DISCUSSION I: Esophageal Causes of Chest Pain

   
1. ESOPHAGEAL CAUSES OF CHEST PAIN
   
  These include:
1. Esophageal dysmotility disorders
2. Esophagitis
3. Esophageal tears
   
1. Esophageal Dysmotility Disorders.
  Classification of Esophageal Motility Disorders
 

a. Primary motility disorders

 

1. Achalasia and variants
2. Diffuse esophageal spasm
3. Nutcracker esophagus
4. Nonspecific esophageal motility disorders (NEMD)
5. Presbyesophagus

 

b. Secondary motility disorders

 

1. Collagen vascular disease
2. Chemical or physical agents

 

a. reflux esophagitis
b. caustic esophagitis
c. radiation effects

 

3. Infectious causes
4. Diabetes mellitus
5. Alcoholism
6. Endocrine disease
7. Neuromuscular disorders

 

a. Cerebrovascular disease
b. Demyelinating disorders
c. Chorea-related disorders
d. Myasthenia gravis
e. Muscular dystrophies

 

8. Secondary neoplasm's

   
  Diffuse esophageal spasm (DES) is an uncommon idiopathic condition that may be part of a spectrum of esophageal dysmotility disorders and has been known to progress to achalasia. It is characterized by intermittent chest pain that may be indistinguishable from angina and by occasional dysphagia. Middle aged patients are most commonly affected. DES involves the smooth muscle of the lower esophagus rather than the striated muscle superiorly. The muscle may be normal or hypertrophied. The intramural neural plexus is normal in the pure form. Since the pain is caused by smooth muscle spasm it may be relieved by nitroglycerin. The pain is unpredictable, unrelated to swallowing and may occur during sleep or be exacerbated during times of stress. Dysphagia for solids or liquids may occur without chest pain.
   
  Radiologic findings are non-specific and clinical correlation and correlation with esophageal manometry are necessary. On esophagram, multiple swallows may be necessary before dysmotility is demonstrated. In DES there is occasional absence of primary peristalsis and multiple simultaneous non-peristaltic tertiary contractions of the esophagus occur, causing segmentation of the barium column and resulting in a "rosary bead" or "corkscrew" appearance. This distinguishes DES from "Nutcracker Esophagus" in which primary peristalsis is preserved and no motility abnormality is usually demonstrated. This condition is diagnosed by extremely forceful contractions on manometry. Nonspecific esophageal motor disorder (NEMD) and presbyesophagus demonstrate the same findings as DES but do not cause symptoms.
   
  Demonstration of esophageal dysmotility on imaging or manometry does not confirm that this is the cause of chest pain. Most patients in whom positive imaging studies are obtained do not experience symptoms to coincide with the findings.
   
  Usual Treatment:
   
  In all but the most severe cases of obstruction and pain, medical therapy is tried initially. The purpose of treatment is symptom relief. Any precipitating foods should be avoided and reflux should be treated.
   
  Nitrates, including sublingual fast-acting preparations for acute spasm, and long term use of slow-release preparations are often helpful. Benzodiazepines are useful as muscle relaxants and anxiolytics. Calcium channel blockers have been used with some success. Chronic analgesic use may be necessary but the risk of addiction is high. Mechanical dilation of the esophagus relieves spasm and prolongs the duration of effectiveness of medical management.
   
  Surgical therapy may be performed for severe recurrent chest pain, disabling dysphagia, secondary diverticula and progression to achalasia. Heller cardiomyotomy may relieve all symptoms if the excision extends proximally to include the most proximal contraction ring.
   
  Prognosis:
  Medical treatment is rarely 100% effective but most patients are adequately improved and are able to tolerate residual symptoms. Surgery cannot be guaranteed to provide total relief either.
   
  Achalasia
  The primary disorder in achalasia is failure of relaxation of the lower esophageal sphincter (LES). There is thus obstruction to the outflow of the esophagus which dilates. Early, there is disordered peristalsis with multiple tertiary contractions. Late there is absent peristalsis. The classic radiographic appearance is a dilated esophagus with air fluid level, "bird beak" GE junction and absent gastric bubble.
   
  Achalasia may be:
1. Primary
2. Secondary
   
  Primary achalasia is the idiopathic form described above.
   
  Secondary achalasia may be due to:
1. Tumor crossing the GE junction
2. Infection, e.g. chagas disease
3. Vagotomy
   
  Scleroderma
  Scleroderma is a disorder of connective tissue with fibrosis of the skin and multiple internal organs including the kidneys, lungs, heart, and GI tract. It may affect all races worldwide with a 3:1 female predominance usually presenting in the 4th to 6th decades.
   
  GI tract:
  The esophagus is involved in approximately 75% of patients. Fibrosis replaces the smooth muscle in the lower 2/3 of the esophagus which becomes aperistaltic. Free reflux makes reflux esophagitis, strictures, and Barrett's metaplasia (10%) common. There is an increase incidence of adenocarcinoma of the esophagus probably secondary to the Barrett's metaplasia.
   
  The small bowel wall may becomes fibrotic and atonic, dilated and often develops pseudo diverticula.
   
