques ^[3][4] 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 peptic strictures or esophageal cancer. Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis Benign esophageal stricture is a narrowing of the Esophagus that causes swallowing difficulties Esophageal cancer is malignancy of the Esophagus. There are various subtypes ^[5]

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. ^[1][6]

Nutcracker esophagus may also be associated with the metabolic syndrome,^[7] obesity,^[8] and gastroesophageal reflux disease. Metabolic syndrome is a combination of medical disorders that increase the risk of developing Cardiovascular disease and diabetes. Obesity is a condition in which excess Body fat has accumulated to such an extent that health may be negatively affected Gastroesophageal reflux disease ( American English and Canadian English) or Gastro-oesophageal reflux disease ( British English, Hiberno-English ^[9] It is uncertain what the effects of treating the underlying conditions will have on improvement of symptoms. ^[10] The incidence of nutcracker esophagus in all patients is uncertain.

Diagnosis

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. Dysphagia should not be confused with the similarly pronounced Dysphasia, a language disorder The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in This usually includes visualization of the esophagus with an endoscope, and can also include barium swallow x-rays of the esophagus. The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in A barium swallow is a Medical imaging procedure used to examine the upper GI (gastrointestinal tract which includes the esophagus and to a lesser extent the stomach ^[11] 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. Gastroesophageal reflux disease ( American English and Canadian English) or Gastro-oesophageal reflux disease ( British English, Hiberno-English ^[9] Barium swallow in nutcracker esophagus is also typically normal. ^[1] Studies on endoscopic ultrasound show slight trend toward thickening of the muscularis propria of the esophagus in nutcracker esophagus, but this is not useful in making the diagnosis. Endoscopic ultrasound is a medical procedure in which an endoscopically directed Ultrasound is used to obtain images of the internal organs in the The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in ^[12]

Esophageal motility studies

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. In the Esophagus After food is chewed into a bolus it is swallowed to move it into the esophagus

The diagnosis of nutcracker esophagus is typically made with an esophageal motility study, which shows characteristic features of the disorder. An esophageal motility study (EMS or esophageal manometry is a study performed to evaluate the pressure of the Esophagus in various stages along its length Esophageal motility studies involve pressure measurements of the esophagus after a patient takes a wet (fluid-containing) or dry (food-containing) swallow. The swallows and martins are a group of Passerine Birds in the family Hirundinidae which are characterised by their adaptation to aerial Measurements are usually taken at various points in the esophagus. ^[11]

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. The cardia (also known as Z-line or esophagogastric junction or gastroesophageal junction) is the anatomical term for the junction orifice of ^{[1] [11] [13][14]}

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. ^[9][14] 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. ^[15] It has been incorporated into a number of clinical guidelines for the evaluation of dysphagia. ^[14] 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. ^[14][16]

Pathophysiology

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. Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis Part of the Enteric nervous system, Auerbach's Plexus (or myenteric plexus) exists between the longitudinal and circular layers of Muscularis externa 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. Nitric oxide or nitrogen monoxide is a Chemical compound with Chemical formula N[[Oxygen O]] Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis ^[1][17] 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. ^[14]

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.

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. ^[10]

Medical therapy for nutcracker esophagus includes the use of calcium-channel blockers, which relax the LES and palliate the dysphagia symptoms. Calcium channel blockers are a class of drugs and natural substances with effects on many excitable cells of the body like the muscle of the Heart, smooth muscles Diltiazem has been used in randomized control studies with good effect. Diltiazem is a member of the group of drugs known as Benzothiazepines which are a class of Calcium channel blockers used in the treatment of Hypertension ^[18] Nitrate medications, including isosorbide dinitrate, given before meals may also help relax the LES and improve symptoms. Isosorbide dinitrate (ISDN (also known as Dilatrate is a Nitrate used pharmacologically as a vasodilator, e ^[1] Phosphodiesterase inhibitors, such as sildenafil have also been tried in case series for treatment. Sildenafil citrate, sold as Viagra, Revatio and under various other trade names is a drug used to treat Erectile dysfunction (male impotence ^[1] Finally, trazodone, an anti-depressant that reduces visceral sensitivity, has also been shown to reduce chest pain symptoms in patients with nutcracker esophagus. Trazodone (trade names Desyrel, Molipaxin, Trittico, Thombran, Trialodine, Trazorel) is a Psychoactive compound ^[1][19][17]

Endoscopic therapy with botulinum toxin, known also as Botox, can also be used to temporarily improve symptoms,^[20] but the effect is temporarily and may only last for weeks. Botulinum toxin is a Neurotoxin Protein produced by the Bacterium Clostridium botulinum. 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. Esophageal dilatation is a therapeutic endoscopic procedure that enlarges the lumen of the Esophagus. ^[1][17]

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. 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 Part of the Enteric nervous system, Auerbach's Plexus (or myenteric plexus) exists between the longitudinal and circular layers of Muscularis externa It is used as a final treatment option in patients who do not respond to other therapies. ^[1][21][22]

References