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Volume: Barrett's Esophagus
Chapter: Etiology and origins of Barrett's epithelium

Are there grounds to support a congenital origin for Barrett's esophagus?

J.M. Streitz (Duluth)

Barrett's esophagus is widely recognized as a condition acquired due to chronic gastroesophageal reflux disease (GERD). Whether there exist congenital cases remains controversial. Most evidence, however, refutes a congenital etiology. Hassal has published refutation of this evidence and a summary of his work is herein presented.

The early reported cases of Barrett's esophagus in infants and children were based upon demonstration of columnar epithelium in a few biopsies (often only one) that did not document the presence of specialized, metaplastic epithelium, which is currently considered essential for the diagnosis of Barrett's esophagus. Borrie and Goldwater [1] proposed a bimodal distribution for Barrett's esophagus, in which cases less than 10 years of age were considered congenital and cases older than 10 years considered acquired. They did not, however, show evidence of such a distribution. In fact, relatively few cases of histologically proven Barrett's esophagus have been reported in children.

No convincing evidence exists of congenital Barrett's esophagus in newborns or infants. The youngest patient with documented Barrett's esophagus was 5 years of age at the time of diagnosis [2]. Claims of Barrett's esophagus in patients younger than this are not supported by histologic evidence [3], and no autopsy study of neonates or stillborns has ever yielded a case of histologically confirmed Barrett's esophagus [4-6]. One report of a newborn autopsy demonstrated a completely columnar-lined esophagus, but the presence of specialized epithelium was not documented, and it likely represented fetal columnar epithelium that had not yet undergone the normal transition to squamous epithelium [5]. A case of a 14-month old with a bleeding distal esophageal ulcer with columnar epithelium at the margins and surrounding squamous epithelium was reported to be Barrett's esophagus [7], but specialized epithelium was not demonstrated, suggesting gastric heterotopia as a cause.

Notably, all children with well documented Barrett's esophagus have also shown evidence of severe GERD. This suggests that the congenital defect in children with Barrett's esophagus is not the abnormal epithelium, but rather, the tendency to develop GERD [8].


1. Borrie J, Goldwater L. Columnar cell-lined esophagus: assessment of etiology and treatment. J Thorac Cardiovasc Surg 1976;71:825-834.

2. Qualman SJ, Murray RD, McClung J, et al. Intestinal metaplasia is age related in Barrett's esophagus. Arch Pathol Lab Med 1990;114:1236-240.

3. Hassall E. Barrett's esophagus: congenital or acquired? Am J Gastroenterol 1993;88:819-824.

4. Rector LE, Connerley ML. Aberrant mucosa in the esophagus in infants and in children. Arch Pathol 1941;31:285-294.

5. Postlethwait RW, Musser AW. Changes in the esophagus in 1,000 autopsy specimens. J Thor Cardiovasc Surg 1974;31:285-294.

6. de la Pava S, Pickren JW, Adler RH. Ectopic gastric mucosa of the esophagus. NY State J Med 1964;65:1831-1835.

7. Heydenrych JJ, Keet AD. Giant lower oesophageal ulcer in a Bushman baby. S Afr Med J 1983;63:331-333.

8. Everhart CW, Holtzapple PG, Humphries TJ. Occurrence of Barrett's esophagus in three members of the same family. Gastroenterology 1978;74:1032A.

Publication date: August 2003 OESO©2015