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Gastric Heterotopia in the Ileum With Ulceration and Chronic Bleeding
66: 113-117, 1974 Copyright © 1974 by The Williams & Wilkins Co.
Vol.·66, No.1
GASTROENTEROLOGY
Printed in U.S.A.
CASE REPORTS GASTRIC HETEROTOPIA IN THE ILEUM WITH ULCERATION AND CHRONIC BLEEDING KARIM NAWAZ, M.D., DAVID
Y.
GRAHAM, M.D., ROBERT
E.
FECHNER, M.D., AND JOHN
M. EIBAND, M.D,
Departments of Medicine and Pathology, Baylor College of Medicine, Houston, Texas
A 43-year-old man had gastric heterotopia of the small bowel with chronic gastrointestinal bleeding secondary to ulceration of the ileum. Only 1 similar case has previously been reported. Heterotopic gastric epithelium of the intestinal tract is an occasional, incidental, gross or microscopic finding at surgery or autopsy. It is most frequently seen as an acquired metaplastic change resembling antral mucosa in response to inflammatory bowel diseases such as regional enteritis. Excluding Meckel's diverticula, congenital heterotopic gastric mucosa in the wall of the small bowel distal to the ligament of Treitz has been reported in 11 previous cases. l These patients were children or young adults presenting with chronic partial small bowel obstruction resulting from a large fold of gastric mucosa acting as the leading part of the intussusceptum. Only 1 patient, an infant, presented with intestinal bleeding secondary to ulceration. We have recently observed a man, who, since childhood, had unexplained anemia which was caused by two discrete areas of heterotopic gastric mucosa in the ileum, with associated ulceration and chronic gastrointestinal blood loss. Case Report M. B., a 43-year-old white male, was admitted to The Methodist Hospital, Houston, Texas Received June 1, 1973. Accepted September 6,
1973.
Address requests for reprints to: Dr. Karim Nawaz, Department of Medicine, Section of Clinical Oncology, Baylor College of Medicine, 1200 Moursund, Houston, Texas 77025. 113
in March, 1971, for surgical repair of a herniated lumbar disc. He was found to be anemic and related the history of anemia which he had had since the age of 14. Previously, he had undergone multiple clinical evaluations and gastrointestinal roentgenographic studies, but no specific etiology for the anemia had been discovered. He had received "liver injections" in the past which had not corrected the anemia. He specifically denied nausea, vomiting, diarrhea, weight loss, or abdominal cramps, but had noted an occasional dark stool. He was not taking any medication and had never taken enteric-coated tablets. The physical examination was normal except for slight pallor. There was no hepatosplenomegaly. Proctosigmoidoscopic examination was normal. The hematocrit was 32 vol per 100 ml and the hemoglobin was 10.2 g per 100 ml. The white blood count was 6,600 with a normal differential. The peripheral smear revealed hypochromic microcytic red cells. The serum iron was 37 JLg per 100 ml with a total iron binding capacity of 462 JLg per 100 m!. Bone marrow iron stores were absent. The stool guaiac examination was positive for occult blood on multiple occasions. Radiographs of the esophagus, stomach, and colon were normal. Radiographs of the small bowel revealed two areas of narrowing of the terminal ileum with dilation of intervening segments (fig. 1). The patient underwent ileocolectomy with primary anastomosis. The resected fresh specimen included 71 cm of distal ileum and 9 cm of contiguous grossly normal cecum and ascending colon. The entire resected small bowel mucosa was normal except for two strictures located 32 and 40 cm proximal to the ileocecal valve. The strictures were 4 and
114
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Vol. 66, No.1
FIG . 1. Small bowel series showing localized area of stenosis in distal small bowel as indicated by solid arrows, and hollow arrows indicate areas of saccular dilation of normal small bowel between areas of stenosis.