  Lungs
  The lungs are often affected with bibasilar interstitial fibrosis. There is an increased incidence of alveolar cell cancer. Pulmonary hypertension may develop due to primary involvement of the pulmonary vasculature independent of the interstitial changes.
   
  Musculoskeletal System
  Cutis calcis (calcification in soft tissues) and resorption of distal phalangeal tufts (acro-osteolysis) are characteristic.
   
  Treatment:
  There is no cure. Palliative therapies include:
 

a. Vaso active therapies, e.g. calcium channel blockers
b. Steroids
c. Immunosuppresants, e.g. cytoxan
d. D-penicillamine
e. H2 blockers for reflux, tetracycline for malabsorption
f. Organ specific therapies

   
  Prognosis:
  30% die within 5 years. Poor prognosticators include:
 
i. Old age
ii. Male
iii. Renal involvement
iv. Cardiac involvement
v. Pulmonary involvement
   
2. Esophagitis
  DDX OF ESOPHAGITIS:
 

1. Candidiasis (Candida Albicans)
2. Herpetic esophagitis
3. Reflux esophagitis
4. Corrosive esophagitis (e.g. lye)
5. Radiation (low doses if on Adriamycin or Actinomycin D)
6. Drug induced esophagitis

 

a. tetracycline
b. potassium chloride
c. quinine
d. ferrous sulphate
e. ascorbic acid
f. theophylline
g. multivitamins
h. aspirin
i. NSAID's

   
  Characteristic findings on barium swallow include diffuse mucosal irregularity and/or ulceration early with possible stricture later.
   
3. Tears and Ruptures
   
  Boerhaave's Syndrome
  Forceful vomiting, especially against resistance develops high intraesophageal pressures capable of rupturing the esophagus. In such a case, rupture usually occurs at the left posterolateral aspect of the distal esophagus, just above the gastroesophageal junction (Boerhaave's syndrome). Esophageal perforation is the most common cause of acute bacterial mediastinitis, either due to vomiting (15%) or due to iatrogenic causes (endoscopy, dilatation, biopsy) (55%), esophageal cancer, corrosive or foreign-body ingestion (14%) and penetrating trauma (10%). Infection following mediastinal surgery is uncommon. Uncommon causes of acute mediastinitis include extension from spinal, neck, pericardial or pleural infection.
   
  Acute mediastinitis usually presents with severe retrosternal chest pain, high fever often with sepsis. Dysphagia is usually present. Examination may demonstrate subcutaneous emphysema in the neck and a mediastinal crunch on auscultation. The most common chest radiograph findings include widening of the upper mediastinum and a left pleural effusion. Frank mediastinal air, extending into the neck and chest wall when severe, or air-fluid levels are less commonly seen. Gas may also extend into the pleural space and abdomen resulting in hydropneumothorax and pneumoperitoneum. Hydropneumothorax is most commonly on the left in distal esophageal perforation and most commonly on the right in mid-esophageal perforation.
   
  When esophageal perforation is suspected, esophogram, performed initially with low-osmolar nonionic water-soluble contrast is a quick and simple method to demonstrate esophageal perforation. The location and extent of perforation can be delineated and continuity with the pleural space can be demonstrated. If the patient does not have a good gag reflex and the risk of aspiration is thought to be high, barium may be preferable. Aspiration of water-soluble contrast can cause severe pulmonary edema. If a leak is not demonstrated initially with water soluble contrast, the swallow should be repeated with barium for improved sensitivity. Detection of perforation is best when the study is performed within 24 hours. If a pleural effusion is present, thoracentesis may be performed. A high concentration of salivary amylase in the pleural fluid is very suggestive of esophageal rupture.
   
  Chest CT is the study of choice for evaluating mediastinitis. Diffuse mediastinitis causes diffuse inflammatory streaking of the mediastinal fat. More focal abscesses may also be seen. Pneumomediastinum, pneumoperitoneum and pneumothorax are all best demonstrated with CT. CT is also useful to evaluate for venous thrombosis and extension of infection into the pericardium or spine. Esophageal thickening suggests an esophageal cause. Extravasation of esophageal contrast is diagnostic.
   
  Chest MRI can display these findings too, and has the added advantage of not requiring intravenous contrast.
   
  Usual Treatment
  Antibiotics, drainage of abscesses (percutaneous or surgical), and drainage of related empyema are required. Small abscesses may respond to antibiotics alone.
   
  Prognosis
   
  Acute diffuse mediastinitis related to esophageal rupture has a mortality approaching 50%. Mediastinitis due to other etiologies, and more focal mediastinal abscesses have a better prognosis. The mortality is lowest when diagnosis is made and therapy instituted within 24 hours.
   
  Mallory Weiss Tear
   
  A Mallory Weiss tear is a vertical mucosal tear of the esophagus across the gastro esophageal junction. The mechanism is the same as Boerhaave's syndrome but the mucosa alone tears with the outer esophageal layers remaining intact. These patients present with profuse hematemesis. Chest x-ray is normal. It is a clinical diagnosis.