Diffuse esophageal spasm overview

# Overview
Diffuse or Distal esophageal spasm (DES) is an uncommon esophageal motility disorder causing chest pain and/or dysphagia. DES was first described by Osgood, in 1889 in 6 patients presenting with chest pain and dysphagia. Creamer et al. (1958) made the first manometric descriptions of DES. Development of high resolution esophageal manometry in 2000 has led to classification of esophageal motility disorders. Diffuse esophageal spasm can be classified as primary or secondary based on presence or absence of other disease associated with it. The exact pathogenesis of DES is not fully understood. Current high-resolution manometric studies suggests impairment of inhibitory neurons. These inhibitory neurons use nitric oxide (NO) as neurotransmitter. Exact cause of diffuse esophageal spasm is unknown. However, may be caused by consequence of various diseases and secondary to conditions like compression of nerves within esophageal wall, inflammation of the esophagus, stricture, GERD, psychological conditions like anxiety or depression. Diffuse esophageal spasm must be differentiated from other diseases that cause dysphagia, chest pain and weight loss such as angina, reflux esophagitis, esophageal carcinoma, systemic sclerosis, nutcracker esophagus, hypertensive LES, esophageal web/stricture, pseudoachalasia, stroke, esophageal candidiasis, Chagas disease etc. Common risk factors in the development of Diffuse Esophageal Spasm include: Age (60-80 years), obesity, mitral valve prolapse, presence of GERD, Hypertension, anxiety or depression, and drinks (eg. red wine, very hot or cold liquid or fluid). If left untreated, most patients  are symptom free over the course of time. Very few cases report of progression to achalasia and nut cracker esophagus. The diagnostic study of choice for DES is manometry. An x-ray of esophagus after barium swallow (esophagogram) is the next best test to support manometric diagnosis. The mainstay of treatment for DES is medical therapy with calcium channel blockers, and/or tricyclic antidepressants.

# Historical Perspective
Esophagus was described by Vasalius in 1543. Diffuse esophageal spasm was first described by Osgood in 1889 in 6 patients presenting with chest pain and dysphagia. Development of high resolution esophageal manometry in 2000 has led to classification of esophageal motility disorders.

# Classification
Diffuse esophageal spasm can be classified as primary or secondary based on its association with other diseases.

# Pathophysiology
The exact pathogenesis of DES is not fully understood. Current high-resolution manometric studies suggests impairment of inhibitory neurons. These inhibitory neurons use nitric oxide (NO) as neurotransmitter.

# Causes
Exact cause of diffuse esophageal spasm is unknown. However, may be caused by consequence of various diseases and secondary to conditions like compression of nerves within esophageal wall, inflammation of the esophagus, strictures, GERD, and psychological conditions like anxiety or depression.

# Differentiating Diffuse esophageal spasm from Other Diseases
Diffuse esophageal spasm must be differentiated from other diseases that cause dysphagia, chest pain and weight loss such as angina, reflux esophagitis, esophageal carcinoma, systemic sclerosis, nutcracker esophagus, hypertensive LES, esophageal web/stricture, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease etc.

# Epidemiology and Demographics
Diffuse esophageal spasm is relatively uncommon disease with incidence of 1 per 100,000 in the USA. DES affects all age groups. There is no racial predilection to DES.

# Risk Factors
Common risk factors in the development of diffuse esophageal spasm include age (60-80 years), obesity, mitral valve prolapse, presence of GERD, hypertension, anxiety or depression, and drinks (eg. red wine, very hot or cold liquid or fluid).

# Screening
There is insufficient evidence to recommend routine screening for DES.

# Natural History, Complications, and Prognosis
If left untreated, most patients remain asymptomatic over the course of time. Very few cases report progression to Achalasia and nut cracker esophagus.

# Diagnosis

## Diagnostic Study of Choice
The diagnostic study of choice for DES is manometry.

## History and Symptoms
The hallmark of DES is esophageal dysphagia for both solids and liquids and chest pain. Symptom onset is sudden, intermittent and non-progressive in nature. Chest pain usually retrosternal in location, which is intense and squeezing in nature and may be mistaken for Angina. Difficulty swallowing, is sometimes related to specific substances like red wine, very cold or hot liquid.

## Physical Examination
Patients with primary diffuse esophageal spasm usually appear normal. Physical examination of patients with DES is usually remarkable for findings related to secondary diseases.

## Laboratory Findings
There are no diagnostic laboratory findings associated with DES.

## Electrocardiogram
There are no ECG findings associated with DES.

## X-ray
An x-ray of esophagus after barium swallow (esophagogram) is the next best test to support manometric diagnosis.

## Ultrasound
There are no echocardiography/ultrasound findings associated with DES.

## CT scan
Chest CT scan may be helpful in the diagnosis of DES. Findings on CT scan suggestive of DES include esophageal wall thickening.

## MRI
There are no MRI findings associated with diffuse esophageal spasm.

## Other Imaging Findings
Endoscopy may be helpful in the diagnosis of DES to exclude other esophageal lesion causing chest pain.

## Other Diagnostic Studies
24-hour esophageal pH monitoring may be helpful in the diagnosis of secondary DES.

# Treatment

## Medical Therapy
The mainstay of treatment for DES is medical therapy with calcium channel blockers, and/or tricyclic antidepressants.

## Surgery
The mainstay of treatment for DES is medical therapy. Surgery is usually reserved for patients with manometrically proven, symptomatic and those cases refractory to medical therapy.

## Primary Prevention
There are no established measures for the primary prevention of diffuse esophageal spasm.

## Secondary Prevention
There are no secondary preventive measures available for insulinoma.