Hyperplastic gastropathy refers to conditions confined to the rugae in the gastric body and fundus, which are associated with an excessive number of mucosal epithelial cells.
Emphysematous gastritis is a rare and severe form of gastritis secondary to mucosal disruption and gas-forming bacterial invasion. It is characterized by air in the wall of the stomach.
We describe a case of a 56-year-old male admitted with chief complaint of persistent abdominal pain, nausea and vomiting.
Comprehensive work up was undertaken and led up to diagnosis of Zollinger- Ellison syndrome. It was felt that the rugal hypertrophy was so severe that it trapped air which appeared on the scan as gastric emphysema. This would also explain the lack of gastric wall edema as would be expected in the latter.
History and examination
A 56-year-old gentleman who was fairly healthy until one month prior, presented to another hospital with epigastric pain and was found to have biliary pancreatitis. During the same hospitalization, when his pancreatitis settled, his gallbladder was removed. Endoscopic retrograde cholangiopancreatography performed the following day, because of an elevated bilirubin, did not find choledocholithiasis.
The patient was discharged but was readmitted soon with mid-abdominal pain that was similar to the pain prior to cholecystectomy; it was described as a steady knife-like pain associated with nausea, vomiting and diarrhea. He did have a remote history of heartburn, but this was without any alarm symptoms. He also had looser bowel movements than normal over the past few years, but this did not lead to any weight loss.
The patient was smoking 2 packs daily until the onset of his symptoms, had minimum alcohol use and denied any drug use. He lived alone on a farm. His family history revealed no gastrointestinal problems. He was on no medicines at home.
On examination, his vital signs were within normal limits. Though he looked unwell, he did not seem critically ill. Neck exam was without any masses, no lymphadenopathy. Abdomen was soft, recent well-healed surgical scars and no tenderness, bowel sounds being heard in all quadrants.
His nausea, vomiting and abdominal pain had resolved after several days of hydration, opioid pain killers and pantoprazole. Laboratory work was significant for normal CBC, creatinine of 1.9 mg/dl, otherwise electrolytes normal. Calcium levels were normal.
CT scan of the abdomen showed stomach wall thickening and air densities within it. This raised the possibility of emphysematous gastritis. However, there was very little surrounding edematous change in the fat adjacent to the stomach.
There were no liver lesions noted. Clinically he was unwell but not critically ill as would be expected of patients with emphysematous gastritis (Figure 1).
Figure 1 CT scan showing emphysematous gastritis.
On upper GI endoscopy he was found to have marked gastric rugal hypertrophy (Figure 2), severe ulcerative esophagitis and gastritis. A hypersecretory stomach was noted. Upon entry, there was a fair amount of fluid in the stomach. This recurred several times, despite evacuation.
Figure 2 Endoscopy showing gastric rugal hypertrophy.
The duodenal bulb and distal duodenum demonstrated multiple small erosions or ulcers, all the way through the distal second part of the duodenum. Biopsies revealed parietal cell hyperplasia without any metaplasia.
Fasting gastrin was high at 948 pg/mL. The gastric fluid's pH was reported as less than 6 (our center's assays of gastric fluid pH are only reported as either less than 6 or more than 6).
Octreoscan was positive for a focus of intense uptake in the duodenum. Endoscopic ultrasound showed hypoechoic lesion in the distal duodenal wall deep to the muscularis propria and caudal to the head of the pancreas and ampulla. Clinical picture of hypergastrinemia, duodenitis, hypertrophied gastric folds and positive octreotide scan were all quite consistent with the diagnosis of a gastrinoma.
Management and outcomeWith hydration the patient's renal function returned to normal, he did not require any pain medicines and was continued on pantoprazole. Six weeks later he underwent resection of the submucosal tissue in the second part of duodenum and adjacent lymph node. On post operative day 3, the gastrin level dropped remarkably to 26 pg/mL. Permanent sections of the lymph node near the right gastroepiploic artery confirm the presence of a well differentiated neuroendocrine neoplasm. Permanent sections of the submucosal mass and deep tissue from the duodenal wall demonstrate ducts and glandular tissue in an apparent lobular architecture associated with fibrosis. This duodenal lesion did not clearly appear neoplastic, and is favored to represent heterotopic pancreatic tissue (Figure 3).
Figure 3a Parietal cell hyperplasia
Figure 3b Metastatic neuroendocrine tumor in lymph node
Figure 3c Heterotopic pancreas in duodenum
The patient is doing very well now, with no further GI symptoms.
