Nutcracker Esophagus or Hypertensive Peristalsis: Causes, Symptoms, Treatment, Pathophysiology
What is Nutcracker Esophagus or Hypertensive Peristalsis?
Nutcracker Esophagus, also known as Hypertensive Peristalsis, is a benign condition and one of the motility disorders of the esophagus where the patient has contractions in the smooth muscles of the esophagus, which occur for excessive duration or amplitude (>180 mm Hg). The sequence, however, of the contractions is normal. Patient experiences dysphagia (difficulty in swallowing) to both liquid and solid foods and significant chest pain and heartburn. In some cases, patient may not have any symptoms at all.
Nutcracker Esophagus or Hypertensive Peristalsis can occur in individuals of any age; however, people in their 60s and 70s are more commonly affected by this disorder. The diagnosis of Nutcracker Esophagus or Hypertensive Peristalsis is made by esophageal manometry (esophageal motility study), a test which assesses esophageal pressure at different points along the length of the esophagus.
The name "nutcracker esophagus" is derived from the increased pressures found in the esophagus during peristalsis where the pressure exceeds 180 mmHg. This has been compared to the pressure exerted by a mechanical nutcracker, which leads to the name of this condition, Nutcracker Esophagus or Hypertensive Peristalsis.
This condition does not worsen and there are usually no complications associated with it, due to which, treatment for Nutcracker Esophagus or Hypertensive Peristalsis mainly comprises of managing its symptoms only.
Pathophysiology of Nutcracker Esophagus or Hypertensive Peristalsis
There was no significant abnormality in the pathology specimens of a Nutcracker Esophagus or Hypertensive Peristalsis. Whereas, in other motility disorders, such as achalasia, there was destruction of myenteric plexus seen. The pathophysiology of nutcracker esophagus can be associated with abnormalities in mediators or neurotransmitters in the distal part of the esophagus. The main problem is thought to be abnormality in the levels of nitric oxide, which are also seen in achalasia. As nutcracker esophagus is also associated with GERD and the variations in the nitric oxide and other chemicals can be in response to the reflux.
Causes of Nutcracker Esophagus or Hypertensive Peristalsis
Gastroesophageal Reflux Disease (GERD) is one of the main causes of Nutcracker Esophagus or Hypertensive Peristalsis. This condition can affect individuals of any age; however, patients who are in their 60s and 70s are at an increased risk to develop Nutcracker Esophagus or Hypertensive Peristalsis.
Signs & Symptoms of Nutcracker Esophagus or Hypertensive Peristalsis
Nutcracker Esophagus or Hypertensive Peristalsis is an esophageal motility disorder, where there is abnormal peristalsis or movement of the esophagus. Patients who suffer motility disorders commonly experience two primary symptoms: Dysphagia (difficulty with swallowing) or chest pain, which is described as non-cardiac chest pain due to its origin in the esophagus. Patients having nutcracker esophagus commonly experience chest pain more than dysphagia. The chest pan experienced in nutcracker esophagus is intermittent in nature and may not be associated with food intake. Other symptoms of Nutcracker Esophagus or Hypertensive Peristalsis are often sporadic in nature and can occur with or without food.
In rare cases, patients can experience sudden obstruction of the esophagus after consumption of food and need immediate medical treatment. This condition is known as food bolus obstruction or 'steakhouse syndrome.'
Unlike other motility disorders, Nutcracker Esophagus or Hypertensive Peristalsis does not progress or worsen or lead to any complications. There are many patients who have nutcracker esophagus, but are asymptomatic. Esophageal manometry studies which are done on asymptomatic patients reveal the same motility findings that of a nutcracker esophagus. Nutcracker Esophagus or Hypertensive Peristalsis can also be related with metabolic syndrome.