7 cm in length. respectively, and normal small bowel mucosal folds stopped abruptly at their margins. The mucosa within the strictures had thick irregular folds with ulcers measuring up to 1.2 em in diameter (fig. 2). The wall of the bowel was 0.4 em thick adjacent to the ulcer due to submucosal fibrosis and mucosal thickening. The serosa was normal without "creeping fat" and the mesenteric lymph nodes were normal. Microscopically, the mucosa of both strictures contained thick gastric mucosa with long coiled branching glands containing both abundant chief and parietal cells resembling the mucosa of the body of the stomach (figs. 3 and 4). There was fibrosis in the ulcer base and it extended into the adjacent submucosa. The muscularis was hypertrophic but not fibrotic. The serosa was normal. No granulomas were present and the mesenteric lymph nodes were
microscopically normal. The patient had been followed in our clinic and his hemoglobin has remained normal with no evidence of recurrent bleeding. His stool has remained negative of occult blood.
Discussion Heterotopic gastric mucosa in the gastrointestinal tract may be either congenital or acquired. The congenital variety is most often found in the esophagus, duodenum, Meckel's diverticulum, or in the remnants of the vitelline duct but is rare in the jejunum or ileum. 2 By contrast, acquired heterotopic gastric mucosa is common in the jejunum and ileum in areas of mucosal regeneration accompanying inflammatory
January 1974
CASE REPORTS
115
FIG. 2. Normal small bowel mucosa (right) stops abruptly at edge of largest stricture (left). A sharply punched out ulcer lies in the gastric mucosa (arrow).
FIG. 3. Thickened mucosa of stricture consists of gastric glands (H & E, x 60).
lesions such as regional enteritis. In this situation, the gastric mucosa can be considered as metaplasia and consists mainly of mucus-secreting cells with the appearance of gastric pyloric glands. Parietal and
chief cells are absent, or, if present, are sparse. This type of metaplasia is usually a microscopic finding but on occasion has been grossly and radiographically detectable. 3 Since our case lacked transmural
116
CASE REPORTS
Vol. 66, No . 1
FIG. 4. Parietal and chief cells characteristic of gastric fundus are present (H & E, x 520),
inflammatory changes, granulomas, or other evidence of inflammatory bowel disease, we have no evidence of regional enteritis-a disease which may be associated with gastric metaplasia resembling pyloric mucosa. Although we lack direct evidence, the history of anemia from childhood, which was relieved by the intestinal resection, supports our belief that our case represents congenital lesion. The frequency of heterotopic gastric mucosa of the small intestine was studied by Taylor. 2 In a careful autopsy study of 150 patients, he found two small areas of heterotopic gastric mucosa in the duodenum, an incidence of 1.4%. There have been other examples of duodenal gastric mucosa occurring as small polypoid masses that were asymptomatic incidental findings at surgery or autopsy. 1, 4 Of the 11 previously reported examples of apparent congenital heterotopic gastric mucosa of the small bowel distal to the duodenum, 7 have been reported to be in the jejunum. Six of these presented as chronic partial small bowel obstruction resulting from intussusception of the mass of gastric mucosa, and 1 was an incidental surgical finding. Of the 4 cases occurring in the ileum, 3 presented as chronic intestinal obstruction, and 1, a 17-monthold boy, had recurrent melena which re-
suIted in death from intestinal bleeding. 2 The described lesions have been either eccentric polypoid or rugosa I masses. The types of involvement have ranged from a 1.5-cm polypoid mass to an eccentric rugosa I mass, 7 cm long, occurring in the jejunum of a 16-year-old male. 5 In only 2 previous patients were there two separate areas of gastric heterotopia. The duration of symptoms prior to surgical intervention ranged from a few weeks to 4 years. The individuals having jejunal involvement included children and adults ranging in ages from 6 to 28 years, with an average of 17 years. On the other hand, with the exception of our case, the patients with ileal disease were children from 1.5 to 11 years of age, with an average of 6 years. Although heterotopic gastric mucosa containing fundic mucosa can presumably secrete both acid and pepsin, ulceration has been described in only 2 patients, including this case. Histological examination of either primary small bowel ulcerations, or ulcers in patients taking enteric-coated potassium chloride preparations, has failed to incriminate heterotopic gastric mucosa as an etiological factor. Microscopic foci of gastric metaplasia are commonly present in idiopathic small bowel ulcers, but histological examination has invariably shown only