Gastrinomas are derived from multipotential stem cells of endodermal origin, known as enteroendocrine cells. They arise mainly in the pancreas as well as the small intestine and have a particular affinity for chromium and silver salts, which are useful markers for the pathologist.
Gastrin is the most common peptide within the secretory granules of gastrinoma cells, but other peptides such as vasoactive intestinal peptide and glucagon have been identified as well.
Approximately 70% of the gastrinomas arise in the duodenum, 25% arise in the pancreas and the remainder in the lymph nodes. Duodenal gastrinomas are frequently multiple and benign, as compared with their solitary pancreatic counterparts.
These tumors are associated with parathyroid and pituitary tumors as a part of Multiple Endocrine Neoplasia-1. Therefore when one is diagnosed, a high index of suspicion must be maintained and further assessment should be carried out for other endocrine tumors.
The gastrin secreted from the tumor causes increased acid production via its action on the parietal cells.
Over 90 percent of patients with the Zollinger-Ellison syndrome develop peptic ulcers.
Diarrhea occurs due to excess acid that is inhibiting absorption of sodium and water by small intestine.
Steatorrhea occurs secondary to the acid excess overcoming the neutralizing action of bicarbonate. The very low pH of the intestinal contents inactivates pancreatic digestive enzymes, interferes with the emulsification of fat.
Metastatic disease is evident at the time of diagnosis in approximately one-third of patients with a gastrinoma, with liver being the most common site. When metastatic disease is seen in the bones, it is seen in patients with hepatic metastases.
Diagnosis is made by either measurements of fasting gastrin, secretin stimulation or gastric acid stimulation studies. Of these, only the fasting serum gastrin concentration and secretin stimulation test are used routinely.
The upper limit of normal for serum gastrin is 110 pg/mL. The majority of the patients have gastrin levels about 10 times upper limits of normal and higher values are seen with larger tumor burden, pancreatic tumors and metastases . In the presence of a gastric pH below 5, a serum gastrin value greater than 1000 pg/mL is virtually diagnostic of the disorder.
Measurement of gastric pH on a single specimen is important to exclude secondary hypergastrinemia due to achlorhydria ( in patients with pernicious anemia or atrophic gastritis) since the serum gastrin level can easily exceed 1000 pg/mL in these circumstances.
Special attention must be paid to patients who are already on PPIs - this class of medicine suppresses acid production and causes an increase in the levels of gastrin . For the diagnosis to be made with certainty, holding off on the medications for one week prior to the test has been recommended by some, but this may be dangerous in the face of unremitting gastric acid hypersecretion.
Octreoscan and SPECT have a higher sensitivity than all other imaging modalities, are particularly useful in identifying liver and bone metastases . Endoscopic ultrasound is especially valuable in imaging small pancreatic endocrine tumors, and permits fine needle aspiration for histological identification. Combining both modalities allows majority of cases to be diagnosed.
After the diagnosis of the Zollinger-Ellison syndrome is made, the gastrinoma must be located, unless the decision is to treat the condition medically (For example, in the MEN-1 group). Sometimes sporadic cases of gastrinomas are associated with MEN-1 gene but no manifestation of the components of MEN-1.
PPIs, especially intravenous pantoprazole, are the medicines of choice . For patients with metastatic disease chemotherapy, octreotide and interferon may be tried. Surgical excision of the tumor is preferred after stabilizing the patient .
Finally, emphysematous gastritis is a rare and severe type of gastritis secondary to mucosal disruption and gas-forming bacterial invasion. It is characterized by air in the wall of the stomach. Causes include ingestion of toxic material such as corrosives, alcohol ingestion, trauma, gastric infarction, ulcer disease.
It presents with sudden onset of chills, nausea, fever, vomiting. Submucosa is invaded by gas forming organisms such as hemolytic Streptococci, Clostridium welchii and Escherichia coli. Prognosis is guarded with mortality of 60 to 80 percent.
In conclusion, though our patient had radiological features of a rare condition, careful assessment revealed very different, albeit equally rare, more treatable condition.
This article was first published on GastroHep.com on 24 April 2008.
Lakshmi Pasumarthy, James Srour, David Speranza and Elisabeth Stonesifer
Department of Internal Medicine/Gastroenterology, York Hospital, York, USA
Department of Internal Medicine/ Gastroenterology
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