Diagnosis of Nutcracker Esophagus or Hypertensive Peristalsis
High resolution videoesophagram and esophageal motility studies are done to confirm the diagnosis of Nutcracker Esophagus or Hypertensive Peristalsis. Esophageal motility studies comprise of measurements taken of the pressures in the esophagus after the patient takes a dry (solid-containing) and wet (liquid-containing) swallow. The measurements are taken at different points in the esophagus. This study shows the characteristic features of Nutcracker Esophagus or Hypertensive Peristalsis. There are different characteristics of Nutcracker Esophagus or Hypertensive Peristalsis the most common is an average peristaltic amplitude in the distal esophagus that is greater than 180 mm Hg. The other characteristic is the presence of repeated contractions which exceed 6 seconds in duration. There is relaxation of the lower esophageal sphincter in nutcracker esophagus, however, with increased pressure of more than 40 mm Hg at baseline.
In patients who are suffering from dysphagia, initially tests are done to rule out an anatomical cause of dysphagia, i.e. whether there is esophageal distortion. An endoscope is used to visualize the esophagus. Barium swallow x-rays of the esophagus can also be done. The findings of an endoscopy are usually normal in patients who have nutcracker esophagus; however, other abnormalities which are related to gastroesophageal reflux disease along with nutcracker esophagus can be visualized. Barium swallow x-rays in patients with nutcracker esophagus are also usually normal. However, a definitive diagnosis can be found if contrast is given. Endoscopic Ultrasound reveals slight tendency towards thickening of the muscularis propria of the esophagus in patients who have nutcracker esophagus. However, this may not be useful in making the diagnosis.
Treatment for Nutcracker Esophagus or Hypertensive Peristalsis
Nutcracker Esophagus or Hypertensive Peristalsis is a non-progressive and benign condition, which means that the patient will not have worsening symptoms or suffer from any significant complications due to this condition. Reassurance is given to the patient that this disease is not likely to progress. However, symptoms, such as chest pain and dysphagia can be severe in some patients and they may need treatment for it which usually consists of medications, and in rare cases, surgery.
Conservative Treatment for Nutcracker Esophagus or Hypertensive Peristalsis
The first step in treatment of Nutcracker Esophagus or Hypertensive Peristalsis and its symptoms is focused on reducing the risk factors. Losing excess weight helps in reducing the symptoms. Extremely hot and cold beverages can also trigger the esophageal spasms and should be avoided.
Medications to Treat Nutcracker Esophagus or Hypertensive Peristalsis
- Medicines for treating Nutcracker Esophagus or Hypertensive Peristalsis comprise of calcium-channel blockers, which help in relaxing the lower esophageal sphincter and relieve the symptoms of dysphagia. Calcium-channel blockers, such as diltiazem is shown to be helpful.
- Sildenafil, which is a phosphodiesterase inhibitor, also can be used for reducing the symptoms, especially pain.
- Nitrate medications, such as isosorbide dinitrate when taken before meals, also help in relaxing the lower esophageal sphincter and alleviate the symptoms of nutcracker esophagus.
- The generic combination of phenobarbital and belladonna helps in preventing the symptoms of nutcracker esophagus and is especially useful in patients who only have occasional episodes of this condition.
- Trazadone, which is an antidepressant, may also help in decreasing the visceral sensitivity, and has also shown to alleviate the symptoms of chest pain in nutcracker esophagus.
- The role of medicines which are used to suppress acid for reducing esophageal reflux is not certain.
Endoscopic Therapy for Nutcracker Esophagus or Hypertensive Peristalsis
- Endoscopic therapy with botulinum toxin (Botox) can be used for alleviating the symptoms of dysphagia, but there is only limited effect seen on other symptoms, such as pain. This is a temporary treatment which lasts only for some weeks.
- An endoscopic technique known as pneumatic dilatation of the esophagus can also be done in which a high-pressure balloon is utilized for stretching the muscles of Lower Esophageal Sphincter. This procedure can help in improving the symptoms, however there is no clinical improvement seen in the esophageal motility.
Surgery to Treat Nutcracker Esophagus or Hypertensive Peristalsis
Surgery is done in those patients who do not benefit from medical or endoscopic therapy. Surgery for Nutcracker Esophagus or Hypertensive Peristalsis comprises of Heller myotomy where an incision is made in the muscles of the lower esophageal sphincter and in the myenteric plexus, which innervates it. This will allow the liquids and food to pass into the stomach. Heller myotomy is done as a final resort in rare cases where patients do not benefit or respond to other treatment modalities for Nutcracker Esophagus or Hypertensive Peristalsis.