Information on the following diseases can be found in the Related Disorders section of this report:

• Achalasia
• Gastroesophageal Reflux
• GERD
• Hiatal Hernia

Barrett esophagus, per se, does not cause any specific symptoms on its own. Since most affected individuals have GERD, they may have symptoms normally associated with that condition including backflow of food and acid from the stomach into the esophagus (reflux), hoarseness, heartburn, a sore throat, a dry cough, shortness of breath, chest pain, loss of appetite and unintended weight loss. Rarely, some individuals may vomit up small amounts of blood.

Some individuals with Barrett esophagus may develop difficulty swallowing (dysphagia), which may indicate narrowing of the esophagus (peptic stricture) or the development of cancer in the esophagus. Individuals with Barrett esophagus are at a greater risk than the general population for developing a form of cancer known as adenocarcinoma of the esophagus. However, the overall risk is still very low; less than 1 percent of individuals with Barrett esophagus develop cancer of esophagus on a yearly basis.

The exact cause of Barrett esophagus is unknown. Most cases appear to occur randomly for no apparent reason (sporadically). Barrett esophagus occurs with greater frequency in individuals with GERD.

Researchers speculate that the tissue changes that characterize Barrett esophagus are caused by chronic damage to the esophagus as is seen in individuals with chronic GERD. In individuals with GERD, backflow of the contents of the stomach including stomach acids and bile salts repeatedly damage the tissue of the lower esophagus. Over time, the tissue normally found lining the lower esophagus (squamous epithelium) is replaced by tissue normally found in the stomach (intestinal columnar epithelium), a process known as specialized intestinal metaplasia. Some researches suggest that this may occur because the intestinal tissue is more resistant to damage from stomach acids.

Some cases of Barrett esophagus have run in families suggesting that some individuals have a genetic predisposition to developing the disorder. A genetic predisposition means a person carries a gene (or genes) for the disease, but it may not be expressed unless it is triggered or "activated" under certain circumstances, such as due to particular environmental factors.

It is likely that several different factors, including environmental and genetic factors as well as lifestyle choices, cause the distinctive tissue changes that characterized Barrett esophagus. Such factors may also be why individuals with Barrett esophagus are more likely than individuals in the general population to develop adenocarcinoma of the esophagus.

Certain risk factors have been indentified for developing Barrett esophagus including individuals who are of advancing age (60 or older), white, male, and obese. Smoking may also increase the risk of developing Barrett esophagus.

Barrett esophagus affects men approximately twice as often as it does women. The disorder can affect individuals of any age, but is much more likely in older individuals. The average age at diagnosis is 60. It occurs in greater frequency in Caucasians. The exact prevalence of Barrett esophagus is not known because many people may have the disorder, but do not develop symptoms and remain undiagnosed. One estimate placed the prevalence of Barrett esophagus as high as approximately 700,000 to 1 million adults in the United States.

Symptoms of the following disorders can be similar to those of Barrett esophagus. Comparisons may be useful for a differential diagnosis.

Achalasia is a rare disorder of the esophagus characterized by the abnormal enlargement of the esophagus, impairment of the ability of the esophagus to push food down toward the stomach (peristalsis), and the failure of the ring-shaped muscle (sphincter) at the bottom of the esophagus to relax. The symptoms of achalasia typically appear gradually. People with this disorder may initially experience an impaired ability to swallow (dysphagia). Early symptoms may include mild pain in the chest, cough, and/or the regurgitation of food from the stomach. (For more information on this disorder, choose "Achalasia" as your search term in the Rare Disease Database.)

Gastroesophageal reflux disease (GERD) is a digestive disorder characterized by reflux of the contents of the stomach or small intestines into the esophagus. Symptoms of gastroesophageal reflux may include a sensation of warmth or burning rising up to the neck area (heartburn or pyrosis), swallowing difficulties (dysphagia), and chest pain. This condition is a common problem and may be a symptom of other gastrointestinal disorders. Approximately 5-15 percent of individuals with GERD eventually develop Barrett esophagus. (For more information about this condition, choose "gastroesophageal" as your search term in the Rare Disease Database.)

Hiatal hernia is a very common digestive disorder. Symptoms may include a backward flow (reflux) of stomach contents into the esophagus (gastroesophageal reflux), pain, and/or a burning sensation in the throat. The opening in the diaphragm becomes weakened and stretched, allowing a portion of the stomach to bulge through into the chest cavity. This disorder can easily be diagnosed through testing by a radiologist. Many people with Barrett esophagus also have hiatal hernia.

Diagnosis
A diagnosis of Barrett esophagus may be made by examination of the esophagus through a device known as an endoscope, a thin flexible tube that has a small camera with a light on its tip. The tube is run down the throat allowing a physician to view the tissue of the lower esophagus and the junction where the esophagus meets the stomach. Healthy tissue in this area is usually a pearly white color; the tissue that characterizes Barrett esophagus is a darker pink color often described as "salmon-colored." A diagnosis of Barrett esophagus may be confirmed by the microscopic examination of tissue samples (biopsy) taken from this discolored tissue lining the esophagus. Under a microscope, the cells have an abnormal "column" shape that is characteristic for this disease.

