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Isolated Crohn's Disease of the Stomach

      Crohn's disease can involve any portion of the digestive tract, but isolated gastric Crohn's disease is a rare entity. In the few previously reported cases, the inflammatory disorder has involved only a portion of the stomach. Herein we describe a patient with diffuse involvement of the entire stomach and an associated gastrosplenic fistula but no evidence of involvement elsewhere in the gastrointestinal tract. Usually, a patient with isolated Crohn's disease of the stomach will have the clinical symptoms of nausea, vomiting, and epigastric pain and radiographic evidence of a small contracted stomach (or, occasionally, a huge dilated stomach). Because the condition may suggest the presence of a malignant lesion and biopsy specimens often reveal nonspecific inflammation, surgical resection is usually necessary for diagnosis of isolated Crohn's disease of the stomach.
      Crohn's disease of the stomach is uncommon and almost always occurs concomitantly with disease elsewhere in the gastrointestinal tract. Of the few reported cases of isolated Crohn's disease of the stomach, most have shown involvement of only a portion of the stomach, usually the antrum. In this report, we describe a patient with Crohn's disease who had diffuse involvement of the entire stomach and a gastrosplenic fistula, without evidence of involvement elsewhere in the gastrointestinal tract.

      REPORT OF CASE

      A 52-year-old woman was hospitalized because of nausea and vomiting of 3 months' duration. An upper gastrointestinal x-ray series showed particulate matter in the stomach, gastric ulceration, and obstruction of the gastric outlet. Gastroscopy disclosed inflamed and scarred gastric mucosa. The pylorus was deformed but widely patent, and the duodenum appeared normal. Gastric mucosal biopsy specimens showed benign inflammation. Because her symptoms diminished, she was dismissed from the hospital and instructed to eat frequent small meals.
      One month later, the patient was rehospitalized because of complaints of upper abdominal pain, nausea, emesis, and abdominal distention. A flat plate roentgenogram of the abdomen showed a huge dilated stomach with retained food. Gastroscopy revealed retained food, diffuse mucosal ulceration, and a patent, deformed pylorus. In endoscopic gastric biopsy specimens, benign chronic inflammation was noted. A computed tomographic scan of the abdomen demonstrated diffuse thickening of the gastric wall (Fig. 1).
      Figure thumbnail gr1
      Fig. 1Computed tomographic scan of abdomen with use of contrast agent, showing diffuse thickening of wall of stomach in 52-year-old woman with isolated Crohn's disease of the stomach.
      After undergoing gastric lavage, the patient was placed on a low-residue diet, and treatment with ranitidine, sucralfate, and domperidone, a pro-motility agent, was instituted. Six months after the onset of her symptoms, she was referred to our institution because of persistent nausea, vomiting, and gastric distention.
      A review of the history of the patient showed that she had undergone vagotomy and pyloroplasty 10 years previously for treatment of an intractable duodenal ulcer. She had been free of digestive symptoms until the onset of her current illness. Although she had taken diazepam for chronic muscle stiffness, she had taken no salicylates or other potentially ulcerogenic medications for 10 years.
      Physical examination revealed a well-developed woman in moderate distress. Her vital signs were stable, and she was afebrile. Her abdomen was soft and nontender, and the bowel sounds were normal.
      Laboratory studies revealed a hemoglobin concentration of 10.1 g/dl with a hematocrit of 29.5% and normal erythrocytic indices. The leukocyte count was 5,200/mm3. Results of the following serum studies were normal: sodium, potassium, calcium, phosphorus, creatinine, gastrin, alkaline phosphatase, glutamic-oxaloacetic transaminase, and bilirubin. A rapid plasma reagin test was nonreactive. Both the serum total protein (6.2 g/dl; normal, 6.3 to 7.9 g/dl) and the serum albumin concentration (3.3 g/dl; normal, 3.5 to 5.0 g/dl) were low. A supine roentgenogram of the abdomen was unremarkable. An upper gastrointestinal barium x-ray study disclosed substantial deformity of the stomach with a lack of distensibility. The pylorus also appeared deformed, and enlarged nodular folds were evident (Fig. 2). The findings on a barium x-ray series of the small intestine were normal. At gastroscopy, the gastric mucosa appeared diffusely friable, erythematous, and grossly edematous, and multiple linear ulcers and scattered superficial ulcers were noted throughout the stomach. The pylorus was deformed, edematous, and widely patent; the duodenum appeared normal. Multiple esophageal and gastric biopsy specimens showed only chronic inflammatory changes. The clinical diagnosis was a probable gastric malignant lesion, and abdominal exploration was recommended.
      Figure thumbnail gr2
      Fig. 2Barium x-ray study of stomach, showing considerable gastric deformity, with enlarged nodular folds and poor distensibility; pylorus and duodenal bulb appear deformed. (Surgical clips are from previous vagotomy and pyloroplasty.)
      At operation, a diffusely thickened and contracted stomach was firmly attached to the spleen at the greater curvature of the gastric body. The duodenum, jejunum, ileum, and colon appeared normal. Total gastrectomy and splenectomy were performed, and gastrointestinal continuity was restored by an end-to-side Roux-en-Y esophagojejunostomy. Pathologic examination of the resected specimen revealed that the wall of the entire stomach was massively thickened and somewhat constricted. Three longitudinal ulcers, up to 4 cm in length, were identified, as were transmural inflammation and a fistula from the greater curvature of the gastric body to the splenic capsule. Microscopic examination of the gastric wall showed intense chronic transmural inflammation featuring lymphocytes, plasma cells, rare eosinophils, and histiocytes, without granuloma formation (Fig. 3). We found no evidence of fungi or other microorganisms or amyloidosis. These gross and microscopic findings were interpreted collectively as consistent with Crohn's disease. The resected margins were free of inflammatory activity.
      Figure thumbnail gr3
      Fig. 3Photomicrograph of gastric mucosa, showing intense inflammation without granuloma formation. (Hematoxylin-eosin; ×400.)
      One year after dismissal from the hospital, the patient was asymptomatic from a gastrointestinal standpoint. A follow-up x-ray series of the small intestine and a single contrast barium enema study showed no evidence of recurrent disease.

