Journal of Pediatric Gastroenterology & Nutrition:
Recurrent Intussusception and Gastrointestinal Bleeding Secondary to Gastric Heterotopia of the Small Intestine
Sari, Sinan*; Dalgic, Buket*; Demirogullari, Billur†; Ozen, I Onur†; Avsarlar, Cagatay†; Vural, Cigdem‡; Poyraz, Aylar‡; Kale, Nuri†
*Departments of Pediatric Gastroenterology
‡Pathology, Faculty of Medicine, In Gazi University, Ankara, Turkey
Accepted 31 July, 2006
Address correspondence and reprint requests to Sinan Sari, Havacilar Sitesi 6. Blok. No. 60, 12. Cad. Demetevler, Ankara, Turkey (e-mail: firstname.lastname@example.org).
Heterotopic gastric mucosa (HGM) may be found anywhere in the gastrointestinal tract. Apart from congenital abnormalities, it is rarely found in the small intestine distal to the ligament of Treitz. HGM may cause gastrointestinal bleeding, perforation, and intussusception (1). We report on a 13-year-old diabetic boy with HGM of the small intestine without congenital abnormalities. He presented with recurrent intussusceptions and severe gastrointestinal bleeding.
A 13-year-old boy came to our clinic with a 4-day history of colicky abdominal pain and melena. His medical history was significant for abdominal surgery for intestinal perforation 6 years previously at a peripheral hospital. After the operation he continued to experience severe abdominal pain and recurrent gastrointestinal bleeding. He had been hospitalized twice in the previous 2 years because of severe gastrointestinal bleeding and intussusception, but no cause could be determined. At the age of 9, he had received a diagnosis of type I diabetes mellitus.
Physical examination revealed a pale and tachycardic boy, who looked unwell. There was no peritoneal sign in the abdomen. Bowel sounds were present, and rectal examination revealed black, tar-like stool. His hemoglobin was 3.9 g/dL. The results of other laboratory tests, including platelet count, coagulation studies, and blood biochemistry were within normal limits except for a high blood glucose level (450 g/dL). Ultrasonography of the abdomen revealed the typical “target”' sign that confirmed the diagnosis of intussusception (Fig. 1). Computed tomography of the abdomen did not reveal any abnormal mass. The bleeding site could not be demonstrated by gastroscopy or colonoscopy. A 99mTc pertechnetate scan showed extensive tracer accumulation throughout the long segment of the small intestine, which appeared simultaneously with the activity of the gastric mucosa (Fig. 2A). Small bowel follow-through demonstrated multiple filling defects in the distal jejunum and proximal ileum (Fig. 3).
Recurrent life-threatening gastrointestinal bleeding, in addition to the abnormal scintigraphic and radiological findings, justified laparotomy. At laparotomy, the bowel wall was edematous and thickened between 60 cm distal to the ligament of Treitz and 210 cm proximal to the ileocecal valve. Polypoid structures were also palpated in this segment. The abnormal bowel segment was resected, and an end-to-end anastomosis was performed. Macroscopic evaluation of the specimen revealed coarse, rugose, partly polypoid mucosa involved globally. Microscopic examination with hematoxylin-and eosin staining and alcian blue-periodic acid-Schiff staining revealed a sharp demarcation between the ectopic gastric mucosa and the normal ileal mucosa (pH 2.5) (Fig. 4). In some areas, superficial ileal epithelium was replaced by gastric epithelium. Other areas were replaced with well-developed full-thickness fundic type glands in the small bowel wall. Helicobacter pylori or inflammation was not identified in these areas.
The patient was discharged on the 10th postoperative day. After 3 months, repeat scintigraphy showed tracer accumulation in the stomach only (Fig. 2B). Proton pump inhibitors were administered to prevent recurrence of complications.
HGM occurs rarely in the small intestine without associated morphological abnormalities such as Meckel diverticulum or duplication (1). The presence of ectopic gastric mucosa has also been documented in all parts of the gastrointestinal tract from the oropharynx to the rectum, gallbladder, and bile duct (2–5). Although the exact mechanism is not known, it is hypothesized that these lesions arise from the epithelium of the primitive gut as a congenital anomaly, become separated from the primordial stomach, and undergo hyperplasia over time (6,7). Acid secreted by the ectopic gastric mucosa results in the clinical findings and complications of HGM. These include inflammation, bleeding, perforation, and intussusception (1).
The first case of HGM in the small intestine without congenital abnormalities was reported in 1912 by Poindecker (8). From 1980 onward, 19 pediatric cases with small intestinal HGM without associated morphological abnormalities have been reported in the literature (1,9–27). The patients' ages ranged from 2.5 months to 17 years; there were 15 male and 4 female patients. The clinical presentations were intussusception in 12 patients, bleeding in 3, perforation and bleeding in 2, and obstruction in 2. The localization of HGM in the small intestine was ileal in 9 patients, jejunal in 9, and all intestinal segments in 1. Macroscopically, the lesions were reported as usually polypoid. Long-segment, rugose, irregular involvement was described in 2 patients (14,17). Hafen et al reported a 17-year-old boy with cystic fibrosis and intussusceptions due to polypoid HGM (10), but we have not found any reports in the literature on an association between HGM and any other systemic disorder such as diabetes mellitus. Therefore, we speculate that diabetes mellitus and HGM were coincidental in our patient.
99mTc pertechnetate scintigraphy is a defined diagnostic method for the evaluation of HGM (28). Although a positive scan is not specific to distinguish the location and size of ectopic gastric mucosa, it can help detect the abnormal area and support the decision for laparotomy (17,28).
Endoscopic methods may help the diagnosis of HGM if the site of the lesion is accessible endoscopically (3). Although new methods such as video capsule endoscopy may be beneficial for the diagnosis of HGM in the small intestine, laparotomy is the logical choice for the diagnosis and management of complicated small intestinal HGM.
The treatment of HGM is surgical resection of the involved segment. Although this segment was easily detected by inspection and palpation in our patient, determination of the involved segment is difficult in some patients. Intraoperative endoscopic examination or enterostomy during laparotomy may be useful for unidentified lesions (1,17). 99mTc pertechnetate with a handheld gamma probe has also been used for intraoperative localization of HGM (13). Histopathological examination of frozen sections or intraoperative scan may be useful for evaluation of the resection border. The lesion may recur in the anastomotic border of the bowel by hyperplasia of microscopic heterotopic gastric rests.
In the long-term follow-up of these patients, the major problem is recurrence of HGM adjacent to the resection border or another segment of the small intestine (9,13,17). These lesions can be detected by 99mTc pertechnetate scintigraphy. The complications caused by remnants of HGM may be prevented by long-term use of proton pump inhibitors (9).
In conclusion, although gastric heterotopy of the small intestine is a rare clinical condition, in situations of unexplained gastrointestinal tract bleeding, perforation, and recurrent intussusception, the possibility of intestinal HGM should be kept in mind.
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