Since Barrett esophagus is associated with an increased risk of cancer of the esophagus, affected individuals should be evaluated periodically by a physician who specializes in treating diseases of the intestines (gastroenterologist). Examination of the esophagus every 2-3 years with a specialized endoscope is recommended to detect early pre-malignant cell changes (dysplasia). Cell dysplasia means that the cells show similarities with cancer cells, but cannot invade tissue or spread. The tissue changes at this stage can still be treated. Dysplasia may be classified as low-grade to high-grade. High-grade dysplasia indicates a greater risk of progression to esophageal cancer.

There are no accepted screening guidelines for individuals suspected of having Barrett esophagus. Some physicians recommend that individuals more than 50 who have had chronic GERD symptoms for several years undergo an endoscopy exam to determine whether they have Barrett esophagus.

Treatment
The treatment of Barrett esophagus is often directed at the symptoms associated with GERD and may include the elevation of the head of the bed and the avoidance of bedtime snacks or liquids. Drug therapy may include the administration of medications that help to relieve the symptoms of GERD and acid reflux. These may include proton pump inhibitors including esomeprazole (Nexium), lansoprazole (Prevacid), omeprazole (Prilosec), and omeprazole powder (Zegerid). Additional drugs that may be prescribed include metoclopramide (Reglan), famotidine (Pepcid), cimetidine (Tagamet), and ranitidine (Zantac).

Individuals with Barrett esophagus are urged not to smoke or drink alcoholic beverages. Some people with Barrett esophagus who do not respond to drug therapy may require surgery to heal areas of ulceration on the esophagus and prevent acid reflux. The procedure, known as laparoscopic Nissen fundoplication, tightens the muscle (sphincter) that connects the esophagus and the stomach preventing the backflow of contents from the stomach into the esophagus.

For some individuals with high-grade dysplasia, the surgical removal of the esophagus may be recommended. However, other physicians believe that the surgical removal of the esophagus should only be performed in individuals who have developed esophageal cancer.

In August of 2003, the Food and Drug Administration (FDA) approved porfimer sodium (Photofrin) as an alternative to surgery for individuals with high-grade dysplasia associated with Barrett esophagus. This photosensitizing agent kills abnormal and potentially precancerous cells. Photofrin is manufactured by Axcan Pharma, Inc. For information, contact:

Axcan Pharma, Inc.
22 Inverness Center Parkway
Birmingham, AL 35242
Tel: (205) 991-8085
Fax: (205) 991-8176

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources contact:
www.centerwatch.com

Additional therapies that are being studied for individuals with high-grade dysplasia associated with Barrett esophagus including radiofrequency ablation, cryotherapy and laser therapy. Radiofrequency ablation is a procedure in which radiofrequency energy is used to destroy the affected tissue. Cryotherapy is a procedure in which extreme cold is used to freeze and destroy affected tissue. Laser therapy uses lasers to destroy the affected tissue. More research is necessary to determine the long-term safety and effectiveness of these procedures for the treatment of individuals with Barrett esophagus.

A procedure known as endoscopic mucosal resection is being studied for the treatment of high-grade dysplasia associated with Barrett esophagus. During this procedure, the affected tissue is removed through the endoscope without damaging the underlying tissue of the esophagus. More research is necessary to determine the long-term safety and effectiveness of this potential therapy for individuals with Barrett esophagus.

TEXTBOOKS
Higgins P, Askari FK. Barrett Esophagus. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:333.

Yamada T, Alpers DH, Kaplowitz N, Laine L, et al. Eds. Textbook for Gastroenterology. 4th ed. Lippincott Williams & Wilkins. Philadelphia, PA; 2003:.

Ballenger JJ., ed. Diseases of the Nose, Throat, Ear, Head & Neck, 14th ed. New York, NY: Lea & Febiger Co; 1991:1315-6.

JOURNAL ARTICLES
Seewald S, Angl TL, Soehendra N. Endoscopic mucosal resection of Barrett's oesophagus containing dysplasia or intramucosal cancer. Postgrad Med J. 2007;83:367-372.

Shalauta MD, Saad R. Barrett's esophagus. Am Fam Phys. 2004;69:2113-2118

Drovdlic CM, Goddard KAB, Chak A, et al. Demographic and phenotypic features of 70 families segregating Barrett's oesophagus and oesophageal adenocarcinoma. J Med Genet. 2003;40:651-656.

Shaheen N, Ransohoff EF. Gastroesophageal reflux, barrett esophagus, and esophageal cancer: scientific review. JAMA. 2002;287:1972-1981.

Spechler S J. Barrett's Esophagus. N Engl J Med. 2002;346:836-842.

FROM THE INTERNET
Azodo IA, Romero Y. Barret's Esophagus. The American College of Gastroenterology. 2006. Available at: http://www.gi.org/patients/gihealth/barretts.asp Accessed on: May 23, 2008.

Johnston MH, Eastone JA. Barrett esophagus and Barrett Ulcer. Emedicine Journal, March 7, 2008. Available at: http://www.emedicine.com/med/TOPIC210.HTM Accessed on: May 23, 2008.

National Digestive Diseases Information Clearinghouse. Barrett's Esophagus. December 2004. Available at: http://digestive.niddk.nih.gov/ddiseases/pubs/barretts/ Accessed On: May 23, 2008.

Mayo Clinic for Medical Education and Research. Barrett's Esophagus. August 14, 2007. Available at: http://www.mayoclinic.com/health/barretts-esophagus/HQ00312 Accessed On: May 23, 2008.