      DISCUSSION

      Crohn's disease is a chronic inflammatory disorder of unknown cause that can involve any portion of the digestive tract from the mouth to the anus. No single feature is pathognomonic of Crohn's disease, and the diagnosis is based on supportive clinical, radiographic, endoscopic, and pathologic findings. Crohn's disease is invariably a diagnosis of exclusion, and infectious, neoplastic, ischemic, infiltrative, and other inflammatory processes must be ruled out.
      Symptomatic gastroduodenal involvement occurs in up to 4% of cases of Crohn's disease, and the gastric involvement is almost always confined to the antrum.
      • Fielding JF
      • Toye DKM
      • Beton DC
      • Cooke WT
      Crohn's disease of the stomach and duodenum.
      Few cases of Crohn's disease with exclusive involvement of the stomach have been reported; indeed, some investigators restrict the diagnosis of Crohn's disease to those cases with confirmed regional enteritis in the small intestine or colon.
      • Beaudin D
      • Da Costa LR
      • Prentice RSA
      • Beck IT
      Crohn's disease of the stomach: a case report and review of the literature.
      In our review of the literature, we found a total of 13 cases of previously reported Crohn's disease with involvement limited to the stomach. Three single case reports have been published
      • Talseth T
      Isolated gastric involvement in Crohn's disease: report of a case simulating scirrhus carcinoma.
      • Guibert HL
      Maladie de Besnier-Boeck-Schaumann: a localisation gastro-ganglionnaire pure (etude histo-pathologique).
      • Fahimi HD
      • Deren JJ
      • Gottlieb LS
      • Zamcheck N
      Isolated granulomatous gastritis: its relationship to disseminated sarcoidosis and regional enteritis.
      and Mylla Netto and associates
      • Mylla Netto G
      • Costa R
      • Pěgo-Fernandes PM
      • de Lima EC
      • Ribeiro FM
      • Stolf NAG
      Crohn's disease with exclusive gastric involvement: a case report and review of the literature.
      reported a case of Crohn's disease with exclusive gastric involvement and reviewed an additional 11 cases, 10 of which had gastric involvement only. Among the 13 reported cases, the distribution of disease within the stomach was as follows: the antrum only in 6 patients, the lower half of the stomach in 1, the antrum and the greater curvature of the stomach in 1, the body of the stomach in 1, the greater curvature and proximal half of the stomach in 1, and the fundus and pylorus in 1; the extent of involvement was not specified in 2 cases. In our case described herein, the entire stomach was involved. In addition, this is the first report of a patient with isolated gastric Crohn's disease and a fistula to the spleen.
      Our patient had undergone a vagotomy and pyloroplasty for intractable duodenal ulcer 10 years before the onset of her current illness. We found no convincing evidence in the literature that patients with Crohn's disease have an increased frequency of peptic ulcer disease. Although Crohn's disease can involve the duodenal bulb, the presence of a discrete ulcer is unusual; more commonly, mucosal nodularity and strictures are noted. In our patient, we found no evidence of disease in the duodenal bulb by endoscopy or by inspection intraoperatively. Because of the previous vagotomy and pyloroplasty in our patient, a question could be raised about whether the identified fistula could have been a complication of the prior surgical treatment. The fistula extended from the greater curvature of the stomach to the splenic capsule, a location well removed from the site of the vagotomy and pyloroplasty. Also, the progression from a hugely dilated stomach to a thickened and contracted stomach during a 5-month period would be difficult to explain as a complication of a vagotomy and pyloroplasty 10 years previously, during which period the patient had been free of symptoms.