January 1974
CASE REPORTS
117
pyloric gland metaplasia. 6 In reviewing the lesions have presented either as recurrent problem of primary ileal ulceration, Brown partial small bowel obstruction, or as uland Pemberton 7 could find no instance of ceration with stricture formation and bleedpeptic ulceration due to heterotopic gastric mg. mucosa. Congenital gastric heterotopia of the REFERENCES small bowel may be more common than is currently appreciated, as microscopic foci 1. Lee SM, Mosenthal WT, Weismann RE: Tumorous heterotopic gastric mucosa in the small intescould be missed easily at surgery or netine. Arch Surg 100:619-622, 1970 cropsy. The cause of gastric heterotopia is unknown but it most likely represents a 2. Taylor AL: The epithelial heterotopias of the alimentary tract. J Pat hoi Bacteriol 30:415-499, developmental error since, embryologi1927 cally, the epithelium of the esophagus, 3, Ming S, Morris S, Tandon BN: Gross gastric stomach, and small bowel is derived from metaplasia of ileum after regional enteritis. Gasmorphologically similar cells. The stimulus troenterology 44:63-68, 1963 for differentiation and proliferation of these 4. Gannon PG, Dahlin DC, Bartholomew LG, et al: Polypoid glandular tumors of small intestine. Surg cells into a mucosa typical for a distinct Gynecol Obstet 114:666-672, 1962 and different region of the gastrointestinal tract is unknown. Irrespective of the stimu- . 5. Gore I, William WJ: Adenomatous polyp of the jejunum composed of gastric mucosa. Cancer Ius required, the mass of heterotopic tissue 164-167, 1953 will remain asymptomatic until it attains 6. 6: Wayte DM, Helwig EB: Small-bowel ulcerationsufficient bulk to act as the leading part of iatrogenic or multifactorial origin? Am J Clin the intussusceptum, or if the volume of Pathol 49:26-40, 1968 acid and pepsin secretion can acidify small 7. Brown PW, Pemberton JDeJ: Solitary ulcer of the bowel contents and promote ulceration. ileum and ulcer of Meckel's diverticulum. Ann This is borne out clinically since these Intern Med 9: 1684-1702, 1936
Vol.·66, No.1
GASTROENTEROLOGY
Printed in U.S.A.
CASE REPORTS GASTRIC HETEROTOPIA IN THE ILEUM WITH ULCERATION AND CHRONIC BLEEDING KARIM NAWAZ, M.D., DAVID
Y.
GRAHAM, M.D., ROBERT
E.
FECHNER, M.D., AND JOHN
M. EIBAND, M.D,
Departments of Medicine and Pathology, Baylor College of Medicine, Houston, Texas
A 43-year-old man had gastric heterotopia of the small bowel with chronic gastrointestinal bleeding secondary to ulceration of the ileum. Only 1 similar case has previously been reported. Heterotopic gastric epithelium of the intestinal tract is an occasional, incidental, gross or microscopic finding at surgery or autopsy. It is most frequently seen as an acquired metaplastic change resembling antral mucosa in response to inflammatory bowel diseases such as regional enteritis. Excluding Meckel's diverticula, congenital heterotopic gastric mucosa in the wall of the small bowel distal to the ligament of Treitz has been reported in 11 previous cases. l These patients were children or young adults presenting with chronic partial small bowel obstruction resulting from a large fold of gastric mucosa acting as the leading part of the intussusceptum. Only 1 patient, an infant, presented with intestinal bleeding secondary to ulceration. We have recently observed a man, who, since childhood, had unexplained anemia which was caused by two discrete areas of heterotopic gastric mucosa in the ileum, with associated ulceration and chronic gastrointestinal blood loss. Case Report M. B., a 43-year-old white male, was admitted to The Methodist Hospital, Houston, Texas Received June 1, 1973. Accepted September 6,
1973.
