THE COLUMNAR-LINED ESOPHAGUS (BARRETT ACQUIRED CONDITION?

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1 GASTROENTEROLOGY Copyright 1966 by The Williams & Wilkins Co. Vol. 50, No.5 Printed in U.S.A. THE COLUMNAR-LINED ESOPHAGUS (BARRETT ACQUIRED CONDITION? S Y N D R O M E ) ~ A N SANFORD M. MOSSBERG, M.D. Department of Gastroenterology, Medical Division, Montefiore Hospital and Medical Center, New York, New York, and the Department of Medicine, New Rochelle Hospital, New Rochelle, New York In 1950, Barrett! described a pathological syndrome characterized by columnar epithelium lining the lower esophagus, esophageal stricture, esophageal ulcer, and hiatal hernia. Evidence that the fetal esophagus is covered by a columnar lining before the 6th month 2 suggested that this condition resulted from a developmental failure of esophageal reepithelialization. In recent years, an acquired etiological basis for the Barrett syndrome has been accepted by several authors,3-7 but too few cases have been presented to establish such a contention firmly. This communication reports a patient who had serial pathological evidence for the acquisition of columnar mucosa in the lower esophagus. Case Report The pertinent history of this white male patient began in 1946, at age 18, when he first experienced daily episodes of nausea and selfinduced vomiting. In 1960, he noted heartburn and black stool and was treated with sedatives and antacids. Because of heartburn, postprandial abdominal pain, and hematemesis, he was hospitalized in January 1961, and an upper gastrointestinal X-ray series showed duodenal bulb deformity, a small hiatal hernia, and questionable esophagitis. At esophagoscopy, slight redness of the distal esophageal mucosa was noted, and a biopsy specimen taken at 38 cm from the incisor teeth, 2 cm proximal to the cardioesophageal junction, revealed squamous epithelium (fig. 1). Despite adherence to a regimen of antacid and anticholinergic drugs and elevation Received October 6, Accepted December 21,1965. Address requests for reprints to: Dr. Sanford M. Mossberg, 33 Quaker Ridge Road, New Rochelle, N ew York. 671 of the head of the bed, he continued to vomit daily and to experience recurrent epigastric pain. On June 19, 1962, he was admitted to Montefiore Hospital with heartburn, mid-dorsal pain regurgitation, and melena. Cinefluorograph; demonstrated an ulcer arising from the posterior wall of the esophagus about 5 cm above the cardioesophageal junction. At this point the esophagus was dilated and the ulcer appeared to lie within an intraluminal filling defect. The radiological diagnosis was "probable ulceration in a leiomyoma but carcinoma cannot be excluded." Esophagoscopy showed reddened esophageal mucosa distal to 37 cm and a normally located esophagogastric junction at 40 cm from the incisor teeth. At 38 cm a deep, posterior wall crater was seen. A biopsy specimen taken from the superior wall of the ulcer was reported as "acute and chronic inflammation in gastric mucosa" (fig. 2). After discharge the patient was instructed to follow an antisecretory regimen, but he continued to have symptoms. On August 31, 1962, after 4 hr of brisk hematemesis and melena, he was admitted to New Rochelle Hospital. The patient was a well-nourished man with mild epigastric tenderness and tarry stool (guaiac, 4+) in the rectum. Blood pressure was 120/80 mm Hg; pulse, 136/min; respirations, 20/min; and temperature, 99 F. Hemoglobin was 8.6 g/loo ml; white blood count, 21,800/mm 3 ; blood urea nitrogen, 37 mg/loo ml; blood sugar, 102 mg/loo ml; and urinalysis, negative. A nasogastric tube was inserted into the stomach, and black, guaiac-positive material was aspirated. After 2 units of whole blood, the patient's hemoglobin increased to 10.3 g/loo ml. Barium swallow roentgenograms demonstrated a large ulcer approximately 2 cm above a sliding esophageal hiatal hernia (fig. 3). The following morning, esophagoscopy revealed red, friable, bleeding esophageal mucosa starting at 20 cm from the incisor teeth and extending to the gastroesophageal junction (40 cm). At 37

672 CASE REPORTS Vol. 50, No.5 FIG.!. January 1961: Esophageal biopsy specimen, taken at 38 cm from the incisor teeth (2 cm proximal to the esophagogastric junction), showing squamous epithelium.

Biopsy specimens taken at 22 cm, 30 cm, and 37 cm revealed columnar epithelium lining the esophagus (fig. 4), whereas tissue obtained at 18 cm was covered by squamous epithelium (fig. 5).

The postoperative course was uneventful, and an insulin-gastric analysis showed no increase in acidity following hypoglycemia. The patient was discharged on September 17, 1962.

