Tracheoesophageal fistula may arise as a congenital connection between the esopha-gus and trachea that is often associated with esophageal atresia. It is often discovered soon after birth because of aspiration. In adults the condition can occur secondary to malignancy, trauma, or iatrogenic causes.
Esophageal webs are web-like protrusions of the esophageal mucosa into the lumen which typically present with dysphagia. Plummer-Vinson syndrome is a disease of middle-aged women characterized by esophageal webs, iron deficiency anemia, and increased risk of carcinoma. Schatzki rings are web-like narrowings at the gastro-esophageal junction.
Achalasia is a failure of the lower esophageal sphincter (LES) to relax with swallow-ing. The etiology is unknown in most cases; in South America, achalasia may be caused by Chagas disease. Presentation is with progressive dysphagia. The esopha-gus is characteristically dilated proximal to the lower esophageal sphincter; barium swallow shows a “bird-beak” sign. Microscopically, there is a loss of ganglion cells in the myenteric plexus. Treatment is LES balloon dilation or myotomy. Achalasia carries an increased risk for esophageal carcinoma.
Mallory-Weiss syndrome is esophageal bleeding due to linear lacerations at the gas-troesophageal junction from severe prolonged vomiting; the most common cause is acute alcohol ingestion and/or chronic alcoholism. Esophageal rupture (Boerhaave syndrome) may result.
Esophageal varices are dilated submucosal veins in the lower third of the esophagus, usually secondary to portal hypertension. The most common cause is cirrhosis. Clinically, the presentation is asymptomatic, though there is massive hematemesis when the varices are ruptured. Complications include potentially fatal hemorrhage. Treatment is generally band ligation, sclerotherapy, or balloon tamponade.
Gastroesophageal reflux disease (reflux esophagitis) (GERD) is esophageal irritation and inflammation due to reflux of gastric secretions into the esophagus. Reflux typi-cally presents with heartburn and regurgitation. Complications include bleeding, stricture, bronchospasm and asthma, and Barrett esophagus.
Barrett esophagus is a metaplasia of the squamous esophageal mucosa to a more protective columnar type (intestinal metaplasia). It occurs because of chronic expo-sure to gastric secretions, usually in the setting of GERD. The endoscopic appearance is of an irregular gastroesophageal junction with tongues of red granular mucosa extending up into the esophagus. Barrett has an increased risk for dysplasia and esophageal adenocarcinoma. The incidence of Barrett esophagus is increasing in the United States.
Squamous cell carcinoma (SCC) of the esophagus is the most common type of esophageal cancer in the world. It affects males more than females, and African Americans more than Caucasians; typical age is usually age >50. Risk factors include:
• Heavy smoking and alcohol use
• Plummer-Vinson syndrome
• Lye ingestion
The presentation of squamous cell carcinoma of the esophagus varies; it is often asymp-tomatic until late in the course. When symptoms do develop they may include progres-sive dysphagia, weight loss and anorexia, bleeding, hoarseness, and cough. Diagnosis is by endoscopy with biopsy. Treatment is surgery though the prognosis is poor.
Adenocarcinoma of the esophagus affects Caucasians more than African Ameri-cans. It arises in the distal esophagus. The progression from Barrett metaplasia to dysplasia and eventually to invasive carcinoma occurs due to the stepwise accumula-tion of genetic and epigenetic changes. The prognosis is poor.
In the United States, adenocarcinoma and squamous cell carcinoma of the esophagus occur with equal frequency.