Address requests for reprints to: Dr. Karim Nawaz, Department of Medicine, Section of Clinical Oncology, Baylor College of Medicine, 1200 Moursund, Houston, Texas 77025. 113
in March, 1971, for surgical repair of a herniated lumbar disc. He was found to be anemic and related the history of anemia which he had had since the age of 14. Previously, he had undergone multiple clinical evaluations and gastrointestinal roentgenographic studies, but no specific etiology for the anemia had been discovered. He had received "liver injections" in the past which had not corrected the anemia. He specifically denied nausea, vomiting, diarrhea, weight loss, or abdominal cramps, but had noted an occasional dark stool. He was not taking any medication and had never taken enteric-coated tablets. The physical examination was normal except for slight pallor. There was no hepatosplenomegaly. Proctosigmoidoscopic examination was normal. The hematocrit was 32 vol per 100 ml and the hemoglobin was 10.2 g per 100 ml. The white blood count was 6,600 with a normal differential. The peripheral smear revealed hypochromic microcytic red cells. The serum iron was 37 JLg per 100 ml with a total iron binding capacity of 462 JLg per 100 m!. Bone marrow iron stores were absent. The stool guaiac examination was positive for occult blood on multiple occasions. Radiographs of the esophagus, stomach, and colon were normal. Radiographs of the small bowel revealed two areas of narrowing of the terminal ileum with dilation of intervening segments (fig. 1). The patient underwent ileocolectomy with primary anastomosis. The resected fresh specimen included 71 cm of distal ileum and 9 cm of contiguous grossly normal cecum and ascending colon. The entire resected small bowel mucosa was normal except for two strictures located 32 and 40 cm proximal to the ileocecal valve. The strictures were 4 and
114
CASE REPORTS
Vol. 66, No.1
FIG . 1. Small bowel series showing localized area of stenosis in distal small bowel as indicated by solid arrows, and hollow arrows indicate areas of saccular dilation of normal small bowel between areas of stenosis.
7 cm in length. respectively, and normal small bowel mucosal folds stopped abruptly at their margins. The mucosa within the strictures had thick irregular folds with ulcers measuring up to 1.2 em in diameter (fig. 2). The wall of the bowel was 0.4 em thick adjacent to the ulcer due to submucosal fibrosis and mucosal thickening. The serosa was normal without "creeping fat" and the mesenteric lymph nodes were normal. Microscopically, the mucosa of both strictures contained thick gastric mucosa with long coiled branching glands containing both abundant chief and parietal cells resembling the mucosa of the body of the stomach (figs. 3 and 4). There was fibrosis in the ulcer base and it extended into the adjacent submucosa. The muscularis was hypertrophic but not fibrotic. The serosa was normal. No granulomas were present and the mesenteric lymph nodes were
microscopically normal. The patient had been followed in our clinic and his hemoglobin has remained normal with no evidence of recurrent bleeding. His stool has remained negative of occult blood.
Discussion Heterotopic gastric mucosa in the gastrointestinal tract may be either congenital or acquired. The congenital variety is most often found in the esophagus, duodenum, Meckel's diverticulum, or in the remnants of the vitelline duct but is rare in the jejunum or ileum. 2 By contrast, acquired heterotopic gastric mucosa is common in the jejunum and ileum in areas of mucosal regeneration accompanying inflammatory
January 1974
CASE REPORTS
115
FIG. 2. Normal small bowel mucosa (right) stops abruptly at edge of largest stricture (left). A sharply punched out ulcer lies in the gastric mucosa (arrow).
FIG. 3. Thickened mucosa of stricture consists of gastric glands (H & E, x 60).