2 672 CASE REPORTS Vol. 50, No.5 FIG.!. January 1961: Esophageal biopsy specimen, taken at 38 cm from the incisor teeth (2 cm proximal to the esophagogastric junction), showing squamous epithelium. The lamina propria contains mild round cell infiltration (X 100). cm a mass of heaped-up mucosa was noted, but no ulcer crater was seen. Biopsy specimens taken at 22 cm, 30 cm, and 37 cm revealed columnar epithelium lining the esophagus (fig. 4), whereas tissue obtained at 18 cm was covered by squamous epithelium (fig. 5). On September 6, 1962, a vagotomy and pyloroplasty were performed, no attempt being made to resect the ulcer. The postoperative course was uneventful, and an insulin-gastric analysis showed no increase in acidity following hypoglycemia. The patient was discharged on September 17, Since surgery he has had no recurrence of symptoms, and his daily episodes of vomiting have ceased. An esophagogram in March 1963 was negative except for a small, residual hiatal hernia. FIG. 2. June 1962: Esophageal biopsy specimen, taken at 38 cm from the incisor teeth (2 cm proximal to the esophagogastric junction), demonstrating typical glandular mucosa containing no parietal cells. Round cells and polymorphonuclear leukocytes are seen in the lamina propria (X 200).

CASE REPORTS May 1966 673 FIG. 3. August 1962: Upper gastrointestinal roentgenogram demonstrating a large, square esophageal ulcer (arrows) above a sliding hiatal hernia.

columnar epithelium of the primitive foregut. 2 11 In the 5th or 6th month epidermoid mucosa begins to replace the cylindrical cells ;2.

3 CASE REPORTS May FIG. 3. August 1962: Upper gastrointestinal roentgenogram demonstrating a large, square esophageal ulcer (arrows) above a sliding hiatal hernia. Discussion Heterotopic mucosa, a common finding in the "normal" esophagus,2, S-1O may take one or more forms: (1) During early fetal life, the lining of the esophagus is derived from the simple columnar epithelium of the primitive foregut In the 5th or 6th month epidermoid mucosa begins to replace the cylindrical cells ;2. 11 this process originates in the midesophagus and extends caudally and orally so that by the end of gestation the fetal esophagus is covered by squamous epithelium. At birth, persisting islands of columnar mucosa may be found in the cervical esophagus,s, 10 which is the last portion to become stratified. (2) By the 7th week of fetal life, caudal descent of the stomach has been completed. When clusters of potentially gastric cells remain within the esophagus, charac-

between simple columnar epithelium on the left and pseudostratified columnar epithelium on the right.

4 674 CASE REPORTS Vol. 50, No.5 FIG. 4 (top). August 1962: Esophageal biopsy specimen, taken at 22 cm from the incisor teeth, revealing a transition between simple columnar epithelium on the left and pseudostratified columnar epithelium on the right. Glandular elements are not noted in the lamina propria (X 280). FIG. 5 (bottom). August 1962: Squamous mucosa is present at 18 cm from the incisor teeth (X 170).

5 May 1966 CASE REPORTS 675 teristic nests of ectopic gastric fundic epithelium may occur after birth at any level of the esophagus (3) The superficial cardiac glands of the lamina propria may also represent true gastric rests,6. 13 although general agreement on this point is lacking. It is difficult to place Barrett-type epithelium into any of the categories noted above. In the original description of the syndrome 1 it was assumed that the intrathoracic organ lined by glandular mucosa represented stomach lying below a congenitally short esophagus. This concept was challenged by Allison and Johnstone, 14 who considered the anomaly to be gastriclined esophagus rather than stomach because of the absence of peritoneal covering, the presence of squamous islands within the columnar mucosa, and the gross resemblance of the external musculature, the arterial supply, and the submucosal glands to that of the normal esophagus. They postulated that the columnar lining might be an upward growth of mucosa from the cardia associated with healing esophagitis. Objective support for an acquired etiology was presented first, however, by Goldman and Beckman,3 who documented the ascent of columnar mucosa in the esophagus over a 7-year period in a 66-year-old man with recurrent esophagitis. Despite endorsement of the concept that columnar lining in the lower esophagus might be acquired rather than congenital,3-7 there has been a paucity of definitive pathological corroboration. This communication describes a young adult with prolonged "psychogenic" vomitiing. Esophagoscopic biopsies performed on three occasions over a 32-month period confirmed the appearance of columnar epithelium in areas of the esophagus that previously contained normal squamous mucosa. Thus, the pathogenetic sequence in this patient appeared to be: (1) forced vomiting, (2) gastroesophageal reflux, (3) esophagitis and esophageal ulceration, (4) regeneration of columnar rather than squamous mucosa, and (5) "ascent" of the abnormal lining with progressive involvement of the lower esophagus. The hiatal hernia could have resulted either from recurrent vomiting or from inflammatory shortening of the esophagus. Failure to demonstrate the hernia endoscopically is not unusual when the protrusion through the esophageal hiatus is small and easily reducible and does not negate the positive radiographic findings. The evidence provided by this report and by that of Goldman and Beckman 3 is not incontrovertible, since small collections of squamous cells occcasionally may be found within Barrett's epithelium, and one of these islands may have been sampled on the initial biopsy. It is more reasonable to assume, however, that the appearance of glandular mucosa at progressively higher levels in the esophagus occurs in response to acid-pepsin injury and represents anomalous regenerative activity of the esophagus. The ascent of an esophageal stricture located at the squamocolumnar junction of a columnar-lined gullet supports this latter thesis. Perhaps, the production of a more resistant columnar lining is "nature's way" of attempting to protect the esophagus from repeated insults. It would be unfair to leave the impression that most of the confusion about the etiology of this fascinating entity has been dispelled. Several important questions remain unanswered. Why have some authors found typical fundic mucosa lining the esophagus, whereas other observers, including ourselves, have noted complete absence of parietal cells either in the surface epithelium or in the glands of the lamina propria? Possibly, what we call the Barrett syndrome is really two different conditions, one acquired and the other congenital. What is the process by which the esophagus acquires a foreign type of lining? Does mucosa of the cardia grow upward into the esophagus, does metaplasia occur, or do the superficial cardiac glands reach the surface to replace the destroyed squamous mucosa? Adler and associates 5 6 favor the latter mechanism but their evidence is not conclusive. Finally, can this anomaly be produced in the laboratory? Although experimental acute esophageal injury in dogs has resulted in regeneration only of squamous epithelium, an intriguing avenue

