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NORD is very grateful to Prateek Sharma, MD, Professor of Medicine, Director of the GI Fellowship Training, University of Kansas School of Medicine, for assistance in the preparation of this report.
Barrett esophagus is a condition in which the cells that make up of the tissue of the lower end of the esophagus are abnormal. The esophagus is the thin tube that connects the back of the throat to the stomach. Chronic inflammation and ulceration of the lower end of the esophagus eventually causes the cells normally found there to be replaced by cells normally found in the intestines (intestinal metaplasia). Since most patients with Barrett esophagus have acid reflux disease, they suffer from heartburn and/or acid regurgitation; Barrett esophagus does not cause any specific symptoms. The disorder is considered a premalignant condition and affected individuals are at an increased risk (although their overall risk remains low), of developing cancer (adenocarcinoma), of the esophagus. Barrett esophagus usually occurs more often in individuals with gastroesophageal reflux (GERD), a condition characterized by backflow (regurgitation), of the contents of stomach into the esophagus. The exact reason these tissue changes occur in Barrett esophagus is unknown.
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 0.5 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 researchers 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 identified for developing Barrett esophagus including individuals who are of advancing age (60 or older), white, male, and obese. Smoking increases 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 1 -1.5 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.
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 an endoscopy orradiologist. The majority of the people with Barrett esophagus also have hiatal hernia.
A diagnosis of Barrett esophagus is 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 3-5 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 consensus accepted screening guidelines for individuals suspected of having Barrett esophagus. Some guidelines suggest that individuals more than 50 who have multiple risk factors such as chronic GERD symptoms for several years, and other risk factors such as obesity, smoking history, family history etc undergo an endoscopy exam to determine whether they have Barrett esophagus.
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), dexlansoprazole (Dexilant), pantoprazole, 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 repair the hiatal hernia and 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 and early cancer, the surgical removal of the esophagus may be recommended. However recent advances in endoscopic therapy including endoscopic mucosal resection and ablation (radiofrequency ablation, cryotherapy, laser, PDT) have them the initial choice of therapy in patients with high grade dysplasia and early cancer.
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.
A procedure known as endoscopic mucosal resection is now widely utilized for the treatment of high-grade dysplasia and early cancer associated with Barrett esophagus. During this procedure, the affected tissue is removed through the endoscope without damaging the underlying tissue of the esophagus. Recently published data using endoscopic therapies for patients with high grade dysplasia and early cancer appear very promising.
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
For information about clinical trials sponsored by private sources contact:
For information about clinical trials conducted in Europe, contact:
Contact for additional information about Barrett esophagus:
Prateek Sharma, MD
Professor of Medicine
Director: GI Fellowship Training
Veterans Medical Center
University of Kansas School of Medicine
Kansas City, KS 66160
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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.
Sharma P. N Barrett's esophagus. NEJM 2009; 361(26):2548-56. Erratum in:NEJM 2010; 362(15):1450.
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. NEJM 2002;346:836-842.
Azodo IA, Romero Y. Barrett's Esophagus. The American College of Gastroenterology. 2006. Available at: http://www.gi.org/patients/gihealth/barretts.asp Accessed Dec 30, 2013.
Johnston MH, Eastone JA. Barrett esophagus and Barrett Ulcer. Emedicine Journal, Updated: Dec 16, 2013. Available at: http://www.emedicine.com/med/TOPIC210.HTM Accessed Dec 30, 2013.
National Digestive Diseases Information Clearinghouse. Barrett's Esophagus. Updated November 20, 2013. Available at: http://digestive.niddk.nih.gov/ddiseases/pubs/barretts/ Accessed Dec 30, 2013.
Mayo Clinic for Medical Education and Research. Barrett's Esophagus. May 25, 2011. Available at: http://www.mayoclinic.com/health/barretts-esophagus/HQ00312 Accessed Dec 30, 2013.
Report last updated: 2014/01/28 00:00:00 GMT+0