      Symptoms of isolated Crohn's disease of the stomach usually include weight loss, nausea, vomiting, and epigastric pain. These symptoms alone are not pathognomonic of Crohn's disease because they are also common in patients with gastric malignant lesions, peptic ulcer disease with obstruction, and infiltrative processes such as eosinophilic gastroenteritis.
      Radiographically, the mucosal lesions of gastric Crohn's disease appear similar to those seen with erosive gastritis.
      • Levine MS
      Crohn's disease of the upper gastrointestinal tract.
      The stomach may be dilated and have multiple superficial ulcerations, or it can be small, rigid, and contracted. Our patient had both manifestations—first a huge dilated stomach and later a thickened stomach with a funnel-shaped antrum. The latter finding has been described as the “ram's horn” sign.
      • Farman J
      • Faegenburg D
      • Dallemand S
      • Chen C-K
      Crohn's disease of the stomach: the “ram's horn” sign.
      The differential diagnosis includes corrosive gastritis due to ingestion of lye, peptic ulcer disease, gastric scirrhous carcinoma, Ménétrier's disease, eosinophilic gastroenteritis, and gastric lymphoma. Pseudolymphoma, amyloidosis, tuberculosis, and sarcoidosis can also mimic Crohn's disease of the stomach. Our patient had no history of ingestion of lye or other corrosives. Although the patient had had a duodenal ulcer 10 years previously, we found no evidence of persistent duodenal disease or a history of chronic symptoms of peptic disease. Although Ménétrier's disease can involve the entire stomach and produce ulcerations, it does not cause transmural disease. Malignant and infiltrative processes were ruled out by the histologic findings.
      The most common indication for surgical intervention in gastroduodenal Crohn's disease is gastric outlet obstruction, and approximately a third of the patients require surgical treatment.
      • Fielding JF
      • Toye DKM
      • Beton DC
      • Cooke WT
      Crohn's disease of the stomach and duodenum.
      In all the reported cases, isolated gastric Crohn's disease was diagnosed after pathologic examination of the surgical specimen. Many of these cases were thought to be carcinoma preoperatively, and the patients underwent total or subtotal gastrectomy. Gastroscopy was performed in three cases, but endoscopic biopsy did not yield the diagnosis because of the small tissue samples.
      • Danzi JT
      • Farmer RG
      • Sullivan Jr, BH
      • Rankin GB
      Endoscopic features of gastroduodenal Crohn's disease.
      Long-term follow-up is not available for the previously reported cases. As noted, our patient had no evidence of recurrent Crohn's disease on upper and lower intestinal barium x-ray studies 1 year after total gastrectomy.

      CONCLUSION

      Isolated Crohn's disease of the stomach usually manifests clinically as nausea, vomiting, and abdominal pain. Radiographically, Crohn's disease of the stomach can manifest as a huge dilated stomach or, more frequently, as a small contracted one. Endoscopic biopsy specimens often show only nonspecific inflammation. Because the condition can mimic the appearance of a malignant process, surgical resection is usually necessary to establish the diagnosis.

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