What is nutcracker esophagus, also known as hypertensive peristalsis (HP) of the esophagus?

From the Guidelines

Nutcracker esophagus is a disorder of esophageal motility characterized by abnormally strong contractions of the esophageal muscles during swallowing, often presenting as squeezing retrosternal pain or spasm, accompanied by dysphagia. This condition is one of the less common esophageal motility disorders, which also include achalasia and distal esophageal spasm, and can be diagnosed through esophageal manometry, as noted in the 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR guideline for the evaluation and diagnosis of chest pain 1. Patients with nutcracker esophagus typically experience symptoms that can mimic myocardial ischemia, such as chest pain described as squeezing or burning, and dysphagia.

• The diagnosis of nutcracker esophagus involves esophageal manometry, which measures pressure changes in the esophagus and reveals high-amplitude peristaltic contractions.
• Treatment options for nutcracker esophagus may include medications that relax the smooth muscle of the esophagus, such as calcium channel blockers or nitrates, as well as lifestyle modifications like eating smaller meals slowly and avoiding trigger foods.
• The exact cause of nutcracker esophagus remains unclear but may involve neurological dysfunction affecting esophageal muscle coordination, as suggested by the complex interplay of mechanisms involved in esophageal motility disorders 1.
• It is essential to differentiate nutcracker esophagus from other causes of chest pain, including gastroesophageal reflux disease, which is the most likely cause for recurring unexplained chest pain of esophageal origin, according to the same guideline 1.

From the Research

Definition and Characteristics of Nutcracker Esophagus

• Nutcracker esophagus is a commonly diagnosed esophageal motility disorder characterized by high-amplitude peristaltic contractions, often of prolonged duration, which can manifest as dysphagia and chest pain 2.
• The disorder is associated with hypertensive peristaltic contractions in the distal esophagus, with significantly elevated mean lower esophageal sphincter pressure, peak peristaltic pressures, mean peristaltic pressures, and contraction durations compared to asymptomatic controls 3.

Clinical Features and Symptoms

• Patients with nutcracker esophagus often present with symptoms of chest pain and dysphagia, with a median time to diagnosis of 24 months 4.
• Other common symptoms include heartburn, gastroesophageal reflux disease (GERD), irritable bowel syndrome (IBS), and fibromyalgia, with many patients also experiencing psychiatric comorbidity 4.
• The symptoms of nutcracker esophagus can be similar to those of cardiac angina, making diagnosis challenging 5.

Pathophysiology and Mechanisms

• Low esophageal mucosal blood flow has been proposed as a potential mechanism for the chest pain experienced by patients with nutcracker esophagus, with tissue ischemia leading to visceral pain 5.
• The high-amplitude peristaltic contractions characteristic of nutcracker esophagus may lead to reduced esophageal wall blood perfusion, resulting in hypoxia and pain 5.

Treatment and Management

• Medical management is often effective in achieving symptomatic control, with calcium channel blockers such as diltiazem showing promise in reducing symptoms and improving esophageal motility 6.
• In some cases, surgical treatment such as extended esophagomyotomy may be necessary for patients who are refractory to conventional treatment 2.