6 676 CASE REPORTS Vol. 50, No.5 for research lies in the study of acute and chronic esophageal mucosal destruction in other species. Summary Columnar epithelium lining the lower esophagus in association with esophageal ulcer, esophageal stricture, and hiatal hernia is a distinct syndrome described originally by Barrett. Although generally considered to be of congenital origin, the columnar mucosa has been thought by some authors to represent an acquired change resulting from injury to the normal squamous lining. Pathological documentation of this latter concept, however, has been scanty. The patient described in this article displayed typical features of the Barrett syndrome. Of particular interest was the demonstration over a 30-month span of glandular epithelium at levels that had been occupied previously by squamous mucosa. This sequence of events appeared to result from recurrent gastroesophageal reflux, esophagitis, and regeneration of the destroyed esophageal lining by columnar rather than squamous epithelium. Support for the Barrett syndrome as an acquired condition, therefore, is strengthened. REFERENCES 1. Barrett, N. R Chronic peptic ulcer of the oesophagus and "oesophagitis." Brit. J. Surg. 38: Johns, B. A. E Developmental changes in the oesophageal epithelium in man. J. Anat. 86: Goldman, M. C., and R. C. Beckman Barrett syndrome: Case report with discussion about concepts of pathogenesis. Gastroenterology 39: Morris, K. N Gastric mucosa within the oesophagus. Aust. New Zeal. J. Surg. 25: Adler, R. H The lower esophagus lined by columnar epithelium. J. Thor. Cardiov. Surg. 4fj: De La Pava, S., J. W. Pickren, and R. H. Adler Ectopic gastric mucosa of the esophagus; a study on histogenesis. New York J. Med. 64: Hayward, J The treatment of fibrous stricture of the oesophagus associated with hiatal hernia. Thorax 16: Schridde, H Uber Magenschleimhaut Inseln vom Bau der Cardialdrusenzone und Fundusdrusenregion und den unteren, oesophagealen Cardialdrusen gleichende Drusen im obersten Oesophagusabschnitt. Virchow. Arch. Path. Anat.175: Taylor, A. L The epithelial heterotopias of the alimentary tract. J. Path. Bact. 30' Rector, L. W., and M. L. Connerley Aberrant mucosa in the esophagus in infants and children. Arch. Path. 31: Raeburn, C Columnar ciliated epithelium in the adult oesophagus. J. Path. Bact. 63: Stewart, M. J., and S. J. Hartfall Chronic peptic ulcer of the oesophagus. J. Path. Bact. 32: Abrams, L., and D. Heath Lower oesophagus lined with intestinal and gastric epithelia. Thorax 20: Allison, P. R., and A. S. Johnstone The oesophagus lined with gastric mucous membrane. Thorax 8: Wolf, B. S., R. H. Marshak, M. L. Som, and A. Winkelstein Peptic esophagitis, peptic ulcer of the esophagus and marginal esophagogastric ulceration. Gastroenterology 29: Som, M. L., and B. S. Wolf Peptic ulcer of the esophagus and esophagitis in gastric-lined esophagus. J. A. M. A. 162: Barrett, N. R The lower esophagus lined by columnar epithelium. Surgery 41: Van de Kerckhof, J., and T. Gahagan Regeneration of the mucosal lining of the esophagus. H enry Ford Hosp. Med. Bull. 11:

gastric epithelia Lower oesophagus lined with intestinal and LEON ABRAMS AND DONALD HEATH burning pain in the epigastrium and he was

gastric epithelia Lower oesophagus lined with intestinal and LEON ABRAMS AND DONALD HEATH burning pain in the epigastrium and he was Thorax (1965), 20, 66. Lower oesophagus lined with intestinal and gastric epithelia LEON ABRAMS AND DONALD HEATH From the Department of Thoracic Surgery, Queen Elizabeth Hospital, Birmingham, and the Department