lesions such as regional enteritis. In this situation, the gastric mucosa can be considered as metaplasia and consists mainly of mucus-secreting cells with the appearance of gastric pyloric glands. Parietal and
chief cells are absent, or, if present, are sparse. This type of metaplasia is usually a microscopic finding but on occasion has been grossly and radiographically detectable. 3 Since our case lacked transmural
116
CASE REPORTS
Vol. 66, No . 1
FIG. 4. Parietal and chief cells characteristic of gastric fundus are present (H & E, x 520),
inflammatory changes, granulomas, or other evidence of inflammatory bowel disease, we have no evidence of regional enteritis-a disease which may be associated with gastric metaplasia resembling pyloric mucosa. Although we lack direct evidence, the history of anemia from childhood, which was relieved by the intestinal resection, supports our belief that our case represents congenital lesion. The frequency of heterotopic gastric mucosa of the small intestine was studied by Taylor. 2 In a careful autopsy study of 150 patients, he found two small areas of heterotopic gastric mucosa in the duodenum, an incidence of 1.4%. There have been other examples of duodenal gastric mucosa occurring as small polypoid masses that were asymptomatic incidental findings at surgery or autopsy. 1, 4 Of the 11 previously reported examples of apparent congenital heterotopic gastric mucosa of the small bowel distal to the duodenum, 7 have been reported to be in the jejunum. Six of these presented as chronic partial small bowel obstruction resulting from intussusception of the mass of gastric mucosa, and 1 was an incidental surgical finding. Of the 4 cases occurring in the ileum, 3 presented as chronic intestinal obstruction, and 1, a 17-monthold boy, had recurrent melena which re-
suIted in death from intestinal bleeding. 2 The described lesions have been either eccentric polypoid or rugosa I masses. The types of involvement have ranged from a 1.5-cm polypoid mass to an eccentric rugosa I mass, 7 cm long, occurring in the jejunum of a 16-year-old male. 5 In only 2 previous patients were there two separate areas of gastric heterotopia. The duration of symptoms prior to surgical intervention ranged from a few weeks to 4 years. The individuals having jejunal involvement included children and adults ranging in ages from 6 to 28 years, with an average of 17 years. On the other hand, with the exception of our case, the patients with ileal disease were children from 1.5 to 11 years of age, with an average of 6 years. Although heterotopic gastric mucosa containing fundic mucosa can presumably secrete both acid and pepsin, ulceration has been described in only 2 patients, including this case. Histological examination of either primary small bowel ulcerations, or ulcers in patients taking enteric-coated potassium chloride preparations, has failed to incriminate heterotopic gastric mucosa as an etiological factor. Microscopic foci of gastric metaplasia are commonly present in idiopathic small bowel ulcers, but histological examination has invariably shown only
January 1974
CASE REPORTS
117
pyloric gland metaplasia. 6 In reviewing the lesions have presented either as recurrent problem of primary ileal ulceration, Brown partial small bowel obstruction, or as uland Pemberton 7 could find no instance of ceration with stricture formation and bleedpeptic ulceration due to heterotopic gastric mg. mucosa. Congenital gastric heterotopia of the REFERENCES small bowel may be more common than is currently appreciated, as microscopic foci 1. Lee SM, Mosenthal WT, Weismann RE: Tumorous heterotopic gastric mucosa in the small intescould be missed easily at surgery or netine. Arch Surg 100:619-622, 1970 cropsy. The cause of gastric heterotopia is unknown but it most likely represents a 2. Taylor AL: The epithelial heterotopias of the alimentary tract. J Pat hoi Bacteriol 30:415-499, developmental error since, embryologi1927 cally, the epithelium of the esophagus, 3, Ming S, Morris S, Tandon BN: Gross gastric stomach, and small bowel is derived from metaplasia of ileum after regional enteritis. Gasmorphologically similar cells. The stimulus troenterology 44:63-68, 1963 for differentiation and proliferation of these 4. Gannon PG, Dahlin DC, Bartholomew LG, et al: Polypoid glandular tumors of small intestine. Surg cells into a mucosa typical for a distinct Gynecol Obstet 114:666-672, 1962 and different region of the gastrointestinal tract is unknown. Irrespective of the stimu- . 5. Gore I, William WJ: Adenomatous polyp of the jejunum composed of gastric mucosa. Cancer Ius required, the mass of heterotopic tissue 164-167, 1953 will remain asymptomatic until it attains 6. 6: Wayte DM, Helwig EB: Small-bowel ulcerationsufficient bulk to act as the leading part of iatrogenic or multifactorial origin? Am J Clin the intussusceptum, or if the volume of Pathol 49:26-40, 1968 acid and pepsin secretion can acidify small 7. Brown PW, Pemberton JDeJ: Solitary ulcer of the bowel contents and promote ulceration. ileum and ulcer of Meckel's diverticulum. Ann This is borne out clinically since these Intern Med 9: 1684-1702, 1936